USMLE Step 2 & 3 UWorld Notes for Quick Revision

USMILE INNER CIRCLE COMMUNITY STEP 2 & 3 NOTES Complete Step 2 & 3 Uworld notes for quick revision 2024 USMLE Inner Circle Community Version 2 These notes are dedicated to the USMLE community, a community of learners and leaders who embody the spirit of resilience and compassion. As we prepare for the challenges ahead, let us remember that we rise by lifting others, and that true success is measured not by what we achieve alone, but by how we empower others to achieve their goals. Let us flock together, not as individuals, but as a team, bound by a common purpose and a spirit of collaboration, and reach new heights of success. Anonymous MD PDF version: 17th March'2024 https://t.me/usmleinnercircle Version 2 USMLE CIRCLE COMMUNITY irc le INNER Step 2 & 3 Notes rC Important Update Read these instruction first ne Renal Pulmonary Endocrine Obs U SM GIT LE Gynae & Reproductive Health In CVS Blood & Oncology Musculoskeletal Dermatology CNS, Eye, Ear Psychiatry & Substance Abuse --------Short Subjects General Paeds Geriatrics Step 2 & 3 Notes 1 https://t.me/usmleinnercircle Version 2 Public Health Ethics & Communication Disclaimer All the copyrights belong to Uworld,LLC. The distribution and/or reproduction of these notes is intended for educational, non-commercial use only. The information provided in these notes is NOT intended as a replacement for Uworld Qbank, but only as a review resource intended to facilitate quick revision of the topics covered in Uworld Qbank. Step 2 & 3 Notes 2 https://t.me/usmleinnercircle Version 2 Important Update 2024 Hello lovely people ! irc le Can you believe it's been a whole year since we started putting together these notes? The response we've received has been nothing short of amazing. We've built a community of nearly 2000 dedicated individuals, all hustling to ace the USMLE. Throughout this past year, our incredible members have generously shared their time and insights, constantly updating and refining these notes to ensure they're as accurate and helpful as possible. rC On a personal note, I recently took my Step 3 exam and made sure to incorporate relevant information into our notes. There weren't too many differences, so I decided to merge the updates into the existing material. I'm confident that these revisions will benefit not only those preparing for Step 3 but also those tackling Step 2. ne And now, for the exciting news! Every single admin who contributed to these notes, including me, has successfully MATCHED to their DREAM residency program this year. This achievement wouldn't have share any updates/corrections. With Love, LE Anonymous MD In been possible without your support and encouragement. To all of you on your USMLE journey, we wish you the very best of luck! Remember, our group is here for you, so don't hesitate to ask questions or to U SM The increase is number of pages is mainly because we increased the size of tables and images on request of people who like to print these notes. 2023 Hey, thankyou all of you for writing to me over mail ! When I started my Step 2 prep I did not find any good resource like ‘First Aidʼ for Step 2. I found that while Uworld is a Gold standard resource for preparing for Step 2, there wasn't a good way to review and re-read information (creating Visuospatial memory) while solving the questions. So, I began creating my own coloured notes. After sharing these notes with friends and others, I discovered that many people had the same issue that I initially faced during my preparation. I was encouraged by the positive feedback I received and it became clear that these notes were helping others as well. As a result of indirectly assisting others through my notes, I was also able to establish mutually beneficial connections with other medical students. These connections led to opportunities such as a Important Update 3 https://t.me/usmleinnercircle Version 2 research paper and an observership for me. I have created the initial version of this rapid review resource, but it needs to be frequently updated to stay current. Therefore, I am inviting all interested medical students preparing for the USMLE to join and collaborate with me to produce the highest quality community-generated notes. Additionally I believe that as we all strive towards the same goal, we can greatly benefit from supporting and assisting each other with study strategies, research experience, mental health support and clinical experience. With this in mind, I have decided to create a Telegram group called "The USMLE Inner Circle" to facilitate these connections and collaborations. I will be uploading the PDF (when it is completed) and all future updates in this group itself. As the links to these notes may change in the future, I strongly encourage you to join the group to stay updated on the latest version and links. The USMLE Inner Circle You can view and join @usmleinnercircle right away. https://t.me/usmleinnercircle Important Update 4 https://t.me/usmleinnercircle Version 2 Read these instruction first  These are notes that I made with the help of Tzanki Deck and solving UWorld questions. irc  Have any other questions? Feel free to join our telegram group and ask. le  The notes Include major tables and concepts from Step 2/3 uworld which I believe are new additions to Step 1 knowledge. I am skipping repeated Step 1 tables, if thereʼs nothing new. Suggest you to keep your step 1 first aid handy for reviewing repeated step 1 concepts. rC Things that I find interesting while doing NBME forms are in these yellow boxes LE My Personal Progress In These are general highlighted points. ne Things I find interesting listening to Divine podcasts are in these orange boxes Uworld Percentage Complete: 100% Uworld Percentage Correct: U SM 75% Assessments: 21/03/23 NBME 10 259 36 incorrects) 24/03/23 NBME 9 257 40 incorrects) 28/03/23 UWSA 1 265 84%  New Free 120 106/120 31/03/23 NBME 11 256 40 incorrects) 03/04/23 NBME 12 261 36 incorrects) 08/04/23 UWSA 2 263 83%  Old Free 120 104/120 13th April 2023 DDAY Predicted Score: 262  12 Actual STEP 2 Score: 267 !!! Read these instruction first 5 https://t.me/usmleinnercircle 1 Version 2 Update: MARCH 2024 Matched to my DREAM UNIVERSITY RESIDENCY PROGRAM !!! Read these instruction first 6 https://t.me/usmleinnercircle Version 2 Renal Metabolic Abnormalities Metabolic Acidosis RTA le Metabolic Alkalosis Electrolyte Disturbances Hypercalcemia irc Hypocalcemia Hypernatremia Hyponatremia Hyperkalemia rC Hypokalemia Hypophosphatemia Renal Cysts Fibromuscular Dysplasia ne Acute Kidney Injury Contrast Induced AKI Rhabdomyolysis Hemodialysis Calcific Uremic Arteriolopathy CardioRenal Syndrome Pyelonephritis LE Renal Stones In Analgesic Nephropathy Asymptomatic Bacteriuria Recurrent UTI Renal Abscess U SM Nephrotic Syndrome HIV-associated nephropathy Glomerulonephritis Acute Interstitial Nephritis Bladder Interstitial cystitis (painful bladder syndrome) Urinary Incontinence Renal Artery Stenosis Renal Vein Thrombosis Renal Cancers Miscellaneous Peripheral Edema Hematuria Renal Osteodystrophy Renal Drugs Renal 7 https://t.me/usmleinnercircle Version 2 Paeds Renal Hematuria Neonatal AKI Nocturnal Enuresis Vesicoureteral Reflux UTI Benign Proteinuria Renal Surgery Genitourinary trauma Kidney Injury Bladder Ureter Injury Urethral Injury Post Operative Urinary Retention Urethral Strictures Metabolic Abnormalities CheckarterialpH I pH< 7.35 pH> 7.45 1 Acidemia Pco2 > 44mmHg Alkalemia ~-<20mEq/L Respiratory acidosis ~< L Checkaniongap=~-(S!:.+HCO,-) i ~"'"..,..;. o,-,;o,,_. 0yperventilation Metabolicalkalosis r I An~iety/p~k Hy22xemia leg,highaltitude) Salicylates (early) CDm~ (!) cJ>'\,o Tu.!!JPr Pulmonary embolism Pr~cy =' i > 12mEq/L .,,,J½ 40 I Aniongap Normalaniongap 73 GOLDMARK: Glycols!ethyleneglycol.proplyeneglycol) Oxoproline!toxicmetaboliteof acetaminophen) L-lactate!lacticacidosis) D-lactate (exogenous lacticacid) HARDASS ,_ c...-~.t;.., titc~ Hyperchloremia/hvperalimentation Addisondisease Renaltubular acidosis 1"tN Diarrhea j Methanol(andother alcoho~I ~lamide<iil,c." o:: ~teettect) Renalfailure Keytones (diabetic,alcohoLic, starvation) Spirooo\actone Salineinfusion c.l 1.,.,.;~ -- --- --- v If+ loss/HCOfexcess Loopdiuretics Vomiting Antaciduse ~aldosteronism Metabolic Pco,= alkalosis 40 mm Hg ( l, -.411.:a; "c-w~ro... . Respiratory acidosis 35 E Metabolic acidosis Respiratory alkalosis ..,_ (J.;.,.<aI.AC~ ....... r-M"C. 6.9 7.0 71 7.2 7.3 75 7.6 77 7.8 7.9 Ii!! V Renal HC03- > 28mEq/L Respiratory alkalosis Metabolicacidosis VHypov~~ Airwayobstruction Acutelungdisease Chroniclungdisease Op~,se~ Weakening of respiratory 36mmHg 8 https://t.me/usmleinnercircle Version 2 Mixed primary acid-base disorders Metabolic acidosis + respiratory acidosis Metabolic alkalosis + respiratory alkalosis Metabolic acidosis + respiratory alkalosis Metabolic alkalosis + respiratory acidosis pH Example Sepsis (lactic acidosis) ! i Very low t ! Very high Hyperemesis gravidarum & hyperventilation of pregnancy ! ! Near normal Salicylate toxicity t t Near normal Overdiuresed heart failure & underlying COPD with respiratory failure .... .... Near normal Metabolic acidosis + metabolic alkalosis Diabetic ketoacidosis with severe vomiting irc COPD = chronic obstructive pulmonary disease. Metabolic Acidosis Common . .. .. . Loss of bicarbonate Severe diarrhea Renal tubular acidosis Elevated anion gap Accumulation of unmeasured acidic compounds Lactic acidosis Diabetic ketoacidosis Excessive saline infusion Renal failure (uremia) Methanol, ethylene glycol Salicylate toxicity In causes . .. . Normal anion gap ne Mechanism rC Metabolic acidosis Type le HC03" PaC02 • pH will decrease 0.08 for every 10 mmHg of acute increase in PaCO2. LE i.e. a patient with a CO2 of 65 (normal 40 should have a pH of 7.2. When compensation kicks in, pH will increase closer to normal. Workup of high anion gap metabolic acidosis U SM High anion gap metabolic acidosis 1/ l Drug ingestion Hypoperfusion j Serum lactic acid • Salicylates (earty respiratory alkalosis) • lsoniazid • Iron i Associated clues i Renal failure Hyperglycemia t Blood urea Urine & serum ketones nitrogen i i Lactic acidosis Uremia i Diabetic ketoacidosis ©UWor1d Renal Osmolal gap • Ethylene glycol (urinary calcium oxalate crystals) • Methanol (blindness) • Propylene glycol 9 https://t.me/usmleinnercircle Version 2 Gl=e ~~~'1!:~ (0ml : 21Na• (meq/L)I BUN Normal osmolal gap (measured - calculated) is  10 (mg/dlJ + (mg/dll """"is """1r osm"Measured osmolality-Calcula1edosmolality  What is the likely cause of anion gap metabolic acidosis in a patient with a recent seizure? Postictal lactic acidosis (transient and typically resolves without treatment within 90 minutes following resolution of seizure activity) Type A • Lactic acidosis is most commonly seen in states of hypo-perfusion and shock (eg, sepsis, cardiogenic shock). Type B Lactic acid levels may also be elevated in patients with end-stage liver disease as lactic acid is Metformin metabolised in the liver.  Why is normal anion gap also referred to as hyperchloremic metabolic acidosis? Cl- levels rise to balance out the low HCO3 Na+] - ([↑ Cl-] + [↓ HCO3]} The risks and benefits of treating acute metabolic acidosis with sodium bicarbonate are not entirely clear; however, it is generally recommended in patients with severe acute metabolic acidosis with pH 7.1. Administration of sodium bicarbonate may cause myocardial depression and increased lactic acid production; therefore, in patients with pH 7.1, the relatively small benefits of sodium bicarbonate do not typically outweigh the risks. RTA  What is the likely diagnosis in an elderly diabetic with non-anion gap metabolic acidosis, hyperkalemia, and mild renal insufficiency? Type 4 (hyperkalemic) renal tubular acidosis most common in elderly patients with poorly controlled diabetes (damage to the juxtaglomerular apparatus results in hyporeninemic, hypoaldosteronism) Top 3 differentials for Non-AG metabolic acidosis is diarrhea, RTA, and Addisonʼs! Renal 10 https://t.me/usmleinnercircle Version 2 Renal tubular acidosis 1 (Distal) 2 (Proximal) 4 Primary defect Poor hydrogen secretion into urine Poor bicarbonate resorption Aldosterone resistance Urine pH ~5.5 <5.5 <5.5 Serum potassium Low-normal Low-normal High Fanconi syndrome (glucosuria, phosphaturia, aminoaciduria) • Obstructive uropathy Causes • Autoimmune disorders (eg, Sjogren syndrome, rheumatoid arthritis) T/t:  Type 2 Alkali Therapy: Potassium citrate ne  Type 4 Furosemide, Mineralocorticoid replacement rC  Type 1 Alkali Therapy  Sodium Bicarbonate, Sodium Citrate • Congenital adrenal hyperplasia irc • Genetic disorders • Medication toxicity le Type In Renal tubular acidosis in infants commonly presents with failure to thrive in the setting of normal anion gap metabolic acidosis. Causes of elevated urine pH (pH  5.5 LE Type I RTA, UTI with a urease (+) bug (proteus mirabilis) (magnesium-ammonium phosphate stones) U SM Metabolic Alkalosis Renal 11 https://t.me/usmleinnercircle Version 2 Clinicalfeaturesof metabolic alkalosis Saline-responsive • Vomiting • Gastric suc~oning • Diuretics • Laxative abuse Causes • Decreased oral fluid intake (volume depletion) Saline-resistant • Primary hyperaldosteronism • Cushing's syndrome • Severe hypokalemia (<2 mEq/L) • Volume depletion: Easy fatigability, postural dizziness, muscle cramps Clinical presentation • Hypokalemia; Muscle weakness, arrhythmias • Urine chloride: <20 mEq/L (saline-responsive), >20 mEq/L (saline-resistant) • Treat underlying cause to rever5e generation phase in all cases Treatment • Saline-responsive- Also give normal saline to correct maintenance phase Differential diagnosis of metabolic alkalosis Metabolic a lkalosis pH >7.45 & HCo,- >24 mEq/l High urine chloride (>20 mEq/l) Low urine chloride (<20 mEq/l) Hypovolem1c • Vomit1ng/nasogastnc aspiration • Diuretic overuse• • Bartter & Gitelman syndromes Saline responsive ·lJrine cNoride wil be htgh if clurede wn Hypervolem1c Excess mineralocorticold activity • Primary hyperaldosteronism • Cushing disease • Ectopic ACTH production Saline unresponsive last Ingested within teveflll h<ut CUWoOd • Loop or thiazide diuretic overuse, which involves loss of Cl- and retention of HCO3 by the kidneys, eventually leading to total body chloride depletion (as well as hypovolemia). Although these patients are overall chloride depleted, urine chloride may be high in the setting of recent diuretic ingestion (eg, within several hours of measurement). Diuretics will produce a "waxing and waning" urinary Cl-. Explanation here is that loop and thiazides block NaCl reabsorption, so while active, they produce a "high" urinary Cl-, and when their effect dissipates, they produce a "low" urinary Cl- Renal 12 https://t.me/usmleinnercircle Version 2  What is the recommended treatment for a patient with metabolic alkalosis and hypokalemia secondary to recurrent vomiting? IV saline and potassium Ammonium chloride is a strong acid that can be used to treat severe metabolic alkalosis in patients unable to receive normal saline (eg, volume overload) le  What is the likely diagnosis in a young female with hypokalemia, metabolic alkalosis, normotension, and low urine Cl-? irc Surreptitious vomiting {low urine Cl- helps distinguish vomiting from other causes of hypokalemia, alkalosis, and rC normotension (e.g. diuretic abuse, Barter syndrome, and Gitelman's syndrome)} ne Low Cl− impairs renal HCO3 excretion. Therefore, Cl−depletion perpetuates metabolic alkalosis In Primary Hyperaldosteronism causes Cl and Na loss in urine through Aldosterone Escape Mechanism I LE Electrolyte Disturbances Electrolyte disturbances ELECTROL VTE LOWSERUM CONCENTRATION ,~tupor, U SM Sodium Renal HIGH SERUM CONCENTRATION Irritability, stupor,~ seizures Potassium U "·a,·cs and Aattcncd T wm-cs on ECC, arrhythmias, muscle cramps, spE.!!!, w~ Wide QRS and peaked T 11·a,-cson ECC, arrhythmias muscle weakness Calcium Tetan)', seizures, QT prolongation, twitching (eg, Chvostek sign), spasm (eg, Trousseau sign) Stones (renal), bones (pain), groans (abdominalv _Eai1J1thrones (t urinary freguency), psychiatric overtones (anxiety, altered mental status) V Magnesium Tetany, torsades de pointes, hypokalemia, °Typocalcemia (when [Mg'+]< 1.0 mEq/L) i DTRs, letharg\', bradycardia, hypotension, cardiac arrest, hypocalcemia Phosphate Bone loss, osteomalacia (adults), rickets (children) Renal stone , metastatic ca lei Iications, hypocalcemia V 13 https://t.me/usmleinnercircle Version 2 Equilibration movements of charged ions under physiologic conditions Ion Charge Sodium Positive Major Equilibrium location potential Equilibration movement at -70 mV Extracellular gradient drives Na+ into cell, Extracellular +60mV making membrane potential more positive (Choice E) Intracellular gradient drives K+ out of cell, Potassium Positive Intracellular -90mV making membrane potential more negative Extracellular gradient drives cI- into cell, Chloride Negative Extracellular -75 mV making membrane potential more negative (Choices B and C) Extracellular gradient drives Ca2 + into cell, Calcium Positive Extracellular +125 mV making membrane potential more positive (Choice A) Hypercalcemia Diagnosis of hypercalcemia Confirm hypercalcemia • Repeat testing • Correct for albumin concentration or measure ionized calcium l Measure PTH level High-normal or elevated PTH (PTH dependent) Suppressed PTH (PTH independent) l Measure PTHrP, 25-hydroxyvitamin D, 1,25-dihydroxyvitaminD Causes Causes • Primary (or tertiary) hyperparathyroidism • Malignancy • Familial hypocalciuric hypercalcemia • Lithium • Vitamin D toxicity • Granulomatous diseases • Drug-induced (eg, thiazides) • Milk-alkali syndrome • Thyrotoxicosis • Vitamin A toxicity • Immobilization PTH = paralhyroid hormone, PTHrP = paralhyroid hormone--rnlated protein. Renal QUWorld 14 https://t.me/usmleinnercircle Version 2 Management of hypercalcemia Short-term (immediate) treatment Severe (calcium >14 mg/dL) or symptomatic Moderate • Normal saline hydration plus calcitonin • Avoid loop diuretics unless volume overload (heart failure) exists Long-term treatment • Bisphosphonate (zoledronic acid) • Usually no immediate treatment required unless symptomatic (calcium 12-14 mg/dL) • Treatment is similar to that for severe hypercalcemia Asymptomatic or mild • No immediate treatment required (calcium <12 mg/dL) • Avoid thiazide diuretics, lithium, volume depletion & prolonged bed rest irc le Hemodialysis is an effective treatment for hypercalcemia. However, it is typically reserved for patients with renal insufficiency or heart failure in whom aggressive hydration cannot be administered safely  What is the typical volume state (hypo, eu-, or hyper-volemic) of patients with hypercalcemia? rC Hypovolemic : due to polyuria and decreased oral intake; loop diuretics should be avoided as they can worsen volume depletion Hypercalcemia on kidney In • Vasoconstriction (decrease GFR ne Hypercalcemia may induce nephrogenic diabetes insipidus leading to polyuria and fluid loss; other symptoms include weakness, GI distress, and neuropsychiatric symptoms • Activation of Ca++-sensing receptor in thick ascending limb, which inhibits Na-K2Cl cotransporter → natriuresis LE • Blocks ADH water reabsorption U SM Natriuresis ⟶ volume depletion ⟶ metabolic alkalosis  What is the likely etiology of hypercalcemia in a patient that develops symptoms 4 weeks after a severe motor vehicle accident left the patient a quadriplegic? Immobilisation due to increased osteoclastic bone resorption, especially in individuals with a high baseline rate of turnover (e.g. young individuals, Paget's disease patients); characterised by high Ca2 and low PTH T/t: Bisphosphonates Renal 15 https://t.me/usmleinnercircle Version 2 Milk-alkali syndrome Pathophysiology • Excessive intake of calcium & absorbable alkali • Renal vasoconstriction & decreased GFR • Renal loss of sodium & water, reabsorption of bicarbonate Symptoms • Nausea, vomiting, constipation • Polyuria, polydipsia • Neuropsychiatric symptoms Laboratory findings • Hypercalcemia • Metabolic alkalosis • Acute kidney injury • Suppressed PTH Treatment • Discontinuation of causative agent • Isotonic saline followed by furosemide Hypercalcemia of malignancy Cause PTHrP* Bone metastases 1,25-dihydroxyvitamin D .. . .. . Tumor type Squamous cell Kidney & bladder Breast & ovarian Breast Multiple myeloma Lymphoma Mechanism . . . PTH mimic t Osteolysis f Calcium absorption Diagnostic .. .. ! PTH f PTHrP ! PTH & PTHrP ! Vitamin D .! . PTH t Vitamin D "PTHrP causes -80% of malignancy-associated hypercalcemia. PTH = parathyroid hormone; PTHrP = PTH-related protein. In hypercalcemia due to malignancy, serum calcium is typically much higher (often 14 mg/dL)  What is the drug class of choice for stabilising bony metastatic lesions and preventing hypercalcemia of malignancy? Bisphosphonates • Hypercalcemia of malignancy and hypervitaminosis D secondary to granulomatous disease both present with similar calcium lab panels (increased Ca, increased PO4, decreased PTH). What lab test can be ordered to discern between the two? 1,25Vitamin D levels (to confirm hypervitaminosis); or PTH-rp levels (to confirm paraneoplastic syndrome) If you're asked this, use the history to discern which one you need (i.e. history suggestive of sarcoid/TB vs. a lung mass) Hypocalcemia Renal 16 https://t.me/usmleinnercircle Version 2 Clinical features Treatment Pancreatitis Sepsis Tumor lysis syndrome Acute alkalosis Chelation: blood (citrate) transfusion, EDTA, foscarnet Muscle cramps Chvostek & Trousseau signs Paresthesias Hyperreflexia/tetany le Causes Neck surgery (parathyroidectomy) Seizures IV calcium gluconate/chloride EDTA = ethylenediaminetetraacetic acid; IV= intravenous. irc . • • .. . .. • .. . Acute hypocalcemia rC As citrate binds ionized calcium, these patients are at risk for symptomatic hypocalcemia due to ionized calcium deficiency. ne An ionized calcium level is required for diagnosis, as serum calcium is often normal. Approach to hypocalcemia T Low magnesium level? Yes f--------+ Is it due to a drug? Recent blood transfusion (t citrate, volume)? U SM LE . . In Low serum calcium Confinn with repeat measurement Correct for serum albumin or measure ionized calcium (if needed) Treat underlying cause Replete magnesium Intravenous calcium for severe symptoms Measure serum PTH Normal or low PTH Hypoparathyroidism Surgical: Parathyroidectomy, thyroidectomy, radical neck surgery Autoimmune: Polyglandular autoimmune syndrome Infiltrative disease: Metastatic cancer, Wilson disease, hemochromatosis Metabolic: Vitamin D deficiency, chronic kidney disease Inflammatory: Pancreatitis, sepsis Oncology: Tumorlysis syndrome PTH resistance: Pseudohypoparathyroidism Genetic: PTH gene or calcium sensing receptor gene mutations ©UWorld Renal 17 https://t.me/usmleinnercircle Version 2 What formula is used to calculate the "corrected Ca2" in a patient with low albumin? Serum Calcium  0.84  serum albumin) Level of Total Ca 2 is decreased, however, ionized Ca2 remains stable and thus patients remain asymptomatic  What electrolyte abnormality is typically the cause of hypocalcemia in an alcoholic patient? Severe hypomagnesemia hypomagnesemia causes decreased PTH release and resistance to PTH, resulting in hypoparathyroidism with low Ca2 and low phosphorus (vs other causes of hypoparathyroidism) Hypocalcemia due to hypomagnesemia is typically refractory to treatment with calcium unless magnesium is replaced as well. Although PTH levels increase rapidly after magnesium replacement, hypocalcemia takes longer to improve because PTH resistance persists despite improvement in magnesium levels. Hypernatremia Hypernatremia Euvolem1c Hypovolemic j Symptomatic• Yes No Hypotonic solution (eg, 5% dextrose) Isotonic solution (eg, 0.9% saline) until euvolemic "Tachycardia,decreased blood pressure,dry mucousmembranes, delayedcapillaryrefill CUWortd  What is the fluid of choice for initial resuscitation of severe hypovolemic hypernatremia in an infant? Isotonic solutions (e.g. normal saline, lactated Ringer's) using hypotonic solutions initially may lower the sodium too rapidly Hyponatremia Renal 18 https://t.me/usmleinnercircle Version 2 Evaluation of hyponatremia Hypovolemic hyponatremia • Marked hyperglycemia • Advanced renal failure Serum osmolality >290 mOsm/kg • Serum sodium <135 mEq/L • Symptoms/signs of hypovolemia: Intake& output of water, orthostatic lightheadedness, dry mucous membranes, poor skin turgor, tachycardia, orthostalic hypotension No • Primary polydipsia • Malnutrition (beer drinker's potomania) Urine osmolality <100 mOsm/kg No (urine sodium QO mEq/ ) No Yes Renal losses (urine sodium >20 m q/L) • Volume depletion • Congestive heart failure • Cirrhosis • SIADH • Adrenal insufficiency • Hypothyroidism OeCleasedeffective ©UWorld • Diuretics • Mineralocorticoid deficiency • CHF • Cirrhosis irc circul tin volum • Diarrhea, vomiting • Bums • Pancrealitis le xtrarenallosses Urine sodium <25 mEq/L rC CH = c-ongestivhe r1f 1lure. Hyponatremia Serum osmolality ECV Urine findings UNa <40 mEq/L UNa >40 mEq/L Low (<275 mOsm/kg) Uosm <100 mOsm/kg Euvolemic In Uosm >100 mOsm/kg & UNa >40 mEq/L Hypervolemic High (>295 mOsm/kg) Variable LE Normal . . .. . . . .. Nonrenal salt loss (eg, vomiting, diarrhea, dehydration) ne Hypovolemic Cause Variable Renal salt loss (eg, diuretics, primary adrenal insufficiency) Psychogenic polydipsia Beer potomania SIADH (rule out hypothyroidism, secondary adrenal insufficiency) CHF, hepatic failure, nephrotic syndrome Pseudohyponatremia (eg, paraproteinemia, hyperlipidemia) Hyperglycemia Exogenous solutes (eg, mannitol) CHF ;;;congestive heart failure; ECV ;;; extracellular volume; SIADH ;;;syndrome of inappropriate antidiuretic hormone; UNa ;;; urine U SM sodium; Uosm ;;; urine osmolality. Renal 19 https://t.me/usmleinnercircle Version 2 Why would a patient with hyperosmolar hyperglycemic state have hyponatremia? Pseudohyponatremia due to hyperglycemia → need to correct (Na+ + 2 mEq/L for each 100 mg/dL that glucose is above 100)) Na+  128 Glucose  800 Actual Na+: 128  7  2  142 mEq/L Iatrogenic hyponatremia . . . . . . . . . • Hypotonic fluid hydration Risk factors Clinical presentation Management Children, premenopausal women, elderly Hypoxia Central nervous system disorders Headache Nausea/vomiting Encephalopathy (eg, mental status changes, seizure) Hypertonic (3%) saline Serial measurement of electrolytes Increase serum sodium 6-8 mEq/L in first 24 hrs  What is the likely diagnosis in a young female with orthostatic hypotension, hyponatremia, and hypokalemia with high urine Na+ and K? Diuretic abuse normally dehydrated patients with hyponatremia and hypokalemia will have reduced urine Na+/K  Correction of serum sodium (both hypo- and hyper-natremia) should not exceed a rate of 0.5 mEq/L/hr. and thus should not exceed 12 mEq/L/24 hours  Adaptations to normalize brain volume (eg, extrusion of osmolytes from brain cells) typically take 48 hours. • Acute hyponatremia (present for 48 hr) is poorly tolerated, and patients are at high risk of brain herniation. Therefore, patients with serum sodium of 130 mEq/L with any symptoms of elevated intracranial pressure should be treated with hypertonic 3% saline boluses to rapidly correct serum Renal 20 https://t.me/usmleinnercircle Version 2 sodium. Because neural adaptations have not occurred, patients are at relatively low risk of osmotic demyelination syndrome ODS. • Chronic hyponatremia (present for 48 hr) is typically better tolerated; therefore, hypertonic saline is often reserved for those with severe hyponatremia 120 mEq/ L, severe symptoms (eg, seizure), or concurrent intracranial pathology (eg, masses, hemorrhagic stroke). Symptoms of hyponatremia are dependent on the severity of depletion and the acuity of onset. Acute onset leads to more severe symptoms, such as seizure and coma, while chronic irc le onset generally has milder symptoms, such as nausea, vomiting, fatigue, and confusion. Diuretics are a common cause of symptomatic hyponatremia, and, thus, patients should have sodium concentrations monitored after initiation. If hyponatremia develops, the diuretic should be discontinued. Potassiumshifts SHIFTS K' INTO CELL (CAUSING HYPOKALEMIA) rC Hyperkalemia SHIFTS K' OUTOFCELL (CAUSING HYPERKALEMIA) Dig a•/K• ATPase) HyperOsmolarity ne Hypo-osmolarity Lysis of cells (eg, crush injury, rhabdomyolysis, tumor lysissyndrome) Alkalosis Acidosis P-blocker ~n High blood Sugar (insulin deficiem;y) In P-adrenergic agonisq(t Na+/K-ATPase) (t Na+/K-ATPase) LE Insulin shifts K• into cells Succinylcholine (f risKin burns/muscle trauma) Hyperkalemia? DO LApss Common medications that cause hyperkalemia U SM Medication ACE inhibitor, ARB Cyclosporine Decreases aldosterone secretion (inhibition of AT II/AT II receptor) + inhibits ENaC Blocks aldosterone activity Digitalis Inhibits Na•tK• -ATPase Heparin Blocks aldosterone production Non selective !3-adrenergic blocker NSAID Mechanism Interferes with Jlrmediated intracellular potassium uptake Decreases renal perfusion --+ decreased potassium delivery to the collecting ducts Potassium-sparing diuretic Inhibits ENaC or aldosterone receptor Succinylcholine Causes extracellular leakage of potassium through acetylcholine receptors Trimethoprim Inhibits ENaC ARB= angiotensin II receptor blocker; AT= angiotensin; ENaC = epithelial sodium channel; Na•tK•-ATPase = sodium-potassium pump; NSAID = nonsteroidal anti-inflammatory drug. Renal 21 https://t.me/usmleinnercircle Version 2 Result of blood sample Etiology Arising from Pseudohyperkalemia Hemolysis Leukocytosis Thrombocytosis Repeat the blood sample K > 5mEq/L Decreased excretion Increased release from tissues Renal failure - Acute or chronic Low aldosterone state - ACE inhibitors/ARBs - RTA IV - Drugs - Addison disease Cell lysis Low insulin Acidosis Drugs - Beta blockers - Digoxin - Heparin Clinical features of hyperkalemia Sequence of ECG changes • • • • Tall peaked T waves with shortened QT interval PR prolongation & QRS widening Disappearance of P wave Conduction blocks, ectopy, or sine wave pattern Cardiac membrane stabilization • Calcium infusion Rapidly acting treatment options • Insulin with glucose • Beta-2 adrenergic agonists • Sodium bicarbonate Removal of potassium from the body (slow-acting) • Diuretics • Cation exchange resins • Hemodialysis  IV calcium infusion (e.g. calcium gluconate or calcium chloride) Hyperkalemia Tall peaked Twave ! K• 6 to 7 mEq/L -- Tall peaked Twave Widened ORS (sine wave pattern) AV node block Fascicle & BB blocks ! K•7 to 8 mEq/L -- K'>8 mEq/L  If patient has CAD, beta-2 agonists are contraindicated because they can cause tachycardia and precipitate angina. • Calcium gluconate or insulin with glucose is typically reserved for hyperkalemia in patients with ECG changes, K  6.5 mEq, or rapidly rising K. Renal 22 https://t.me/usmleinnercircle Version 2 Hypokalemia  What is the likely diagnosis in a patient that presents with muscle cramps/weakness and hyporeflexia with broad, flat T waves on EKG? Hypokalemia ECG changes: T-wave flattening, ST depression, Presence of U Waves le  What is the likely underlying cause of refractory hypokalemia in an alcoholic patient? Hypomagnesemia features ECG findings rC Hypomagnesemia Hyperaldosteronism Increased beta-adrenergic activity (eg, albuterol) Muscle weakness & cramps Hyporeflexia Rhabdomyolysis Cardiac palpitations/arrhythmias Broad, flat T waves ProminentU waves Potassium replacement LE Treatment .. .. .. . Diuretic therapy (eg, thiazides) Vomiting/diarrhea ne Clinical "U"..waves. in Hypokalernia Hypokalemia In Etiologies .. .. . irc (intracellular Mg2 typically inhibits potassium secretion by renal outer medullary potassium ROMK channels) U SM Hypophosphatemia Internal redistribution Decreased intestinal Causes of hypophosphatemia • Increased insulin secretion (especially refeeding malnourished patients) • Acute respiratory alkalosis (stimulates glycolysis) • Hungry bone syndrome (after parathyroidectomy) • Chronic poor intake • Aluminum- or magnesium-containing antacids (bind phosphate) absorption • Steatorrhea or chronic diarrhea Increased • Primary & secondary hyperparathyroidism urinary excretion • Vitamin D deficiency (i GI absorption, t urinary excretion) • Primary renal phosphate wasting syndromes • Fanconi syndrome Renal Cysts Renal 23 https://t.me/usmleinnercircle Version 2 Characteristics of renal cysts Simple renal cyst Malignant cystic mass Thin, smooth, regular wall Thick, irregular wall Unilocular Multilocular No septae Multiple septae, occasionally thick & calcified Homogeneous content Heterogeneous content (solid & cystic) Absence of contrast enhancement on CT/MRI Presence of contrast enhancement on CT/MRI Usually asymptomatic May cause pain, hematuria, or hypertension No follow-up needed Requires follow-up imaging & urological evaluation for malignancy .. .• • .. • .. Autosomal dominant polycystic kidney disease Clinical presentation Ultrasound characteristics of autosomal dominant polycystic kidney disease Known family history but unknown genotype Age 15-39: ≥3 unilateral or bilateral kidney cysts Age 40-59: ≥2 cysts in each kidney Age ≥60: ≥4 cysts in each kidney Unknown family history Multiple (usually ≥10) in each kidney regardless of age Extrarenal features Diagnosis Most patients asymptomatic until age 30-40 Flank pain, hematuria Hypertension Palpable abdominal masses (usually bilateral) Chronic kidney disease (CKD) Cerebral aneurysms Hepatic & pancreatic cysts Mitral valve prolapse, aortic regurgitation Colonic diverticulosis Ventral & inguinal hernias • Ultrasonography showing multiple renal cysts • Aggressive control of risk factors for CV & CKD Management . • ACE inhibitors preferred for hypertension Hemodialysis, renal transplant for ESRD CV= cardiovascular;ESRD = end-stage renal disease. I  What are the common extra-renal complications of ADPolycystic Kidney Disease?  Hepatic cysts  Berry aneurysm : routine screening is not recommended Renal 24 https://t.me/usmleinnercircle Version 2  MVP  Arterial hypertension ("morning headaches") ADPKD can also produce hypertension through activation of RAAS. Can present with pain and hematuria and be mistaken for stones)  What is the preferred medication for hypertension associated with autosomal dominant PKD? ACE inhibitors le Fibromuscular Dysplasia irc  Non-inflammatory and non-atherosclerotic condition that commonly involves the renal, carotid, and vertebral arteries Males = females (vs. adults 81 female:male) Clinical features? • Renal rC  What is the gender disparity of fibromuscular dysplasia in children? Secondary Hypertension - Due to Secondary Hyperaldosteronism 0 Abdominal bruit at costovertebral angle 0 CKD symptoms In ne 0 • Cerebrovascular (involves carotids) Headache, pulsatile tinnitus, TIA 0 Subauricular Bruit Diagnostics? LE 0 • Duplex ultrasound and/or CT angio Non-invasive imaging) U SM Initial for renal: Duplex U/S and/or CT angio Cerebrovascular FMD CT angio Gold standard: Digital Subtraction Angiography (“string of beadsˮ) Renal 25 https://t.me/usmleinnercircle Version 2 Fibromuscular dysplasia • 90% women (in adults) Internal carotid artery stenosis . Clinical presentation . Physical examination Diagnosis Treatment . . . . . o Recurrent headache o o Pulsatile tinnitus Transient ischemic attack o Stroke Renal artery stenosis o Secondary hypertension o Flank pain Subauricular systolic bruit • Abdominal bruit Imaging preferred (eg, duplex US, CTA, MRA) Catheter-based arteriography Antihypertensives (ACE inhibitors or ARBs 1st line) PTA • Surgery (if PTA unsuccessful) ARB = angiotensinII receptorblocker;CTA = CT angiography; MRA = MR angiography;PTA = percutaneous transluminal Iangioplasty;US = ultrasonography. Clinical features of FMD Women age <50 with 1 of the following: • Severe or resistant hypertension Patients to screen • Onset of hypertension before age 35 • Sudden increase in blood pressure from baseline • Increase in creatinine (;;>,0.5-1mg/dl) after starting angiotensin-converting enzyme inhibitor or angiotensin receptor blocker & without significant effect on blood pressure • Systolic-diastolic epigastric bruit • Resistant hypertension from renal artery involvement • Cerebrovascular FMD with symptoms of brain ischemia (eg, amaurosis fugax, Homer's Clinical presentation syndrome, transient ischemic attack, stroke) • Nonspecific symptoms (eg, headache, pulsatile tinnitus, dizziness) from carotid or vertebral artery involvement • Can also involve iliac, subclavian & visceral arteries • Noninvasive testing preferred (eg, computed tomography angiography, duplex ultrasound) Diagnosis & follow-up • Catheter-based digital subtraction arteriography for patients with inconclusive noninvasive testing • Medically treated patients need follow-up blood pressure & creatinine every 3-4 months & renal ultrasound every 6-12 months FMD = fibromusculardysplasia. Acute Kidney Injury Renal 26 https://t.me/usmleinnercircle Version 2 Evaluation of acute kidney injury Evidence of volume depletion? ] r-Yes No--i Normal Urinalysis with microscopy No - Abnormal! I Yes Sterile pyuria Hematuria ± proteinuria j Granular casts &for epithelial cells j ! Monitor to resolution l j ! Evaluate for AIN Evaluate for glomerulonephritis le -- irc Improvement with IV fluids? Evaluate for ATN rC ·Renal ultrasonography. AIN = acute lnterstlllal nephritis; ATN = acute tubular necrosis, IV= Intravenous. Exclude urinary obstruction• QUWorld Give a trial of IV fluids first if evidence of volume depletion else go for UA ne Management of acute oliguria History & physical examination (eg, assessfor benign pro static hyperplasia) In No significant urine retention -------1 Bedside bladder scan to assessfor urinary retention LE Serum & urine biochemistry ±imaging Pre-renal causes, U SM Hypovolemia Sepsis Low cardiac output (eg, heart failure) Fluid administration if appropriate or treat underlying cause Renal causes, Acute tubular necrosis Interstitial nephritis Glomerular disease Treat underlying cause Significant urine retention Urethral catheter to decompress bladder Serum & urine biochemistry ± imaging Treat underlying cause (eg, benign prostatic hyperplasia. malignancy) with urologic consultation Normal Postvoid residual ≤150 mL in women, ≤50 mL in men  What is the next step in evaluation of acute kidney injury in an elderly patient with BPH who presents with rising creatinine and urinary urgency without hematuria, weight loss, or elevated PSA? Renal ultrasound: helps assess for hydronephrosis and other causes of obstruction Renal 27 https://t.me/usmleinnercircle Version 2  What is the likely diagnosis in an afebrile elderly male with BPH who experiences agitation and lower abdominal tenderness 2 days after surgery? Acute urinary retention : diagnosis is confirmed by bladder ultrasound (  300 mL of urine ) ; treatment is insertion of a Foley catheter Prerenal acute kidney injury • Decreased renal perfusion o True volume depletion o Decreased EABV (eg, heart failure, cirrhosis) Etiology o Displacement of intravascular fluid (eg, sepsis, pancreatitis) o Renal artery stenosis o Afferent arteriole vasoconstriction (eg, NSAIDs) • Increase in serum creatinine (eg, 50% from baseline) • Decreased urine output Clinical features • Blood urea nitrogen/creatinine ratio >20:1 • Fractional excretion of sodium <1% • Unremarkable ("bland") urine sediment Treatment • Restoration of renal perfusion IV normal saline : need to restore renal perfusion and prevent development of acute tubular necrosis Clinical features of crystal-induced acute kidney injury . . . . . . . . • Acyclovir Common etiologies Clinical presentation Methotrexate Ethylene glycol Protease inhibitors Uric acid (tumor lysis syndrome) Usually asymptomatic • AKI g days of starting drug . Management Sulfonamides . UA: Hematuria, pyuria & crystals Increased risk with volume depletion, CKD Discontinuation of drug • Volume repletion Loop diuretic AKI = acute kidney injury; CKD = chronic kidney disease; UA = urinalysis. Also excessive vitamin C ACE inhibitors (eg, lisinopril) are used with caution in acute kidney injury because of the risk of exacerbating hyperkalemia. • Acute tubular necrosis : characterised by muddy brown casts; distinguishing features from pre-renal AKI include BUN:creatinine ratio  201, urine Na+  40 mEq/L, and FeNA  2% Look at your fucking BUNCr. Do not let these fuckerʼs trick you— ATN can be produced by ischemia, so donʼt always jump to pre-renal azotemia BUNCr 201 Renal 28 https://t.me/usmleinnercircle Version 2 Acute kidney injury Acute tubular necrosis BUN/creatinine ratio Typically >20 Typically normal (-10-15) Urine sodium <20 mEq/L >40 mEq/L Fractional excretion of sodium <1% >2% Urine osmolality >500 mOsm/kg -300 mOsm/kg Urine specific gravity >1.020 <1.020 Microscopy Bland Muddy brown casts le Prerenal  Most cases of renal failure result in hypocalcemia due to reduced renal phosphorus excretion; irc because phosphorous combines with calcium to form calcium phosphate salts, serum calcium levels usually decline. rC Patients who have acute renal failure and hypercalcemia often have an underlying malignancy that is driving calcium release. This is most often seen in the setting of multiple myeloma Contrast Induced AKI Risk factors Creatinine elevation 24-48 hr after arterial contrast administration Gradual return to baseline within 3-7 days Chronic kidney disease (particularly diabetic nephropathy} High contrast load Hypovolemia NSAID use Prerenal vasoconstriction Direct tubular cytotoxicity Clinical diagnosis (ie, biopsy usually not needed) LE Pathophysiology .. .• • . .. .. . • .. In Presentation ne Contrast-associated acute kidney injury Diagnosis U SM Prevention FeNa <1% & muddy brown. granular casts Patchy necrosis of renal tubular cells on biopsy Optimize renal perfusion (eg, 0.9% saline) Hold NSAIDs Use lowest volume of contrast agent possible FeNa ;;;fractionalexcretionof sodium;NSAID;;; nonsteroidalanti-inflammatorydrug. Clinical Diagnosis, Biopsy not needed • Metformin is typically held before administering contrast to patients at risk for CAAKI. done in order to prevent metformin accumulation (eg, lactic acidosis) from reduced renal elimination. It does not reduce the risk for CAAKI itself. Rhabdomyolysis Renal 29 https://t.me/usmleinnercircle Version 2 Rhabdomyolysis Drug-induced rhabdomyolysis Major mechanism Direct myotoxicity Vasoconstrictive ischemia Prolonged immobilization (compression ischemia) .. .. .. .. . Examples Skeletal muscle lysis/necrosis due to: Stalins, fibrates Colchicine Etiology Ethanol Cocaine • Intense muscle activity (eg, seizure, exertion) • Drug/medication toxicity (eg, statins) • Muscle pain & weakness Cocaine Amphetamines Ethanol • Crush injury or prolonged immobilization • Dark urine (myoglobinuria/pigmenturia) Clinical features • + Blood on urinalysis & no RBCs on microscopy • l Serum K & PO4 , t serum Ca, l AST > ALT Opioids Benzodiazepines • Acute kidney injury Diagnosis Management • Serum creatine kinase >1,000 U/L • Consistent clinical features • Aggressive intravenous fluid resuscitation • Sodium bicarbonate in some cases ALT= alanine aminotransferase; AST= aspartate aminotransferase; RBCs = red blood cells .  Other laboratory abnormalities indicative of rhabdomyolysis include: • Hyperkalemia and hyperphosphatemia, which result from the release of these electrolytes from lysed muscle cells into the bloodstream. • Hypocalcemia, which is often present due to deposition of calcium in damaged muscle tissue. • Moderate transaminase elevations, which characteristically show greater elevation in aspartate aminotransferaseAST than alanine aminotransferase ALT due to relatively higher levels of AST in skeletal muscle.  The intravascular volume depletion causes an initial prerenal insult, which is followed by a subsequent insult from direct tubular toxicity of heme (pigment nephropathy). Affected muscle groups must be monitored closely because the initial tissue damage and subsequent volume replacement create a risk for acute compartment syndrome.  What is the likely diagnosis in a patient who overdosed on cocaine and presents with elevated K and creatine phosphokinase CPK? Rhabdomyolysis • Statins can cause many adverse effects on skeletal muscle (eg, asymptomatic inflammation, myopathy, rhabdomyolysis), possibly by impairing regulatory proteins (eg, coenzyme Q. Colchicine inhibits microtubule function, leading to myocyte waste product accumulation. Synergistic toxicity can lead to widespread skeletal muscle necrosis. • Creatine phosphokinase is a marker of skeletal muscle damage, and serum elevation 1,000 U/L confirms the diagnosis in the appropriate clinical setting. Renal 30 https://t.me/usmleinnercircle Version 2 • Opioids and other CNS depressants (eg, ethanol, benzodiazepines) can cause rhabdomyolysis by inducing impaired consciousness with prolonged immobilization and ischemic compression of dependent areas of skeletal muscle (eg, mottled skin over back, buttocks, and posterior thighs). Analgesic Nephropathy Analgesic nephropathy • Prolonged use of combination analgesics o Acetaminophen: direct oxidative renal toxicity o Aspirin/NSAIDs: depletion of glutathione impairs deactivation of reactive acetaminophen metabolites • • History of chronic pain (eg, headaches, lower back pain) • Often asymptomatic, may have malaise, fatigue, flank pain le Pathophysiology Clinical presentation • Urinalysis: WBCs & WBC casts ± mild proteinuria • Hematuria & urine RBCs if papillary necrosis is present • Imaging: small kidneys ± papillary calcification & irregular contours rC NSAIDs = nonsteroidal anti-inflammatorydrugs; RBCs = red blood cells; WBCs = white blood cells. irc Manifests as chronic interstitial nephritis ± papillary necrosis • NSAID-induced acute kidney injury: ne Patients with intravascular volume depletion (eg, vomiting, diarrhea) normally have increased renal prostaglandin production to dilate the afferent arteriole and maintain the glomerular filtration rate. Nonsteroidal anti-inflammatory drugs inhibit prostaglandin synthesis, which can cause prerenal In azotemia. Hemodialysis LE e.g: Cirrhosis Which is a state of reduced intravascular volume Indicationsfor urgent dialysis (AEIOUl U SM Acidosis ~lectrolyte abnormalities Ingestion Qverload !,!_remia • Metabolic acidosis . 0 Symptomatic hyperkalemia 0 ECG changes or ventricular arrhythmias • Severe hyperkalemia .• • . • . 0 Potassium >6.5 mEq/L refractory to medical therapy Toxic alcohols (methanol, ethylene glycol) Salicylate Lithium Sodium valproate, carbamazepine Volume overload refractory to diuretics Symptomatic: 0 0 0 Renal pH <7.1 refractory to medical therapy Encephalopathy Pericarditis Bleeding 31 https://t.me/usmleinnercircle Version 2 Cardiovascular risk factors in ESRD patients • Hypertension, diabetes mellitus & dyslipidemia Traditional risk factors • Left ventricular hypertrophy • Advanced age & low physical activity • Anemia of chronic kidney disease ESRD-specific risk factors • Vascular calcifications (i phosphorus, j calcium) • Oxidative stress related to uremia and dialysis ESRD = end-stage renal disease. oxidative stress; resulting in accelerated atherogenesis and inhibition of nitric oxide synthesis.  What is the most common cause of death in dialysis and renal transplant patients? Cardiovascular disease : accounts for approximately 50% of all deaths in dialysis patients Patients with chronic renal failure are prone to volume overload, hyperkalemia, hyperphosphatemia, metabolic acidosis, hyperparathyroidism, osteodystrophy, and anemia. These patients should minimize their intake of fluids, potassium and phosphate  Patients with chronic kidney disease CKD are at increased risk of coronary artery disease CAD) and cardiovascular events. Physicians should have a low threshold for pursuing cardiac stress testing (eg, stress echocardiography) in CKD patients with atypical CAD symptoms (eg, exertional dyspnea). Calcific Uremic Arteriolopathy Calcific uremic arteriolopathy (calciphylaxis) Pathophysiology • Arteriolar & soft tissue calcification • Local tissue ischemia & necrosis • End-stage renal disease Risk factors • Hypercalcemia, hyperphosphatemia • Hyperparathyroidism • Oral anticoagulants (eg, warfarin) • Painful nodules & ulcers Clinical manifestations • Soft tissue calcification on imaging • Diagnosis requires skin biopsy: arteriolar calcification/occlusion, subintimal fibrosis • Analgesia & wound care Management • Treatment of risk factors (eg, high phosphorus, high calcium) • Optimization of dialysis Vitamin K promotes synthesis of matrix proteins that prevent vascular calcification and reduce the risk of calciphylaxis. Therefore, patients with vitamin K deficiency or who are taking vitamin K inhibitors (eg, warfarin) are at increased risk of developing calciphylaxis. Renal 32 https://t.me/usmleinnercircle Version 2  Calciphylaxis is most common in areas of adiposity (eg, trunk, thighs) and, in contrast to atherosclerotic ischemia, usually presents with intact peripheral pulses. Laboratory studies can show high serum parathyroid hormone and hyperphosphatemia (which is common in patients with ESRD due to reduced renal clearance of phosphate). Although hypercalcemia (not hypocalcemia) is also a risk factor, normal serum calcium levels do not rule out calciphylaxis because tissue calcium concentrations may be much higher due to local inflammatory changes le CardioRenal Syndrome irc Cardiorenal syndrome r rC LV & RV failure T Central venous & renal venous pressure Vasoconstriction & T Na & H2O reabsorption ( r Reduced forward flow ne Venous congestion !Cardiac output ! Glomerular capillary filtration gradient RAAS activation l ! Renal artery perfusion In r GFR. LE • Diuretics are usually the most effective therapy for AKI due to cardiorenal syndrome; the resulting reduction in renal venous pressure restores the glomerular capillary filtration gradient and improves U SM Renal Stones Renal 33 https://t.me/usmleinnercircle Version 2 Management of symptomatic ureteral stones Initial symptom control: • Analgesia • Antiemetics IV fluids Hematuria, Colicky Flank Pain Radiating to the Groin, Nausea / Vomiting Consider Something Else (Highly Sensitive) 1 Pass Stone Assess for the following: • Urinary infection • Acute kidney injury • Complete obstruction (anuria) • Intractable pain, nausea, vomiting Analyze Stone NOW + 24-hr Urine >6 weeks later Children may present with Painless Hematuria + l l Inpatient admission Urgent urology consultation • IV medications (analgesics, fluids± antibiotics) • Alpha blocker if stone >5 mm and s10 mm • Strain urine Outpatient management • Pain control • Oral hydration • Alpha blocker if stone >5 mm and s10 mm • Strain urine 1 Outpatient urology consultation for: Stone >10 mm No stone passage in 4-6 weeks Persistent pain ©UWa<ld Pain Control: morphine (narcotic) and ketolorac NSAID Renal 34 https://t.me/usmleinnercircle Version 2 Evaluation of renal colic during pregnancy History & physical examination, urinalysis, serum chemistry panel I Stone suspected 1 Renal & pelvic ultrasound ] Positive Treat as indicated } 1 Transvaginal ultrasound ] Positive • Treat as indicated ] I irc Negative Treat empirically for a stone & observe closely OR Low-dose CT urogram (2nd & 3rd trimester only) rC OR Magnetic resonance urogram le Negative ne  Uric acid stones : radiolucent therefore must be evaluated by abdominal CT, ultrasound, or IV pyelography (not X-ray) • LE In  Be careful! Do not choose CT with contrast! For test purposes, don't pick U/A  presence of hematuria is nonspecific U SM An intravenous pyelogram IVP) uses IV contrast and plain x-ray to visualize the urinary system. IVP was previously the test of choice for diagnosing urinary stones. However, due to the risk of contrast administration (eg, allergy, acute kidney injury), non-contrast CT is now preferred.  What is the likely diagnosis in a patient with intermittent flank pain, low-volume voids, and occasional episodes of high-volume voids? Obstructive uropathy e.g. due to renal calculi; high-volume voids occur due to large volume of retained urine overcoming the obstruction (post-obstructive diuresis) Excessive diuresis may lead to potassium wasting and dehydration, both of which can cause weakness Renal 35 https://t.me/usmleinnercircle Version 2 Prevention of recurrent nephrolithiasis Dietary measures • Increase fluids (produce >2L urine/day) • Reduce sodium (<100 mEq/dL) • Reduce protein • Normal calcium intake (1200 mg/day) • Increase citrate (fruits & vegetables) • Reduced-oxalate diet for oxalate stones (dark roughage, vitamin C) Drug therapy • Thiazide diuretic • Urine alkalinization (potassium citrate/bicarbonate salt) • Allopurinol (for hyperuricosuria-related stones) Pyelonephritis Treatment of urinary tract infection in nonpregnant women • Nitrofurantoin Uncomplicated UTI Complicated UTI* • Trimethoprim-sulfamethoxazole • Fosfomycin (single dose) • Fluoroquinolones only if previous options cannot be used • Urine culture only if initial treatment fails • Outpatient: fluoroquinolones • Inpatient: ceftriaxone, piperacillin-tazobactam, carbapenems (eg, imipenem) • Culture obtained prior to therapy, with adjustment of antibiotic as needed *Infection above the bladder (eg, pyelonephritis), pelvic pain in men, other signs or symptoms of systemic illness.  When is imaging indicated with pyelonephritis? Not indicated unless complicated (obstruction, sepsis) or no response to antibiotics after 4872 hours  What is the next step in management for a patient with pyelonephritis that has clinically resolved after two days of IV ceftriaxone? Urine culture reveals E. coli sensitive to ceftriaxone and TMPSMX. Switch to oral TMPSMX most hospitalized patients can be transitioned to culture-guided oral antibiotics if symptoms are improved at 48 hours Renal 36 https://t.me/usmleinnercircle Version 2 Pyelonephritis in pregnancy . • Asymptomatic bacteriuria Risk factors Diabetes mellitus • Age <20 • Escherichia coli (most common) Common • Klebsie/la pathogens • Enterobacter • Groue B Stree_tococcus . • Preterm labor Complications Low birth weight • Acute respiratory distress syndrome le • Intravenous antibiotics • Supportive therapy irc Treatment  What is the treatment of choice in a pregnant patient with pyelonephritis? rC Hospitalize + ceftriaxone ne UTI prophylaxis for rest of the pregnancy if pregnant female has pyelonephritis In Urine culture is performed for all pregnant patients with acute cystitis and is repeated a week after completion of antibiotics to ensure infection resolution (eg, test of cure). U SM LE Asymptomatic Bacteriuria Renal 37 https://t.me/usmleinnercircle Version 2 Asymptomatic bacteriuria in pregnancy Definition . 2:100,000 CFU/ml bacteria • Pregestational diabetes Risk factors mellitus • History of urinary tract infection • Multiparity • Escherichia coli ( most Common pathogens Potential complications Treatment common) • Klebsiella • Enterobacter • Group B Streptococcus • Acute pyelonephritis • Preterm labor & delivery • Cefpodoxime • Fosfomycin • Amoxicillin-clavulanate • Nitrofurantoin* •Avoided in first & third trimesters. CFU = colony-formingunits. Increase risk of pyelonephritis, preterm birth, and low birth weight. Cystitis & asymptomatic bacteriuria during pregnancy Condition . . Asymptomatic bacteriuria . Clinical features Management Asymptomatic patient with positive urine • Amoxicillin* or amoxicillin-clavulanate for 5-7 culture (2:100,000 CFUs/mL) Screening usually performed at 12-16 weeks gestation Treatment reduces progression to UTI, pyelonephritis & complications (eg, preterm birth, low birth weight) . Acute cystitis . Symptomatic patient (eg, dysuria, urgency) with positive urine culture Considered a complicated UTI days . . . .. OR Cephalexin for 5-7 days OR Fosfomycin as a single dose OR Nitrofurantoin for 5-7 days (avoid in 1st trimester & at term) No fluoroquinolones in any trimester No trimethoprim-sulfamethoxazole in 1st trimester or at term •Limited use as a single agent due to potential resistance among gram-negativebacteria. CFU = colony-formingunit; UTI = urinary tract infection. Recurrent UTI Renal 38 https://t.me/usmleinnercircle Version 2 Recurrent urinary tract infection Definition • .1:2infections in 6 months • .1:3infections in 1 ):'.ear • History of cystitis at age S15 Spermicide use Risk factors • • New sexual partner • Postmenopausal status Urinalysis Evaluation • Prevention Urine culture • Behavior modification • Postcoital or daily antibiotic prophylaxis • Topical vaginal estrogen for postmenopausal patients irc Same for recurrent cystitis le . rC Renal Abscess LE In ne Renal abscess U SM  Renal and perinephric abscesses manifest with insidious onset of flank pain and systemic symptoms (eg, fever, weight loss), typically in patients with a history of urinary tract infection or extrarenal infection (eg, bacteremia) in the prior 12 months. In most cases, the urinalysis demonstrates pyuria, bacteriuria, and proteinuria, but it may remain normal if the abscess is not in contact with the collecting ducts. Nephrotic Syndrome Renal 39 https://t.me/usmleinnercircle Version 2 Nephrotic syndrome Glomerular injury t Glomeru lar permeability l t Liver protein & lipid synthesis I ! Hyperlipidemia I I Oncotic pressure t Aldosterone & t ADH secretion I Edema ADH = antidiuretichonnone. I+-- L I Blood flow to the kidneys l l Na• & water retention t Renin tHydrostaticpressure ! - Vasoconstriction IDUWorld Patients with nephrotic syndrome are at risk of developing atherosclerosis due to the loss of albumin, which triggers a compensatory increase in hepatic synthesis of cholesterol/triglyceride levels.  What is the earliest renal abnormality in patients with diabetic nephropathy? Glomerular hyper-filtration (elevation in GFR  First change that can be quantitated is thickening of GBM ⟶ mesangial expansion ⟶ nodular sclerosis  The pathologic hallmark of diabetic nephropathy is nodular glomerulosclerosis (with KimmelstielWilson nodules), but diffuse glomerulosclerosis is more common.  Which type of nephrotic syndrome is most commonly associated with renal vein thrombosis & Malignancy? Membranous nephropathy may present acutely with abdominal pain, fever, and hematuria or gradually as worsening renal function and proteinuria in an asymptomatic patient (more common) Also, patients with SLE can get membranous nephropathy, making them even more hypercoagulable Look for proteinuria, hyperlipidemia, hypoalbuminemia, and edema in a patient with cancer. HIV-associated nephropathy  Usually advanced HIV but can also occur with normal CD4 count. Renal 40 https://t.me/usmleinnercircle Version 2  Can develop relatively quickly and typically presents with heavy proteinuria and rapidly progressive renal failure. Edema, hematuria, and hypertension may also occur. The diagnosis is confirmed with renal biopsy that demonstrates collapsing focal segmental glomerulosclerosis; tubuloreticular inclusions are usually visible on electron microscopy. Antiretroviral therapy ART may help kidney recovery and should be initiated in patients who develop HIVAN and are not already on therapy le prognosis for HIVAN is poor irc Glomerulonephritis Glomerulonephritis serologic evaluation l rC Glomerular hematuria (dysmorphic erythrocytes ± erythrocyte casts) ne Serum complement Low + Antibody mediated • Lupus nephritis: ANA, anti-dsDNA antibodies • Membranoproliferative GN: cryoglobulins, anti-HCV • Infection-related GN • Poststreptococcal GN: ASO antibodies • Staphylococcus-associated GN: +tissue/blood culture • Endocarditis-associated GN: +blood culture • Goodpasture disease: anti-GBM antibodies • Pauci-immune GN (GPA, MPA, EGPA):ANCA LE In Immune complex mediated• 'lgA nephropathy Is Immune comptex mechated; however, serum complement Is typically normal. Lower complement consumption may result from igA's plimanly local complement activation & rts limited effects on the classical pathway. U SM ANA= anbnudear anbbod1es;ANCA = anbneutrophil cytoplasmic ant,bodoes;ASO = antistreptolysln O; dsDNA = double-stranded DNA; EGPA = eosonophilocgranulomatosis with polyang11bs; GBM = glomerular basement membrane; GN = glomerulonephnbs, GPA= granulomatosls with polyanglitis, HCV = hepatrtis C virus; MPA = microscopic polyangubs C,UWorld What diseases will have a low C3? PSGN, SLE, MPGN, Bacterial endocarditis MPGN is classically associated with cryoglobulinemic small-vessel vasculitis (eg, palpable purpura, arthralgia) Renal 41 https://t.me/usmleinnercircle Version 2 Glomerular hematuria Type of hematuria • Microscopic > gross • Gross > microscopic • Glomerulonephritis • Nephrolithiasis Common etiologies o Poststreptococcal • Cancer (eg, renal, prostate) o lgA nephropathy • Polycystic kidney disease • Basement membrane disorder o Clinical presentation Urinalysis Nonglomerular hematuria Alpert syndrome • Infection (eg, cystitis) • Papillary necrosis • Nonspecific or no symptoms • Dysuria • Nephritic syndrome • Urinary obstruction • Blood & protein • Blood but no protein • RBC casts, dysmorphic RBCs • Normal-appearing RBCs • Glomerulonephritis general key points: • the differential for the type of glomerulonephritis is extensive • RBC casts are typical of GN • Definitive diagnosis is via biopsy • Important to rule out nephrotic syndrome with UA spot test or 24 hr urine collection Presence of hematuria and pulmonary edema helps distinguish nephritic syndrome from other causes of edema (e.g. hypoalbuminemia, cirrhosis, heart failure)  What is the likely diagnosis in a young male with hemoptysis and hematuria without history of sinusitis? Goodpasture's disease granulomatosis with polyangiitis can cause hemoptysis and hematuria with upper airway involvement (e.g. sinusitis, otitis media, etc.) . Clinical . features lgA nephropathy Onset: spontaneous or several days after URI Episodic gross hematuria o ± Flank pain, low-grade fever, j BP • Asymptomatic, microscopic hematuria can occur Laboratory findings Diagnosis Prognosis/ risk factors .. . . . .. Urinalysis: protein, blood, red blood cell casts Normal serum C3 & C4 i Serum creatinine Usually clinical Confirmed by kidney biopsy: mesangial lgA deposition Often self-resolves End-stage renal disease in some patients 0 Risk factors: j creatinine, j BP, persistent proteinuria 0 Slowly progressive (>10 yr) BP = blood pressure; URI = upper respiratory infection. Renal 42 https://t.me/usmleinnercircle Version 2 Acute Interstitial Nephritis Management Infections (eg, Legionella, tuberculosis, CMV) New medication exposure Acute kidney injury Arthralgias, malaise Classic triad of~. s~ & eosinophilia rarely present** Clinical presentation Urinalysis: WBCs & WBC casts ± mild RBCs & proteinuria Peripheral eosinophilia ± urine eosinophils le Diagnosis Rheumatologic disease (eg, SLE, Sjogren syndrome, sarcoidosis) irc Clinical features Medications (eg, antibiotics, NSAIDs, PPls)* ± Renal biopsy: tubulointerstitial inflammation & edema Discontinue offending drug or treat underlying condition Systemic glucocorticoids Supportive hemodialysis if needed rC .. . .. .. .. .. .. . Causes Acute interstitial nephritis *Medicationsaccount for -75% of cases. **Classic triad is present in only -10% of cases. CMV = cytomegalovirus;NSAIDs = nonsteroidalanti-inflammatorydrugs; PPls = proton pump inhibitors; RBCs = red blood cells; SLE = systemic lupus ne erythematosus;WBCs = white blood cells.  Treatment of suspected medication-induced AIN is prompt discontinuation of the offending drug In with serial monitoring of renal function studies for spontaneous resolution of AIN. Bladder LE Patients whose renal function does not improve rapidly and those who have significant renal failure (eg, requiring hemodialysis) should be treated with glucocorticoids. U SM Interstitial cystitis (painful bladder syndrome) Epidemiology Clinical presentation Interstitialcystitis (painful bladdersyndrome) • More commonin women • Associatedwith psychiatricdisorders (anxiety)& pain syndromes(fibromyalgia) . • Bladderpain with filling, relief with voiding Frequency,urgency • Dyspareunia Diagnosis • Bladderpain with no other attributablecause for :esweeks • Normalurinalysis Treatment • Not curative;focus is on quality of life • Behavioralmodification& triggeravoidance • Amitriptyline • Analgesicsfor exacerbations • Chronic, noninfectious cystitis with unknown etiology Renal 43 https://t.me/usmleinnercircle Version 2 Pain can be exacerbated with sexual intercourse (dyspareunia) Urinary Incontinence Differential diagnosis of urinary incontinence Etiology Symptoms Stress L Urethral sphincter tone Urethral hypermobility Leakage with coughing, lifting, sneezing Urge Detrusor hyperactivity Sudden, overwhelming urge to urinate Overflow Impaired detrusor contractility Bladder outlet obstruction Incomplete emptying & persistent involuntary dribbling Urinary incontinence Type Symptoms Treatment • Lifestyle modifications Stress Leakage with Valsalva (coughing, sneezing, laughing) • Pelvic floor exercises • Pessary • Pelvic floor surgery Urgency Mixed Overflow Sudden, overwhelming, or frequent need to void Features of stress & urgency incontinence Constant involuntary dribbling & incomplete emptying • Lifestyle modifications • Bladder training • Antimuscarinic drugs • Variable treatment depending on predominant symptoms • Identify & correct underlying cause • Cholinergic agonists • Intermittent self-catheterization First-line treatment for any type of urinary incontinence includes bladder training and pelvic floor muscle Kegel) exercises Key idea: Neurogenic bladder can range from bladder atony to spastic bladder, but importantly these patients would have a clear neurological insult on NBME Multiple sclerosis, stroke, etc.)  Initial evaluation of mixed incontinence includes a voiding diary, which tracks fluid intake, urine output, and leaking episodes in order to classify the predominant type of urinary incontinence and determine optimal treatment. All patients with mixed incontinence generally require bladder training with lifestyle changes (eg, weight loss, smoking cessation, decreased alcohol and caffeine intake) and pelvic floor muscle exercises (eg, Kegels).  What type of catheterization is recommended for patients with neurogenic bladder to reduce the risk of catheter-associated UTI? Clean intermittent catheterization Renal 44 https://t.me/usmleinnercircle Version 2 i.e. insertion/removal of a clean urinary catheter every 4  6 hours; can be performed by patient or caregiver .. • Causes of urinary incontinence in the elderly Potentially reversible Urethral sphincter or pelvic floor weakness • Urogenital fistula • Multiple sclerosis • Dementia (eg, Parkinson, Alzheimer, normal pressure hydrocephalus) • Spinal cord injury, disc herniation • Delirium .. • • .. le Neurologic Bladder or urethral obstruction (eg, tumor, BPH) Infection (eg, UTI) Atrophic urethritis/vaginitis Pharmaceuticals (eg, alpha blockers, diuretics) Psychological (eg, depression) irc Genitourinary i Detrusor contractilily, detrusor overactivity Excessive urine output (eg, diabetes mellitus, CHF) Restricted mobility (eg, postsurgery) rC • Stool impaction BPH = benign prostatic hyperplasia;CHF = congestiveheart failure; UTI = urinary tract infection. In Urine analysis with culture ne  What is the next best step in management of a patient with acute urinary incontinence? The patient has a history of dementia and has been less active and sleeping more for the past week. Vitals are normal. Most common cause of UI in elderly patients are UTI's. Elderly patients often lack typical symptoms of UTI (like dysuria, fever). Patient may have delirium secondary to a UTI. LE Once infection is excluded, additional testing can be done to rule out other causes.  A continence pessary is used to treat stress urinary incontinence SUI and symptomatic pelvic organ prolapse (eg, anterior vaginal wall bulge); U SM it works by stabilising the pelvic floor in its anatomic position and compressing the urethra against the pubic symphysis. Midurethral sling procedures are performed for SUI due to urethral hyper-mobility or when pessary fails. Vaginal estrogen therapy is used in patients with genitourinary syndrome of menopause (eg, vaginal dryness, atrophy) due to estrogen deficiency. In postmenopausal patients, localized estrogen can relieve urinary symptoms (eg, stress and/or urge incontinence) related to atrophy. Renal 45 https://t.me/usmleinnercircle Version 2 Postmenopausal estrogen deficiency usually causes Urge Incontinence . • Stress urinary incontinence is often associated with urethral hyper-mobility. characterized by urethral angle of  30 degrees from horizontal with an increase in abdominal pressure (Q-tip test); SUI may also be caused by decreased urethral sphincter tone NORMAi. Resung STRESSURINARY INCONTINENCE • Postpartum SUI increases the risk of chronic SUI, but most cases are self-limited because the pelvic floor muscles and pudendal nerve heal after delivery; therefore, patients 6 weeks postpartum are managed with observation and reassurance and encouraged to perform Kegel exercises to strengthen pelvic floor muscles. Renal Artery Stenosis HTN-related symptoms .. .. . .. . . Clinical clues to renovascular disease Resistant HTN (uncontrolled despite 3-drug regimen) Malignant HTN (with end-organ damage) Onset of severe HTN (>180/120 mm Hg) after age 55 Severe HTN with diffuse atherosclerosis Recurrent flash pulmonary edema with severe HTN Physical examination Supportive evidence Asymmetric renal size (>1.5 cm) Abdominal bruit Laboratory results Unexplained rise in serum creatinine (>30%) after starting ACE inhibitors or ARBs Imaging results Unexplained atrophic kidney ARBs = angiotensinII receptor blockers;HTN = hypertension. Dx: Renal USG with Doppler Renal 46 https://t.me/usmleinnercircle Version 2  RAS causes Secondary Hyper Aldosteronism A systolic-diastolic abdominal bruit lateralizing to one side can be heard in 40% of patients; this finding has very high specificity(99%) for RAS. Treatment:  ACE, ARB  1st Line; Nephroprotective le -there is a higher risk of AKI; therefore, renal function should be monitored closely. irc Given for both unilateral and bilateral RAS ( continued if the creatine rise remains acceptable ie, 30%).  Revascularisation with stenting or angioplasty done only for refractory cases rC Transplant RAS typically occurs in the first 2 years after kidney transplantation. ne Renal Vein Thrombosis Renal vein thrombosis • Hypercoagulability In o Nephrotic syndrome (particularly membranous nephropathy) o Malignancy (particularly renal cell) Causes o Other (eg, inherited thrombophilia, oral contraceptive pills) • Trauma • Acute: renal infarction symptoms (flank pain, hematuria, j LDH, j kidney size on imaging) LE Clinical features • CT scan or MR angiography • Renal venography • Anticoagulation U SM Diagnosis • Chronic: renal symptoms typically absent (may cause pulmonary embolus) Treatment • Local thrombolysis/thrombectomy (if AKI present) AKI = acute kidney injury; LOH = lactate dehydrogenase. Renal Cancers • Renal cell carcinoma and hepatocellular carcinoma  Secrete EPO (causing polycythemia vera)  Patients age  35 with gross hematuria should be evaluated for urological neoplasms with CT urogram (diagnostic imaging) and cystoscopy. Renal 47 https://t.me/usmleinnercircle Version 2 Indications for cystoscopy • Gross hematuria with no evidence of glomerular disease or infection • Microscopic hematuria with no evidence of glomerular disease or infection but increased risk for malignancy • Recurrent urinary tract infections refractory to antibiotic therapy • Obstructive symptoms with suspicion for stricture, stone • Irritative symptoms without urinary infection • Abnormal bladder imaging or urine cytology  In a patient with with unexplained hematuria and risk factors for bladder cancer, cystoscopy is the preferred next step to examine the bladder and urethral linings. Eg. patient with BPH has gross and microscopic hematuria with extensive smoking history — Cystoscopy should be obtained to rule out bladder cancer  What is the most common malignancy associated with painless hematuria in adults? Bladder tumors especially patients age  35 with a smoking history Epidemiology Manifestations Diagnosis Staging Treatment .. .. . .. .. .. . Bladder cancer >90% urothelial carcinoma j Risk with smokers & exposure to industrial carcinogens Painless hematuria throughout micturition Irritative voiding symptoms (eg, frequency, urgency, dysuria) Regional pain Flexible cystoscopy with biopsy (gold standard) Urine cytology TURBT Upper urinary tract imaging (eg, IVP, MRI, CT) No muscle invasion: TURBT & intravesical immunotherapy Muscle invasion: radical cystectomy & systemic chemotherapy Metastatic: systemic chemotherapy & immunotherapy IVP = intravenouspyelogram;TURBT = transurethralresectionof bladdertumor.  What is the current recommended screening regimen for bladder cancer? Not recommended, even in patients with significant smoking and family histories bladder cancer screening does not provide survival benefit, even among high-risk populations. This is likely due to the following: • Current screening tests such as dipstick urinalysis (for hematuria), cytology of urine sediment, and urinary tumor biomarkers have relatively low sensitivity/specificity, leading to missed cases (low sensitivity) or unnecessary workup (false-positive testing). • Most bladder cancers are detected at an early stage, progress slowly, and are associated with relatively high rates of 5-year survival; therefore, earlier detection (via screening) provides minimal mortality benefit. Renal 48 https://t.me/usmleinnercircle Version 2 Ideal screening program • Common Disease characteristics • Serious • Long asymptomatic phase • Correctable if identified early Patient characteristics Test characteristics • High prevalence in screened population • Appropriate age range & gender • Low cost relative to disease severity • High sensitivity & specificity • Identifies population to be screened characteristics • Avoids "overdiagnosis" (detects benign/indolent disease) • Avoids "lead-time" bias (detects disease early but does not alter ultimate prognosis) irc Program le • Acceptable to broad range of patients • Improves outcomes of screened vs nonscreened population rC Miscellaneous ne Peripheral Edema Causes of peripheral edema Primary mechanism • Heart failure (left ventricular & cor pulmonale) • Primary renal sodium retention (renal disease & drugs) In Increased capillary hydrostatic pressure Clinical examples • Venous obstruction (eg, cirrhosis & venous insufficiency) Decreased capillary oncotic pressure • Protein loss (eg, nephrotic syndrome & protein-losing enteropathy) • Decreased albumin synthesis (eg, cirrhosis & malnutrition) LE (hypoalbuminemia) • Burns, trauma & sepsis U SM Increased capillary permeability Lymphatic obstruction/increased interstitial oncotic pressure • Allergic reactions • Acute respiratory distress syndrome • Malignant ascites • Malignant ascites • Hypothyroidism • Lymph node dissection Hematuria Renal 49 https://t.me/usmleinnercircle Version 2 Evaluation of gross hematuria Gross hematuria l History of trauma or suspected stone Imaging (CT scan/ultrasonography) -Yes-+ I No t Obtain urinalysis & urine culture Evidence of infection proteinuria, ood cell casts Give antibiotics j Other etiology suspected (eg, cancer) Evaluate for glomerular causes Imaging (CT scan) Cystoscopy Urine cytology C,UWorld Hematuria throughout urinary stream: Evaluation of red urine • Renal mass (benign/malignant) • Glomerulonephritis Red/brown urine, heme-positive dipstick • Urolithiasis • Polycystic kidney disease • Pyelonephritis Urinalysis • Urothelial cancer • Trauma ~3 RBC 0-2 RBC I Upper collecting system. Hematuria ) Hemoglobinuria • lntravascu1ar hemolysis • l Hemoglobin & haptoglobin CK = creatine lonase; RBC = red blood cells Myoglobinuria • Rhabdomyolysis • T CK Terminal hematuria: Lower collecting system • Urothelial cancer • Urolithiasis • Benign prostatic hyperplasia • Prostate cancer Initial hematuria: • Urethritis • Trauma (eg, catheterization) ©UWorld • Initial hematuria is characterised by blood at the beginning of the voiding cycle and often reflects a urethral source. • Total hematuria is characterised by blood during the entire voiding cycle and can reflect bleeding from anywhere in the urinary tract (eg, bladder, kidneys). • Terminal hematuria is characterised by blood at the end of voiding cycle and often suggests bleeding from the prostate, bladder neck or trigone, or posterior urethra. Significant exertion (eg, long-distance running) can lead to dark urine due to the following: • Myoglobinuria from skeletal muscle injury (ie, rhabdomyolysis) Renal 50 https://t.me/usmleinnercircle Version 2 • March hemoglobinuria Exertional hemoglobinuria) (eg, mechanical red blood cell RBC damage/hemolysis in the vasculature of the plantar surface vasculature of the feet) (rare) • Benign hematuria from bladder due to traumatic injury to the bladder mucosa Dx: clinical, T/t: Self Limited Dx of exclusion: Repeat UA after a week. le Renal Osteodystrophy Renal osteodystrophy t t,25-Dihyd<O><YC•~-t, fil1rat0e t Phosphorus rC y l Calcium irc Chronic kidney disease (l GFR) OP'mal L1moet• treatm7 In tea<,q~t, ne l PTH (secondary hyperparathyroidism) LE Oste1tis fibrosa cystica • High PTH, high bone turnover • l Mineralization with fibrosis, t fracture risk ""=•i~treatmeet Adynam1c bone disease • Low PTH, low bone turnover • l Cellularity & mineralization, l fracture risk Normal bone turnover U SM ·Treatment involves dietary phosphate restriction t phosphate binders Once phosphorus is nonnalized, vrtamin D can be given (while dosely monitonng for hypercalcem,a). C)UW..ld GFR = glomerular filtration rate; PTH = parathyroid honnone. Renal Drugs Renal 51 https://t.me/usmleinnercircle Version 2 Diuretic effects on total body electrolyte levels Diuretic type Loop (eg, furosemide) Thiazide (eg, HCTZ,metolazone) Uric Na• K+ HC03- ca 2 • U! u if ! i u ! i i i ! i ! - - ! ! ! ! - Potassium sparing (eg, spironolactone, amiloride) Carbonic anhydrase inhibitor (eg, acetazolamide) acid HCTZ = hydrochlorothiazide. • Acute nitrofurantoin-induced pulmonary injury is due to a hypersensitivity that can present with fevers, shortness of breath, dry cough, and erythematous rash. Symptoms usually begin 39 days from medication initiation. Bilateral basilar opacities and pleural effusions are common. Treatment involves cessation of nitrofurantoin. Paeds Renal • In utero hydronephrosis: if sufficient amniotic fluid is present, dont do anything. if there is oligohydramnios, you can try in utero surgical diversion of kidneys. (only in severe cases though) Hematuria Renal 52 https://t.me/usmleinnercircle Version 2 Evaluationof hematuriain children Hematuna (~3 RBC) J Glomerular' Nonglomerular Symptomatic Urinary tract infection (~ Urine culture, antibiotics J Reassurance Kidney stones (fl pain aySla ) Conside. • Renal ultrasound • Urine culture • Urine Ca Cr Renal ultrasound J rC CTscano~f abdomen pyuna) Perineal/meatal imtation le Trauma history irc Crea1J01ne, complement levels,CBC Asymptomabc 'Findings of glomelula- d.sease,ndude bro#n unne, edema. hypenens,on_1)(0Ceino.N, and RBC ca11S C.,,;Cr: calcMn 10ae t,.,. ra1JO,CBC : c:anc,lece blood C0Un1.RBC : blood 0111 ne • Young children are more likely to have stones located in the kidney, which are painless and cause an atypical presentation of gross hematuria alone, In The preferred imaging study for evaluation of nephrolithiasis in children is a renal and bladder ultrasound, which can detect some stones and ureteral obstruction while avoiding radiation exposure. LE CT scan of the abdomen without contrast is more sensitive, particularly for identifying small calculi, and may be required if the ultrasound is nondiagnostic Glomerular disease should be suspected with any of the following: U SM • Red blood cell casts (pathognomonic) • Proteinuria • Hypertension • Edema • Brown, cola-colored urine (in contrast to red/pink urine with lower urinary tract bleeding) Neonatal AKI Renal 53 https://t.me/usmleinnercircle Version 2 Common causes of neonatal acute kidney injury Prerenal disease Intrinsic renal disease Postrenal disease • Hypovolemia • Cardiac disease • Sepsis • Acute tubular necrosis (eg, ischemic injury) • Nephrotoxins (eg, maternal NSAID use, aminoglycosides) • Renal vascular disease (eg, renal vein thrombosis) • Glomerular & cystic disease (eg, polycystic kidney disease) • Congenital anomalies (eg, renal agenesis) • Obstructive uropathy (eg, posterior urethral valves) NSAID = nonsteroidal anti-inflammatory drug . All neonates with oliguria require further evaluation with the following: • History and physical examination to evaluate for possible risk factors (eg, nephrotoxins, family history, renal anomaly) and volume status (volume overload vs hypovolemia) • Renal and bladder ultrasound RBUS, which documents the number, shape, and size of the kidney(s) and detects vascular abnormalities or congenital anomalies. Nocturnal Enuresis Primary nocturnal enuresis Definition Causes of secondary enuresis • Nighttime urinary incontinence age ;?5 Etiology • No prior prolonged period of overnight dryness Psychologicalstress • Delayed maturation of bladder control Pathogenesis Risk factors • ; Nocturnalurine output (eg, j eveningfluids, ! ADH) • ! Bladder capacity • Family history Urinary tract infection Diabetes mellitus Diabetes insipidus • Boys age 5-8 Obstructive sleep apnea Evaluation • Urinalysis (to exclude other causes) • Voiding diary • Treatmentof comorbid conditions(eg, constipation) Management . . . . . Associated symptoms Behavior regression, mood !ability Oysuria, hesitancy, urgency, abdominal pain Polyuria, polydipsia, polyphagia,weight loss, lethargy,candidiasis Polyuria, polydipsia Snoring, dry mouth, fatigue, hyperactivity,irritability Secondary nocturnal enuresis is more likely to be due to a medical condition • Behavioral modifications (eg, restrict evening fluids) • Enuresis alarm • Desmopressin therapy ADH = antidiuretic hormone. Family history of bed wetting is the greatest risk factor. Constipation is commonly associated with bladder dysfunction and should be considered in any patient with enuresis. Because of the close proximity of the bladder to the rectum, fecal retention can lead to a decreased functional bladder capacity and instability of the detrusor muscle of the bladder. Although often mistaken as diarrhoea, encopresis (ie, fecal incontinence) is usually a sign of constipation and is characterised by leakage around impacted stool. Renal 54 https://t.me/usmleinnercircle Version 2 Left-sided firmness, or a palpable fecal mass, on abdominal examination further supports the diagnosis of constipation. Medical conditions causing enuresis Urinary tract infection Chronic kidney disease Diabetes mellitus Diabetes insipidus Obstructive sleep apnea Infrequent & hard stools • ± Palpable stool mass Encopresis • Daytime incontinence • ± Recurrent urinary tract infections • Weak stream, urgency, straining . .. • Dysuria, urgency, frequency • Abdominal pain • Daytime incontinence . . . • Weight loss, fatigue Polyuria, polydipsia, polyphagia Positive urine culture Hypertension Proteinuria, hematuria le Bladder dysfunction* Findings • Glucosuria • Weight loss, fatigue Polyuria, polydipsia • Low urine specific gravity • Snoring Hyperactivity, inattention *Often a clinical diagnosis. rC Constipation* Symptoms irc .. Etiology • Adenotonsillar hypertrophy ne Evaluation of bladder dysfunction in children Bladder dysfunction in children In 1 p .. _u_rin_a_l_ys_i_s..,ositive--+ I Treat underlying cause (eg, UTI, diabetes mellitus) I LE Negative U SM Nighttime symptoms only Manage nocturnal enuresis Daytime symptoms 1 Red flags for spinal cord anomaly? • Neurologic deficits• • Cutaneous lumbosacral abnormality No Yes +- +- Obtain spinal MRI for spinal dysraphism Consider VUR, constipation, behavioral etiologies un = urinary tract infection;VUR = vesicoureteralreflux. 'Lower extremityweakness.hypotonla,hyporefleXJa.  CKD in children is most commonly due to a congenital urinary tract abnormality such as posterior urethral valves PUV. PUV is an obstructive uropathy that prevents the outflow of urine from the bladder, causing symptoms of bladder dysfunction such as urinary incontinence (during the day and night) and a weak stream. Urinary stasis and associated vesicoureteral reflux from the distended Renal 55 https://t.me/usmleinnercircle Version 2 bladder into the ureters can cause damage to the kidneys and predispose patients to recurrent UTIs, which further contribute to renal scarring. Fatigue, poor growth, and hypertension in a child, in addition to urinary symptoms, should also raise suspicion for CKD. Proteinuria (and/or hematuria) on urinalysis may be present and cause edema if in the nephrotic range (typically 3. The next step in evaluation of CKD is to check a serum creatinine level as an indicator of renal function. Imaging with renal ultrasound and voiding cystourethrogram may also be considered. The first step in evaluation of all patients with nocturnal enuresis (both primary and secondary) is a urinalysisUA. Vesicoureteral Reflux Symptoms? Recurrent UTI's in neonate/child Diagnosis? • UA, urine culture • U/S • Contrast voiding cystourethrogram (test of choice for definitive diagnosis) indicated in: 0 2 episodes of febrile UTIs 0 Renal anomaly detected on U/S • Findings: retrograde reflux of contrast into ureters during micturition is diagnostic of VUR. Cystourethrogram: contrast instilled into bladder through foley ⟶ images obtained via fluoroscopy while child is voiding Primary vesicoureteral reflux in children Pathogenesis Presentation Diagnosis Management Prognosis • Retrograde urine fiow through a short intravesical ureter • Febrile urinary tract infection • Renal ultrasonography: hydronephrosis • Voiding cystourethrography: ureteral filling ± dilated collecting system • Mild (grade 1-11)*:observation ± prophylactic antibiotics • Severe (grade Ill-IV)*: prophylactic antibiotics± surgery • Mild: spontaneous resolution with growth • Severe (without intervention): renal scarring and chronic kidney disease *Plus screening/treatment of concomitant bladder & bowel dysfunction (eg, stool softeners/laxatives). Spontaneous resolution of VUR by age 5 is common due to growth of the intravesical ureteral segment Renal 56 https://t.me/usmleinnercircle Version 2 Vesicoureteral reflux Grade I Grade Grade II Grade Ill Grade IV Grade V le Normal Description Into a nondilated ureter Into the pelvis & calyces without dilation Ill Mild to moderate dilation of the ureter, renal pelvis & calyces, with minimal blunting of the fornices IV Moderate ureteral tortuosity & dilation of the pelvis & calyces V Gross dilation of the ureter, pelvis & calyces; loss of papillary impressions;ureteral tortuosity rC irc I II Renal scintigraphy with dimercaptosuccinic acid is the preferred modality for long-term ne evaluation for renal scarring. In UTI Urinary tract infection (UTI) in children LE • Female sex • Uncircumcised male infants • Urologic abnormalities (eg, vesicoureteral reflux) • Bowel/bladder dysfunction (eg, • Infants: fever, fussiness, poor feeding • Older children: dysuria, suprapubic/flank pain • Urinalysis: leukocyte esterase, nitrites, WBCs • Urine culture: bacteriuria • Antibiotics (eg, cephalosporin) • First febrile UTI: Risk factors U SM Clinical features Laboratory findings o Age <2: RBUS, followed by VCUG if abnormal Management o Age ;;,2: observation alone • Recurrent febrile UTls: RBUS & VCUG RBUS = renal & bladder ultrasound; VCUG = voiding cystourethrogram;WBCs = white blood cells. Recurrent UTI 2 episodes. Renal 57 https://t.me/usmleinnercircle Version 2 Indications for renal & bladder ultrasound Infants and children age< 24 months with a first febrile UTI Recurrent febrile UTls in children of any age UTI in a child of any age with a family history of renal or urologic disease, hypertension, or poor growth Children who do not respond to appropriate antibiotic treatment Ciprofloxacin is reserved for treatment of UTIs caused by Pseudomonas aeruginosa, which typically occur in patients with an indwelling catheter UTI's in Children 1. Urine analysis and urine culture 2. Renal and bladder U/S a. 224 mo w/ febrile UTI b. Recurrent UTI's (any age) 3. Voiding cystourethrography a. 1 mo. b. 2 years with recurrent UTI's (2 episodes of febrile UTIs) c. Organism other than E. coli d. if any anomaly seen in U/S  What is the next step in management for a 12-month-old girl with a confirmed E. coli UTI that is resolving after initiation of antibiotics? Renal and bladder ultrasound  What is the first step of diagnosis of a UTI in infants in diapers? Straight catheterization to obtain sterile specimen sample for UA and urine culture clean-catch specimens are unreliable in diapered patients due to high likelihood of contamination with stool or skin flora The presentation of UTI in infants is nonspecific and can include fever, fussiness, and poor feeding. Benign Proteinuria Renal 58 https://t.me/usmleinnercircle Version 2  What is the next step in management for a child that is found to have isolated proteinuria on urine dipstick? Repeat dipstick testing on two subsequent occasions if subsequent tests are negative, the diagnosis is transient proteinuria; if subsequent tests reveal continued proteinuria, further workup is warranted Benign proteinuria rC irc le • Defined as isolated proteinuria < 3.5 g/day i;;::i • Important, benign differential diagnosis in the evaluation of proteinuria • Very common; mostly affects younger individuals i;:::1 • Types of benign proteinuria • Orthostatic proteinuria (postural proteinuria): increased protein excretion only in the upright position • Transient proteinuria Most common cause of isolated proteinuria in children Causes: Heavy exertion/stress Fever Exposure to cold temperatures • A dipstick test should be repeated to exclude underlying disease. • No treatment necessary; excellent prognosis Most common cause of proteinuria in children ne • Orthostatic proteinuria is the most common cause of proteinuria in adolescents. The degree of proteinuria is mild and other significant renal abnormalities (eg, hematuria, acute kidney injury, hypertension) are absent. In Orthostatic proteinuria can be diagnosed by split (day and night) 24-hour urine collection showing elevated daytime but normal nighttime protein excretion rates. If diagnosed, NO TREATMENT NEEDED. LE Renal Surgery Genitourinary trauma U SM Gross hematuria following blunt trauma suggests an injury to what structures? Kidney or bladder 1. Thus, need to first do a CT scan to identify any renal trauma 2. Injury to the bladder can be determined by either a retrograde cystogram or CT cystogram 3. Blood at the urethral meatus suggests urethral injury -- perform retrograde urethrogram • Ability to insert a Foley catheter helps distinguish Bladder Injury from urethral injury Kidney Injury  Evaluation of kidney injury in blunt genitourinary trauma should include urinalysis and contrastenhanced CT of abdomen/pelvis in hemodynamically stable patients with evidence of flank pain and Renal 59 https://t.me/usmleinnercircle Version 2 ecchymosis, costovertebral area tenderness, and hematuria. may warrant retrograde cystourethrogram if patients have gross hematuria, difficulty urinating, blood at the meatus, or suprapubic pain Bladder  Rupture of the dome of the bladder can cause urine to leak into the peritoneal cavity and can lead to chemical peritonitis. Irritation of peritoneal lining of right or left hemidiaphragm may cause referred pain to shoulder ("Kehr sign") Retrograde cystography, in which the bladder is passively filled (eg, via Foley catheter) with water-soluble contrast and then imaged (eg, CT scan), can confirm the diagnosis Ureter Injury  Usually due to gynae procedures  Spl Hysterectomy  Ureter obstruction → Hydronephrosis → nonradiating back pain and costovertebral angle tenderness  Only 1 ureter involved usually → Creatinine normal  Dx: Renal USG  Unilateral ureteral laceration, during hysterectomy, may present with Intrabdominal Urine Collection and abdominal distention. might overflow through vaginal sutures as vaginal discharge. Urethral Injury Urethral injuries • The goal is to maintain urinary continence and sexual function. • Place suprapubic catheter to decompress bladder (diverts urine from the healing urethra and anastomosis) • Anterior urethral injury • Partial injury: place Foley catheter for healing by secondary intention • Penetrating injury: surgical exploration with debridement and defect repair with a direct anastomosis over a catheter • Posterior urethral injury • Endoscopic approach: early realignment (within 1 week) with combined transurethral and percutaneous transvesical approach • Surgical approach: place suprapubic catheter - delayed urethroplasty (6-12 weeks after initial injury)  What imaging modality is used to diagnose urethral injury? Retrograde urethrogram Extravasation of contrast from the urethra is diagnostic of urethral injury. Renal 60 https://t.me/usmleinnercircle  Any of the following should prompt evaluation for urethral injury: • Blood at the urethral meatus rC • Hematuria (eg, pinkish-red urine) irc le Version 2 • Difficulty voiding (due to disruption of urethral continuity) ne  Are most cases of Posterior urethral injury treated with immediate or delayed surgical repair? Delayed In typically treated with temporary urinary diversion by suprapubic catheter, followed by delayed repair • Anterior urethral injuries (eg, penile fracture, straddle injury) are typically repaired urgently (eg, within 24 hours) LE  Foley catheter NOT recommended for patients with suspected urethral injury U SM Post Operative Urinary Retention Postoperative urinary retention Risk factors Clinical features Management .. .. .. . .. . .. Age >50 years Surgery >2 hours duration >750 ml intraoperative fluids Regional anesthesia Neurologic disease Underlying bladder dysfunction Previous pelvic surgery Decreased urine output Abdominal distension Suprapubic pressure/pain Indwelling catheter Clean intermittent catheterization  Urinary retention is a common postoperative complication. Factors associated with an increased risk of POUR include: Renal 61 https://t.me/usmleinnercircle Version 2 • Patient factors: increasing age and male sex (eg, prostatic enlargement) • Type of surgery: hernia repair, joint arthroplasty, or anorectal operations • Anesthetic factors: prolonged anesthetic duration, excessive fluid administration, and use of medications (eg, opioids, anticholinergics) that impair bladder emptying Dx: Suprapubic Fullness, USG 300ml urine) Urethral Strictures Etiology Symptoms Complications Diagnosis Management .. .. .. . .. . .. . .. Urethral stricture Male > female Urethral trauma (eg, catheterization) Urethritis Radiotherapy Weak or spraying stream Incomplete emptying Irritative voiding (eg, dysuria, frequency) Acute urine retention Recurrent urinary tract infection Bladder stones Postvoid residual, uroflowmetry Urethrography Cystourethroscopy Dilation Urethroplasty Commonly Idiopathic Renal 62 https://t.me/usmleinnercircle Version 2 Pulmonary Physiology Ventilator Pulmonary Function Tests le Capnography PIP DLCO irc Hypoxemia Methemoglobinemia Cyanide Toxicity CO Poisoning rC Hemoptysis Head, Neck & Nose Stridor Laryngomalacia ne Torus Palatinus URI Epiglottitis Peritonsillar Abscess Sinusitis Tracheitis Septal Hematoma Neck Masses Mediastinum U SM Masses LE Laryngeal Papillomatosis Rhinitis In Retropharyngeal Abscess Acute Mediastinitis Obstructive Acute Bronchitis COPD Treatment Asthma Status Asthmaticus Occupational Asthma Exercise Induced Treatment Bronchiectasis Allergic Bronchopulmonary Aspergillosis Restrictive Interstitial Lung Disease Pulmonary 63 https://t.me/usmleinnercircle Version 2 Hypersensitivity Pneumonitis Sarcoidosis Pulmonary Hypertension OSA Pneumonia VAP Aspiration Pneumonia Recurrent Pneumonia Treatment Pleural Effusion Lung Abscess Pneumothorax Spontaneous Pneumomediastinum Pulmonary Embolism DVT Air embolism Fat Embolism ARDS Lung Cancer Pancoast Tumor Lung Carcinoid Tumor Head and Neck Cancer Nasopharyngeal Carcinoma Oral Leukoplakia Miscellaneous Chronic Cough Hyperventilation Syndrome Decompression Sickness High Altitude Sickness Diffuse Alveolar Hemorrhage Pulmonary Paeds Choanal Atresia CHARGE Syndrome Foreign Body Aspiration Nasal Foreign Body NRDS Persistent Pulmonary Hypertension of Newborn OSA Breath holding spells Sudden Infant Death Syndrome Drowning Pulmonary Surgery Post-operative Atelectasis Flail Chest Blunt Chest Trauma Hemothorax Tracheobronchial Injury Pulmonary Contusion Pulmonary 64 https://t.me/usmleinnercircle Version 2 Diaphragmatic Rupture Diaphragmatic Paralysis Physiology Topography of the lungs Lower border of Midclavicular Midaxillary Paravertebral pleura line line line 6th rib pleura) 8th rib 10th rib 10th rib Right - upper border 12th rib irc 8th rib Parietal pleura Left- lower border Type2 pneumocyte Ciliated airway particulate matter) Alveolar epithelial lining for gas exchange Surfactant production Stem cell reservoir for type 1 pneumocytes Mucociliary escalator Salt, water & moisture homeostasis LE epithelial .• (surfactant, pathogens, inhaled cell Club cell U SM Goblet cell Fibroblast . . Example disease association(s) . . PAP:impaired macrophage clearance of surfactant ne Type 1 pneumocyte Clearance of alveolar debris In Alveolar macrophage . . .. Normal function(s) rC Lung cells & functions Cell type le Lungs (& visceral Protection & repair of distal airway ARDSdue to inhalational injury • Hyaline membrane disease . . . . Secretion of mucins of prematurity Ciliary dyskinesia (Kartagener syndrome) Cystic fibrosis: airway desiccation COPD:tobacco smoke detoxification COPD & asthma: goblet cell metaplasia & mucus hypersecretion . Maintenance of interstitial lung tissue . . IPF:increased fibroblast activity Emphysema: decreased fibroblast activity ARDS= acute respiratory distress syndrome; COPD = chronic obstructive pulmonary disease; IPF = idiopathic pulmonary fibrosis; PAP= pulmonary alveolar proteinosis. Pulmonary 65 https://t.me/usmleinnercircle Version 2 Spectrum of ventilation/perfusion (V/Q) mismatch Intrapulmonary shunt Perfusion without venlilation (V=O) Normal V & a matched Dead-space ventilation Ventilation without perfusion (Q=O) UV !V 10 ua Diffuse pulmonary edema, ARDS Lobar pneumonia, localized atelectasis Segmental PE Massive PE, R-to-L shunt, severe PAH Consolidated regions ., 0 > Normally ventilated .;; 0 ... NormalV/Q UL_ 0 0 Unperfused regions 0 V/Qratio V/Qratio Normally perfused V/Qratio ARDS = acute respiratorydistress syndrome;PAH = pulmonaryarterial hypertension:PE = pulmonaryembolism. Ventilator  When using a ventilator, the goal is to keep SpO2 between 88  95%. PaO2 between 55  80 mmHg  When using a ventilator, prolonged FiO2 levels  60% are associated with oxygen toxicity. thus it is usually preferable to increase PEEP to allow for lower levels of FiO2 if oxygenation needs to be increased. 02 is changed with altering FiO2 or PEEP. CO2 is changed with tidal volume or respiratory rate (preferred)  In general, tidal volumes in mechanically ventilated patients should be about 68 ml/kg of ideal body weight. 70kg patient = tidal volume of 420 Pulmonary 66 https://t.me/usmleinnercircle Version 2 • Positive end-expiratory pressure (PEEP): ventilator setting that can be chosen to increase !~.~-~J.i.?.~.~1 __ (~~-i-~-~-~1 ..~.~P.?.~()Y. (:._~~) by reducing alveolar collapse during mechanical ventilation • Definition: Positive pressure(~ 5 cm H2 O) I;] in the lung is aintained over the entire inhalation and expiration phases. I;] • Mechanism of action PEEP j alveolar pressure and alveolar volume --, collapsed or unstable alveoli reopen __,improves ventilation/perfusion relation 60%) --> reduces the risk of oxygen toxicity • Advantages i Oxygenation j Gas exchange area j Functional residual capacity rC j Pulmonary compliance irc Avoidance of .................. atelectasis le Provides an adequate arterial l'a_Q2 at a low and safe concentration of oxygen (< • Complications of PEEP: • Barotrauma; typically involves worsening SOB after PEEP has been given to the pt • Pneumothorax; suspect this if the patient becomes hypotensive and mediastinal shift/tracheal ne deviation; diagnosed with CXR; treatment with needle decompression; can be secondary to In barotrauma. Noninvasive positive pressure ventilation LE .. . U SM Indications (strongest evidence) Contraindications . COPD (severe exacerbation, prevent extubation failure) Cardiogenic pulmonary edema Acute respiratory failure 0 Postoperative hypoxemic respiratory failure 0 lmmunosuppressed patients Facilitate early extubation .. . Medical instability Cardiac or respiratory arrest (or impending arrest) Severe acidosis (pH <7.1) Acute respiratory distress syndrome Nonrespiratory organ failure 0 Unstable cardiac arrhythmia/hemodynamic instability 0 Encephalopathy (Glasgow Coma Score <10) 0 Gastrointestinal bleed Inability to protect airway Uncooperative or agitated Inability to clear secretions/high aspiration risk Mechanical issues Recent esophageal anastomosis Facial or neurologic surgery, deformity, or trauma Upper airway obstruction . .. .. . COPD = chronic obstructive pulmonary disease. Somnolent patient or coma: inability to protect airway = Intubation Pulmonary 67 https://t.me/usmleinnercircle Version 2 • Positive pressure mechanical ventilation causes an acute increase in intrathoracic pressure. in the setting of decreased CVP (e.g. hypovolemic shock), this can cause acute loss of right ventricular preload with resultant sudden cardiac death  Continuous positive airway pressure CPAP predisposes users to recurrent epistaxis because of its drying effect on the nasal mucosa. Humidification is often effective in preventing CPAP-associated epistaxis. Effects of positive pressure ventilation in cardiogenic pulmonary edema t lntrathoracic pressure l RV preload T RV afterload (l Central venous return) (Pulmonary capillary compression) L l LV pre load l l MAP' J l LV transmural pressure gradient l LV afterload J Displaces interstitial lung water ! .... ~F~· t LV performance Pulmonary decongestion (Improved stroke volume---------- T PaO, (I Intrapulmonary •OOr l i End-organ o, delivery & survival •Aorticcompression triggersbaroreceptor reflexto lowerbtoodpressure. co • cardiacoutput; LV • lett ventricle;MAP• meanarterialpressure.Pao,• arterialpartialpressureof oxygen; PVR • pulmonaryvascularresistance;RV: right ventride. 0UWOl'ld Major Mortality Benefit  An endotracheal tube that is advanced too far will preferentially enter into the right mainstem bronchus. causes asymmetric chest expansion and markedly decreased breath sounds on the left side. Ideal Position: 26 cm above Carina  The initial criteria for extubation readiness include: • pH 7.25 • Adequate oxygenation (eg, PaO 60 mm Hg) on minimal support (ie, fraction of inspired oxygen FiO 40% and positive end-expiratory pressure PEEP 5 cm HO • Intact inspiratory effort and sufficient mental alertness to protect the airway Pulmonary 68 https://t.me/usmleinnercircle Version 2 Because there is short-term risk of recurrent respiratory failure requiring reintubation, most patients who meet the above criteria should undergo a spontaneous breathing trial SBT to help confirm readiness for extubation. During an SBT, patients remain intubated but ventilatory support is turned off Candidacy for successful extubation may be further assessed during an SBT using the rapid shallow breathing index RSBI, which is calculated by dividing respiratory rate (per minute) by tidal volume (liters). irc le Patients with a high RSBI (eg, 105 are breathing fast and shallow and are unlikely to do well without continued ventilatory support. rC Because an endotracheal tube can cause damage to the larynx and upper trachea over time (eg, tracheal stenosis), tracheostomy is indicated when prolonged intubation (eg, 710 days) is required. Mechanical ventilation can lead to respiratory muscle atrophy and insufficiency. ne Exercises such as physiotherapy, coughing, repositioning (sitting), and moving can improve respiratory sufficiency after mechanical ventilation. In Pulmonary Function Tests LE Pulmonary function tests in chronic lung disease COPD Interstitial lung diseases Pulmonary arterial hypertension Restrictive chest wall disease TLC Normal/j i l Normal l FEV1/FVC l* l Normal Normal Normal it l l Normal U SM Asthma DLCO Normal/j COPD = chronic obstructive pulmonary disease; DLCO = diffusing capacity for carbon monoxide; TLC = total lung capacity. *With positive bronchodilator response. tNormal in early COPD. Pulmonary 69 https://t.me/usmleinnercircle Version 2 Pulmonary auscultation examination findings Condition Breath sounds Tactile fremitus Percussion Mediastinal shift Normal lung Bronchovesicular (hilar), vesicular (peripheral) Normal Resonance None Consolidation (eg, lobar pneumonia) Increased (crackles & egophony present) Increased Dullness None Pleural effusion Decreased or absent Decreased Dullness Away from effusion (if large) Pneumothorax Decreased or absent Decreased Hyperresonant Away from tension pneumothorax Emphysema Decreased Decreased Hyperresonant None Atelectasis (eg, mucus plugging) Decreased or absent Decreased Dullness Toward atelectasis (if large) Diagnostic pulmonary tests Test Indications Disadvantages Spirometry Gold standard in evaluating pulmonary function (eg, FVC, forced expiratory volume in 1 second) May be difficult to perform in unstable patients Peak flow meter Assessment of airflow out of the lungs (peak expiratory flow rate) Less accurate than spirometry Chest x-ray First-line imaging of tracheal position, lung fields, bones & heart size with relatively low radiation Insensitive for small tumors & pulmonary embolus; no information about lung function Chest CT Rapid & detailed visualization of tracheal position, lung fields, bones & heart size Significant radiation exposure; no information about lung function Pulse oximeter Rapid assessment of oxygenation at fingertip, earlobe, or foot (infant) Inaccurate if the extremity is cold, calloused, or moving; cannot assess ventilation Arterial blood gas Quantitative measurement of arterial pH, oxygen/carbon dioxide/ bicarbonate levels & base deficit Slight risk of bleeding, infection & radial artery thrombosis FVC is the gold standard for assessing ventilation; decline in FVC (especially  20 mL/kg) indicates impending respiratory failure Capnography Pulmonary 70 https://t.me/usmleinnercircle Version 2 Common capnography waveforms Inspiration J~ Anatomical dead space Anatomical alveolar dead space Alveolar plateau t t Expiration Inspiration Hyperventilation Hypoventilation Bronchospasm I Increased2 ETC0 ... le ....................................... Increase of phases 2 and 3 ETCO2 baseline irc Tube displacement Loss of a angle Sudden loss in all phases or reduction of ETCO2 ! rC j CPR Apnea ne Absence of phases 0, 1, 2, 3 ETC0 2 = end•tidal CO2 @Uv\lorld In A normal capnogram has a characteristic rectangular waveform with 4 phases. A flat waveform indicates that the sensor (located at the hub of the ETT is not detecting CO2. This most commonly reflects 2 situations: LE • Because CO is not produced in the stomach or esophagus, esophageal intubations are classically represented by a flat line. An uncuffed ETT can easily become displaced during transport and move the relatively short distance from the trachea to the esophagus. Uncuffed ETTs are commonly used in children because they are thought to be associated with less mucosal injury.) U SM 0 • In prolonged cardiac arrest (production of CO has ceased), a flat waveform can occur. Capnography is the most reliable method for verification of endotracheal tube ETT placement in the trachea rather than the esophagus. PIP • The peak airway pressure (the maximum pressure measured as the tidal volume is being delivered) equals the sum of the resistive pressure (flow x resistance) and the plateau pressure. Peak airwaypressure= resistivepressure+ plateaupressure Pulmonary 71 https://t.me/usmleinnercircle Version 2  The plateau pressure is measured during an inspiratory hold maneuver, when pulmonary airflow and thus resistive pressure are both 0. It is determined by the compliance of the lung. i.e. if there is a problem with compliance, plateau pressure will rise. ( increased plateau pressure = decreased compliance.) Elastic pressure is inversely related to compliance, so decreased compliance causes a higher elastic pressure Plateau pressure = elastic pressure + PEEP Increasedpeak pressure PIP f Peak pressure Normal plateau pressure pplat t Plateau pressure Bronchospasm Pneumothorax Mucus plug Pulmonary edema Biting ETtube l Resistance flow Pneumonia PEEP l Auto-PEEP Total-PEEP Atelectasis Right mainstem intubation  The alveolar end-expiratory pressure is typically equal to the atmospheric pressure. In obstructive lung disease, however, the alveoli cannot empty completely, resulting in higher than normal endexpiratory pressures. This is called intrinsic, or auto-PEEP. The end-expiration hold maneuver measures auto-PEEP. DLCO Pulmonary 72 https://t.me/usmleinnercircle Version 2 Differential diagnosis based on carbon monoxide diffusing capacity of the lung Obstructive pattern (FEV1/FVC <70% predicted) Low DLCO • Emphysema Restrictive pattern (FEV1/FVC >70% predicted, FVC <80% predicted) Normal spirometry • Interstitial lung diseases • Anemia • Sarcoidosis • Pulmonary embolism • Asbestosis • Pulmonary hypertension • Musculoskeletal deformity • Chronic bronchitis Increased DLCO • Asthma • Neuromuscular disease • Asthma • Morbid obesity • Pulmonary hemorrhage irc Normal DLCO • Polycythemia rC Hypoxemia A-a gradient Corrects with supplemental 02? Normal Yes High altitude Hypoventilation CNS depression, neuromuscular weakness Normal Yes Dead-space ventilation (V/Q=oo) Pulmonary embolism Increased Yes Emphysema, ILD Increased Yes Intrapulmonary shunt (V/Q=0) Pneumonia, pulmonary edema, atelectasis Increased No lntracardiac shunt (right to left) Tetralogy of Fallo!, Eisenmenger syndrome Increased No U SM LE Diffusion limitation ne Reduced PiO2 In Example le • Heart failure Methemoglobinemia History Clinical examination Clinical presentation of methemoglobinemia Exposure to oxidizing substances (eg, dapsone, nitrites, local/topical anesthetic) • Cyanosis • Pulse oximetry saturation -85% • Dark chocolate-colored blood Laboratory findings • Saturation gap (>5% difference between oxygen saturation on pulse oximetry & ABG) • Normal Pa02 • Standard pulse oximetry is unable to detect methemoglobin, thus creating a falsely low oxygen saturation (versus co-oximetry, which can detect hemoglobin, methemoglobin, and Pulmonary 73 https://t.me/usmleinnercircle Version 2 carboxyhemoglobin) Arterial blood gas (ABG) detects free, unbound O2, which is not affected by methemoglobin. Cyanide Toxicity Cyanide poisoning Common etiologies Pathophysiology Clinical features • Structure fires (eg, combustion of plastics) • Occupational exposure (eg, mining) • Cyanide-containing medications (eg, sodium nitroprusside} • Inhibits oxidative phosphorylation & forces anaerobic metabolism • Rapidly lethal if untreated • Hypertension, tachycardia, tachypnea --+ circulatory collapse, death • Headache, confusion, anxiety --+ seizures, coma • Cherry-red skin • Elevated anion gap metabolic acidosis with t lactic acid Management • Decontamination • Supportive care (eg, 100% oxygen, intravenous fluids, vasopressors) • Empiric treatment with hydroxocobalamin ± sodium thiosulfate* *Sodium nitrite+ sodium thiosulfate is an alternate treatment but is contraindicated in carbon monoxide poisoning. Treatment overview for suspected cyanide poisoning Dermal exposure • Removal of clothing • Skin decontamination Ingestion • Activated charcoal Decontamination All exposures • Antidote o Hydroxocobalamin preferred o Sodium thiosulphate as alternate therapy • Antidote not available o Nitrites to induce methemoglobinemia Respiratory support • No mouth-to-mouth resuscitation • Supplemental oxygen • Airway protection (intubation) Cardiovascular support • Intravenous fluids for hypotension  Risk factors for cyanide toxicity due to nitroprusside include prolonged infusion  24 hours) and high rates 510 μg/kg/min), as well as chronic kidney disease To prevent cardiorespiratory arrest and permanent neurologic disability, victims of smoke inhalation should be treated empirically for cyanide toxicity. CO Poisoning Pulmonary 74 https://t.me/usmleinnercircle Version 2 Carbon monoxide poisoning Carbon monoxide poisoning Epidemiology • Smoke inhalation • Defective heating systems • Gas motors operating in poorly ventilated areas Mild-moderate . • Headache, confusion Manifestations Malaise, dizziness, nausea Severe • ABG: carboxyhemoglobin level • ECG ± cardiac enzymes Treatment • High-flow 100% oxygen • lntubation/hyperbaric oxygen (severe) ABG = arterial blood gas. ne • Diagnosis is confirmed by measuring carboxyhemoglobin levels ( 3% in non-smokers; > 10% in smokers) rC Diagnosis irc le • Seizure, syncope, coma • Myocardial ischemia, arrhythmias In A standard pulse oxymetry is unreliable and may appear normal because it cannot differentiate carboxyhemoglobin from oxyhemoglobin LE  What is the most likely diagnosis in a patient with headache for 2 months with a recent decline in his performance in school? He lives in the basement of his house. Labs show a Hb 19.6. Cerebral hypoxia secondary to carbon monoxide U SM CO binds to Hb 240x stronger than O2 and prevents normal O2 delivery to cells, leading to a chronic hypoxic state ⟶ stimulation of EPO production • Carbon monoxide CO poisoning induced–myocardial infarction chest pain, ECG changes, elevated troponin, and bilateral radiographic infiltrates no acute coronary occlusion Pulmonary 75 https://t.me/usmleinnercircle Version 2 Pathophysiology Concerning features Strong indicators of airway injury Management .. .. .. .. . Inhalation Injury Upper airway thermal injury± lower airway chemical injury Concomitant CO & cyanide poisoning common Historical: smoke exposure in enclosed space Physical: singed hair, facial bums, carbonaceous sputum, wheezing Oropharyngeal blistering or edema Retractions, respiratory distress, hypoxia 100% oxygen to displace CO Stable patients with concerning features but no strong indicators: bedside fiberoptic laryngoscopy Unstable patients or patients with strong indicators: endotracheal intubation CO= carbon monoxide. - - - - - - - - - - - - - -- -- - - - -- - Hemoptysis Causes of hemoptysis Pulmonary Cardiac Infectious • Bronchitis • Lung cancer • Bronchiectasis • Mitral stenosis/acute pulmonary edema • Tuberculosis • Lung abscess • Bacterial pneumonia • Aspergillosis Hematologic Vascular Systemic disease Other • Coagulopathy • Pulmonary embolism • Arteriovenous malformation • Granulomatosis with polyangiitis • Goodpasture syndrome • Trauma • Cocaine use (inhalation)  What position should a patient with massive hemoptysis be placed? 1. Put patient in dependent positioning (lateral position) (on side of bleeding) to avoid blood collection in the airways of the opposite lung. 2. Secure airway 3. Stabilize 4. Stop bleeding (bronchoscopy, embolization) Pulmonary 76 https://t.me/usmleinnercircle Version 2 History & physical to rule out other causes (eg, oropharynx, gastrointestinal) Massive Chest x-ray, CBC,coagulation studies, renal function, urinalysis, Secure airway, stops breathing & circulation irc rheumatologic workup if suspected Bleeding CT scan± bronchoscopy Bleeding (dependent on imaging result continues Treat cause; persistent bleeding treated via bronchoscopic interventions, embolization, or resection ne rC & if intervention is needed) Head, Neck & Nose le (>600 ml/24 hr OR 100 mUhr) In • Adenoid hypertrophy is hyperplasia of the pharyngeal tonsils and is the most common cause of nasal obstruction in children. LE Stridor Causes of stridor in infants & toddlers Acute • Parainfluenza virus, most cases in fall/winter • lnspiratory or biphasic strider, "barky" cough, infectious symptoms ± Choking episode Foreign body aspiration • • lnspiratory strider &/or wheeze, focally diminished breath sounds U SM Croup Laryngomalacia Vascular ring Airway hemangioma . Chronic "Floppy" supraglottis, prominent age 4-8 months • lnspiratory strider worsens when feeding, C!:}'.ing,or sueine; improves when prone • Great vessels encircle & compress trachea • Biphasic strider that improves with neck extension • Hemangiomas enlarge in the first few weeks of life • Worsening biphasic strider, concurrent skin hemangiomas ("beard distribution") • Tracheal stenosis (ie, rigid narrowing) or tracheomalacia (ie, weakness and collapsibility) are complications of prolonged endotracheal intubation, and can cause dyspnea and noisy breathing. Tracheal stenosis is associated with prolonged 2 weeks) mechanical ventilation, presents with Pulmonary 77 https://t.me/usmleinnercircle Version 2 slowly progressive (rather than episodic) dyspnea, and is potentially accompanied by expiratory stridor.  Adolescent boy with VACTERL presenting with subacute cough accompanied by expiratory stridor, forced vital capacity < slow vital capacity and a scooped-out flowvolume loop, all of which are associated with tracheal collapse Key idea: FORCED vital capacity (amount of air that can be FORCEfully expired) is almost always about equal to SLOW vital capacity (amount of air expired in passive breathe), with one exception being tracheal collapse because forceful expiration leads to obstruction ⟶ less air expired • Vocal cord dysfunction (ie, paradoxical closure during inspiration) can present with intermittent dyspnea and noisy breathing (ie, inspiratory stridor) triggered by exercise or psychosocial stress. It is more common in young women. Dx: Laryngoscopy showing vocal cord adduction during inspiration T/t: Acute episodes : sniff or pant, which activates the posterior cricoarytenoid to abduct the vocal cords, noninvasive positive-pressure ventilation Endotracheal intubation ideally should be avoided Long-term treatment: primarily consists of education and therapy with a speech-language pathologist. • Velopharyngeal insufficiency VPI is a common sequela of cleft palate, with or without surgical repair. VPI is defined as an inability to properly close the velopharyngeal port separating the nasopharynx and oropharynx during the production of speech, which is frequently caused by a shortened palate or persistent palatal defects. VPI can lead to highpitched, hypernasal, or whistling speech with reduced intelligibility. Laryngomalacia Pulmonary 78 https://t.me/usmleinnercircle Version 2 Pathophysiology Clinical presentation Diagnosis . • . . Laryngomalacia Laryngomalacia Start of inspiration Mid-inspiration Collapse of supraglottic tissues on inspiration lnspiratory stridor that worsens when supine Peak at age 4-8 months Laryngoscopy (eg, omega-shaped [OJ epiglottis) • Reassurance with close follow-up (± concurrent GER Management . treatment) for most cases Supraglottoplasty for severe symptoms rC irc le GER= gastroesophagealreflux. • Laryngomalacia is characterized by floppy supraglottic structures that collapse during inspiration due to laryngeal hypotonia (possibly from delayed maturation). However, it presents in infancy and ne typically resolves by age 18 months.  What imaging test/study may be used to confirm the diagnosis of laryngomalacia? U SM LE Torus Palatinus In Fibrooptic laryngoscopy  What is the most likely diagnosis for a hard, immobile mass on the hard palate of a young individual? Torus palatinus (benign bony growth) can increase in size throughout life; no medical or surgical therapy is required unless the growth becomes sympatomatic Pulmonary 79 https://t.me/usmleinnercircle Version 2 It can be congenital or develop later in life URI Distinguishing features of common upper respiratory illnesses Viral upper respiratory syndrome Influenza Streptococcal pharyngitis Abrupt & often dramatic Variable Usually mild Predominantly pharyngeal symptoms Prominent with possible high fever, Variable with possible fever & myalgias, headache myalgias Variable but often unremarkable hypertrophy & exudates, tender Onset of symptoms Slow, stepwise, migratory, or evolving Upper respiratory Rhinorrhea, coryza, sneezing, mild symptoms pharyngitis Systemic Usually mild symptoms Examination Nasal edema with normal or slightly findings erythematous pharynx Pharyngeal erythema, tonsillar cervical lymph nodes Important respiratory tract infections in children Common etiologic Clinical illness Presentation Nasopharyngitis Nasal congestion & discharge, sneezing, {common cold) cough & sore throat Laryngotracheitis {croup) Upper respiratory tract symptoms followed by hoarseness, barking cough, strider & respiratory distress Sore throat, cervical lymphadenopathy, Diphtheria coalescing pseudomembrane Sore throat, dysphagia, drooling & Epiglottitis respiratory distress Upper respiratory tract symptoms Bronchiolitis followed by wheezing, cough & .. • . . . . respiratory distress agents Rhinovirus Influenza virus Coronavirus Parainfluenza virus Corynebacterium diphtheriae Haemophilus influenzae Respiratory syncytial virus Key respiratory tract infections in children Diagnosis Laryngotracheitis (croup) Pulmonary Classic pathogen • Parainfluenza virus Presentation • Age 6 months to 3 years • Barking cough, stridor, hoarseness • Unvaccinated children • Sore throat, dysphagia, drooling, tripod position Epiglottitis • Haemophilus influenzae Bronchiolitis • Respiratory syncytial virus • Wheezing, coughing • Age <2 80 https://t.me/usmleinnercircle Version 2 Evaluation of acute pharyngitis in children Evaluation & management of pharyngitis Viral symptoms present (eg, cough, conjunctivitis, oral ulcers) Yes Cantor criteria • Fever by history • Tender anterior cervical lymphadenopathy • Tonsillar exudates • Absence of cough No Rapid streptococcal antigen testing Negative Throat culture I Supportivecare 2-3 present POS1tive Streptococcal pharyngitis No testing/treatment for streptococcal infection Rapid streptococcal antigen test 4present j le Viral pharyngitis 0-1 present Positive Empiric penicillin or amoxicillin irc Negative Oral penicillin oramoxlclllin Penicillin or amoxicillin for positive results 0l1Wofld rC because the risk of rheumatic fever is high, negative results should be confirmed with throat culture in kids Penicillin allergy? Macrolides; or Clindamycin ne The benefits of a 10-day course of penicillin for streptococcal pharyngitis include decreased symptom severity and duration, prevention of spread to close contacts, and prevention of In acute rheumatic fever  What is the next step in management for a child that presents with fever, pharyngitis with tonsillar exudates, and tender anterior cervical lymphadenopathy? LE Rapid streptococcal antigen testing GAS pharyngitis in children should always be confirmed by rapid streptococcal antigen testing or throat culture prior to initiation of antibiotics U SM (versus adults, who can be treated empirically if they meet all Centor criteria) Epiglottitis Pulmonary 81 https://t.me/usmleinnercircle Version 2 Epidemiology Clinical Diagnosis Treatment .. .. .. . .. .. Infectious epiglottitis Streptococcus pneumoniae, Haemophilus innuenzae Risk reduced with H innuenzae vaccination Rapidly progressive & life-threatening Fever, sore throat, drooling, muffled voice Airway obstruction (stridor, dyspnea) Pooled oropharynx secretions Laryngotracheal tenderness Direct visualization Imaging (lateral neck x-ray) Early artificial airway (if needed) Intravenous antibiotics (ceftriaxone plus vancomycin) Epiglottitis nflamedandswollenepigtoms, mostcommonly causedby Haemophilus lnnuenzae Adult epiglottitis The presence of drooling helps differentiate epiglottitis from laryngotracheobronchitis (croup) Pulmonary 82 https://t.me/usmleinnercircle Version 2  Patients with epiglottitis who develop rapid-onset respiratory failure (eg, tripod position, hypoxia, drooling, tachypnea) require urgent airway management. This includes bag-valve-mask ventilation with 100% oxygen followed by endotracheal intubation with advanced equipment (eg, video laryngoscope). A single failed attempt at video-assisted endotracheal intubation should prompt surgical cricothyrotomy, which bypasses the epiglottal swelling and potential obstruction. Retropharyngeal abscess • Age 2-4 but can occur in any age group • Polymicrobial (group A Streptococcus, Staphylococcus aureus & irc Epidemiology respiratory anaerobes) Diagnosis In Management rC Examination • Fever • Odynophagia/dysphagia • Neck pain • Drooling, muffled "hot potato" voice, trismus • Retropharyngeal bulge • Limited neck extension • Lateral neck x-ray (increased prevertebral thickening) • CT neck with contrast • Airway protection • Intravenous antibiotics (eg, ampicillin-sulbactam, clindamycin) • ± Surgical drainage ne Symptoms le Retropharyngeal Abscess often preceded by viral infection or direct spread from pharyngitis LE • Retropharyngeal abscess often presents with neck pain, odynophagia, and fever following penetrating trauma to the posterior pharynx (e.g. fish bone). may also have nuchal rigidity and bulging of the pharyngeal wall U SM  What is the likely diagnosis in a child that presents with an inability to extend the neck with a widened prevertebral space on X-ray after having fever and sore throat for one week? Retropharyngeal abscess complication of a viral upper respiratory infection Pulmonary 83 https://t.me/usmleinnercircle Version 2 Retropharyngeal abscess • D/D Peritonsillar Abscess  What life-threatening complication may occur due to contiguous spread of a retropharyngeal abscess? Acute necrotizing mediastinitis Deep neck space anatomy Buccopharyngeal Prevertebral fascia fascia Pharyngeal mucosa I I Anterior spinal lrment Alar fascia I Retropharyngeal space i Superior mediastinum 1 "Danger" space i Posterior c:=;' Prevertebral space i Extends to mediastinum the coccyx (internal jugular vein, (potential space CNs IX, X, XI & XII) between pericardium & vertebral column) Peritonsillar Abscess Pulmonary 84 https://t.me/usmleinnercircle Version 2 Peritonsillar abscess Clinical features Fever Sore throat, difficulty swallowing Trismus Muffled "hot potato" voice Uvula deviation away from enlarged tonsil Incision & Drainage and IV antibiotics* U SM LE In ne rC Make sure airway is not compromised first! irc  What is the recommended treatment for peritonsillar abscess? le Pooling of saliva Pulmonary 85 https://t.me/usmleinnercircle Version 2 Management of suspected peritonsillar abscess Clinical features • Severe sore throat • Fever • "Hot potato" voice • Dysphagia Impending airway obstruction Secure airway • ENT & anesthesia j Drainage in OR ] j +-Yes- • Tripod position • Inability to lie fiat • Severe respiratory distress (eg, nasal fiaring, noisy breathing, retractions) No • Signs of deep neck space infection • Neck pain or stiffness on extension • Neck tenderness or swelling • Bulging of posterior pharyngeal mucosa • Chest pain - Yes _.. CT scan of the neck with contrast No • Antibiotics ] Clinical diagnosis of PTA (at least one ol): • Trismus • Unilateral swelling • Uvular deviation • Fluctuant bulging of soft palate - No - Likely infectious pharyngitis I Yes • Needle aspiration or incision and drainage ~ioticsJ ENT= ear, nose.throat; OR = operatingroom; PTA = peritons,llarabscess. CUWor1d Trimus is the ddx feature between this and tonsillitis  What are the differentials for "hot-potato voice"? • Epiglottitis • Retropharyngeal abscess • Peritonsillar abscess Sinusitis  What are the most common causative organisms 2 associated with acute bacterial rhinosinusitis? Nontypeable Haemophilus influenzae and Streptococcus pneumoniae Moraxella catarrhalis is the third most common and accounts for 10% of cases Pulmonary 86 https://t.me/usmleinnercircle Version 2  Pseudomonas: in Cystic fibrosis patients  S. Aureus: Chronic sinusitis (sinus inflammation for 12 weeks) Acute rhinosinusitis in children .. Treatment Facial pressure/pain ± Fever, cough, headache, loss of smell, ear pain Viral 0 No fever or early resolution of fever 0 Mild symptoms (eg, well-appearing, mild facial pain) 0 Improvement & resolution by day 5-10 Bacterial 0 Fever 2:3 days OR 0 New/recurrent fever after initial improvement OR 0 Persistent symptoms 2:10 days Intranasal saline, saline irrigation, NSAIDs Antibiotics if bacterial NSAIDs = nonsteroidal anti-inflammatory drugs. .. . .. .. Fever ne .. Acute bacterial rhinosinusitis Nasal congestion/obstruction Purulent nasal discharge Maxillary tooth discomfort Facial 12ain/pressureworsens with bending forward In Symptoms .. . le . Etiology Nasal congestion &/or purulent drainage irc .. . . rC Clinical features Diagnosis requires any of the following: criteria Worsening symptoms ~5 days after an initially improving viral upper respiratory infection 1st-line therapy: Amoxicillin-clavulanate Alternate agent: Doxycycline or fluoroquinolones Supportive care: Analgesics, decongestants, saline irrigation, topical glucocorticoids Not recommended due to resistance: Amoxicillin, macrolides, trimethoprim-sulfamethoxazole, 2nd- or 3rd- U SM Treatment Persistent symptoms ~10 days without improvement Severe symptoms, high fever (>39 C [102.2 Fl), purulent nasal discharge, or facial pain ~3 days LE Diagnostic generation cephalosporins Most common predisposing factor: Viral URI or sometimes allergic rhinitis Hard to distinguish viral vs bacterial sinusitis Viral rhinosinusitis generally improves by days 710 Pulmonary 87 https://t.me/usmleinnercircle Version 2 Both endotracheal tubes and nasogastric tubes can cause acute sinusitis in critically ill patients as a result of impaired sinus drainage. Symptoms include fever, cough, headache, and purulent nasal drainage. Diagnosis is made with CT scan of the sinuses, which will show opacification of the sinuses, and culture of the sinus fluid. Management is with antibiotic therapy and removal of the obstruction, although operative sinus drainage may also be required. Tracheitis  What is the organism is most likely in a patient that presents with fever and croup-like symptoms that do not resolve with racemic-epi? S. aureus (tracheitis) Work-up requires? Endoscopy (laryngoscopy or bronchoscopy) with tracheal culture Path: S. aureus most common following viral prodrome Presentation? • 4 years • Follows viral prodrome • Croup that does not resolve • Toxic (sick as shit) Diagnosis? • Endoscopy (laryngoscopy or bronchoscopy) with Tracheal culture • Imaging: steeple sign (just like Croup so not sensitive/specific) • No improvement with racemic epi (i.e. it is not Croup) Treatment? • Maintenance of airway • IV Antibiotics Laryngeal Papillomatosis Pulmonary 88 https://t.me/usmleinnercircle Version 2 Normal vocal cords irc le Recurrent respiratory papillomatosis • Vertical transmission of human papillomavirus subtypes 6 and 11 can cause recurrent respiratory papillomatosis, which results in hoarseness due to finger-shaped growths on the true vocal cords. rC T/t: Surgical Debridement Treatment - Ocular itching & tearing "Allergic shiners" (infraorbital edema & darkening) "Allergic salute" (transverse nasal crease) Pale, bluish, enlarged turbinates Pharyngeal cobblestoning "Allergic facies" (high-arched palate, open-mouth breathing) Allergen avoidance Transverse nasal crease Intranasal corticosteroids can also have thick, green nasal discharge U SM - Cough secondary to postnasal drip In Physical examination Allergic rhinitis Rhinorrhea, nasal congestion, sneezing, nasal itching LE Symptoms .. . .. .. . .. ne Rhinitis  During peak allergy seasons, patients can experience systemic (eg, fever) or neuropsychiatric (eg, fatigue, irritability) symptoms. Nonallergic rhinitis • Nasal congestion, rhinorrhea, sneezing, postnasal Clinical features . drainage Later onset (age >20) common • No obvious allergic trigger • Perennial symptoms (may worsen with season changes) Pulmonary . .. • .. Watery rhinorrhea, sneezing, eye symptoms Earlier age of onset Identifiable allergen or seasonal pattern Pale/bluish nasal mucosa Associated with other allergic disorders (eg, eczema, asthma, eustachian tube dysfunction) • Erythematous nasal mucosa Treatment Allergic rhinitis Mild: intranasal antihistamine or glucocorticoids • Moderate to severe: combination therapy • Intranasal glucocorticoids • Antihistamines 89 https://t.me/usmleinnercircle Version 2  Patients with NAR typically lack the sneezing and allergic conjunctivitis (eg, itchy eyes, injected conjunctivae) that classically accompany allergic rhinitis.  Be careful with decongestants (sympathomimmetics like phenylephrine) -- do not use for more than 3 days as hey can produce rebound congestion (rhinitis medicamentosa) Physical examination often shows beefy-red nasal mucosa as opposed to the edematous, pale mucosa often seen in allergic rhinitis. T/t: Cessation of medication; Nasal Glucocorticoids Septal Hematoma Septal hematoma Soft fluctuant _____ swelling _  A septal hematoma presents after nasal trauma as fluctuant swelling of the nasal septum. It should be recognized and promptly drained to avoid complications of infection, septal perforation, and nasal deformities.  If a patient develops a whistling noise during respiration following rhinoplasty, what is likely the diagnosis? Nasal septal perforation likely resulting from a septal hematoma (more common) or septal abscess (less common) Neck Masses Pulmonary 90 https://t.me/usmleinnercircle Version 2 Reactive adenopathy Mycobacterium avium lymphadenitis Cystic hygroma Lateral Lateral Lateral Posterior . Often eresents after upper reseirato~ tract infection Cystic mass with trapped epithelial debris Occurs along embryologic fusion planes No displacement with tongue protrusion Tract may extend to the tonsillar fossa (2nd branchial arch) or pyriform recess (3rd branchial arch) Anterior to the sternocleidomastoid muscle Firm, often tender Multiple nodules le Branchial cleft cyst .. . . . .. .. . Midline Cystic, moves with swallowing or tongue protrusion Necrotic lymph node Violaceous discoloration of skin Frequent fistula formation Dilated lymphatic vessels irc Dermoid cyst . Midline Pediatric neck masses Tract between foramen cecum & base of anterior neck rC Thyroglossal duct cyst .. Thyroglossal duct cyst LE In ne Branchial cleft cyst U SM Cystic hygroma  Similar to a thyroglossal duct cyst TDC, which is also formed from incomplete involution of an embryologic remnant, a BCC is often detected when it becomes secondarily infected after an upper respiratory tract infection (URI), leading to erythema, tenderness, and sometimes drainage of fluid from a sinus tract. Pulmonary 91 https://t.me/usmleinnercircle Version 2 Embryology Clinical presentation Management .. • . Thyroglossal duct cyst Forms along path of thyroid descent Foramen cecum (base of tongue) to base of anterior neck Midline cystic neck mass Moves superiorly with swallowing or tongue protrusion • Often present after upper respiratory tract infection (secondary infection) • Associated with ectopic thyroid tissue • Confirm presence of normal thyroid tissue • Surgical resection of cyst, associated tract & central portion of hyoid bone Mediastinum Masses 1 Anterior mediastinum: 4 T's 3 Posterior mediastinum • -- Thymoma •  Neurogenic tumors (neurofibroma, • -- Thyroid neoplasm schwannoma) • -- Teratoma •  Meningocele • -- Terrible lymphoma •  Enteric cyst 2 Middle mediastinum •  Lymphoma •  Bronchogenic cyst •  Diaphragmatic hernia •  Tracheal tumor •  Esophageal tumor •  Pericardial cyst •  Aortic aneurysm •  Lymphoma •  Lymph node enlargement •  Aortic arch aneurysm Pulmonary 92 https://t.me/usmleinnercircle Version 2 Structures Masses Anterior • Thymus • Lymph nodes' • Thymic neoplasms (eg, thymoma) • Lymphoma • Germ cell tumors • Teratomas • Seminomas, nonseminomas • Thyroid tissue (eg, ectopic, substernal goiter) • Lymph nodes• • Pericardium • Heart & great vessels • Trachea & main bronchi • Esophagus • Lymphadenopathy (eg, sarcoidosis, lung cancer), lymphoma • Benign cystic masses (eg, pericardia! cyst, bronchogenic cyst) • Vascular masses • Esophageal tumors • Neural tissue •Vertebrae • Lymph nodes• • Neurogenic tumors (eg, schwannoma, neurofibroma), meningocele • Spinal masses (eg, metastases) • Lymphoma irc Compartment le Mediastinal Compartments, Structures, & Masses rC *Lymph nodes, from which lymphoma may arise, are present in all 3 compartments. "Some sources define the middle & posterior compartments based on their relationship to the posterior pericardia! surface rather than the posterior thoracic wall. @UWorld U SM LE In ne Normal thymus in a neonate ©UWorld  A large thymic silhouette is a normal finding on frontal CXR in children  3 years In question stems, if you find this missing in a young person, consider things like DiGeorge or SCID!  The thymus normally atrophies and is replaced by fat after puberty, when it has completed the production of T lymphocytes. Therefore, adults with mediastinal opacities on x-ray should undergo further work-up for a pathologic cause (eg, lymphoma, germ cell tumor). Acute Mediastinitis  What is the treatment for post-operative acute mediastinitis? drainage, debridement, and antibiotics Pulmonary 93 https://t.me/usmleinnercircle Version 2 symptoms include fever, chest pain, leukocytosis, and mediastinal widening on CXR Obstructive Conditions commonly associated with digital clubbing lntrathoracic neoplasms lntrathoracic suppurative diseases Bronchogenic carcinoma Metastatic cancers Malignant mesothelioma Lymphoma • • • • • Lung abscess Empyema Bronchiectasis Cystic fibrosis Chronic cavitary infections (eg, fungal, mycobacterial) • Idiopathic pulmonary fibrosis • Asbestosis • Pulmonary arterio-venous malformations Lung disease Cardiovascular • • • • disease • Cyanotic congenital heart disease  The presence of digital clubbing in patients with COPD should prompt a search for occult malignancy (i.e. chest CT). COPD itself, with or without hypoxemia, does NOT cause clubbing Asthma vs COPD Asthma COPD Late-stage COPD FVC Normal/L Normal/L LIH FEV1 L L H FEV1/FVC L L H Bronchodilator response Reversible Partially reversible/ nonreversible Usually nonreversible Chest x-ray Normal Normal Hyperinflation, loss of lung markings DLCO Normal/j Normal/L L COPD = chronic obstructive pulmonary disease; OLCO = diffusion capacity of the lung for carbon monoxide. Acute Bronchitis Etiology Clinical presentation Diagnosis & treatment . .. . .. . Acute bronchitis Preceding respiratory illness (90% viral) Cough for >5 days to 3 weeks (± purulent sputum) Absent systemic findings (eg, fever, chills) Wheezing or rhonchi, chest wall tenderness Clinical diagnosis, CXR only when pneumonia suspected Symptomatic treatment (eg, NSAIDs &/or bronchodilators) Antibiotics not recommended CXR = chest x-ray; NSA!Ds = nonsteroidalanti-inflammatorydrugs. Pulmonary 94 https://t.me/usmleinnercircle Version 2 • Sputum production occurs in roughly half of patients; the typical yellow/purulent sputum is due to epithelial sloughing and is not a sign of bacterial infection. Small amounts of blood in the sputum can occur due to inflammation and epithelial damage D/D Pneumonia which has systemic signs of inflammation COPD le • Jugular venous distention may be observed on physical examination of acute COPD exacerbation during expiration due to increased intrathoracic pressure. -Phys. exam often shows wheezing, tachypnea, prolonged expiration, and use of accessory respiratory muscles irc M/C Trigger : Upper respiratory infection URI  When should intubation be considered in patients with COPD/Asthma exacerbation? rC Profound acidemia (pH 7.1, poor mental status (somnolence, lack of cooperation), hemodynamic instability ne Otherwise, begin with initiation of noninvasive positive-pressure ventilation in patients with respiratory failure. Endotracheal intubation with mechanical ventilation is recommended for patients who fail a trial of NPPV. In Acute exacerbation of chronic obstructive pulmonary disease • Infectious (70%): respiratory viruses (eg, rhinovirus) & commensal bacteria (eg, nontypable Pathophysiology & • j WOB (hyperinflation) & impaired gas exchange (hypoxemia [LV/Q], hypercapnia [jV 0]) • Maximize expiratory flow: inhaled bronchodilators U SM presentation Haemophilus influenzae) • Noninfectious (30%): sterile inflammation (underlying disease), pulmonary embolism, inhaled irritants • Bronchial inflammation: systemic response (eg, fever, leukocytosis) usually absent • Cardinal symptoms: j dyspnea, j sputum volume, j sputum purulence LE Precipitants Management • Reduce airway inflammation: systemic glucocorticoids • Treat underlying triggers:± antibiotics• &/or antivirals (influenza, COVID-19) • Maintain adequate oxygenation: SpO 2 target 88%-92% • Maintain adequate ventilation: NIPPV or invasive mechanical ventilation *Antibiotics generally indicated if: (1) increased sputum purulence+ ~1 other cardinal symptoms; or (2) patient requires hospitalization. NIPPY= noninvasive positive pressure ventilation; V/Q = ventilation-perfusionratio; Vo= alveolar dead space ventilation; WOB = work of breathing. Pulmonary I: Ii 1, Systemic Glucocorticoids: IV or Oral ! NOT INHALED 95 https://t.me/usmleinnercircle Version 2 Global Initiative for Chronic Obstructive Lung Disease GOLD recommend antibiotic therapy for patients who have a COPD exacerbation with any two of the following features: increased sputum purulence, increased sputum volume, or increased dyspnea. Antibiotics are also recommended for those requiring mechanical ventilation (noninvasive or invasive).  What is the most likely diagnosis in a patient with extensive smoking history, CAD, and hypertension who presents with one week history of exertional dyspnea, bilateral lung crackles, and occasional wheezes? ABG shows respiratory alkalosis. Congestive heart failure CHF will have respiratory alkalosis -- pulmonary edema reduces ventilation, causing tachypnea ⟶ loss of CO2 COPD will have respiratory acidosis and widespread wheezing. Pulmonary embolism is acute onset High CO2 and Respiratory Acidosis help differentiate from CHF exacerbation, which causes Low CO2 and Respiratory Alkalosis  What CXR finding may be found on patient with COPD? Hyperinflation with flattening of diaphragm; 8 ribs counted = hyperinflation What physical exam finding does this produce? Distant heart sounds (listen at subxiphoid process) Chronic obstructive pulmonary disease ~UWortd Pulmonary Nonna! chest x-ray Chronic obstructive pulmonary disease 96 https://t.me/usmleinnercircle Version 2  Patients with an acute exacerbation of chronic obstructive pulmonary disease are at risk for Symptomatic Hypercapnia and should be promptly investigated with arterial blood gas analysis. Clinical manifestations are predominantly neurologic and include headache and hypersomnolence with mild to moderate hypercapnia; higher levels of hypercapnia (eg, PaCO2 7580 mm Hg) can cause confusion, lethargy, and eventually coma CO2 narcosis) or seizures. le Severe COPD is commonly accompanied by pulmonary cachexia, which is characterized by loss of lean muscle mass due to energy imbalance and systemic inflammation. Treatment irc Treatment ImprovesMortality and DelaysProgressionof Disease Smoking cessation p0 2 <55/sat S88% with pulmonary HTN, high HCT, or cardlomyopathy: po, <60/sat s90% Oxygen therapy for those with p0 2 ;;55 or saturation S88%;mortality benefit is directly proportional to the number of hours that the oxygen is used. rC O2 use: Influenzaand pneumococcalvaccinations DefinitelyImprovesSymptoms(But DoesNot Decrease DiseaseProgressionor Mortality) Short-acting beta agonists (e.g., albuterol) Inhaled antlchollnerglc agents are most effective in COPD. ne Anticholinergic agents: tiotropium, ipratropium Steroids Long-acting beta agonists (e.g., salmeterol) Pulmonary rehabilitation In Asthmatics not controlled with albuterol -, inhaled steroid (e.g., tiotropium) LE COPD not controlled with albuterol -, anticholinergic -> inhaled steroid  What are the (2) interventions to improve mortality and delay progression of disease in COPD? 1. Smoking cessation U SM 2. Long-term oxygen therapy (PO2 55 or O2 Sat 88) If O2 sat is fine, then O2 is not indicated Survival benefits of home oxygen therapy are significant when it is used for 15 hours a day MTB says pneumococcal and influenza vaccines decrease mortality, UWorld says they just decrease the rate of COPD exacerbations. 1. Resting arterial oxygen tension (PaO2) ~55 mm Hg or pulse oxygen saturation (SaO2) ~88% on room air 2. PaO2 ~59 mm Hg or SaO2 ~89% in patients with cor pulmonale, evidence of right heart failure, or hematocrit >55%  The primary long-term intervention for COPD is a daily long-acting anticholinergic inhaler. Pulmonary 97 https://t.me/usmleinnercircle Version 2 e.g. ipratropium, tiotropium; may be combined with a SABA for greater symptom relief inhaled steroids and LABA can be used for patients with more severe COPD (vs Oral/IV steroids for Acute exacerbation) Selection of initial bronchodilator for stable COPD a; .c Exacerbations in prior year Group E Cl I ~1 requiringhospitalization LABA+ LAMA t Ics· OR ., ~2 requiring outpatient systemic corticosteroids C: 0 -~ Group A Group B Bronchodilator (eg, SAMA as needed) LABA+ LAMA -e 0 .. " <) w None requiring hospitalization & <2 requiring outpatient systemic cortiocosteriods Higher .. Lower Symptoms •Ics recommendedif penpheral bloodeoslnophllia(>300/mm3) Is present but avoided If recurrent pneumoniais presenL ··Assess using validated onl/umenlsuch as COPD AssessmentTest (CAT)or standard dyspnea scale Higher symptomseverity = dyspnea causing slower walking or worse. COPD = chronic obstructive pulmonarydisease:ICS = inhaled corticosteroid,LABA = long.actingfl·2 agonist: LAMA = long-actingmuscarinicantagor<st SAMA = short-actingmuscarinicanlagonis Cuworld Asthma Evaluation of suspected asthma Intermittent respiratory symptoms (cough, dyspnea, wheezing, chest tightness) Spirometry Obstruction (FEV1/FVC <70%) Normal Inhaled bronchodilator (SABA) Methacholine bronchoprovocatoon test Reversibte• Asthma confirmed Nonreversible Negative Asthma unlikely Positive Asthma possible Seek alternatediagnosis (eg. CHF, PH, VCD) •Reversible:At least 12% and 200 ml increase FEV1 or FVC, wilh postbronchodllatorFEV1/FVCrise lo ~70% CHF = congestive heartfailure;PH = pulmonaryhypertension: COPO= chronicobstructive pulmonary disease; SABA = short-actingbeta agonist (albulerol);VCD = vocal cord dysfunction. CUWoM Pulmonary 98 https://t.me/usmleinnercircle Version 2 The diagnosis of asthma can be made in patients with normal or increased DLCO & a 12% increase in FEV1 following bronchodilator administration. patients with COPD typically have a decreased DLCO and  12% increase in FEV1 after bronchodilators le  What is the likely diagnosis in an inmate who smokes and presents with rapidly worsening shortness of breath, tachycardia, tachypnea, and 87% O2 sat? He is poorly adherent to his asthma medications and began taking ibuprofen for shoulder pain the previous night. Urticarial rash is seen over the trunk and extremities. irc Anaphylaxis (laryngeal edema) Anaphylaxis may be confused with an asthma exacerbation if skin involvement is minimal or missed by the clinician. rC NSAIDs can worsen anaphylaxis Flow (L /sec) Flow (L /sec) ne 8 C: 0 LE In ·ew C: .2 e ! Lung volume (L) Upper Airway Obstruction (e.g. laryngeal edema): U SM Characterized by flattening of the top and bottom of the curve due to limited airflow during both inspiration and expiration 8 4 0 Lung volume (L) ©UWond Scooped out pattern in Asthma • Mechanisms by which GERD exacerbates Asthma 1 Increased vagal tone 2 Heightened bronchial reactivity 3 Microaspiration of gastric contents into the upper airway Patient with asthma has several clues in their history suggesting comorbid GERD, including sore throat, morning hoarseness, worsening cough only at night, and increased need for her albuterol inhaler following meals. -Proton-pump inhibitorPPI therapy has been shown to improve both asthma symptoms and peak expiratory flow rate in asthma patients with evidence of comorbid GERD Pulmonary 99 https://t.me/usmleinnercircle Version 2 Status Asthmaticus Features of respiratory failure in acute asthma • Absent/minimal wheezing (poor air movement -, silent chest) Clinical features • Accessory muscle use • Altered mental status .. . . • ABG*: poor ventilation (i PaCO2 & L pH), poor oxygenation (! PaO2) Laboratory findings Management Lactate: transient t due to muscle WOB (type A) &/or Jl-agonist-induced (type B) K•: transient L due to Jl-agonist &/or respiratory alkalosis Nebulized albuterol & ipratropium, IV corticosteroids ± IV magnesium • Short trial (<2 hr) of NIPPV Low threshold for intubation & invasive mechanical ventilation *Impending respiratory failure: ii respiratory effort to maintain normal/borderline PaC02 & pH. ABG = arterial blood gases; IV= intravenous; NIPPY = noninvasive positive pressure ventilation; WOB = work of breathing.  Additional bronchodilation with one-time infusion of IV magnesium sulfate may be added if no improvement appreciated after an hour. Management of asthma exacerbation during pregnancy Supplemental 0 2 Bronchodilators Systemic corticosteroids Additional therapy . .. .. .. . Maintain SaO 2 2'95% Nebulized or inhaled albuterol Inhaled ipratropium If incomplete response to bronchodilators Oral preferred (eg, prednisone) MgSO 4 or terbutaline if severe Epinephrine contraindicated Intubate for respiratory failure MgSO 4 = intravenous magnesium sulfate. Patient history Clinical status (impending respiratory failure) .. .. . Fatal asthma: risk factors Prior respiratory failure: NIPPV, intubation, ICU admission Poor asthma control: t inhaler use, frequent oral corticosteroids or acute care visits Examination: respirations >30/min, SaO2 S90%, accessory muscle use, AMS, "silent chest" ABG: t or inappropriately normal PaCO2 relative to WOB Peak flow: S50% of baseline ABG = arterial blood gas; AMS= altered mental status; ICU= intensive care unit; NIPPY= noninvasive positive pressure ventilation; Sa0 2 = arterial saturation of oxygen; woe;;; work of breathing.  What is the next step in management for a patient with severe asthma exacerbation with an elevated PaCO2? Endotracheal intubation Patients with asthma exacerbation often have tachypnea, which should lead to low CO2 -a normal or elevated PaCO2 suggests impending respiratory failure (inability to meet increased respiratory demands) • Systemic glucocorticoids (eg, oral prednisone, dexamethasone) reduce late-phase inflammation in Status Asthamaticus. Pulmonary 100 https://t.me/usmleinnercircle Version 2 Because the anti-inflammatory activity of glucocorticoids is delayed by several hours (due to the time required to change nuclear gene expression), they are often given with bronchodilators in the emergency department. B agonsist + anticholi ) Corticosteroids are continued at home (eg, prednisone 4060 mg daily for 57 days) to prevent relapse and decrease the need for hospitalization Occupational Asthma & high-risk occupations • Workplace antigens--+ inflammation (lgE-dependent or independent) --+ bronchoconstriction • Classic antigens: animal proteins (eg, animal workers, seafood processors), grain carbohydrates (eg, bakers), isocyanates (eg, painters), metals (eg, welders) • Latency (months/years) due to gradual sensitization irc Pathogenesis le Occupational asthma • Initially, symptoms clearly exacerbated at workplace & relieved at home Clinical • Over time, symptoms persist throughout week & subside only after sustained work absence features • Diagnosis: confirm asthma or BHR (eg, methacholine bronchoprovocation)--+ confirm rC workplace-specific worsening of obstruction (eg, serial PEFR at home & work) • Antigen avoidance (change of workplace if possible) or reduction (eg, respirator, shift rotation) Management • Bronchodilators & inhaled corticosteroids • Desensitization (immunotherapy) PEFR = peak expiratory flow rate . ne BHR = bronchial hyperresponsiveness;  Diagnosis requires a detailed occupational history, careful symptom timeline, and 2-step confirmatory testing: In • First, asthma must be confirmed (eg, reversible obstruction on spirometry). For patients with normal baseline spirometry, bronchoprovocation (eg, methacholine) is performed to confirm bronchial hyperresponsiveness. LE • Next, an occupational relationship is established by confirming workplace-specific worsening of airflow obstruction. This can be accomplished with serial peak expiratory flow rate PEFR measurements using a portable peak flow meter. U SM Patients record their PEFR at home and at work (along with their symptoms): a PEFR decline by 20% at the workplace relative to at home is consistent with occupational asthma. Exercise Induced Pulmonary 101 https://t.me/usmleinnercircle Version 2 Exercise-induced bronchoconstriction • Hyperventilation -+ incomplete heating & humidification of inspired air-+ cooler, dry air triggers mast cell degranulation & bronchospasm Pathophysiology • Can occur in isolation or in underlying asthma • ! Exercise tolerance; asthma symptoms appear within 5-10 min & improve* after 20 min of exercise Clinical features • Diagnosis: o Supportive: empiric response to preexercise bronchodilator & diagnosis o Confirmatory: bronchoprovocation testing (eg, spirometry before & after exercise) showing 2'15% decline in FEV 1 • Improve control of underlying asthma (ie, step-up therapy) Management • Premedicate before exercise: ICS-formoterol (10 min prior) preferred over SABA,LTRA (2 hr prior) • Daily ICS-beta agonist or LTRA may be needed for frequent, prolonged exercise *Refractoryperiod U preformedmast cell mediators& j inhibitoryprostaglandins). ICS; inhaledcorticosteroid;LTRA; leukotrienereceptorantagonist;SABA; short-actingbeta agonist.  What is the first-line treatment for patients with exercise-induced bronchoconstriction who exercise daily? Inhaled corticosteroids or anti-leukotriene agents 1020 minutes before exercise use short-acting β-agonists if only needed a few times per week Treatment Asthma severityfor patients not on controllermedication Asthma severity Intermittent Mild persistent Symptom frequency/ SABA use s2 days a week Pulmonary Indicated therapy initiation S2 times a month Step 1 >2 days a week but not daily 3-4 times a month Step2 Moderate persistent Daily Severe persistent Nighttime awakenings >1 time a week but not nightly Step 3 Throughout the day 4-7 times a week Step 4 ors 102 https://t.me/usmleinnercircle Version 2 Step-up strategy for asthma treatment (adults) Still uncontrolledafter Step 4 Frequentneed for OCS Daily symptoms waking with asthma DecreasedFEV1 Most days waking with asthma Step 5 Step4 Less than daily ICS-formoteroldaily (high-doseICS) Step 3 Reliever= ICS-formoterol PRN Considerbiologictherapy: Anti-lgE or Antl-lLS/SRmAb le ICS-formoteroldaily (low-doseICS) ICS-formoterolPRN irc Steps 1• & 2 +LAMA ICS-formoteroldaily (medium-to high-doseICS) Adjunctive controller therapies = LTRA. allergy immunotherapy •step 1: Alternatively,ICS + SABAmay be used PRN. rC ICS • inhaled conicosteroid: LAMA• long-actingmuscarinicantagonist; LTRA • leukotrienereceptor antagonist: mAb • monocolonalantibody: OCS • oral corticosteroid:PRN • as needed: SABA • short-actingbeta-2 agonist. .. . .. ne Adverse effects of inhaled corticosteroids -75% of dose deposited in oropharynx Dysphonia: steroid-induced laryngeal myopathy; common (50%) & usually minor Thrush: affects tongue & pharynx (esophagus: very rarely); prevent by rinsing & gargling with water, using spacer (decrease local deposition) Other: contact dermatitis (budesonide hypersensitivity); cough & reflex bronchoconstriction In Local -25% of dose deposited in peripheral lung & systemically absorbed . HPA axis: secondary adrenal insufficiency (usually subclinical); can coexist with !CS-induced Cushing syndrome Skeletal: small t osteoporosis & fracture risk Other: dermal thinning (fragile skin, purpura), ocular effects (cataract formation, j IOP), small j CAP risk in COPD patients LE Systemic* *Uncommon. Typically only seen with prolonged (years) of high-dose ICS, superimposed oral glucocorticoid use, or concurrent administration of potent cytochrome P-450 3A4 inhibitor. U SM CAP= community-acquired pneumonia; COPD = chronic obstructive pulmonary disease: HPA = hypothalamic-pituitary-adrenal; ICS = inhaled corticosteroid; IOP = intraocular pressure. You cannot give LABA without an ICS/steroids. That will increase mortality and is always the wrong answer.If the patient is on a LABA, they must be on steroids. NEVER USE LABA FIRST OR ALONE.  What is the likely cause of leukocytosis with neutrophilic predominance in a patient being treated for asthma exacerbation? Glucocorticoid side effect  The primary long-term intervention for persistent asthma is daily inhaled corticosteroids. versus a long-acting anticholinergic inhaler COPD Pulmonary 103 https://t.me/usmleinnercircle Version 2 Bronchiectasis Bronchiectasis • Pathophysiology: airway insult (eg, infection, inhalation) with impaired clearance (eg, mucostasis, Clinical features Etiologies/contributory conditions immunodeficiency) • • Acute exacerbations: recurrent infections with mucopurulent sputum ± frank hemoptysis Chronic: daily production of voluminous, thick ± blood-tinged mucus • Airway obstruction (eg, cancer, foreign body) & mucostasis (eg, CF, ABPA) • Immunodeficiency (eg, tlg) & autoinflammatory disease (eg, Sjogren syndrome) • Chronic/past infection (eg, mycobacteria) or toxic inhalation Evaluation • High-resolution CT scan of the chest (needed for diagnosis): airway dilation • Pulmonary function testing: obstructive pattern • Investigation of etiologies (eg, lg levels, respiratory cultures, bronchial obstruction) Treatment • Address underlying disorders (eg, lg replacement) • Airway clearance (eg, chest physiotherapy), mucolytics • Antibiotics to suppress bacterial overgrowth & treat exacerbations ABPA = allergic bronchopulmonaryaspergillosis;CF = cystic fibrosis; lg = immunoglobulin;RA= rheumatoidarthritis. Focal bronchiectasis is usually due to upstream airway obstruction (eg, neoplastic lesion or foreign body), whereas diffuse bronchiectasis typically reflects systemic disease (eg, cystic fibrosis). Bronchoscopic airway inspection is required to evaluate focal bronchiectasis, allowing for direct visualization along with diagnostic and therapeutic interventions on the obstructing lesion. Bronchiectasis Pulmonary 104 https://t.me/usmleinnercircle Version 2  What test/imaging study is the best diagnostic test for bronchiectasis? high-resolution CT scan of the chest characteristic findings include bronchial dilation, lack of airway tapering, and bronchial wall thickening le May present similarly to chronic bronchitis, but bronchiectasis has more prominent sputum production and exacerbations are typically bacterial (viral in chronic bronchitis) irc Upper lung lobe involvement is characteristic of bronchiectasis due to CF and helps differentiate it from bronchiectasis due to other causes. rC Allergic Bronchopulmonary Aspergillosis Allergic bronchopulmonary aspergillosis Risk factors & pathogenesis • Structural airway disease (eg, asthma, cystic fibrosis) • Fungal spore colonization -> Th2-based sensitization .....allergic inflammation &diagnosis 0 Elevated serum lgE (usually >1,000 IU/ml) 0 Positive Aspergillus skin test &/or lgE 0 Suggestive: eosinophilia, positive Aspergillus lgG • Systemic glucocorticoids-> t allergic inflammation • Antifungal drugs (eg, voriconazole) .....t spore burden • Treatment of underlying asthma (eg, bronchodilators) LE Treatment • Aspergillus sensitization: In Clinical features ne • Difficult-to-control asthma, thick sputum • Chest imaging: fleeting infiltrates, bronchiectasis, bronchial mucoid impaction  Difficult to differentiate from CF Pneumonia. U SM ABPA must be suspected if there is an unexplained lung function decline despite an appropriate antibiotic course 1 wk) Restrictive Interstitial Lung Disease Pulmonary 105 https://t.me/usmleinnercircle Version 2 Interstitial lung disease • Sarcoidosis, amyloidosis, alveolar proteinosis Common etiologies • Vasculitis (eg, granulomatosis with polyangiitis) • Infection (eg, fungal, tuberculosis, viral pneumonia) • Environmental exposure (eg, silicosis, HP) • Connective tissue disease (eg, SLE, scleroderma) • IPF, cryptogenic organizing pneumonia • Progressive exertional dyspnea, dry cough Clinical presentation Diagnosis • >50% of patients have significant smoking history • Fine inspiratory crackles ± digital clubbing • Chest x-ray: reticulonodular interstitial opacities • HRCT: fibrosis, honeycombing, traction bronchiectasis • PFT: restrictive pattern with J DLCO DLCO = diffusion capacity of the lung for carbon monoxide; HP = hypersensitivitypneumonitis; HRCT = high resolution CT scan; IPF = idiopathic pulmonaryfibrosis; PFT = pulmonaryfunction testing; SLE = systemic lupus erythematosus. • Inspiratory "Velcro" (eg, fine, dry) crackles are sensitive for interstitial fibrosis, which is present in many forms of ILD Early into disease progression, interstitial lung abnormalities may not be seen on CXR. Therefore, patients with suspected ILD should undergo high-resolution computed tomography HRCT, a thinslice (usually 1 mm) CT scan protocol developed to visualize subtle interstitial lung features (eg, reticulation, honeycombing). In ILD, hypoxemia, especially with exertion, is more characteristically observed than hypercapnia. (vs COPD Hypersensitivity Pneumonitis Pulmonary 106 https://t.me/usmleinnercircle Version 2 Hypersensitivity pneumonitis • Immunologic response to inhaled antigen (eg, mold, animal protein) Etiology Acute • Abrupt-onset fever, chills, cough, dyspnea, fatigue, leukocytosis • Episodes often recurrent and self-resolving • Chest x-ray: scattered micronodular interstitial opacities Clinical presentation Chronic • Progressive cough, dyspnea, fatigue, weight loss • Hypoxemia that worsens with exertion • Chest x-ray: diffuse reticular interstitial opacities le • PFT: restrictive pattern, J DLCO (chronic only) • BAL: high relative lymphocyte count Diagnosis • Lung biopsy: lymphocytic infiltrate, poorly formed noncaseating granulomas, interstitial inflammation or fibrosis irc (chronic only) • Remove antigen exposure (resolves acute disease) • Glucocorticoids and/or lung transplantation (chronic only) Treatment rC BAL= bronchoalveolarlavage; DLCO = diffusioncapacity of the lung for carbon monoxide;PFT = pulmonaryfunction testing.  Acute symptoms recur and resolve within 12 days (vs other infectious causes like Pneumonia) ne Sarcoidosis Sarcoidosis with mediastinal and hilar lymphadenopathy Cutaneous Ophthalmologic • Interstitial infiltrates • Papules, nodules & plaques • Erythema nodosum• • Anterior & posterior uveitis • Keratoconjunctivitis sicca • Facial nerve palsy • Central diabetes insipidus U SM Neurologic • Hilar lymphadenopathy* LE Pulmonary In Manifestations of sarcoidosis • Hypogonadotropic hypogonadism Cardiovascular Gastrointestinal Other • AV block • Dilated or restrictive cardiomyopathy • Hepatosplenomegaly • Asymptomatic LFT abnormalities • Hypercalcemia • Peripheral lymphadenopathy • Parotid gland swelling • Polyarthritis* • Constitutional symptoms (fever•, malaise) *Manifestations of Lofgren syndrome. AV = atrioventricular; LFT = liver function test. Pulmonary 107 https://t.me/usmleinnercircle Version 2 Sarcoidosis key points: • common in young, AA, females • presents as SOBOE with occasional fine rales on exam; no wheezing • can be associated with erythema nodosum and lymphadenopathy; if present, the diagnosis is likely • best initial test: CXR (hilar adenopathy seen in  95% of cases) • most accurate test: lymph node biopsy to look for noncaseating granulomas • treatment is prednisone; if asymptomatic, do not treat Although lung involvement (eg, hilar lymphadenopathy) is common in sarcoidosis, it is often 50% asymptomatic, and patients may present with extrapulmonary disease. -osis: causes restrictive disease of some organ e.g: Sarcoidosis = restrictive lung disease, restrictive heart disease possible  Patient with sarcoidosis who presents with RUQ pain Liver involvement (granulomas in liver) Pulmonary Hypertension Management of pulmonary hypertension Group 1 PAH Group 2 Left-sided . . Group 3 chronic hypoxemia Group 4 CTEPH General measures Pulmonary vasodilator therapy, treat underlying (eg, autoimmune) cause • Echocardiography (± RHC if diagnosis unclear) • Optimize left ventricular function (eg, heart failure therapy), treat significant valve heart disease Lung disease &/or Evaluate for autoimmune CTD, toxins (eg, amphetamines), HIV, schistosomiasis, etc. disease • Pulmonary function tests, chest imaging, polysomnography • Treat underlying respiratory condition (eg, obstructive sleep apnea) . .. .. • V/Q scan, pulmonary angiography Pulmonary thromboendarterectomy surgery (curative), anticoagulation Refer to accredited PH center Maintain normoxia, euvolemia & sinus rhythm Contraceptive counseling, immunization, cardiac rehabilitation Refractory PH: bilateral lung transplant CTD = connectivetissue disease (eg, scleroderma);CTEPH = chronicthromboembolicpulmonaryhypertension;PAH = pulmonary arterial hypertension;PH= pulmonaryhypertension;RHC = right-sidedheart catheterization;V/Q = ventilation-perfusion. Pulmonary 108 https://t.me/usmleinnercircle Version 2 .. .. .. . Symptoms Signs ! Cardiac output: exertional syncope/presyncope, fatigue, weakness t PA pressure: chest tightness, hemoptysis (rare) RV demand ischemia: exertional angina/tightness Venous congestion: abdominal distension (bowel edema), early satiety Precordial heave due to RV hypertrophy Loud P2, right-sided S3 &/or S4 Holosystolic murmur of tricuspid regurgitation • JVD, ascites, peripheral edema, hepatomegaly In ne rC irc le Pulmonary arterial hypertension 1.:::.s1.r1.?.120282755?.,,,s U SM LE Muscular hypertrophyin pulmonaryarteries/arterioleselevates right-sidedheart pressures Pulmonary 109 https://t.me/usmleinnercircle Version 2 Evaluation of pulmonary hypertension Suspected PH (dyspnea, lethargy, fatigue) ! Echocardiography (ECHO) ---i No ----~---Yes High PH clinical suspicion Left-sided heart disease seen on ECHO rYes__LN07 Exercise ECHO or right-sided heart catheterization rYes__LN07 Evaluate for other causes Cardiology consult for left-sided heart disease evaluation Further evaluation l ! Consider the following based on history: • Polysomnography • V/Q scan to rule out CTEPH • Pulmonary function studies • HIV serology • ANA, RF, ANCA: in patients with suggestive symptoms • Urine toxicology (amphetamines) Abnormal& suggets PH ANA = antinuclearantibodies,ANCA = antlneutrophllcytoplasmicantibodies;CTEPH = chronic thromboembollcPH; PH = pulmonaryhypertensioo;RF = rhel.lTlatoidfactor;VQ = ventilation-perfusion CUWorld OSA 1 point for each characteristic present .. ... ... STOP-Bang survey for obstructive sleep apnea Snoring Excessive daytime tiredness Observed apneas or choking/gasping High blood pressure BMI >35 kg/m 2 Age >50 Neck size: men >17 in (43.2 cm), women >16 in (40.6 cm) Male gender Scoring: 0-2 points: low risk; 3-4 points: intermediate risk; .:5 points: high risk. Have poor positive predictive value but high negative predictive value for OSA; patients with a score 3 are unlikely to have OSA and do not require diagnostic testing  Patients with obstructive sleep apnea are at increased risk of perioperative respiratory failure from procedures involving sedation, neuromuscular blocker, opioids, or anesthesia. When respiratory failure occurs, it results from hypoventilation and typically presents with hypercapnia and hypoxia. Pulmonary 110 https://t.me/usmleinnercircle Version 2  Many patients who snore do not have obstructive sleep apnea OSA; in the absence of other evidence suggesting OSA, snoring is not an indication for diagnostic testing for OSA. Smoking cessation and elimination of alcohol intake before bedtime are preferred initial management strategies for snoring. Pneumonia le Fever+ Cough+ Sputum Amoxicillin Cephalosporin FluoroQuin l&D Clinda rC Typical Sxs Strep Pneumo Something Else 3'" Gen Cephalo (if CAP) Macrolides (if CAP) Zosyn (ifl!AP} Vancomycin ne (ifl!AP) irc e Bronchitis Yes PCP Risk In Bronchoalvcolar uivagc Bactrim Steroids According to the most recent guidelines published by the Infectious Diseases Society of America, LE pneumonia is classified as follows: • Community-acquired – develops in a nonhospitalized setting M/C S. Pneumoniae) • Hospital-acquired – develops 48 hours after admission to the hospital U SM • Ventilator-acquired – develops 48 hours after endotracheal intubation Etiology Clinical features Treatment Pulmonary Community-acquired pneumonia in school-aged children Lobar Bilateral • Increased work of breathing Focal crackles Oral amoxicillin (outpatient) or intravenous ampicillin or ceftriaxone (if hospitalized) Chlamydia pneumoniae • Viruses (rare) • Abrupt onset of fever, cough, chest pain . . . . • Mycoplasma pneumoniae • Streptococcus pneumoniae Fever, malaise, headache, sore throat • Prolonged, gradually worsening cough • Patient can often continue normal activities • Bilateral crackles, wheezing • Macrolide (eg, azithromycin) 111 https://t.me/usmleinnercircle Version 2 Patients with HIV are at increased risk for community-acquired pneumonia CAP "Rusty sputum" is classic for pneumococcal pneumonia but may not always be present. Can present with Clear Sputum Only select if there are no viral options in question)  Immunocompromised Patients with CAP may have no alveolar infiltrate on initial chest x-ray d/t less cytokine response. DO a CT scan if CAP in suspected in such patients and X-ray is Normal. Common causes of recurrent pneumonia Bronchial obstruction • Extrinsic: neoplasm, adenopathy • Intrinsic: bronchiectasis, foreign body Same lung lobe* Recurrent aspiration • Altered consciousness: seizure, sedatives, antipsychotics, alcohol, illicit drugs • Dysphagia: neurologic disorder, esophageal motility issue • Poor dental hygiene • Gastroesophageal reflux Different lung lobes • Immunodeficiency: HIV, leukemia, CVID • Sinopulmonary disease: cystic fibrosis, immotile cilia • Noninfectious: vasculitis, BOOP *Recurrent aspiration can lead to pneumonia in different dependent regions of the lung depending on the position of the patient during the aspiration event. BOOP = bronchiolitis obliterans with organizing pneumonia; CVID = common variable immunodeficiency. What imaging modality is typically used to diagnose pneumonia? Chest X-ray diagnosis requires presence of lobar, interstitial, or cavitary infiltrate on imaging; CXR should be **acquired before administering empiric antibiotics** What test/imaging study should be ordered first to evaluate an older patient with a 30 pack-year smoking history and recurrent episodes of pneumonia in the same anatomic location? CT scan of chest in patients  50 with significant smoking history  30 pack-years), it is essential to evaluate for lung malignancy; CT scan has better sensitivity than CXR Recurrent pneumonia in the same anatomic location is a red flag for lung cancer.  What is the pathophysiologic mechanism underlying worsening hypoxemia when a patient with lobar pneumonia lies on the affected side? Pulmonary 112 https://t.me/usmleinnercircle Version 2 Increased intrapulmonary shunting lying on the affected side increases blood flow Q to this area due to gravity, where ventilation V is poor due to pneumonia, thus worsening the V/Q ratio Effects of positioning in a patient with pneumonia Pneumonia down t perfusion of poorly ventilated region le ! t V/Q mismatch & irc worsened hypoxemia rC Pneumoniaup ! perfusion of poorly ventilated region ! ! VIQ mismatch & ne improved hypoxemia In  What is the likely diagnosis in a patient with pneumonia that presents with continued symptoms despite adequate antibiotic coverage and loculation on CXR? Complicated parapneumonic effusion LE Do chest X-ray to Dx; requires drainage in addition to antibiotics U SM Pneumonia unresponsive to treatment despite adequate Antibiotic coverage; evaluate for complications like Effusion, Abscess Characteristics of high-altitude pulmonary edema versus multifocal pneumonia High-altitude pulmonary edema • Recent arrival at high altitude (<1 week) • Absent or mild leukocytosis • • Marked early improvement with 0 2 Procalcitonin normal Multifocal pneumonia • Persistent stay at high altitude • Leukocytes >15,000 mm3 with bands • Procalcitonin elevated • Minimal early improvement with 0 2 Both conditions can cause fever, leukocytosis, hypoxemia, and bilateral lung crackles. VAP Pulmonary 113 https://t.me/usmleinnercircle Version 2 Evaluation of suspected ventilator-associated pneumonia Suspected VAP • Abnormal chest x-ray Lower respiratory tract endotracheal tube sample • Culture • Microscopy Empiric antibiotics* Negative cultures Discontinue antibiotics & evaluate for other causes • Gram-positive coverage • Antipseudomonal & gramnegative coverage • Consider MRSA coverage Positive cultures with clinical improvement • Narrow antibiotics according to culture results Positive cultures without clinical improvement • Likely VAP • Consider changing antibiotics • Assess for VAP complications (eg, abscess, empyema) • Consider evaluating for other causes MRSA • mettwcilri-resista~ Staphylococcusaureus;VAP • ventilator-associatedpneumonia, ·empmccoverage depends on drug-resistance pattern at institution. • Ventilator-associated pneumonia is a type of hospital-acquired pneumonia that develops after more than 48 hours of mechanical ventilation (intubation). Presentation? Pulmonary infiltrates with one or more of the following: fever, purulent secretions, leukocytosis, difficulty with ventilation Major risk factors for ventilator-associated pneumonia .. .. .. Acid suppression (eg, PPI, H2R blocker, antacid) Supine position Pooled subglottic secretions Paralysis & excessive sedation Excessive patient movement while intubated Frequent ventilator circuit changes H2R = histamine-2 receptor; PPI = proton pump inhibitor. Aspiration Pneumonia Pulmonary 114 https://t.me/usmleinnercircle Version 2 Conditions associated with aspiration pneumonia Predisposing conditions for aspiration pneumonia Trigger Related conditions • Altered consciousness impairing cough reflex/glottic closure (eg, dementia, drug intoxication) . .. . .. . Reduced consciousness Dysphagia Neurologicdisorders Esophagealmotility defects Protractedvomiting • Gastroesophagealreflux • Upper gastrointestinal tract disorders (eg, GERO) • Mechanical compromise of aspiration defenses (eg, nasogastric & endotracheal tubes) • Protracted vomiting • Large-volumetube feedings in recumbent position • Tracheostomy Intubation Nasogastricfeeding • Gingivitis Poor dental hygiene irc Pharyngeal or glottal dysfunction le • Dysphagia due to neurologic deficits (eg, stroke, neurodegenerativedisease) • Sedatives or antipsychotics Illicit drugs or alcohol • Anesthesia • Generalizedseizure rC Dental issues Bacterial aspiration pneumonia • Oropharyngeal/gastric microbes aspirated into lungs -+ host defenses overwhelmed due to Pathophysiology large inoculum size .. . • . Impaired glottic closure (eg, intubation) Protracted vomiting Poor dental hygiene Fever & cough ± foul-smelling sputum • Infiltrate in dependent portions of lung • Aerobic + anaerobic pathogens on sputum studies Clinical features LE . Management Dysphagia (eg, neuromuscular disorder) In Major risk factors ne • Reduced consciousness (eg, anesthesia) • No empyema or lung abscess: treat for community-acquired pneumonia Empyema or lung abscess present: extend coverage to include anaerobes (eg, ampicillin- U SM sulbactam)  Most patients have foul-smelling sputum and symptoms that progress over 12 weeks Strategies to prevent aspiration pneumonia in hospitalized patients .. .. . Recommended Not recommended Elevation of head of bed to 30-45 degrees Enteral feeding Speech & swallow evaluation Drainaoe of subolottal secretions• Decontamination of oror2hartnx/digestive tract* .. .. Prokjnetjc agents Gastrostomy tube or nasogastric tube Gastric volume measurement Probiotic medications *In intubated patients. Pulmonary 115 https://t.me/usmleinnercircle Version 2 • Aspiration pneumonia prophylaxis can include oral care, diet modification for patients with dysphagia, and elevating the head of the bed to 3045 degrees. Also can benefit from Thickened Liquids. Normal swallowing involves a complex sequence of voluntary and reflexive processes that move food from the mouth to the esophagus while preventing it from entering the airway (ie, aspiration). Three main airway-protective movements occur during normal swallowing: • Displacement of the larynx superiorly and anteriorly under the base of tongue, which allows food to be directed into the more posteriorly located esophagus • Tilting of the epiglottis to block the airway • Closing of the glottis by adduction of the vocal folds Because of the complexity of swallowing, patients who sustain strokes often have persistent dysphagia and/or aspiration. If a neurologic deficit cannot be corrected, behavioral modifications can sometimes improve the safety of swallowing. A chin-tuck maneuver (ie, flexion of the head and neck during swallowing) sometimes helps. The maneuver seems to decrease the distance from the hyoid bone to the larynx (simulating elevation of the larynx), as well as to narrow the distance of the laryngeal entrance, leading to decreased aspiration. Recurrent Pneumonia Pulmonary 116 https://t.me/usmleinnercircle Version 2 Recurrentpneumonia Organisms Aspiration Seizures, dysphagia, alcohol intoxication Clinical features • Right middle/lower lobe • Dysphagia/dysarthria • Altered mentation • Anaerobes • Polymicrobial • Streptococcus pneumoniae Chronic obstructive lung disease Chronic bronchitis, emphysema, asthma, bronchiectasis • Haemophilus influenzae • Smoking history • Chronic cough • Chronic dyspnea • Moraxella catarrhalis le • Pseudomonas spp (especially bronchiectasis) Immunodeficiency HIV, primary immune deficiency, hypogammaglobulinemia, hematologic malignancy • S pneumoniae • H influenzae • Pneumocystis • Atypical organisms Tuberculosis • Mycobac/erium tuberculosis Treatment In -·-·· of CAP • Macrolide or doxycycline (healthy) • Fluoroquinolone• or beta-lactam + macrolide (comorbidilies) Inpatient (non-ICU) • Fluoroquinolone• (IV) • Beta-lactam + macrolide (IV) Inpatient (ICU) • Beta-lactam + macrolide (IV) • Beta-lactam + fluoroquinolone• (IV) LE Outpatient U SM • Hemoptysis • Weight loss/cachexia • Pneumonia in the same location rC • Polymicrobial • Variable depending on specific condition • • • • ne Post-obstructive Empiric treatment irc • Viral Upper lobe/apical Recent immigrant Institutionalized patient Homeless/lower socioeconomic status CURB-65 to determine hospitalization 1 point for each of the following· Confusion Urea >20 mg/dL Respirations 2:30/min Blood pressure (Systolic blood pressure <90 mm Hg or diastolic <60 mm Hg) • Age2:65 *Respiratory fluoroquinok>nes(eg, levofloxacin, moxifloxacin) are required. CAP = community-acquired pneumonia; ICU = int~nsive care unit; IV = intravenous. T M/C Cause: S. Pneumoniae Outpatient treatment Pulmonary T Low mortality High mortality Likely inpatient treatment Urgent inpatient admission; possibly ICU if score >4 117 https://t.me/usmleinnercircle Version 2 Outpatient treatment of community-acquired pneumonia Can tolerate penicillins -Yes---> • Amoxicillin or amoxicillin-clavulanate• PLUS • Macrolide (preferred) or doxycycline - • Third generation cephalosponn PLUS • Macrolide (preferred) or doxycycline I No ! Can tolerate cephalosporins Yes ___. No • Respiratory fluoroquinolone ·use amoXJcillln-davulanate whennskfactorsforseverediseaseare present(eg, smoking,age >65, recentantibiotics. majorcomorblditles. alcoholuse disorder). CUWorld HAP Vancomycin + Piperacillin-Tazobactam • Fluoroquinolones (eg, levofloxacin, moxifloxacin) that target respiratory tract organisms can also be used for CAP in the inpatient setting, this class of medication is generally avoided (when possible) in elderly patients due to increased risk of Clostridium difficile infection, tendon rupture, and aortic dissection.  Empiric treatment of pneumonia in a young child with cystic fibrosis should include coverage against methicillin-resistant S. aureus. e.g. IV vancomycin Rates of bacterial colonization in cystic fibrosis based on age <2 DUWorld 10 15 20 25 Age (years) 30 35 40 >45 Pleural Effusion Pulmonary 118 https://t.me/usmleinnercircle Version 2 Management of Parapneumonic Effusions Parapneumonic effusions Etiology Uncomplicated Complicated Sterile exudate in pleural Bacterial invasion of pleural space space Pleural fluid analysis • pH ~7.2 • Glucose ~60 mg/dl • pH <7.2 • Glucose <60 mg/dl 3 • WBC >50,000/mm Negative Negative• Antibiotics Antibiotics + drainage Pleural fluid Gram stain & culture Treatment Respiratory distress No respiratory distress or hypoxia OR 3 • WBC 550,000/mm Hypoxia Oral antibiotics Close monitoring Ultrasound IV antibiotics Drainage ClUWorid le *Gram stain & culture is typically a false negative due to low bacterial count. Both are Etiology Uncomplicated parapneumonic Complicated parapneumonic effusion effusion Inflammatory fluid from pneumonia --+ Bacterial invasion into pleural Empyema Bacterial colonization- fluid purulent fluid pH = 7.20, !/normal glucose, LDH ratio pH <7.20, ! glucose, LDH ratio pH <7 .20, ! glucose, LDH ratio >0.6 >0.6 >0.6 Can be positive or negative Positive pleural space Pleural fluid analysis rC Parameter irc typically positive in empyema. WBC = white blood cells. Pleural fluid Gram stain & Negative ne culture LDH = lactate dehydrogenase. In Positive gram stain & culture helps distinguish empyema from complicated pleural effusion; both are managed with antibiotics and drainage LE • An uncomplicated parapneumonic effusion forms when lung interstitial fluid increases during pneumonia and moves across the adjacent visceral pleural membrane. The pleural fluid is characterized by "exudative" chemistries and an influx of neutrophils into the pleural space. U SM • A complicated parapneumonic effusion develops when there is bacterial invasion of the pleural space. Bacterial invasion typically leads to an increased number of neutrophils and the development of pleural fluid acidosis, which results from anaerobic utilisation of glucose by the neutrophils and bacteria. In addition, lysis of neutrophils increases the LDH concentration in the pleural fluid to values often in excess of 1000 IU/L.  Normal pleural fluid pH is 7.60; -Exudative effusions typically have lower pH 7.30  7.45 -Transudative effusions typically have higher pH 7.40  7.55  What is the next step in management for a smoker with progressive dyspnea, weight loss, and evidence of pleural effusion on CXR? Diagnostic thoracentesis Pulmonary 119 https://t.me/usmleinnercircle Version 2 undiagnosed pleural effusion is best evaluated with thoracentesis to determine if the fluid is transudative or exudative; exception: in patients with evidence of CHF (e.g. weight gain, pedal edema, bibasilar crackles), the first step is a trial of diuretics. Can quickly determine CHF by BNP level 500  CHF  What are the etiologies for pleural effusion with high lymphocytic count 50%? Cancer, Tuberculosis, Fungus  High lymphocyte count with high ADA? Tuberculosis • Tuberculosis pleural effusions are typically lymphocytic and exudative with an elevated adenosine deaminase level. Smears are often negative for acid-fast bacilli; diagnosis usually requires pleural biopsy with histopathologic demonstration of pleural granulomas. Transudate Physical appearance Specific gravity Glucose i;;:i Exudate Does not froth or form clots Straw-colored fluid (may rarety be hemorrhagic), which froths on shakng and forms clots on standing still s 1.016 > 1.016 2 60 mg/dl • 3~ mg/dl: suggests malignant effusion, tuben:ulous pleurisy, empyema, pneumonia, esophageal ":'~l:'~· or lupus pleuritis • < 30 mg/dl: suggests rheumatoid pleurisy or empyema Light's criteria Cholesterol < 60 mg/dl Total protein s30g/l >30g/L Pleural fluid protein: serum protein ratio s0.5 :> 0.5 Pleural fluid LOH: serum LOH ratio s 0.6 > 0.6 p < % the upper limit of normal serum LOH Pleural fluid LOH > ¾ the upper lim~ of normal serum LOH i;;:i Pleural fluid LOH (lactate dehydrogenase) I Pulmonary 2 60 mg/dL (strongly elevated in chylothorax) Pleural fluid with a bloody appearance suggests a malignant etiology! 120 https://t.me/usmleinnercircle Version 2 . • . • . . . .• . Pediatric empyema Clinical features Management resulting in fibrinopurulent consolidation Streptococcus pneumoniae Staphylococcus aureus (eg, MRSA) Pneumonia symptoms (eg, fever, dyspnea, pleuritic chest pain) No improvement with routine pneumonia treatment Signs of pleural effusion (eg, dullness to percussion) Laboratory evidence of inflammation le Common organisms Bacterial invasion of pleural space (eg, leukocytosis, thrombocytosis) Supportive care Empiric antibiotics + drainage (ie, chest tube or surgical) irc Etiology lntrapleural fibrinolytics may aid drainage MRSA = methicillin-resistant Staphylococcus aureus. rC Ceftrixone  Vancomycin/ Clindamycin Exudative effusions • Pleural protein/serum protein ratio >0.5 • Pleural LOH/serum LDH >0.6 ne Pleural fluid analysis • Pleural LDH > 2/3 upper limit of normal for serum LDH • Empyema (purulent fluid, neutrophil-predominant, + gram stain/culture) • Chylothorax (milky white fluid, j triglycerides) • Malignancy (abnormal cytology) In Etiologies • Tuberculosis(+ acid-fast bacterium stain/culture) LE LOH = lactate dehydrogenase.  A pancreaticopleural fistula (between the pancreatic duct and the pleural space) resulting in an amylase-rich exudative pleural effusion occurs most commonly as a result of acute or chronic pancreatitis. U SM Management includes bowel rest to promote fistula closure; endoscopic retrograde cholangiopancreatography with sphinceterotomy may be required to drain pancreatic fluid through ampulla of vater rather than fistula. D/D Boerhaave Syndrome; pleural fluid ph is very low 6 Lung Abscess Pulmonary 121 https://t.me/usmleinnercircle Version 2 Lung abscess Pathophysiology Manifestations Diagnosis Treatment • Aspiration of oropharyngeal/gingival anaerobes • Risk factors: dysphagia, substance abuse, seizures • Pneumonilis ---+pneumonia---+ abscess/empyema • Subacute fever, night sweats, weight loss . . • • . Cough with putrid sputum Cavitary infiltrates with air-fluid levels Cultures rarely useful Ampicillin-sulbactam, imipenem, meropenem Alternate: clindamycin Lung Abscess key points: • abscesses don't just form out of nowhere; these patients typically have an aspiration risk • empiric treatment with Ampicillin-sulbactum is recommended (again, due to the aspiration risk; most lung abscesses involve anaerobes) • aspiration risk is typically secondary to seizure, alcoholism, MS or stroke • give proper treatment once culture and sensitivity return. eparating and lower I Pulmonary 122 https://t.me/usmleinnercircle Version 2 irc le Lung abscess rC Pneumothorax Pneumothorax Spontaneous pneumothorax • Primary: No preceding event or lung disease; thin, young men . In Breath sounds, jchest movement LE • lpsilateral hyperresonance to percussion U SM Imaging Management • Often due to trauma or mechanical ventilation • Secondary: Underlying lung disease (eg, COPD) • Chest pain, dyspnea Signs & symptoms • Life-threatening ne Associated features Tension pneumothorax Same as spontaneous with: • Hemodynamic instability • Tracheal deviation away from affected side • Absent lung markings Same as spontaneous with: • Visceral pleural line • Contralateral mediastinal shift • lpsilateral hemidiaphragm flattening • Small ($2 cm): Observation & oxygen • Urgent needle decompression or chest tube placement • Large & stable: Needle aspiration or chest tube COPD = chronic obstructive pulmonary disease. Pulmonary 123 https://t.me/usmleinnercircle Version 2 Left pneumothorax  What is the likely diagnosis in a patient on a ventilator who develops hypotension, tachycardia, and unilateral absence of breath sounds? Pneumothorax  What is the diagnosis in Premature infant with unilateral absence of breath sounds, heart sounds on right, increased transillumination on left side of the chest? Tension Pneumothorax on left side Illumination test can be used in infants to diagnose it quickly, or Chest X-ray can be used if not sure. Pneumothorax on left pushed the heart to the right → Urgently Needle Decompression needed Meconium aspiration syndrome (most common in post-mature neonates) is associated with pneumothorax because meconium plugging of airways traps distal gas, promoting alveolar overdistension and rupture. Bedside ultrasonography can be rapidly performed and has high sensitivity and specificity for pneumothorax. It has become the test of choice for evaluation of tension pneumothorax in the acute setting (eg, trauma bay, intensive care unit). Key idea: Intubation is CONTRAINDICATED in a patient with a pneumothorax Put a tube or needle first Pulmonary 124 https://t.me/usmleinnercircle Version 2 Spontaneous Pneumomediastinum Spontaneous pneumomediastinum . .. . . • Asthma exacerbation Risk factors Respiratory infection • Tall, thin, adolescent boy • Acute chest pain, shortness of breath, cough Diagnosis Treatment Subcutaneous emphysema Hamman sign (crunching sound over heart) Mediastinal gas on chest x-ray Rest, analgesics le Clinical features irc • Avoid Valsalva maneuvers In ne rC Pneumomediastinum & pneumopericardium U SM LE Pulmonary Embolism Pulmonary 125 https://t.me/usmleinnercircle Version 2 Approach to patient with suspected pulmonary embolism Stabilize patient with oxygen & IV fluids Evaluate for absolute contraindications to anticoagulation Yes Obtain diagnostic test to evaluate for PE No Assess clinical suspicion of PE with modified Wells criteria PE unlikely Consider IVCfilter No further evaluation needed Consider anticoagulation, especially if patient has: • No relative contraindications • Moderate to severe distress IV = intravenous; IVC = inferiorvena cava; PE = pulmonary embolism. Start or continue anticoagulation; consider surgery or thrombolytics if indicated Stop anticoagulation If there is no contraindication to anticoagulation, it should precede diagnostic imaging in patients with likely PE, especially when in moderate distress Recent clinical guidelines by the American College of Chest Physicians recommend ≥3 months of an oral factor Xa inhibitor (eg, rivaroxaban) for patients with DVT or pulmonary embolism (PE). Pulmonary 126 https://t.me/usmleinnercircle Version 2 Predictors of 30-day mortality in pulmonary embolism ,. Diagnostic strategy in suspected pulmonary embolism . Clinical assessment for pulmonary embolism Hypotension, SBP <90 mm Hg • Tachycardia, >110/min Modified Wells criteria .. . . .. U"""'» 0 • Tachypnea, >30/min Clinical Hypoxemia, <90% • Altered mental status PE likely PE unlikely Hypothermia, <30 C (<86 F) History of cancer • Age >80 J I PE excluded j j CT pulmonary angiography Negative j t PE confirmed PE = pulmonaryembolism Brain natriuretic peptide SBP = systolic blood pressure. Positive PE excluded Troponin irc >500 ng/ml Laboratory Right ventricular dysfunction j ne C,IJWolld rC ssoong/ml Radiological le j D-dimer assay ) Diagnostic approach to pulmonary embolism In Suspected PE J Hemodynamically unstable' Hemodynamically stable LE i i Determine pretest probability Low or intermediate l I Positive D-dimer screen ----- j U SM Negative PE excluded Assess RV with emergent TTE High l l l l I l Confirmatory testing (CTAorV/Q scan) New dysfunction (dilation. hypokinesis) Normal PE diagnosed or excluded Presumed massive PE Evaluate for other causes of shock j ·sBP <90 mm Hg for ~15 min, or requirementfor vasopressorflnotropesupport. CTA =CT angiography:PE = pulmonaryeembolism:RV =right ventride: SBP = systolicblood pressure: TTE = transthoracicechocardiography:V/Q = ventilation-perfusion. PE inhospital mortality Risk Factor: AGE & Obstructive Shock  What is the test of choice to diagnose pulmonary embolism in clinically stable patients with a high likelihood of PE (modified Wells  4? Pulmonary 127 https://t.me/usmleinnercircle Version 2 CT angiography in patients with low likelihood of PE (modified Wells  4, D-dimer testing can help rule out PE due to high negative predictive value  A patient is thought to have a pulmonary embolism but cannot tolerate IV contrast due to severe renal disease. What other diagnostic test can be ordered to help in the workup? V/Q scan Also done in Pregnant patients to avoid fetal radiation Pathophysiology of submassive/massive pulmonary embolism Modified Wells criteria for pretest probability of pulmonary embolism Massive PE +3 points RV outflow obstruction f---------, t RV pressure RV hypokinesis & dilation t RV wall tension + RV myocardial 0 2 demand septal deviation toward LV ! LV preload & cardiac output • Alternate diagnosis less likely than PE • Previous PE or DVT +1.5 points ! RV cardiac output & • Clinical signs of DVT ! Coronary perfusion & ! RV myocardial supply +1 point • Heart rate > 100 • Recent surgery or immobilization • Hemoptysis • Cancer S4 = PE unlikely Total score • • >4 = PE likely DVT = deep vein thrombosis; PE = pulmonary embolism. Patients with acute massive PE can present with syncope and hemodynamic collapse • 4  likely = immediate anticoagulation → CT Angiography • 4  D dimer  500 ⟶ CT Angiography only if +ve, reassurance if -ve  What is the likely diagnosis in a post-operative patient with hypotension, JVD, and new-onset right bundle branch block? Massive pulmonary embolism -massive PE is defined as PE complicated by hypotension and/or acute right heart strain (e.g. JVD, RBBB Pulmonary 128 https://t.me/usmleinnercircle Version 2 Anticoagulation Inferior vena cava filter . .. . . All patients unless specific contraindications to anticoagulation Anticoagulation contraindicated or ineffective Low cardiopulmonary reserve Pulmonary embolism with hypotension (systolic blood pressure <90 mm Hg) AND Embolectomy (percutaneous or surgical) . . Low bleeding risk Shock likely to cause death within hours OR Failed thrombolysis (or thrombolysis contraindicated} with persistent hypotension le Thrombolysis Management options for pulmonary embolism .. . Submassive Massive . No RV dysfunction No hypotension (SBP ~90 mm Hg) RV dysfunction (dilation &/or hypokinesis) 0 . .. Typical management Hjgher risk· elevated biomarkers (troponin I &/or BNP) . Anticoagulation unless contraindicated rC Low risk Definition Individualized strategies ranging from anticoagulation to tailored (eg, catheter-directed) thrombolysis if higher risk No hypotension RV dysfunction . ne Classification irc Pulmonary embolism management Hypotension (SBP <90 mm Hg) Systemic thrombolysis &/or embolectomy In BNP = B-type natriuretic peptide; RV= right ventricle; SBP = systolic blood pressure. Acute pulmonary embolism PE may present with syncope due to right ventricular dysfunction, cardiac arrhythmia, or vasovagal response to PE. LE Can also present with mild atelactesis and bloody pleural effusion. U SM It may be difficult to differentiate PE from RV myocardial infarction MI, which can also cause RV dysfunction; however, RVMI is less likely to cause dyspnea or syncope and more likely to cause bradycardia or arrhythmias). Pulmonary 129 https://t.me/usmleinnercircle Version 2 Hampton hump (from pulmonary embolism) Chest CT scan showing a wedge-shaped infarction (red arrows) is virtually pathognomonic for PE Hamptonʼs Hump) Fleischner sign of pulmonary embolism Pulmonary 130 https://t.me/usmleinnercircle Version 2 ne rC irc le Westermark sign atelectasis, pleural effusion) In Sometimes, chest x-ray shows nonspecific findings that may be associated with PE (eg, LE Patients with PE commonly develop small pleural effusions due to hemorrhage or inflammation. The effusions tend to be exudative and grossly bloody, and they can be associated with pain due to pleural irritation. Leukocytosis and/or peripheral neutrophilia U SM may be seen, as with any acute inflammatory process. Acute pulmonary embolism PE is estimated to cause fever in approximately 15% of cases, possibly due to tissue necrosis in the setting of pulmonary infarction.  What ECG findings are classically associated with pulmonary embolism? "S1Q3T3" Or right heart strain  ST depression and T wave inversion in the right leads (aVR, II, V5 and V6. This is rarely seen (sinus tachy is the most common! "S1Q3T3" • prominent S in lead I • Q wave in lead III Pulmonary 131 https://t.me/usmleinnercircle Version 2 • T-wave inversion in lead III • indicative of R heart strain Common Symptoms: Acute-onset dyspnea, Pleuritic chest pain, Atrial fibrillation ( due to atrial dilation ) DVT  What is the diagnostic test of choice for a patient with moderate-high probability of DVT (modified Wells  2? Compression ultrasonography may use D-dimer in patients with low pretest probability; diagnosis of DVT should be confirmed before anticoagulation is started Modified Wells criteria for pretest probability of deep venous thrombosis Upper extremity deep venous thrombosis • Previously documented DVT • Active cancer Risk factors • Recent immobilization of the legs Score 1 point for each feature present • Recently bedridden >3 days • Localized tenderness along vein distribution • Swollen leg • Pitting edema • Collateral superficial nonvaricose veins • Alternate diagnosis more likely (-2 points) Total score for clinical probability Manifestations • Calf swelling >3 cm compared to other leg . Diagnosis Treatment • O points = Low probability .. .. .. . . .. Central venous catheters Repetitive arm motions (eg, baseball pitching) Weight lifting Malignancy Acute am, edema, heaviness, pain & erythema Dilated subcutaneous collateral veins in chest/upper extremity Pulmonary embolism Duplex or doppler ultrasonography 3 months of anticoagulation Thrombolysis (non--<:atheter-related) 1 or 2 points = Moderate probability • ~3 points = High probability OVT = deep venous thrombosis.  In Upper arm DVT, Catheter site is clean with swelling of arm. Patients who inject drugs into the femoral vein can develop DVT due to iliofemoral venous wall trauma, chemical irritation, and/or infection. Pulmonary 132 https://t.me/usmleinnercircle Version 2 Management of lower extremity proximal DVT Lower extremity proximal DVT (ie, above the knee) Limb-threatening DVT • Compartment syndrome • Phlegmasia cerulea dolens· Yes No • le Anticoagulation contraindicated No Yes * Thrombolysis or thrombectomy rC * IVC filter•• (retrievable prefe1Ted) irc • Active/difficull-to-treat major bleeding • Intracranial hemo1Thage Antlcoagulatlon • Systemic or catheter-directed thrombolysis • Percutaneous or surgical thrombectomy ne ·Large occlusiveiliofemoralOVT- venous limb ischemia& gangrene(cyanosis,bullae, massiveedema,extremepain). .. Lesser (relative) indications. dot propagationdespite anticoaguation, JJ cardiopulmonaryreserve (eg, impending right ventncularfailure). DVT = deep vein tlYombosis:IVC = inferiorvena cava. CUWOJld In Treatment of acute deep vein thrombosis/pulmonary embolism Oral factor Xa inhibitors Warfarin Direct factor Xa inhibition Vitamin K antagonism Therapeutic onset 2-4 hr 5-7 days Overlap needed? No Laboratory monitoring No Mechanism Yes, overlap with UFH or LMWH for -5 days PT/INR U SM LE of action LMWH = low molecular weight heparin; UFH = unfractionated heparin. IVC filters are inherently thrombogenic; although they halve the risk for recurrent pulmonary embolism, they double the risk for recurrent DVT. The increased DVT risk appears to be intrinsic to the filter itself, possibly from a combination of the thrombogenic mesh surface and stasis of venous flow.  What is the most common source of symptomatic pulmonary embolism? Proximal deep leg veins (e.g. femoral, popliteal, iliac) Pulmonary 133 https://t.me/usmleinnercircle Version 2 Distal veins (e.g. calf veins) are less likely to embolize What underlying cause of DVT must be ruled out in an older patient with their first episode of DVT and no history of immobilization, surgery, or provocative medications? Malignancy e.g. age-appropriate cancer screening (colonsocopy, mammogram) and CXR; more detailed testing may be indicated depending on the patient's symptoms Test for inherited causes (eg. protein c deficiency) if 45, multiple sites, or family history In a patient who develops a DVT as a result of a reversible or time-limited risk factor (eg, surgery, pregnancy, oral contraceptive use, or trauma), warfarin anticoagulation should be continued for a minimum of three months. Treatment for longer than six months, however, is not necessary. Air embolism Venous air embolism Etiologies Clinical manifestations Management .. • . • . Trauma, certain surgeries (eg, neurosurgical) Central venous catheter manipulation Barotrauma (eg, positive-pressure ventilation) Sudden-onset respiratory distress Hypoxemia, obstructive shock, cardiac arrest Left lateral decubitus positioning • High-flow or hyperbaric oxygen  A small VAE often causes minimal sequelae, traveling to the pulmonary capillaries where it can diffuse into the alveoli without consequence. However, a large VAE (eg, 50 mL can lodge in the right ventricle to cause right ventricular outflow tract obstruction or lodge within the pulmonary arterioles to obstruct pulmonary blood flow. Pulmonary 134 https://t.me/usmleinnercircle Version 2 Left lateral decubitus positioning for venous air embolism rC irc le Rightventricular outflowtract OUWood ne Traps the VAE on the lateral wall of the right ventricle, preventing RVOT obstruction  Manifestations of arterial air embolism include stroke and myocardial infarction. Patients with suspected arterial air embolism should be placed in supine positioning, as it helps In prevent the embolism from traveling to the brain and causing a stroke. LE Fat Embolism U SM Etiology .. .. .. Clinical presentation Diagnosis Prevention & treatment . .. Fat embolism syndrome Fractures of marrow-containing bones (eg, femur, pelvis) Orthopedic procedures Pancreatitis Sickle cell disease Onset usually 24-72 hr following inciting event Classic triad: 0 Respiratory distress (>90%): hypoxemia, dyspnea, tachypnea 0 Neurologic dysfunction (>50%): altered mentation, seizures 0 Petechial rash (<50%): head, trunk, subconjunctiva Based on clinical presentation Early fracture immobilization & fixation Supportive care ARDS Pulmonary 135 https://t.me/usmleinnercircle Version 2 Acute respiratory distress syndrome: pathogenesis & diagnosis Risk factors & • Direct (eg, pneumonia, inhalation) or indirect (eg, sepsis, pancreatitis, trauma) lung injury pathogenesis • Inflammatory cell activation & j permeability .... fluid & cytokine leakage into alveoli • t Lung compliance (alveolar flooding) --+ i work of breathing Pathophysiology • Severe V/Q mismatch (intrapulmonary shunt)--+ severe hypoxemia • i Hypoxic pulmonary vasoconstriction --+ i RV afterload & acute PHTN • New bilateral alveolar opacities within 1 week of inciting insult Diagnosis • Edema not explained by cardiac failure or volume overload • Hypoxemia with PaO2/FiO 2 S300 PHTN = pulmonary hypertension; RV= right ventricular; V/Q = ventilation/perfusion. Acute respiratory distress syndrome: management & prognosis • Lung protection: limit alveolar distending volume (VT 6 ml/kg) & pressure (Pplat S30 cm H2O) Mechanical ventilation • Ventilation: tolerate permissive hypercapnia (ie, j PaCO2 & t pH acceptable) to avoid excessive VT • Oxygenation: set lowest feasible FiO2 (goal SpO2 92%-96%) to avoid 0 2 toxicity • Treat underlying etiology: source control (eg, sepsis) Supportive care • Prevent iatrogenic harm: negative fluid balance, timely extubation (eg, minimize sedation) • ± Corticosteroids: select patients with moderate-to-severe early ARDS Prognosis • Mortality rate: 40% in hospital, death mostly due to multiorgan failure • Morbidity rate: 50% with chronic cognitive impairment & physical debility, 25% with chronic pulmonary dysfunction (restriction & t DLCO) ARDS = acute respiratory distress syndrome; DLCO = diffusion capacity of lung for carbon monoxide; Pplat = plateau pressure; SpO2 = oxygen saturation as measured by pulse oximetry; Vy= tidal volume. Pulmonary 136 https://t.me/usmleinnercircle Version 2 Acute respiratory distress syndrome: Initial ventilator management Intubation ) i Initial ventilator settings • Oxygenation: FiO2 = 100% & PEEP= 5 cm H2O • Ventilation: V, = 6 ml/kg IBW & RR= 14-18/min Adjust ventilation Pao, <60 mm Hg (hypoxia) LFiO2 tPEEP TTPaCO2 & pH <7.25 TRR tV, as last resort !Paco, & pH .?7.45 le Pao, >90 mm Hg (hyperoxia) !V, !RR tSedation as last resort irc Adjust oxygenation ] Ensure lung-protective ventilation (avoid alveolar overdistension) rC Evaluate lung compliance: measure Pplat with inspiratory hold• Goal Pplat S30 cm H2O: !V,&Jor adjust PEEP "Pause venhlator Mefly after hdal volume is delivered and measure pressure required to hold the lungs at distension on currentsettings. ne ABG = anenal blood gas; ET= endotracheal; FiO, = fraebon of inspired oxygen, IBW = ideal body weight; Pplat = plateau pressure; PEEP = positive end-expiratory pressure; RR = resplratoty rate; V, = tidal volume • Clinical indicator of hypoxemia In  What is the PaO2/FiO2 ratio? C)UWorld • Ratio of O2 tension (O2 in blood) to fraction of inspired O2 (fraction O2 in inspired air, usually .21 Normal vs. ARDS? Normal  300500, LE (21%) U SM 300  ARDS 100  severe ARDS • Mechanical ventilation in patients with ARDS should use low tidal volumes and high PEEP. In ARDS, a significant number of the alveoli remain collapsed despite positive airway pressure, and any tidal volume delivered by the ventilator is distributed to those remaining open (functional "baby lung"). LTV prevents overdistension of these alveoli and improves mortality PEEP  10 cm H2O • Prone positioning distributes the ventilation from the ventral to the dorsal (dependent) lung regions, where the majority of alveoli are located. This improves the homogeneity of ventilation throughout the lungs and decreases mortality in patients with ARDS Pulmonary 137 https://t.me/usmleinnercircle Version 2 • Fluid balance refers to the net sum of all fluid intake and output from the time of admission. Patients with ARDS often have an initial positive fluid balance (eg, volume resuscitation for sepsis, blood transfusion for trauma). During hospitalization, a conservative fluid strategy aimed at achieving a neutral or negative fluid balance accelerates recovery from ARDS, with a trend toward improved survival rate ("dry lungs = happy lungs"). This goal is accomplished by: • Minimizing intake: avoiding unnecessary fluid boluses, concentrating intravenous drips • Promoting removal: diuretics, renal replacement therapy Patients with refractory hypotension (eg, septic shock) should be assessed for fluid-responsiveness prior to blind volume loading (eg, via passive leg raise to simulate a fluid bolus). Vasopressors (eg, norepinephrine) may be required to support hemodynamics to permit volume removal. Lung Cancer  A lobectomy is the standard approach to surgical management of lung cancers and can be tolerated if the FEV1 1.5L and DLCO >60%. FEV1 and DLCO, obtained by preoperative pulmonary function testing, are the best predictors of postoperative outcomes following lung resection surgery. Pulmonary 138 https://t.me/usmleinnercircle Version 2 Adenocarcinoma 40%-50% Squamous cell carcinoma 20%-25% Small cell carcinoma 10%-15% Large cell carcinoma 5%-10% Location . .. . Peripheral Central Necrosis & cavitation • Central • Peripheral Clinical associations Assessment of malignancy risk for solitary pulmonary nodule Clubbing • Hypertrophic osteoarthropathy Variable Low risk Intermediate risk High risk Nodule size (cm) <0.8 0.8-2.0 ~2.0 • Hypercalcemia Age(yr) <40 40-60 >60 Smoking status Never smoked Current Current Smoking cessation (yr) >15 5-15 <5 Nodule margin characteristics Smooth Scalloped Corona radiate .. . Cushing syndrome SIADH Lambert-Eaton syndrome . • Gynecomastia Galactorrhea Solitary pulmonary nodule on routine chest x-ray Solif:cK)Ipulmonaryncxtule I Stable lesion over 2-3 years INo further testing I High malignancy risk Surgicalexcision No previous imaging or possible nodule growth Indeterminate or suspicious for malignancy ISerial CT scans I Further investigation with biopsy or PET Highly suspicious for malignancy ne l Benign features I Suspiciousfor maliglancy ISurgical excision I In lchestCT I~ Low to Intermediatemalignancy risk rC Previous chestx-ray or spiculated le Incidence irc Type of tumor Not suspicious for malignancy rr-··T Malignancyrisk Low I Surgical excision ,----Sen-.a-1 C-Tsc_an_s----,1 No follow up LE If a solid lesion revealed on prior imaging is stable in size for 2 years, malignancy is effectively ruled out and U SM no further testing is necessary Squamous cell carcinoma of the lung  Certain patterns of calcification within the pulmonary nodule are strongly suggestive of benign lesions, including popcorn, concentric or laminated, central, and diffuse Pulmonary 139 https://t.me/usmleinnercircle Version 2 homogeneous calcifications. Popcorn calcification is characteristically seen on radiographic imaging in patients with pulmonary hamartoma. • Eccentric calcification (area of asymmetric calcification), as well as reticular or punctate calcification, should raise suspicion for malignancy Pancoast Tumor Clinical presentation of Pancoast tumors • Shoulder pain (most common) • Homer syndrome (ipsilateral ptosis, miosis, enophthalmos & anhidrosis) from involvement of paravertebral sympathetic chain & inferior cervical ganglion • C8-T2 neurological involvement o Weakness &/or atrophy of intrinsic hand muscles o Pain & paresthesias of 4th & 5th digits, medial arm & forearm • Supraclavicular lymph node enlargement • Weight loss Lung Carcinoid Tumor Bronchial carcinoid tumor Epidemiology Manifestations • Most common lung cancer in adolescents/young adults • Neuroendocrine tumor derived from bronchial Kulchitsky cells • Proximal airway obstruction (eg, dyspnea, wheezing, cough) • Recurrent pneumonia distal to obstruction • Hemoptysis • Carcinoid syndrome less common than with midgut carcinoid Diagnosis • Chest imaging: contrast enhanced (vascular) tumor with endobronchial component • Bronchoscopy with biopsy Head and Neck Cancer  What is the best initial test in a patient found to have a cervical lymph node with metastatic squamous cell carcinoma? Panendoscopy (esophagoscopy, bronchoscopy, laryngoscopy) helps detect the primary tumor, which can then be biopsied to determine further management Pulmonary 140 https://t.me/usmleinnercircle Version 2 Referred otalgia __ Referred pain felt in externalauditorycanal - Vagus nerve (CN X) rC irc Posteriorpharyngeal walltumor le Glossopharyngeal nerve (CN IX) Head and neck cancer can present with Cervical LN enlargement or Referred Otalgia ne  An enlarged, ulcerated tonsil with ipsilateral cervical adenopathy is likely oropharyngeal (head and neck) squamous cell carcinoma. In Human papillomavirus is the likely etiology in the absence of traditional risk factors (smoking, alcohol) in younger patients.  A laryngeal ulcer in a smoker is likely squamous cell carcinoma. LE Persistent hoarseness should always be evaluated by laryngoscopy to ensure no delay in diagnosis of possible cancer. U SM Nasopharyngeal Carcinoma Epidemiology Manifestations Diagnosis Treatment .. . .. . . .. Nasopharyngeal carcinoma Endemic to Asia Linked with Epstein-Barr virus reactivation Risk factors: Diet (salty fish), smoking, genetics Obstruction: Nasal congestion, epistaxis, headache Mass effect: Cranial nerve palsy, otitis media Spread: Neck mass (cervical lymphadenopathy) Endoscope-guided biopsy Radiation therapy Chemotherapy Oral Leukoplakia Pulmonary 141 https://t.me/usmleinnercircle Version 2 Risk factors Clinical features Increased risk of cancer Management . .. .. . .. .. Oral leukoplakia Tobacco & alcohol use Painless white mucosal patch Cannot be wiped off Nonhomogeneous gross appearance Large size (>4 cm) Dysplasia seen on biopsy Biopsy (at diagnosis & if appearance changes) Risk factor modification (eg, tobacco cessation) Close monitoring ± Surgical excision  D/D Oral hairy leukoplakia typically presents as multiple white lesions on the lateral tongue with a distinct corrugated appearance which cannot be scrapped off. Caused by EBV Because it occurs almost exclusively in patients with significant immunodeficiency, HIV testing should be performed, especially in patients with signs of systemic illness. NOT premalignant and can occur in young people (vs Oral Leukoplakia) Miscellaneous Chronic Cough Common etiologies of chronic cough Upper airway disorders Lower airway & parenchymal disorders Other causes .. .. .. .. .. Approach to chronic cough in children Upper airway cough syndrome (postnasal drip) Chronic sinusitis Chronic cough• (>4 weeks) Asthma Post-respiratory tract infection Chronic bronchitis Bronchiectasis Lung cancer Nonasthmatic eosinophilic bronchitis Spirometry Evidence of~ airway obstruction Gastroesophageal reflux ACE inhibitors +Yes Improvement with trial of SABA+ ICS No+ Chest x-ray Abnonmal Treat for asthma Treat underlying diagnosis Normal Watch &wait •Focal examination findings or specific cough reatures may guide alternate approaches, such as paroxysmal (pertussis), suppressible (habtt), choking/sudden onset (foreign body), red nags (tuberculosis. immunodeficiency). C)Ull'lorld IC$ = inhaled corticosteroid; SABA = shOrt-aCbngbeta agonisl Pulmonary 142 https://t.me/usmleinnercircle Version 2 Evaluation of subacute (3-8 weeks) or chronic (>8 weeks) cough Suspected etiology on H & P I Evaluate& treat asindicated Stop ACE UACS First-generation Hlblodcer AsthmaPFTs GERDEmpiric PPI • Parenchymaldisease • Purulent sputum/ tmmunocompromised • Nospeciftcetiology ACEinhibitors- le No improvement after intervention Chest x-ray GERO= gastroesophageal reflux disease; H & P"" history& physical; PFTs"" pulmonaryfunction tests; PPI"" proton pump inhibitor; irc UACS = upper airway coughsyndrome.  What is the next step in management for a young patient who presents with chronic nocturnal dry cough with a sensation of liquid dripping into the back of the throat? rC Oral first-generation H1 blocker Hyperventilation Syndrome ne this patient likely has upper-airway cough syndrome (post-nasal drip) and should be treated empirically In Hyperventilation syndrome, a diagnosis of exclusion characterized by intermittent episodes of hyperventilation without any obvious cardiac or pulmonary etiology. Neurologic symptoms, including paresthesias, headache, lightheadedness, and carpopedal spasms, are often present and may be related to cerebral vasoconstriction or alkalosis-induced hypocalcemia LE and hypophosphatemia. U SM If the episode does not improve with breathing retraining, a small dose (not a high dose) of a shortacting benzodiazepine (eg, lorazepam) is appropriate second-line therapy. Breathing into a paper bag was previously recommended to improve hypocarbia by rebreathing CO2; however, this can also result in hypoxia and therefore should not be done Decompression Sickness Pulmonary 143 https://t.me/usmleinnercircle Version 2 Pathophysiology Risk factors Presentation Treatment . .. . .. .. Decompression sickness Abrupt i in ambient pressure causes formation of nitrogen gas bubbles within the body Rapid ascent to surface following deep dive Obesity (nitrogen is fat soluble), male sex Air travel soon after diving (may further reduce ambient pressure) Type 1 (mild illness): musculoskeletal (the bends), cutaneous & lymphatic Type 2 (severe): neurologic (the staggers) & pulmonary (the chokes) Intravenous fluids & 100% oxygen Hyperbaric oxygen therapy as soon as possible  Symptoms usually begin within 12 hours of surfacing.  Small air bubbles in the venous bloodstream can lodge in the capillaries of the skin to cause pruritus or mottling and cyanosis of the extremities. The air bubbles can also lodge in the pulmonary capillaries to cause respiratory distress and localized ischemia, with resulting pulmonary edema. A relatively large volume of coalesced air (eg, 50 mL can lodge in the right ventricular outflow tract and cause obstructive shock.  Air can also pass into the arterial circulation by overwhelming the pulmonary capillary filtering capacity or via a right-to-left-shunt (eg, patent foramen ovale). Small air bubbles in the arterial circulation can travel to the brain to cause confusion, gait ataxia, and dysarthria. A small volume (eg, 12 mL of coalesced air can cause localized stroke (eg, left arm weakness) or myocardial infarction. • Emergency treatment: IV hydration, Trendelenburg positioning, administration of 100% oxygen. Optimal management is with hyperbaric oxygen therapy. High Altitude Sickness Pathogenesis Complications Treatment . . . . .. . High-altitude illness Reduced PiO2 at high altitude (>2,500 m [-8,000 ft]) AMS 0 Headache, fatigue, nausea HAGE 0 i PaO2 ---+ j cerebral blood flow 0 Lethargy, confusion, ataxia HAPE 0 Uneven hypoxic vasoconstriction 0 Dyspnea, cough± hemoptysis, respiratory distress Supplemental oxygen Acetazolamide for AMS, dexamethasone for HAGE Descent to lower altitude (definitive treatment for all HAI) AMS = acutemountainsickness;HACE = high-altitudecerebraledema;HAI = high-altitudeillness;HAPE = high-altitudepulmonaryedema;PI02 = partialpressureof inspiredoxygen. Diffuse Alveolar Hemorrhage Pulmonary 144 https://t.me/usmleinnercircle Version 2 Etiology & pathogenesis Clinical presentation & diagnosis Management .. . .. . .. Diffuse alveolar hemorrhage Pulmonary capillaritis: ANCA vasculitis, SLE, antiphospholipid antibodies Bland hemorrhage: mitral stenosis, anticoagulation Alveolar damage: viral pneumonitis, ARDS, drug-induced (eg, cocaine, amiodarone) Dyspnea, hypoxemia, hemoptysis (absent in ~50%) & blood loss anemia CXR or CT: diffuse ground-glass opacities Bronchoscopy: progressive blood on serial lavage Treat underlying (eg, rheumatologic, infectious) cause Supportive care: oxygen, mechanical ventilation; avoid anticoagulation Diffuse airspace opacification Blood Others • Exudative edema: noncardiogenic (ARDS) • Neutrophils (pus): infectious pneumonia • Lymphocytes & macrophages: hypersensitivity pneumonitis • Eosinophils: acute eosinophilic pneumonia • Malignant cells: carcinomatosis • Diffuse alveolar hemorrhage rC Cells • Transudative edema: cardiogenic • Aspirated nonalveolar hemorrhage • Fat: lipoid pneumonia (eg, vaping oils) • Protein: pulmonary alveolar proteinosis ne Fluid irc Definition: Alveolar filling processes that can involve multiple lobes le ANCA = antineutrophilcytoplasmicantibody; ARDS = acute respiratorydistress syndrome; CXR = chest x-ray; SLE = systemic lupus erythematosus. Pulmonary Paeds Choanal atresia Unilateral(most common) 0 Chronic nasal discharge 0 Symptomaticduring childhood Bilateral 0 Cyanosisthat worsenswith feeding & improveswith crying 0 Noisy breathing(stertor) 0 Symptomaticshortly after birth May be associatedwith CHARGEsyndrome U SM . . LE Choanal Atresia In ARDS= acute respiratory distress syndrome. Clinicalfindings Diagnosis Treatment . .. .. Inabilityto pass catheterpast nasopharynx Confirmationwith CT scan or nasal endoscopy Oral airway Surgicalrepair CHARGE= Coloboma, Heart defects, Atresia choanae, growth Retardation, Genital and Ear abnormalities. coloboma [missing eye tissue] Pulmonary 145 https://t.me/usmleinnercircle Version 2 Choanal atresia Bonyor membranous obstruction Normal anatomy Choanal atresia ll>UWond  What is the likely diagnosis in a generally healthy newborn that presents with cyanosis that worsens with feeding and is relieved with crying despite a normal cardiac and respiratory exam? Choanal atresia failure to pass a catheter through the nose into the oropharynx is suggestive of this diagnosis; CT scan confirms the diagnosis Because neonates are obligate nasal breathers (ie, preferentially breathe through the nose), the complete obstruction of bilateral choanal atresia causes intermittent cyanosis, even at rest CHARGE Syndrome CHARGE syndrome Characteristic features Additional key findings Diagnosis .. .. .. .. . .. Qoloboma !::!eart defects (eg, TOF, VSD) t,tresia choanae Retardation of growth/development §enitourinary anomalies J;:ar abnormalities (eg, hearing loss) Anosmia Cleft lip/palate Hypotonia Clinical CHD7 gene testing TOF = tetralogy of Fallot; VSD = ventricular septal defect. Foreign Body Aspiration Pulmonary 146 https://t.me/usmleinnercircle Version 2 Foreign body aspiration • ± History of choking event with sudden-onset cough • Symptoms depend on location Clinical features o Trachea/main bronchus: acute respiratory distress, cyanosis, stridor (trachea), o Lower airway: chronic/recurrent cough hemoptysis (bronchial) Examination findings • Focal, unilateral, and/or monophonic wheeze • Focal area of diminished breath sounds • Hyperinflation of affected side± mediastinal shift toward unaffected side X-ray findings • Atelectasis if complete obstruction • ± Foreign body • Bronchiectasis • Bronchoscopic removal irc Management • Recurrent pneumonia le Complications Wheezing and decreased breath sounds on the affected side are characteristic, rC and hyperresonance to percussion can occur over the hyperexpanded lung.  What is the next step in management for a child in respiratory distress due to foreign body aspiration? ne Bronchoscopy characterized by sudden-onset respiratory distress; most aspirated foreign bodies end up in In the right mainstem bronchus Normal radiograph does not rule out FB aspiration because at least 30% of radiographs are normal, particularly if a radiolucent FB (eg, food) is lodged in a small airway. LE Therefore, if clinical suspicion remains high, bronchoscopy is indicated to confirm the diagnosis and remove the aspirated object. U SM Sometimes, CT Scan can also be used if Xray is normal in asymptomatic child. Nasal Foreign Body Clinical manifestations Treatment Complications Pulmonary .. . .. .. . . Nasal foreign body Inorganic substance (eg, toy): mild pain/discomfort Organic substance (eg, food): unilateral, foul-smelling, purulent, bloody discharge Button battery: epistaxis, purulent or black discharge Positive pressure expulsion (eg, forceful exhalation with unaffected naris occluded) Mechanical extraction Local irritation Infection (eg, sinusitis) Aspiration Nasal septa! perforation (eg, button battery, multiple magnets) 147 https://t.me/usmleinnercircle Version 2 NRDS Management of neonatal respiratory distress syndrome Neonatewith respiratorydistress syndrome l Apnea or gasping? i Yes t Bag valve mask ventilation j l Continuedapnea ]--No-. I Noninvasivepositive airway pressure (eg CPAP") Consider surfactant Yes t Intubate Chest compressions Consider surfactant ·CPAP = oontmuous posibveairway pressure CIUWorld What is the treatment? • Nasal CPAP with PEEP of 38 cm H2O prevents collapse) • Administration of artificial surfactant within 2 hours postpartum. DDx: Transient tachypnea of the newborn (wet lung disease) • Full-term neonates born by cesarean section (lungs full of fluid) • Therapy should focus on supportive care Common causes of neonatal respiratory distress Diagnosis Pathophysiology Transient tachypnea Respiratory distress Persistent pulmonary of the newborn syndrome hypertension • Inadequate alveolar fluid clearance at birth • Tachypnea shortly after Clinical features birth • Bilateral, perihilar linear • Prematurity & cyanosis • Diffuse, ground-glass appearance with low lung streaking • Fluid within interlobar fissures atelectasis • Severe respiratory distress • Resolves by day 2 of life Chest x-ray • Surfactant deficiency • Alveolar collapse & diffuse volumes • Air bronchograms • High pulmonary vascular resistance • Right-to-left shunt • Tachypnea & severe cyanosis • Clear lungs with decreased pulmonary vascularity • Meconium Aspiration Syndrome: • Post-term neonates with meconium aspiration • Unresponsive neonate and green amniotic fluid establish diagnosis Pulmonary 148 https://t.me/usmleinnercircle Version 2 • Emergency intubation Others: Congenital Diaphragmatic hernia, Pneumothorax, Neonatal pneumonia, Sepsis, Lung hypoplasia D/D for Early Differential Cyanosis: le 1. PDA with RDS 2. Persistent Pulmonary Hypertension irc  What is the next step in management for a newborn with respiratory distress and hypoxia secondary to a suspected congenital diaphragmatic hernia? Endotracheal intubation after to decompress the stomach and bowel rC ABCs take precedence over diagnostic studies; a gastric tube should be placed immediately This is likely due to intrauterine hypoxia. ne  Infant 2 hours after birth has elevated hematocrit 65%, respiratory distress, hypoglycemia, cyanosis, and plethora. In Risk factors? Smoking, maternal diabetes, small or large for gestational age Bronchopulmonary dysplasia . • . . Clinical diagnosis U SM Chest x-ray Treatment Complications Premature arrest of pulmonary development • Alveolar hypoplasia with t septation LE Pathogenesis Impaired vasculogenesis Premature infant with continued supplemental oxygen requirement ~28 days from birth* Mild: diffuse hazy infiltrates, low/normal lung volumes • Severe: fibroclstic chan!les, hleerinflation . . • Supportive (eg, oxygen, nutrition, fluid restriction/diuretics) Pulmonary artery hypertension • Cardiovascular disease (eg, hypertension) Recurrent respiratory infections •some definitionsincludean oxygen requirementat 36 weeks postmenstrualage (ie, gestational+ chronologicage). Pulmonary 149 https://t.me/usmleinnercircle Version 2 Transient tachypnea of the newborn Prominent interlobar fissure Respiratory distress syndrome Diffuse alveolar collapse (atelectasis) due to surfactant deficiency C)UWo,ld Persistent Pulmonary Hypertension of Newborn Persistent pulmonary hypertension of the newborn • Abnormal persistence of elevated fetal pulmonary vascular Pathogenesis Risk factors Examination Treatment . .. . .. . .. resistance Right-to-left shunting across ductus arteriosus Lung hypoplasia (eg, congenital diaphragmatic hernia) Meconium aspiration syndrome Infection (eg, neonatal pneumonia) ! Postductal relative to preductal oxygen saturation Respiratory distress & cyanosis Prominent S2 Oxygenation & ventilation Inhaled nitric oxide (pulmonary vasodilator) OSA Pulmonary 150 https://t.me/usmleinnercircle Version 2 Pediatric obstructive sleep apnea Pathophysiology • Adenotonsillar hypertrophy • Night symptoms Clinical manifestations o Loud snoring, pauses in breathing, gasping o Enuresis, parasomnias (eg, sleepwalking, sleep terrors) • Day symptoms o Inappropriate naps or falling asleep during school o Irritability, inattention, learning problems, behavioral problems o Mouth breathing, nasal speech • Poor growth (ie, failure to thrive) Complications • Poor school performance Management • Tonsillectomy & adenoidectomy le • Cardiopulmonary (eg, hypertension, structural heart changes) irc  The mechanism of enuresis is unclear, but it may be related to the elevated levels of B-type Breath holding spells rC natriuretic peptide seen with OSA, possibly due to intermittent obstruction causing increased cardiac volume and pressure. Breath-holding spell (BHS) vs seizure Clinical t Risk with iron deficiency anemia Crying/frustration Apnea & cyanosis --+ LOC Rapid return to baseline .. . . .. .. .. Minortrauma Pain or fear Bradycardia, apnea & pallor --+ LOC ± Brief (<5 min) confusion or sleepiness Any age f Risk with history of febrile seizure or developmental delay Often unprovoked Sleep deprivation LOC --+ tonic-clonic movements Prolonged (>5 min) postictal confusion LE features . . . Age 6 months to 2 years Seizure ne Triggers .. Pallid BHS In Epidemiology Cyanotic BHS LOC :;;;loss of consciousness. U SM  What is the next step in management for an infant that experienced a breath-holding spell? Reassurance; no further testing breath-holding spells are considered normal development  Resolution by 5 Years age) screening for associated IDA is recommended because iron-deficient patients typically experience improvement in BHS frequency with iron therapy. • No murmur and very brief cyanosis (vs TOF which has left upper sternal murmur and improvement in cyanosis with squatting) Sudden Infant Death Syndrome Pulmonary 151 https://t.me/usmleinnercircle Version 2 . .. .. Risk factors for sudden infant death syndrome Maternal/antenatal Infant . Substance use (eg, cigarettes, alcohol, recreational drugs) Maternal age <20 Inconsistent prenatal care Prematurity or low birth weight Sleep environment 0 Prone-/side-sleep position 0 Soft sleep surface, loose bedding 0 Bed sharing Smoke exposure • Impaired cardiovascular reflexes (eg, increased heart rate due to hypercarbia) and diminished arousal responses may account for elevated risk with Smoke Exposure. Sudden infant death syndrome is the leading cause of mortality in infants age 1 month to 1 year in the United States Drowning Drowning injuries . • Children age <5 & males age 15-25 Risk factors Inability to swim &/or inadequate supervision • Concomitant drug/alcohol use • Acute respiratory distress syndrome ..~.,._l • Cerebral edema c,,,...._,. • Arrh~hmia I Complications . Poor prognostic indicators Submersion time >5 min • Delay in initiation of cardiopulmonary resuscitation • Prolonged resuscitative efforts • Age >14 • Arterial blood pH <7.1 A patient with purposeful movements demonstrates good functional neurologic ability and indicates a good prognosis. (basically shows less hypoxic damage to brain) Pulmonary 152 https://t.me/usmleinnercircle Version 2 Management of drowning Acute interventions • Administer rescue breaths first, then chest compressionsfor cardiacarrest • Remove wet clothing to improve hypothermia • Transport to emergency department ! Emergency department care Asymptomatic ! Observe (.:8 hours) Continuous cardiopulmonary monitoring Supportive care as needed (eg, supplemental oxygen, bronchodilators, NIV, intubation) Evaluate • CXR • ECG • ABG, CBC, electrolytes • Drug screen (in adolescents/adults) Continuous cardiopulmonary monitoring • Monitor for signs of ARDS (eg, dyspnea, wheeze) Evaluate CXR at end of observation (to assess for pulmonary edema) ± ABG, CBC, electrolytes, drug screen rC Maintain oxygenation & ventilation irc ! le Symptomatic OUW><td ne ABG = arterialbloodgasanaly~s;CBC = completebloodcount;CXR = Chestx-ray;NIV = noninvasive venblation  Even if the fluid is coughed out quickly and normal ventilation is restored, the fluid may have already caused damage capable of resulting in delayed pulmonary complications. This damage may include: In • Direct tissue injury from chemicals or contaminants in the fluid, leading to inflammation • Washout of alveolar surfactant, leading to alveolar collapse permeability LE • Disruption of the osmotic gradient of the alveolar-capillary membrane, leading to increased fluid U SM Together, these insults can progressively impair oxygen exchange and cause atelectasis, decreased lung compliance, and non-cardiogenic pulmonary edema. Pulmonary Surgery  In all patients anticipating elective surgery, immediate smoking cessation is recommended as those who quit smoking 48 weeks prior to surgery substantially decrease their postoperative pulmonary risk. Lesser durations of preoperative smoking cessation do not reduce the risk, likely because the airway inflammation and increased bronchial mucus production induced by smoking requires some time to improve. Pulmonary 153 https://t.me/usmleinnercircle Version 2 Postoperative pulmonary complications Complications Risk factors Perioperative prevention • Atelectasis, bronchospasm, pneumonia • Prolonged ventilator requirement • Age >50, active smoking • Underlying heart failure or obstructive lung disease • Emergency surgery or surgery duration >3 hr • Smoking cessation >4-8 weeks prior to surgery • Symptomatic control of underlying lung disease • Pain control, deep-breathing exercises, incentive spirometry  Patients with the following underlying medical conditions are at the greatest risk for developing PPC • COPD • Cigarette smoking • Sleep apnea • Heart failure Prior to undergoing an elective procedure, these conditions should be optimized. This typically includes smoking cessation (ideally 4 weeks prior to the procedure) and the treatment of any heart failure or COPD exacerbation. Uncontrolled postoperative pain often presents with patient discomfort, tachycardia, tachypnea, hypertension, and respiratory splinting, particularly for thoracic and upper abdominal incisions. Adequate pain control following a surgical procedure is necessary to decrease the risk of postoperative complications, such as pneumonia. Patient-controlled analgesia is a frequently used option to provide opioid-based pain relief to patients after surgery. Pulmonary 154 https://t.me/usmleinnercircle Version 2 Common causes of postoperative hypoxemia Approximate time after Airway .. .. Immediate obstruction/edema Residual anesthetic Immediate effect Bronchospasm Typically early Pneumonia 1-5 days Atelectasis 2-5 days Pulmonary embolism . .. .. .. Stridor is common Often due to endotracheal intubation or pharyngeal muscle laxity Anesthetic agents, benzodiazepines, opiates Diminished respiratory drive (decreased respiratory rate or tidal volume) Wheezing Fever, elevated white blood cell count, purulent secretions Infiltrate on chest x-ray Thoracoabdominal surgeries Splinting, reduced cough, retained secretions Chest pain, tachycardia Little improvement on supplemental oxygen rC b~f.lm: J diill:i (thromboembolic or fat) Features le surgery irc Diagnosis Post-operative Atelectasis ne • Atelectasis commonly occurs post-operatively due to shallow breathing and weak cough secondary to pain. Typically manifest on post-operative day 2 and 3 In shallow breathing causes hypoxia with resultant tachypnea and low CO2 (respiratory alkalosis)  Arterial blood gas results typically reveal an increased alveolar-arterial gradient due to intrapulmonary shunting. LE Chest x-ray characteristically shows linear opacifications in the bilateral lung bases, sometimes with an accompanying shift of structures toward the opacification (if the atelectasis is large).  What breathing instrument is useful for preventing post-operative atelectasis? U SM Incentive spirometer An incentive spirometer is a device that teaches patients how to take slow, deep breaths, which opens up airways and prevents collapse Pulmonary 155 https://t.me/usmleinnercircle Version 2 Once atelectasis develops, it can be treated with continuous positive airway pressure CPAP to help open collapsed alveoli  For patients with minimal respiratory secretions, the administration of continuous positive airway pressure CPAP is often effective. However, those with more copious secretions (such as a patient with cough productive of white sputum) are most appropriately managed with aggressive pulmonary hygiene, including chest physiotherapy and suctioning, rather than CPAP. CPAP may stent the airways open, but if this is not sufficient to promote ventilation, escalation to BiPAP or invasive positive pressure ventilation may be required. Incentive Spirometry also helps to prevent Post op Pneumonia Flail Chest Flail chest Pathophysiology • ~3 contiguous ribs fractured in ~2 locations - flail chest segment Findings • Paradoxical chest wall motion with respiration • Chest pain, tachypnea, rapid shallow breaths • CXR: Rib fractures +/- contusion/hemothorax Management • Pain control, supplemental oxygen • PPV (+/- chest tube) if respiratory failure CXR = chestx-ray; PPV = positivepressureventilation. Flail chest 10UWo<ld  How does the flail chest segment move with inspiration? Moves inward (paradoxical chest wall motion) due to negative intrathoracic pressure Pulmonary 156 https://t.me/usmleinnercircle Version 2  What is the most essential part of management for an uncomplicated rib fracture (e.g. no hypotension, pneumothorax, etc.? Pain control essential to maintain deep breathing and adequate cough, which helps prevent atelectasis and pneumonia  The extreme blunt force required to create flail chest typically injures the underlying lung resulting in le pulmonary contusion (eg, seen on x-ray as infiltrates underlying patient's rib fractures) decreases oxygen diffusion (due to alveolar hemorrhage and edema). As a result, patients must breathe harder to maintain oxygenation. The combination of increased irc work of breathing and decreased oxygenation causes many patients to fatigue and develop respiratory failure, requiring mechanical ventilation. rC Blunt Chest Trauma Blunt chest trauma Hemodynamically unstable Hemodynamically stable -----Yes----- ! High-risk mechanism or serious injury on examination In +/- stabilizing intervention (eg, chest tube) if indicated ne ! Resuscitation and evaluation eFAST • Chest x-ray • ECG I No l Hemodynamic stability achieved/maintained? Abnormal findings on evaluation, chest x-ray, ECG U SM LE ! I I No No l l OR thoracotomy Additional tests (eg, CT chest) Possible discharge or observation eFAST = extended Focused Assessment with Sonography for Trauma; OR= operating room. OUWorld Hemothorax  What is the underlying cause of hemorrhagic shock in a patient with decreased breath sounds, tracheal deviation, and dullness to percussion? Hemothorax each hemithorax is capable of holding up to 50% of circulating blood volume! Pulmonary 157 https://t.me/usmleinnercircle Version 2  Life-threatening hemoptysis (eg, jeopardized airway, severe hypoxemia, hemodynamic instability) generally occurs with bleeding rates 100 mL/hour. It is managed by securing the airway, placing the patient's bleeding "bad lung" (if lateralization is known) down to prevent spillover into the "good lung," and obtaining urgent hemostatic control via bronchoscopy, embolization, or surgery.  What is the treatment of a hemothorax? 1. Chest tube insertion (thoracostomy) 2. Thoracotomy indicated if 1500 mL or 200 mL/hr for 2 hours or Continuous need for transfusion to maintain hemodynamic stability. A hemothorax, however small, must always be drained because blood in the pleural cavity will clot if not evacuated, resulting in a trapped lung or an empyema! Tube thoracostomy, or chest tube insertion, involves placing a hollow plastic tube between the 4th or 5th intercostal space at the midaxillary line into the chest to decompress a hemothorax and/or pneumothorax. Rib fractures with hemothorax Bleeding from torn intercostal vessels and lung parenchyma CUWorld  May result from injuries to large (eg, aorta, hilar vessels) or small intrathoracic structures (eg, intercostal blood vessels, lung parenchyma) Tracheobronchial Injury Pulmonary 158 https://t.me/usmleinnercircle Version 2 Tracheobronchial injury (TBI) • Clinical features • Dyspnea, hoarseness, dysphonia, bloody tracheal secretions • Subcutaneous emphysema • Treatment-resistant pneumothorax (not a tension pneumothorax!) • Pneumomediastinum (air in the mediastinum): Hamman's sign c;;::J • Diagnostics • Chest x-ray: air in surrounding soft tissue • Bronchoscopy: visualization of the lesion • TBI vs tension-pneumothorax: TBI usually does not feature midline shift and distended neck veins. • Complications: chylothorax, chylopericardium, chylomediastinum • Treatment: mostly surgical repair le • Persistent pneumothorax and significant air leak following chest tube placement in a patient with blunt chest trauma suggests tracheobronchial rupture. irc other findings include pneumomediastinum and subcutaneous emphysema Definitive Dx: Bronchoscopy rC T/t: Surgical Repair Pulmonary Contusion Clinical features Management • Present <24 hours after blunt thoracic trauma • Tachypnea, tachycardia, hypoxia • Rales or decreased breath sounds • CT scan (most sensitive) or CXR with patchy, alveolar infiltrate not restricted by anatomical borders . Pain control In Diagnosis ne Pulmonary contusion • Pulmonary hygiene (eg, incentive spirometry, chest PT) • Supplemental oxygen & ventilatory support Pulmonary contusion U SM LE CXR ; chest x-ray; PT ; physiotherapy. Pulmonary 159 https://t.me/usmleinnercircle Version 2 • Pulmonary contusion may present with noncardiogenic pulmonary edema. Intiail CXR may be negative. The trauma can produce leaky capillaries, leading to dyspnea and a non-cardiogenic pulmonary edema, which is characterized by a normal PCWP. Diaphragmatic Rupture  What imaging modality can confirm the diagnosis of diaphragmatic rupture in stable patients with suggestive X-ray findings? CT scan (chest, abdomen) X-ray can be done initially and CT scan confirms the diagnosis. Most patients require surgery. Diaphragmatic rupture CUWorld Look for NG tube in the pulmonary cavity-other signs include deviation of mediastinal contents to opposite side and elevation of the hemidiaphragm  Some patients (especially children) with traumatic diaphragmatic injury may initially have no symptoms and can present months to years later after progressive expansion of the diaphragmatic defect. Diaphragmatic Paralysis Pulmonary 160 https://t.me/usmleinnercircle Version 2 Etiology Clinical features Diagnosis .. .. .. . . Unilateral diaphragmatic paralysis Phrenic nerve injury (eg, cardiac surgery, trauma, radiation therapy, compressive tumor) Viral infection (eg, herpes zoster, poliomyelitis) Systemic neurologic disease (eg, ALS, GBS) Idiopathic Typically asymptomatic at rest Dyspnea on exertion Orthopnea Fluoroscopic "sniff" test (1:>aradoxicalmovement of the diaphragm seen during Q[i~~ le ALS = amyotrophic lateral sclerosis; GBS = Guillain-Barre syndrome. irc  One helpful clue on physical examination suggestive of diaphragmatic paralysis is paradoxical abdominal wall retraction during inspiration when the patient is lying supine, which occurs because U SM LE In ne rC the diaphragm is not contracting. Pulmonary 161 https://t.me/usmleinnercircle Version 2 CVS Hypertension Infective Endocarditis Rheumatic Heart Disease Cholesterol Aorta Aortic Trauma Aortic Dissection Aortic Aneurysm Coarctation of Aorta Aortoiliac Occlusion Leriche Syndrome) Carotid Artery Carotid Artery Dissection MI Post MI Complications Angina Stable Angina CAD/ ACS Diagnosis Stress Test Perfusion Test Murmur & Valvular Defects Mitral Regurgitation Mitral Stenosis Aortic Regurgitation Aortic Stenosis Biscuspid Aortic Valve Pulmonary Regurgitation Pulmonary Stenosis ECG Tracings PSVT Atrial Fibrillation PAC MAT WPW Ventricular PVC Monomorphic Ventricular Tachycardia Torsade de Pointes Bradycardia Tachyarrythemia and Cardiac Arrest Heart Block 1st Degree CVS 162 https://t.me/usmleinnercircle Version 2 2nd Degree Complete Heart Block Sick Sinus Syndrome Heart Failure Systolic failure Diastolic Failure Cor Pulmonale High Output Heart Failure Cardiomyopathy Peripartum Cardiomyopathy le Takotsubo cardiomyopathy HOCM Carditis irc Acute Pericarditis Constrictive Pericarditis Myocarditis Cardiac Tamponade rC Peripheral Vascular Diseases Venous Insufficiency Acute Limb Ischemia Shock ne Anaphylactic Hypovolemic & Hemorrhagic Shock Fluid Replacement Obstructive Shock In CVS Drugs Anti-Arrhythmic Miscellaneous Syncope Pulsus Paradoxus Stent JVP U SM Prosthetic Valve LE Anomalous Aortic Origin of a Coronary Artery Prosthetic Valve Thrombosis Hereditary Hemorrhagic Telangiectasia Exercise Induced Postural Hypotension Paeds CVS VSD Tricuspid Atresia Persistent Pulmonary Hypertension of the Newborn Hypoplastic Left Heart Syndrome CVS Surgery CABG Postpericardiotomy Syndrome Sternal Dehiscence Preoperative Cardiac Other Tests CVS 163 https://t.me/usmleinnercircle Version 2 Vascular Trauma Blunt Cardiac Injury Myocardial Contusion Hypertension In the absence of end-organ damage, the diagnosis of hypertension must be confirmed by one of the following: • Ambulatory BP readings, as measured by an automatic device worn continuously by the patient for 2448 hours. The device monitors BP at regular intervals (eg, 1560 min). • If continuous ambulatory BP monitoring is not available, an acceptable alternative is home BP monitoring done twice a day (morning and evening) for a week. • If home BP monitoring is not possible, 3 office readings (preferably by an automated machine while the patient is alone) at least a week apart are needed. Determinants of blood pressure Systolic blood pressure= DBP + p, ure) ai 120 I E .§. [ i Pulse pressure Increases with t SV or l aortic compliance 100 80 Time Diastolic blood pressure (DBP) Increases with t SVR or t arterial blood volume SV • stroke volume: SVR • systemic vascular resistance. Choice of antihypertensive drug for comorbid conditions Laboratory evaluation of hypertension • Serum electrolytes (Na, K, Ca) Renal function tests • Serum creatinine Coronary • Urinalysis atherosclerosis • Urine albumin/creatinine ratio (optional) • Fasting glucose or hemoglobin A 1c Endocrine tests ejection fraction • TSH Cardiac tests Other tests Heart failure with reduced • Lipid profile Atrial fibrillation • ECG or flutter • Echocardiography (optional) Chronic kidney • Complete blood count disease • Uric acid (optional) Gout Urinalysis: for occult hematuria and urine protein/creatinine ratio Osteoeorosis Migraine .. . . . . . . Angina pectoris: @-blocker,CCB Post-myocardial infarction: ACE inhibitor or ARB, P-blocker ACE inhibitor or ARB, P-blocker, diuretic, aldosterone antagonist P-Blocker, nondihydropyridine CCB ACE inhibitor or ARB Losartan, other ARB, CCB (avoid diuretics) Thiazide diuretic P-Blocker, CCB ARB:;;;angiotensin II receptor blocker; CCB = calcium channel blocker. CVS 164 https://t.me/usmleinnercircle Version 2 Initiate Rx Goal blood pressure Age ?60 ~150 mm Hg systolic BP or >90 mm Hg diastolic BP <150/90 mm Hg Age <60, chronic kidney disease, diabetes ~140 mm Hg systolic BP or >90 mm Hg diastolic BP <140/90 mm Hg Black Thiazide diuretic or CCB, alone or in combination (ACEI/ARB, not first-line} Other ethnicities Thiazide diuretic, ACEI, ARB, or CCB, alone or in combination All ethnicities with chronic kidney disease AGEi or ARB, alone or in combination with other drug classes Initial treatment choice Hypertension stages Category & Comments rC Management definition Elevated BP (SBP 120-129; Weight loss, exercise, dietary changes (reduced salt, Lifestyle changes alcohol in moderation, DASH diet) DBP <80) DBP 80-89) Stage 2 HTN (SBP ;?140; DBP;?90) . . ne (SBP 130-139; An antihypertensive drug is needed if: Lifestyle changes ± 1 antihypertensive drug Lifestyle changes In Stage 1 HTN irc ACEI = ACE inhibitor; ARB = angiotensin II receptor blocker; BP = blood pressure; CCB = calcium channel blocker, le Joint National Committee 8 recommendations for treating hypertension AND 1-2 antihypertensive drugs Comorbid DM, CKD, or ASCVD OR 10-year risk of ASCVD >10% A 2-drug combination is recommended if BP is ;?20/10 mm Hg above target ASCVD = atheroscleroliccardiovasculardisease;BP = blood pressure;CKD = chronic kidney disease;DASH = DietaryApproachesto Stop LE Hypertension;DBP = diastolic blood pressure;DM = diabetesmellitus;HTN = hypertension;SBP = systolic blood pressure. U SM First-line antihypertensive drug classes include ACE inhibitors, angiotensin II receptor blockers, calcium channel blockers, and thiazide diuretics. Do not combine ACE inhibitors with ARBʼs Evaluation for secondary causes of hypertension is advised for patients who have resistant hypertension (ie, persistent hypertension despite an appropriate 3-drug regimen), have an abrupt onset of hypertension, or develop hypertension at an unusual age (eg, age 30. CVS 165 https://t.me/usmleinnercircle Version 2 Secondary causes of hypertension Condition Renal parenchymal disease Renovascular disease Primary hyperaldosteronism Obstructive sleep apnea Pheochromocytoma Cushing syndrome Thyroid disease Primary hyperparathyroidism Coarctation of the aorta Clinical clues/features • Elevated creatinine level • Abnormal urinalysis (proteinuria, red blood cell casts) • Recurrent flash pulmonary edema • Elevated creatinine level (particularly with ACE inhibitor use) • Abdominal bruit • Hypokalemia (spontaneous or thiazide induced) • Metabolic alkalosis • Daytime somnolence • Increased neck circumference • Paroxysmal hypertension & tachycardia • Headaches, palpitations, diaphoresis • Cushingoid body habitus & proximal muscle atrophy • Hyperglycemia • Hyperthyroidism: anxiety, heat intolerance, weight loss, tachycardia • Hypothyroidism: fatigue, cold intolerance, weight gain, bradycardia • Mild hypercalcemia ± symptoms (eg, constipation) • Kidney stones • Upper extremity hypertension with brachial-femoral pulse delay (common) • Lateralizing hypertension (less common) Renovascular HTN is common cause of secondary HTN in peds patients, dx using Renal USG with Doppler T/t : CCB → Renal Artery Stenting/ Angioplasty. Hyperparathyroidism is a cause of secondary hypertension and should be suspected in patients who have hypertension associated with hypercalcemia, renal stones, abdominal pain, or neuropsychiatric symptoms. Hypertension can be induced by both hyperthyroidism (predominantly systolic hypertension) and hypothyroidism (predominantly diastolic hypertension). CVS 166 https://t.me/usmleinnercircle Version 2 Mediterranean diet DASH diet Other .. . .. . . Any exercise lowers risk; typical recommendations include: 0 150 min/week moderate aerobic exercise OR 75 min/week vigorous exercise o Strength training 2 times/week Increased intake: Fruits, vegetables, whole grains, legumes, nuts, olive oil, poultry, fish Decreased intake: Red meat,~ saturated fats Moderate intake: Red wine (optional) Increased intake: Fruits, vegetables, whole grains, low-fat dairy, nuts, fish, poultry Decreased intake: Red meat, fats, sweets Sodium: <2,300 mg/day {<1,500 lower-sodium DASH diet) Smoking cessation & moderate alcohol intake le . Exercise Lifestyle interventions to reduce cardiac risk Lifestyle interventions for hypertension Modification Recommended plan irc DASH = DietaryApproachesto Stop Hypertension. Approximate ! systolic BP (mm Hg) Diet high in fruits & vegetables & low in saturated & total fats Reduction of BM I to <25 kg/m2 6 per 10-kg loss Aerobic exercise 30 minutes/day for 5+ days/week 7 Dietary sodium <1.5-2.3 g/day (response varies) 5-8 Alcohol limitation S2 drinks/day in men, S1 drink/day in women 5 ne rC DASH diet Weight loss DASH= DietaryApproachesto Stop Hypertension. 11 In Distractor: Smoking cessation (not shown to significantly reduce blood pressure but should be recommended to reduce overall risk of cardivascular complications) LE ARBs provide the greatest reduction in LVH of all first-line antihypertensive agents U SM  What is the next step in management for a patient with extensive alcohol use and hypertension that is not well controlled on two medications? Counsel for reduction in alcohol intake while adding a third medication is a reasonable option, counseling to reduce alcohol intake should be attempted first  What is the likely physiologic cause of isolated systolic hypertension in an elderly patient? Increased stiffness of the arterial wall associated with increased CV morbidity and mortality and should be managed in the same way as primary hypertension CVS 167 https://t.me/usmleinnercircle Version 2 Hypertensivecomplications Hypertensive urgency • Severe hypertension (usually ~180/120 mm Hg) with no symptoms or acute end-organ damage Severe hypertension with acute, life-threatening, end-organ complications Hypertensive emergency • Malignant hypertension.- Severe hypertension with retinal hemorrhages, exudates, or papilledema • Hypertensive encephalopathy.- Severe hypertension with cerebral edema & non-localizing neurologic symptoms & signs Oral meds in Urgency, IV drugs in emergency. • Isolated Systolic BP   Aortic Stiffening in old age  Aortic Valve insufficiency  Aortic Regurgitation ) Infective Endocarditis Infective endocarditis Complications of infective endocarditis • Valvular insufficiency - common causeof death Cardiac • Perivalvular abscess Risk factors • Conductionabnormalities • Mycoticaneurysm • Embolicstroke • Cerebralhemormage Neurologic • Brain abscess Physical examination • Acuteencephalopathy or meningoencephalitis Renal Musculoskeletal C>UWood CVS • Renalinfarction • Glomerulonephritis • Drug-inducedacuteinterstitial nephritisfrom therapy • Vertebralosteomyelitis • Septicarthritis • Musculoskeletal abscess Diagnostic testing Treatment • Congenital heart disease or prosthetic valve • Previous endocarditis • lntravascular catheters • Intravenous drug use • New regurgitant murmur • Skin: Janeway lesions, Osler nodes • Roth spots (eyes), splinter hemorrhages (nails) • Splenomegaly • ± Signs of embolic phenomenon • Hematuria/proteinuria (glomerulonephritis) • Positive blood cultures • TEE > TTE for detecting vegetation • Acute: Empiric treatment with vancomycin • Subacute: Treatment based on culture results TEE = transesophageal echocardiogram; TTE = transthoracic echocardiogram. 168 https://t.me/usmleinnercircle Version 2 Infective endocarditis with intravenous drug use • Injected particles cause microdamage to right-sided Pathogenesis valves before being filtered out by the lungs • Microdamage facilitates bacterial attachment • Increased risk with HIV • Tricuspid valve most commonly affected • Staphylococcus aureus is the most common organism • Septic pulmonary emboli present in up to 75% of cases Clinical features • Audible murmur may be absent in 50% of cases • Few peripheral manifestations • Heart failure uncommon U SM LE In ne rC irc Septic emboli le (eg, splinter hemorrhages, Janeway lesions) Roth Spots Evaluation begins with blood cultures from 3 different venipuncture sites. First Culture then Antibiotics • Perivalvular abscess should be suspected with the development of conduction abnormalities in patients with infective endocarditis. Perivalvular abscess: extends into adjacent cardiac conduction tissues, leading to heart block What valve is most commonly involved? Aortic valve  What is the common vascular phenomena seen in right-sided endocarditis (S. aureus IVDU? Septic pulmonary emboli multiple nodular, peripheral opacities, often with cavitation seen on CT splinter hemorrhages, Janeway lesions not produced in right-sided CVS 169 https://t.me/usmleinnercircle Version 2 • Most Viridans group streptococci are highly susceptible to penicillin and should be treated with IV aqueous penicillin G NOT ORAL or IV ceftriaxone x 4 weeks (preferred) Oral antibiotics are generally not recommended as initial therapy in pt. With IE Ceftriaxone is preferred due to daily dosing versus 46x per day for penicillin Prevention of infective endocarditis with antimicrobial prophylaxis* High-risk procedures (Prophylaxis needed) Low-risk procedures (No prophylaxis) • Gingival/apical tooth manipulation (amoxicillin) • Respiratory mucosa incision (amoxicillin) • Surgery on infected skin or muscle (vancomycin) • GI or GU procedure in setting of active infection (ampicillin) • GI or GU endoscopy/procedure in absence of infection • Most vaginal/caesarian deliveries *Indicatedfor high-riskpatients(eg, prostheticheart valve, previousinfectiveendocarditis,certain congenitalcyanoticheart diseases). GI = gastrointestinal;GU = genitourinary. Prophylactic antibiotic therapy is usually administered in one dose 3060 minutes prior to the procedure. High-risk conditions for infective endocarditis Common surgical indications in infectious endocarditis • Prosthetic heart valve • Previous infective endocarditis • Structural valve abnormality in transplanted heart • Unrepaired cyanotic congenital heart disease • Repaired congenital heart disease with residual defect • Acute heart failure (eg, aortic/mitral valve regurgitation) • Extension of infection (eg, abscess, fistula, heart block) • Difficult-to-eradicate organism (eg, fungus, MDR pathogen) • Persistent bacteremia on antibiotics • Large vegetation/persistent septic emboli MDR = multidrug-resistant. Overall, the risk of IE following dental procedures in patients with acquired valvular defects due to rheumatic fever is low, and antibiotic prophylaxis is not indicated. However, prophylaxis is indicated in patients with a history of IE. CVS 170 https://t.me/usmleinnercircle Version 2 Infective endocarditis-modified Duke criteria Major criteria • Blood culture positive for typical microorganism (eg, Staphylococcus aureus, Enterococcus, viridans streptococci) • Echocardiogram showing valvular vegetation Minor criteria • Predisposing cardiac lesion • Intravenous drug use • Temperature >38 C (100.4 F) Diagnostic criteria for IE • Positive blood culture not meeting above criteria Definite IE • 2 major OR 1 major + 3 minor criteria Possible IE • Fever (>90%) • Heart murmur (85%) • Petechiae (S50%) • Subungual splinter hemorrhages (<50%) • Osler nodes, Janeway lesions (<50%) • Neurologic phenomena (embolic) (S40%) • Splenomegaly (S30%) • Roth spots (retinal hemorrhage) (<5%) rC Clinical findings (frequency) irc • 1 major+ 1 minor OR 3 minor criteria le • Embolic phenomena • Immunologic phenomena (eg, glomerulonephritis) ne IE = infectiveendocarditis. .. .• .. .• . . . • • .. Culture-positive infective endocarditis lntravascular catheters Implanted devices (eg, pacemaker/defibrillator) In Staphylococcus aureus Prosthetic valves LE Viridans streptococci Staphylococcus epidermidis U SM Enterococci Streptococcus gallolyticus (formerly S bovis) Fungi (eg, Candida) Intravenous drug users Gingival manipulation Respiratory tract incision or biopsy Prosthetic valves lntravascular catheters Implanted devices Nosocomial urinary tract infections Colon carcinoma Inflammatory bowel disease lmmunocompromised host lntravascular catheters Prolonged antibiotic therapy • Gram-negative HACEK organisms can produce subacute endocarditis and are seen in patients with poor dental hygiene and/or periodontal infection. Haemophilus Aggregatibacter actinomycetemcomitans Cardiobacterium hominis Eikenella corrodes CVS 171 https://t.me/usmleinnercircle Version 2 Kingella Rheumatoid factor: may be elevated in patients with infective endocarditis rheumatoid factor is a non-specific finding; don't let it throw you off the diagnosis if symptoms otherwise point to infective endocarditis! Etiology Manifestations . • . • . Nonbacterial thrombotic endocarditis Hypercoagulable state due primarily to underlying malignancy (80%) or SLE Noninfectious thrombi form on healthy valve Usually asymptomatic until embolism to kidney, spleen, skin, extremities, or brain occurs Uncommon: fever, leukocytosis, significant valve insufficiency (eg, murmur) Blood cultures: no growth Diagnosis • Echocardiography: small, mobile vegetations on aortic/mitral valves • Evaluate for hypercoagulable state &/or malignancy Treatment • Anticoagulation SLE = systemic lupus erythematosus. Rheumatic Heart Disease  What is the likely diagnosis in an immigrant with progressive dyspnea, orthopnea, palpitations, and an elevated left main bronchus on CXR? Rheumatic heart disease mitral stenosis results in left atrial enlargement, predisposing to arrhythmia (palpitations) and elevation of the left main bronchus CVS 172 https://t.me/usmleinnercircle Version 2 Acute rheumaticfever Epidemiology • Peak incidence: Age 5-15 • Twice as common in girls • Joints (migratory arthritis) • • (Carditis) Major • Nodules (subcutaneous) • Erythema marginatum le • Sydenham chorea Clinical features • Fever • Arthralgias irc • Elevated erythrocyte sedimentation rate/ C-reactive protein Minor Erythema Marginatum Late sequelae Mitra! regurgitation/stenosis Prevention Penicillin for group A streptococcal (Streptococcus pyogenes) pharyngitis ne 2 Major Jones criteria or 1 Major and 2 minor criteria In Treatment/prevention? • Oral penicillin V to prevent ARF rC • Prolonged PR interval • ARF should still be treated with long-acting IM benzathine pencillin G until adulthood LE Antibiotic prophylaxis for secondary prevention of rheumatic fever Duration of therapy Severity following last attack 5 years or until age 21* With carditis but no valvular disease 10 years or until age 21 * With carditis & valvular disease 10 years or until age 40* U SM Uncomplicated rheumatic fever *Whicheverduration is longer. IntramuscularpenicillinG benzathineevery 3-4 weeks is preferred.  Can acute rheumatic fever be prevented with treatment of Streptococcal pharyngitis (e.g. oral penicillin)? Yes versus PSGN, which can occur with or without treatment Acute Rheumatic fever DX based on JONES. Two major or 1 major and 2 minor criteria are required, although the diagnosis can be made on the presence of Sydenham chorea (SC) or carditis alone. The classic form of SC (also known as Saint Vitus dance), the most common acquired chorea in children. Emotional lability and decline in school performance are typically the earliest neurologic manifestations, followed by distal hand movements that progress to facial grimacing and feet jerking. Movements are typically irregular and rapid. Patients also have decreased strength throughout, and the relaxation phase of the patellar reflex is usually delayed. In addition, pronator drift (involuntary hyperpronation of extended arms) is present. The diagnosis is based on clinical presentation. CVS 173 https://t.me/usmleinnercircle Sydenham chorea Pathophysiology Clinical features Evaluation Treatment Prognosis • Preceding GAS infection • Molecular mimicry between anti-GAS antibodies & neuronal antigens in basal ganglia • Involuntary, jerky movements (worse while awake & with action) • Hypotonia • Emotional !ability, obsessive-compulsive behaviors • ± Symptoms of acute rheumatic fever • GAS testing: throat culture, ASO & anti-DNAse B titers • Cardiac testing: echocardiography, ECG • Chronic antibiotics (eg, penicillin G) • Symptomatic (antidopaminergics (eg, haloperidol]) • Spontaneous remission • Recurrence common • j Risk of rheumatic heart disease anti-DNAse = antideoxyribonuclease;ASO = antistreptolysinO; GAS = group A Streptococcus. Cholesterol • Cholesterol emboli may result in skin complications, such as livedo reticularis and blue toe syndrome. CVS 174 https://t.me/usmleinnercircle Version 2 Cholesterol crystal embolism (atheroembolism) • Comorbid conditions (hypercholesterolemia, hypertension, type 2 diabetes mellitus) Risk factors • Cardiac catheterization or vascular procedure • Dermatologic (livedo reticularis, ulcers, gangrene, blue toe syndrome) • Renal (acute or subacute kidney injury) Clinical features • Central nervous system (stroke, amaurosis fugax) • Ocular involvement (Hollenhorst plaques) • Laboratory findings irc o Elevated serum creatinine, eosinophilia, hypocomplementemia le • Gastrointestinal (intestinal ischemia, pancreatitis) o Urinalysis - typically benign with few cells or casts, may have eosinophiluria Diagnosis • Skin or renal biopsy rC o Biconvex, needle-shaped clefts within occluded vessels o Perivascular inflammation with eosinophils ne • Treatment is supportive and involves statin therapy to prevent recurrent cholesterol embolism Indications for statin therapy Established ASCVD LE Acute coronary syndrome Stable angina Arterial revascularization (eg, CABG) Stroke, TIA, PAD U SM Secondary prevention .. .. In NBME question had 2 proteinuria, Blood, RBC and granular casts in UA of Cholesterol emboli syndrome. LDL i:?:190 mg/dL Age i!:40with diabetes mellitus Primary prevention Estimated 10-year ASCVD risk >7.5%-10% in the prevention .. . . . . of ASCVD Age :575: High-intensity statin Age >75: Moderate-intensity statin High-intensity statin 10-year ASCVD risk .:20%: High-intensity statin 10-year ASCVD risk <20%: Moderate-intensity statin Moderate- to high-intensity statin* (Pooled Cohort Equations) *High-intensity stalins include atorvastalin 40-80 mg & rosuvastatin 20-40 mg; moderate-intensitystalins include atorvastalin 10-20 mg, rosuvastalin 510 mg, simvastatin 20-40 mg, pravastalin 40-80 mg & lovastatin 40 mg. ASCVD = atherosclerolic cardiovascular disease; CABG = coronary artery bypass grafting; PAD = peripheral artery disease; TIA = transient ischemic attack. ALL 4075 diabetic patients should be started on statin therapy, regardless of other risk factors CVS 175 https://t.me/usmleinnercircle Version 2 Hypercholesterolemia is commonly asymptomatic and increases risk for significant cardiovascular and cerebrovascular disease. Therapeutic options include dietary modifications, weight reduction, increased physical activity, and lipid- lowering pharmacotherapy. The normal Optimal range for LDL cholesterol levels is less than 100 mg/dL BEWARE THEY MENTION “NORMAL LEVEL 160ˮ IN THEIR LAB VALUES SECTION BUT THEY WANT YOU TO KEEP IT BELOW 100 IN CMS FORMS Those who develop rhabdomyolysis, a rare side effect of severe statin-induced myopathy, should discontinue statin use entirely. However, most muscle toxicity is mild (eg, myalgias), and the majority of patients can tolerate a different statin. (Prefer moderate intensity) Statin therapy can potentiate muscle injury and elevation of creatine kinase CK levels following prolonged and vigorous exercise. Most such patients should be restarted on statin therapy after CK levels have normalized Treatment of hypertriglyceridemia Hypertriglyceridemia ! Evaluate for secondary causes ! l 150-500 mgldl >1.000 mg/dl • Initial goal is pancreatitis prevention o Fibrates o Fish oil o Abstinence from alcohol • Lifestyle modifications o \Neight loss o Moderate alcohol intake o Increased exercise • Known cardiovascular disease or high risk o Statm therapy l CVS Once levels ssoo mg/dl 176 https://t.me/usmleinnercircle Version 2 Treatment of hypertriglyceridemia Triglycerides 500-999 mg/dl 150-499 mg/dl 2:1,000 mg/dl • Limit dietary sugar/tight glycemic control in diabetes • Limit saturated fat General • Regular aerobic exercise measures • Weight loss of 5%-10% of body weight • Treat with statins based on ASCVD risk • Abstain from alcohol • Limit alcohol intake • 0-3 acids if high risk of ASCVD measures • 0-3 acids or fibrates, depending on ASCVD risk • Abstain from alcohol • Fibrates to reduce pancreatitis risk le Specific Triglyceride-induced pancreatitis Triglyceride levels (mg/dl) • 500-999: mild risk • 1,000-1,999: moderate risk Risk • 2:2,000: high risk rC • <500: minimal risk irc ASCVD = atheroscleroticcardiovasculardisease; 0-3 acids = omega-3 fatty acids. Other risk factors: pregnancy, alcoholism, obesity, uncontrolled diabetes • Acute epigastric pain radiating to back • ± Fever, nausea, vomiting • Elevated serum lipase (>3 times upper limit of normal) • Intravenous fluid hydration, pain control ne Clinical features • Triglycerides 2:500 mg/dl: consider insulin infusion Management In • Triglycerides >1,000 mg/dl or severe pancreatitis (eg, lactic acidosis, hypocalcemia): Aorta Aortic Trauma LE consider apheresis (therapeutic plasma exchange) U SM  What is the initial screening test for blunt aortic trauma? Chest X-ray should be ruled out in patients with blunt deceleration trauma MVA or fall from  10 feet) confirm via CT Scan or TTE  What is the most sensitive finding indicative of blunt aortic trauma? Mediastinal widening common symptoms include anxiety, tachycardia, and hypertension; CXR may also show left-sided effusion due to hemothorax CVS 177 https://t.me/usmleinnercircle Version 2 Thoracic aortic injury Normal chest x-ray Widened mediastinum: supine chest x-ray >8 cm; upright chest x-ray >6 cm ~UWor1d In hypotensive patients, systolic blood pressure is generally kept at 100 mm Hg to prevent injury extension and rebleeding while awaiting emergent operative repair. Aortic Dissection Risk factors Clinical presentation Diagnosis Treatment .. . .. . .. . .. .. Acute aortic dissection Chronic hypertension* Underlying aortopathy (eg, Marfan syndrome) Cocaine use Severe, tearing chest or back pain, maximal at onset ± Variation in SBP between arms >20 mm Hg Hypertension usually present** ECG: normal or nonspecific ST-segment & T-wave changes Chest x-ray: mediastinal widening CT angiography or TEE for definitive diagnosis Pain control (eg, morphine) Intravenous beta blockers (eg, esmolol) ± Sodium nitroprusside (if SBP >120 mm Hg) Emergency surgical repair for ascending dissection *Strongest overall risk factor. '*Hypotension on presentation suggests aortic rupture or other complication (eg, cardiac tamponade, acute aortic regurgitation). SBP = systolic blood pressure; TEE= transesophagealechocardiography. CVS 178 https://t.me/usmleinnercircle Version 2 Diagnosis & management of acute aortic dissection Suspected aortic dissection (eg, abrupt-onset severe chest or back pain, widened med,astinum on chest x-ray) l Anti-Impulse therapy to reduce aortic wall shear stress • Intravenous beta blockade (eg, esmolol, labetalol) • Pain control (eg, morphine) l Emergency surgical repair Type B dissection confirmed (dissection confined to descending aorta) J l Admit for blood pressure control & monitoring Ct.llM)rld rC "Transesophageal echocardiography often prefamtd for emergently hypolens,ve patients irc Type A dissection confirmed (ascending aorta involved) le Confinmatory imaging with CT angiography of the aorta or other modality•  Involvement of the subclavian or iliac arteries can result in asymmetry of pulses or blood pressure ne (pulses and blood pressure are typically symmetric if these arterial branches are not involved). Complications due to extension of acute aortic dissection In • Stroke (carotid artery) • Horner syndrome (carotid sympathetic plexus) • Acute aortic regurgitation (aortic root/valve) • Myocardial ischemia/infarction (coronary artery ostia) Pericardia! effusion/tamponade (pericardium) (involved structure) • • Hemothorax (pleural cavity) • Renal infarction (renal arteries) • Intestinal ischemia (mesenteric arteries) • Lower extremity paralysis (spinal arteries) LE Complication U SM • Aortic regurgitation may result in sudden onset worsening chest pain, hypotension, and pulmonary edema Cardiac tamponade can occur as a complication as well, but those patients with clear lung fields Chronic systemic hypertension is the most important predisposing risk factor for aortic dissection. Marfan is common cause for dissection in younger patients 40 (vs HTN 60) CVS 179 https://t.me/usmleinnercircle Version 2 Diagnostic approach for suspected aortic dissection Suspected aortic dissection Risk factors (eg, Marfan syndrome, connective tissue disease, hypertension) Tearing chest/abdominal pain radiating to the back Perfusion deficits (eg, pulse deficit, >20 mm Hg blood pressure difference between right & left arm, aortic regurgitation murmur) Chest x-ray or ECG Yes Evaluate & treat appropriately suggesting other diagnoses? No Normal serum creatinine Pulse deficit or differential blood pressure 20 mm Hg difference) in the upper extremities is noted in only 20%30% of the patients and its absence should not be used to exclude the diagnosis. & no contrast allergy No I TEEpreferred I Yes Imaging based on availability TEE Chest CT with contrast MRI (only if nonemergency & patient can lie still) ©UWorld  What is the work-up of a stable and unstable aortic dissection? 1 Initial: CXR mediastinal widening, tracheal deviation) 2 Stable: CT angiography 3 Unstable/contrast allergy/renal dysfunction: TEE (reveals intimal flap) CXR shows widened mediastinum, aortic knob TEE noninvasive, performed at bedside CT Angiography: invasive, but best test for determining extent of dissection for surgery CVS 180 https://t.me/usmleinnercircle Version 2 Aortic Aneurysm Abdominal .. .. Management Advanced age (eg, >60) Smoking, male sex, hypertension History of atherosclerosis or CTD Mostly asymptomatic Rapid expansion 0 Dull abdominal/back pain 0 Distal embolization Rupture 0 Sudden, severe abdominal/back pain ± shock 0 Umbilical/flank hematoma Smoking cessation Elective repair for size >5.5 cm (asymptomatic) Urgent repair for symptomatic & HD stable patients Emergency repair for symptomatic & HD unstable patients Abdominal aortic aneurysm {AAA) Most commonly affects infrarenal aorta {2:3 cm) Smoking Male sex Older ne Risks . .. . . . rC CTD = connectivetissuedisease;HD = hemodynamically. Anatomy le Clinical presentation .. . .. . irc Risk factors aortic aneurysm Patient is white Family history of AAA Screening • Abdominal ultrasound in men age 65-75 who have ever smoked .. .. . Mostly asymptomatic May have abdominal, back, or flank pain LE Symptoms In • Atherosclerotic disease . Lower limb ischemia &/or thromboembolism Rupture often presents with abdominal distension & shock Smoking cessation • Aspirin & statin therapy U SM Management Follow-up imaging Elective repair recommended for: 0 Large (2:5.5 cm) aneurysms 0 Rapidly enlarging aneurysms (2:0.5 cm in 6 months) o AAA associated with peripheral artery disease or aneurysm . . Medium (4-5.4 cm): ultrasound every 6-12 months Smaller: ultrasound every 2-3 years  AAA rupture usually occurs posteriorly into the retroperitoneum, which can delay the onset of hemodynamic instability (eg, 1 h of symptoms in a patient); however, rupture may occur anteriorly with direct leakage into the peritoneum and rapid onset of hemodynamic instability and shock CVS 181 https://t.me/usmleinnercircle Version 2 Evaluation of suspected unstable abdominal aortic aneurysm Symptoms suggesting unstable AAA abdominal/flank/groin pain pulsatile mass flank ecchymosis limb ischemia Hemodynamically stable I ! Yes Obtain CT of abdomen Known AAA No Yes Obtain focused abdominal ultrasound AAA identified AAA identified ! Yes Medical optimization and repair Yes Explore other diagnoses l Emergency repair AAA= abdominalaorticaneurysm. CUWotld  Other clues to the diagnosis of AAA include a pulsatile abdominal mass (present in slightly over half of patients) and prevertebral aortic calcification on plain x-ray, consistent with extensive atherosclerosis. Ruptured AAA  What is the likely diagnosis in a male smoker with chest discomfort and the CXR findings below? Thoracic aortic aneurysm Suggestive findings include a widened mediastinum, increased aortic knob, and tracheal deviation Initial test: CXR CVS 182 https://t.me/usmleinnercircle Version 2 Best confirmatory test: CT with contrast  What is the imaging modality of choice for diagnosis and follow-up of abdominal aortic aneurysms? Abdominal ultrasound cheap and does not require contrast (vs. CT and MRI The strongest predictors of abdominal aortic aneurysm rupture are large aneurysm le diameter(> 5.5 cm), rapid rate of expansion ( 1 cm per year), and current cigarette smoking. rC irc Active smoking is the strongest modifiable influence for AAA development and progression. What is the recommended screening protocol for abdominal aortic aneurysm? ne One-time abdominal ultrasound for male active or former smokers aged 6575 years  What abdominal aortic aneurysm repair complication results in progressive abdominal Bowel ischemia/infarction In pain and bloody diarrhea? due to inadequate perfusion after loss of IMA during aortic graft placement LE  What are the most common peripheral artery aneurysms? Popliteal (#1 and Femoral (#2 frequently associated with abdominal aortic aneurysms U SM Coarctation of Aorta CVS 183 https://t.me/usmleinnercircle Version 2 Coarctation of the aorta Etiology • Congenital • Acquired (rare) (eg, Takayasu arteritis) • Upper body o Well developed o Hypertension (headaches, epistaxis) • Lower extremities Clinical features o Underdeveloped o Claudication • Brachial-femoral pulse delay • Upper & lower extremity blood pressure differential • Left interscapular systolic or ccntinuous murmur • ECG: Left ventricular hypertrophy • Chest x-ray Diagnostic studies o Inferior notching of the 3rd to 8th ribs o "3" sign due to aortic indentation • Echocardiography: Diagnostic confirmation Treatment • Balloon angioplasty± stent placement • Surgery often presents a couple days after birth when the ductus arteriosus closes Aortic coarctation Patent ductus Closed ductus Leftcommon carotid  Left ventricular hypertrophy LVH signs: high-voltage QRS complexes, lateral ST segment depression, lateral T wave inversion Pathology .. .. . Clinical features Coarctation of the aorta Thickening of tunica media of aortic arch Can be associated with Turner syndrome or bicuspid aortic valve j BP & strong pulses in upper extremities i BP & weak pulses in lower extremities Neonates• (severe narrowing) 0 Heart failure (eg, poor feeding, diaphoresis) 0 Cardiogenic shock • Children/adults (mild narrowing) 0 Complications Treatment .. • . Lower extremity claudication 0 Palpable pulsations of intercostal vessels (collaterals) 0 Secondary hypertension (upper arms) Aortic aneurysm, dissection & rupture Cerebral aneurysm & subarachnoid hemorrhage Prostaglandin E1 for neonates with severe narrowing Surgical repair *Afterclosureof ductusarteriosus. BP = blood pressure. CVS 184 https://t.me/usmleinnercircle Version 2  Increased LV afterload • Continuous murmur at the back due to collaterals  Patients with suspected severe aortic coarctation should immediately receive prostaglandin E1 to maintain patency of the DA. This allows blood from the pulmonary artery to supply the descending aorta (right-to-left shunting), restoring distal perfusion. Aortoiliac occlusion (Leriche syndrome) is characterized by a triad of irc  bilateral hip, thigh, and buttock claudication, le Aortoiliac Occlusion (Leriche Syndrome)  impotence, and rC  symmetric atrophy of the bilateral lower extremities. Carotid Artery ne Management of carotid atherosclerotic disease Intensive medical therapy Aspirin In Stalin Blood pressure control + LE Evaluation for carotid revascularization l Asymptomatic Symptomatic U SM ! ! j l ! j l <50%· 50-79% 80-99% <50% 50-69% 70-99% No benefit of CEA CEA typically not recommended- CEA recommended No benefit of CEA CEA recommended in select patients•- CEA recommended 'Degree or stenosis. "May be recommendedin select patients with low perioperabverisk (eg, <3%). '"Men likely benefit from CEA, whereas women likely benefit from ,ntens,vemedical therapy only. CEA = carotid endarterectomy;TIA = transient ,schem,cattack. ©UW0<ld Patients with stenosis 80% should receive periodic (eg, annual) carotid duplex surveillance to detect any progression. CVS 185 https://t.me/usmleinnercircle Version 2 • Carotid endarterectomy should be considered for symptomatic patients (e.g. TIA, stroke) with carotid stenosis between 70 - 99% to reduce future stroke risk. patients with disabling neurologic deficits, 100% occlusion of the carotid artery, or life expectancy  5 years are unlikely to benefit Carotid Artery Dissection Carotid artery dissection • Trauma; spontaneous occurrence • Underlying contributors: HTN, smoking, CTD Etiology Clinical presentation Diagnosis .. • . Unilateral head & neck pain, transient vision loss lpsilateral partial Homer syndrome 0 Ptosis & miosis without anhidrosis Signs of cerebral ischemia (eg, focal weakness) Neurovascular imaging (eg, CT angiography) • Thrombolysis (if QI s hr after sl(mptom onset) • Antiplatelet therapy (eg, aspirin) ± anticoagulation Treatment CTD = connectivetissue disease;HTN = hypertension.  What is the likely diagnosis in a child that presents with hemiparesis and aphasia hours after falling down with an object in their mouth? internal carotid artery dissection or thrombus formation Penetrating or blunt posterior pharyngeal trauma Traumatic carotid injuries Internal carotid • Penetrating trauma Mechanism • Fall with object in mouth (eg, toothbrush, pencil) • Neck manipulation (eg, yoga, sports) • Gradual-onset hemiplegia Presentation • Aphasia • Neck pain • "Thunderclap" headache Tearing in tunica intima results in artery dissection Diagnosis • CT or MR angiography • What is the next best step in a young woman who runs a marathon and complains of neck pain, headache, right-sided weakness, and vision loss? She recently completed a marathon. CT scan is normal and duplex ultrasound shows absence of flow in the internal carotid artery. Heparin and oral anticoagulation for 36 mo. Also can do with anti-platelet agents for 1 year Treatment should be initiated after an intracerebral hemorrhage has been ruled out. MI CVS 186 https://t.me/usmleinnercircle Version 2  The following ECG findings are diagnostic of ST-elevation myocardial infarction STEMI • New ST elevation at the J point in 2 anatomically contiguous leads with the following threshold: 0 1 mm 0.1 mV in all leads except V2 and V3 0 1.5 mm in women, 2 mm in men age 40, and 2.5 mm in men age 40 in leads V2 and V3 • New left bundle branch block with clinical presentation consistent with acute coronary syndrome ACS • Perioperative MI is common in patients undergoing noncardiac surgery; le intraoperative hemorrhage requiring blood transfusion increases the risk (likely due to reduced oxygen delivery to the myocardium). irc Patients with perioperative MI often lack chest pain, possibly due to receipt of postoperative pain control (eg, morphine). rC Initial stabilization of acute ST-segment elevation Ml ne • Supplemental oxygen (if SaO2 <90% or dyspnea) • Aspirin 325 mg • P2Y12 inhibitor (eg, clopidogrel) • Nitrates (sublingual) • Beta blocker (unless hypotension, bradycardia, acute heart failure, heart block) LE Persistent pain, hypertension, or heart failure In • High-dose statin (eg, atorvastatin 80 mg) • Anticoagulation (drug depends on planned revascularization) U SM IV nitroglycerin (not if hypotension, right ventricular infarct, or severe aortic stenosis is present) Persistent severe pain Unstable sinus bradycardia l l IV morphine IV atropine Pulmonary edema IV furosemide (not if patient is hypotensive or hypovolemicl Reperfusioo: • PCI within 90 min preferred • Thrombolysis (if PCI not available within 120 min) IV = intravenous; Ml = myocardial infarcbon; PCI = percutaneous coronary intervention, CVS C)UWorld 187 https://t.me/usmleinnercircle Version 2 Acute management of acute coronary syndrome (STEMI, NSTEMI, or unstable angina) .. .. . Nitrates Beta blocker .. .. Antiplatelet therapy Anticoagulation .. .. . Statin therapy Rapid chest pain relief Caution with hypotension (eg, RV infarction) Cardioselective (eg, metoprolol, atenolol) Decreases myocardial 0 2 demand to limit infarct size Contraindicated in cardiogenic shock & bradycardia Aspirin+ P2Y 12 inhibitor (eg, prasugrel, clopidogrel)* Reduces platelet activity Unfractionated heparin, bivalirudin, or enoxaparin Limits thrombus expansion High potency (eg, atorvastatin, rosuvastatin) Stabilizes atherosclerotic plaque STEMI Coronary reperfusion PCI <90 min from 1st medical contact Fibrinolytics (eg, alteplase) if PCI is unavailable NSTEMI or unstable angina Coronary angiography often within 24 hr •In patients with NSTEMI or unstable angina, P2Y12 inhibitor therapy is often held until after coronary angiography in case the atheroscleroticcoronary anatomy indicates the need for coronary artery bypass grafting. NSTEMI = non-ST-segment elevation myocardial infarction; PCI = percutaneouscoronary intervention; RV= right ventricular; STEMI = ST-segment elevation myocardial infarction. Management of unstable angina/non-ST elevation myocardial infarction Perform risk assessment Thrombolysls In Myocardial Infarction risk score Clinical variables at presentation (1 point for each): • Age ;?65 • ;?3 risiifactors for CAD • Known CAD with >50% stenosis • Use of aspirin in the past 7 days • 2;2 angina! episodes within the precedin~ • Elevated serum cardiac biomarkers (eg, troponin I) • ST-segment deviation >0.5 mm on admission ECG Low risk (0-2) l Stress test Intermediate (3-4) or high (5-7) risk ! Early coronary angiography (within 24 hr) CAD = coronary artery disease; MR = mitral regurgitation. • Hemodynamic instability • Heart failure or new MR • Recurrent chestpain • Ventricular arrhythmia Immediate coronary angiography ~UWorld  What pharmaceutical treatment should be avoided in patients with MI that present with CHF or bradycardia? CVS 188 https://t.me/usmleinnercircle Version 2 Beta blockers  What is the recommended reperfusion therapy for patients with NSTEMI that present within 12 hours of symptom onset but cannot undergo PCI? Fibrinolysis fibrinolysis is associated with higher rates of recurrent MI, intracranial hemorrhage, and mortality compared to PCI irc le Percutaneous coronary intervention PCI is recommended for patients with acute STEMI within 12 hours of symptom onset and within 90 minutes from first medical contact to device time at a PCI-capable facility. rC or 120 minutes from first medical contact to device time at a non-PCI-capable facility (to allow time for transport).  What is the most important factor for survival in a patient with an out-of-hospital sudden cardiac Elapsed time to effective resuscitation ne arrest? e.g. adequate bystander CPR, prompt rhythm analysis, and defibrillation in patients found to In be in ventricular fibrillation  What is the initial management for hypotension in a patient with a right ventricular MI that has lownormal JVP? LE IV saline bolus  Nitrates should be avoided ) U SM RVMI often creates high sensitivity to intravascular volume depletion; hypotension with low JVP suggests inadequate RV preload Marked hypotension is a characteristic feature of RVMI CVS 189 https://t.me/usmleinnercircle Version 2 Myocardial infarction location based on coronary vessel involvement Involved myocardium Blocked vessel Anterior Ml LAD • Some or all of leads V1-V6 Inferior Ml RCA orLCX • ST elevation in leads II, lll & aVF Posterior Ml LCX or RCA • ST depression in leads V1-V3 • ST elevation in leads I & aVL (LCX) • ST depression in leads I & aVL (RCA) Lateral Ml LCX, diagonal • ST elevation in leads I, aVL, VS & V6 • ST depression in leads 11,Ill & aVF Right ventricle Ml (occurs in ½ of inferior Ml) RCA ECG leads involved • ST elevation in leads V4-V6R Posteroinferior ST-elevation myocardial infarction (STEMI) I aVR VI V4 VI Pathophysiology Key features Most commonly due to RCA occlusion Less commonly due to LCx occlusion ST elevation in II, Ill, & aVF (inferior wall STEMI) ST depression in V1 & V2 (posterior wall STEMI) Reciprocal ST depression 2:1 AV block (every other P wave not conducted) AV= atrioventricular; LCx = left circumflex artery; RCA= right coronary artery. ©uworld AV Block because RCA supplies AV node, hence bradycardia CVS 190 https://t.me/usmleinnercircle Version 2 Comparison of left ventricular & right ventricular myocardial infarction Left ventricular Ml ECG findings Hemodynamic findings Management • Pulmonary edema • Clear lungs & JVD • S3&S4 • Marked hypotension • lschemic changes in anterior, lateral, or inferior leads • Inferior ischemic changes • ST-segment elevation in V 4 R • Bradyarrhythmias t LV preload • j LV & RV preload • j RV preload, • j SVR • j SVR • Fluid restriction • Fluid resuscitation • Preload & afterload reduction • Avoid preload reduction • Reperfusion therapy • Reperfusion therapy le Clinical features Right ventricular Ml JVD = jugular venous distension; LV = left ventricular; Ml = myocardial infarction; RV = right ventricular; SVR = systemic vascular irc resistance. rC •Sinus bradycardia and atrioventricular block are common and typically transient complications of acute inferior wall myocardial infarction. Intravenous atropine is the initial treatment of choice in patients with hemodynamically significant bradycardia (eg, pulmonary edema, hypotension) due to inferior wall myocardial infarction Temporary cardiac pacing is the treatment of choice in patients with persistent symptomatic ne bradyarrhythmias (eg, hypotension, dizziness, heart failure, syncope) that are not responsive to atropine. DO NOT GIVE FLUIDS IN SYMPTOMATIC PATIENTS, FIRST GIVE ATROPINE TO TREAT In BRADYCARDIA. LE The failing RV becomes reliant on hydrostatic pressure to force blood through the pulmonary circulation and is highly sensitive to a reduction in preload U SM Whenever inferior wall MI is suspected based on ischemic changes in the inferior ECG leads, RV involvement should be evaluated using a Right-sided precordial ECG, which is obtained via precordial lead placement in a mirror image on the right side of the chest. ST-segment elevation in lead V4R is highly accurate in confirming RVMI. CVS 191 https://t.me/usmleinnercircle Version 2 Optimal medical management following myocardial infarction Medication Indication • All patients (regardless of stent placement) Dual antiplatelet therapy • Low-dose aspirin continued indefinitely (low-dose aspirin+ P2Y 12 inhibitor) • P2Y 12 inhibitor continued for 12 months • All patients, continued indefinitely Beta blocker • Reduces myocardial oxygen demand, decreases arrhythmia risk (eg, metoprolol, carvedilol) & inhibits remodeling ACE inhibitor or angiotensin II receptor blocker High-intensity statin • All patients, continued indefinitely for reduced LVEF • Inhibits post-Ml remodeling • All patients, continued indefinitely (ie, atorvastatin, rosuvastatin) • Stabilizes atherosclerotic plaque & reduces recurrent Ml Mineralocorticoid antagonist • Reduced LVEF with symptoms or comorbid DM (eg, spironolactone) • Inhibits post-Ml remodeling DM = diabetes mellitus; LVEF = left ventricular ejection fraction; Ml = myocardial infarction. Beta blockers reduce short-term morbidity (recurrent symptoms or reinfarction) and improve long-term survival in patients with prior MI and/or left ventricular systolic dysfunction, and are an integral part of pharmacotherapy for secondary prevention of cardiovascular events following MI. Use of NSAIDʼs increases the risk for Acute Coronary Syndrome and MI Post MI Complications CVS 192 https://t.me/usmleinnercircle Version 2 Mechanical complications of acute myocardial infarction Papillary muscle Involved coronary artery Acute or within 3-5 rupture/ RCA days dysfunction lnterventricular Acute or within 3-5 LAD (apical septal) or RCA septum rupture days (basal septal) Within 5 days* or up Free wall rupture LAD to 2weeks Left ventricular Upto~I aneurysm LAD months . .. .. . .. . .. . Echocardiography findings Severe pulmonary edema, respiratory distress New early systolic murmur Severe MR Hypotension/cardiogenic shock Chest pain New holosystolic munmur Left-to-right ventricular Hypotension/cardiogenic shunt** shock Chest pain Distant heart sounds Shock, rapid progression to cardiac arrest Heart failure Angina Pericardia! effusion with tamponade Ventricular arrhythmias Thin & dyskinetic myocardial wall rC *50% occur within 5 days. Clinical findings le Time course irc Complication **Right heart catheterizationshows step up in 0 2 concentrationfrom right atrium to right ventricle. ne LAD = left anterior descending;MR= mitral regurgitation;RCA = right coronaryartery. Left ventricular aneurysm Diagnosis Ml = myocardial infarction. . Heart failure & angina In Clinical presentation • Scar tissue deposition following transmural Ml • Several months following Ml • Ventricular arrhythmia (eg, ventricular tachycardia) • Systemic embolization (eg, stroke) • ECG: Persistent ST elevation, deep Q waves • Echocardiograph: Thin and dyskinetic myocardial wall LE Etiology U SM What are the complications? Heart failure, arrhythmias, mitral regurgitation (distortion of LV geometry results in papillary muscle dysfunction), mural thrombus CVS 193 https://t.me/usmleinnercircle Version 2 Left ventricular aneurysm Pathophysiology Key features Fibrous scarring of myocardial wall following transmural (ST-elevation) myocardial infarction Most commonly affects the LAD territory Deep Q waves & persistent ST elevation in leads corresponding with previous myocardial infarction LAD= left anterior descending artery. @UWorld  What is the most common cause of sudden cardiac arrest in the immediate post-infarction period in patients with acute MI? Re-entrant ventricular arrhythmias (e.g. ventricular fibrillation) Post-myocardial infarction ACE inhibition r--- + ! ACE inhibitor Angiotensin II remodeling (over weeks to months) LV dilation Reduced contractilefunction + Reduced LV dilation More preservedcontractilefunction LV " left ventricle ©UWorld Middle-aged man with ST-elevation MI complicated by extensive anterolateral akinesis who on day 5 of hospitalization develops bilateral lower extremity pain/paresthesias, cyanosis, hypotension, and weak upper extremity pulses with absent lower extremity pulses, most consistent with aortic embolism.  In general, based on cardiovascular disease CVD status, patients can be classified into 3 categories with regard to sexual activity: CVS 194 https://t.me/usmleinnercircle Version 2 • Low-risk patients can perform light-intensity exercise without symptoms and should be able to initiate or resume sexual activity. Examples include those with few CVD risk factors, controlled hypertension, asymptomatic left ventricular dysfunction, or successful revascularization of clinically significant lesions 50%60%. • High-risk patients should be referred for a detailed assessment prior to advising on activity. Examples include those with refractory angina, New York Heart Association class IV heart failure, significant arrhythmias, or severe valvular disease. • For indeterminate/intermediate-risk patients, stress testing is recommended to reclassify them as le low- or high-risk and to help guide decisions. In the period immediately following an acute MI, the myocardium may be vulnerable to increases in irc oxygen demand. Nonetheless, MI patients who have undergone successful revascularization or have no evidence of ischemia on exercise stress testing can be considered low-risk. They may safely resume sexual intercourse soon after the MI, within 34 weeks Princeton rC guidelines) and possibly as early as 1 week American Heart Association guidelines). Angina ne Classification of angina • Typical location (eg, substernal), quality & duration • Provoked by exercise or emotional stress • Relieved by rest or nitroglycerin Atypical • 2 of the 3 characteristics of classic angina Nonanginal In Classic • <2 of the 3 characteristics of classic angina U SM Atypical angina LE  What is the likely diagnosis in a male with a history of SLE that presents with epigastric burning provoked by exertion and relieved by rest? CVS 195 https://t.me/usmleinnercircle Version 2 Differential diagnosis & features of chest pain Coronary artery disease Pulmonary/pleuritic (pleurisy, pneumonia, pericarditis, PE) Aortic (dissection, intramural hematoma) Esophageal Chest wall/ musculoskeletal • Substernal • Precipitated by exertion . • . Relieved by rest or nitroglycerin Sharp/stabbing pain Worse with inspiration • Pericarditis: worse when lying flat • Abrupt (maximal at onset), severe "tearing" pain • May radiate to back • Hypertension and/or inherited aortopathy • Substernal, may refer to neck • Associated with regurgitation • Provoked by recumbent position • Nonexertional, relieved by antacids • Persistent pain • Worse with movement or change in position • Often follows repetitive activity PE = pulmonaryembolism.  What is the likely underlying etiology of chest pain for  1 hour, unrelated to activity in a young patient with normal cardiac and pulmonary exam? Esophageal disease (e.g. GERD features suggestive of esophageal origin include episodes lasting  1 hour, post-prandial symptoms, associated heartburn, and relief by anti-reflux therapy Pain radiation is a less reliable distinguishing factor. Pain fibers from the esophagus and heart (visceral afferents) overlap at multiple different spinal cord levels, where they converge with somatic nerves. Therefore, chest pain caused by esophageal or cardiac pathology can be referred to the arms, jaw, neck, and/or back. The Mediterranean diet is high in fresh fruits, vegetables, and legumes. It is proven to reduce cardiovascular morbidity and mortality in patients both with and without established coronary artery disease. CVS 196 https://t.me/usmleinnercircle Version 2 Risk factors for coronary heart disease (CHD) Most Important risk factors CHD risk equivalents . .. .. .. . Noncoronary atherosclerotic disease (eg, carotid, peripheral artery, abdominal aortic aneurysm) Diabetes mellitus Chronic kidney disease • Age (especially >50 in men & menopause in women) Hypertension Dyslipidemia Cigarette smoking le . Family history of CHD in first-degree relative age <50 (men) or age <60 (women) Obesity Vasospastic Angina Vasospasticangina irc CHD established risk factors Male sex rC • Hyperreactivity of coronary smooth muscle Pathogenesis • Young patients (age <50) • Smoking (minimal other CAD risk factors) Clinical presentation • Recurrent chest discomfort Occurs at rest or during sleep ne o o Spontaneous resolution s15 minutes • Ambulatory ECG: ST elevation Diagnosis • Coronary angiography: No CAD In • Calcium channel blocker (preventive) Treatment • Sublingual nitroglycerin (abortive) U SM Stable Angina LE • Propanolol is a β-blocker that may exacerbate vasospasm in Vasospastic Prinzmetal) angina. Treatment of chronic stable angina • First-line therapy Beta blockers • L Myocardial contractility & heart rate Nondihydropyridine CCBs • Alternative to beta blocker • L Myocardial contractility & heart rate • Added to beta blocker when needed Dihydropyridine CCBs • Coronary artery vasodilation • L Afterload by systemic vasodilation Nitrates Ranolazine • Long-acting added for persistent angina • L Preload by dilation of capacitance veins • Alternative therapy for refractory angina • L Myocardial calcium influx CAD/ ACS Diagnosis CVS 197 https://t.me/usmleinnercircle Version 2 Evaluation of chest pain Pretest probability of coronary artery disease Pretest probability of coronary artery disease Low (<10%) • Asymptomatic people of all ages • Atypical chest pain in women age <50 Intermediate (20%-80%) • Atypical angina in men of all ages • Atypical angina in women age ;,50 • Typical angina in women age 30-50 High (>90%) • Typical angina in men age ;,40 • Typical angina in women age ;,50 High Low Start pharmacologic therapy for CAD No additional diagnostic testing Pharmacologic stress imaging test I Expert evaluation I No Exercise imaging test Positive Positive Coronary angiography , UWorld  What is the best initial test for chest pain? ECG  What is the recommended diagnostic test for patients with chest pain that have low-risk for CAD? No further testing for CAD. (explore other pathologies) a positive stress test in low-risk patients is likely to be a false positive  What is the recommended diagnostic test for patients with chest pain that have high-risk for CAD? Coronary angiography CVS 198 https://t.me/usmleinnercircle Version 2 Diagnostic evaluation of suspected stable CAD Symptoms & risk factors suggest stable CAD Unable to exercise Exercise ECG stress testing* Pharmacologic stress testing irc le Able to exercise Positive No significantCAD** CAD present Medical management ± CA with revascularization (high-risk patients) rC Negative Risk factor reduction ne •Exercise imaging if significant baseline ECG abnormalities (eg, baseline ST depression). ··Pertorm corona,y angiography if high suspicion of false-negative result. CA = coronary angiography: CAD= coronary artery disease. ©UWOtld LE In Evaluation of chest pain in the emergency department • Focused history & physical examination • Assess vital signs • Obtain venous access Stable patient Unstable patient U SM • Obtain ECG & chest x-ray • Administer asprin if the risk for aortic dissection STEM! Treat with emergency catherization or thrombolysis • Stabilize hemodynamics • Check for under1yingcauses is tow ECG consistent with ACS? I No Yes I I Chest x-ray ragnostic? NSTEMI Treat with appropriate anticoagulation I Yes No Treat cause • Assess for pulmonary embolism • Check cardiac markers & risk stratify for ACS • Assess for pericarditis • Assess for aor1icdissection l l ACS= acutecoronarysyndrome;NSTEMI= non-ST-segmentelevationmyocardial,nfarclion, STEMI= ST-segmentelevationmyocardialinfarction. CVS 199 https://t.me/usmleinnercircle Version 2 • Aspirin: reduces the rate of myocardial infarction and overall mortality in patients with ACS Evaluation of suspected acute coronary syndrome in the emergency department Initial assessment: •ECG • Troponin ST elevation Ml or new LBBB Other ischemic changes or elevated troponin Negative findings Serial ECG and troponin Positive Admit for further management Urgent reperfusion ._l Negative ! Noninvasive stress test LBBB = left bundle branch block; Ml = myocardial Infarction. C>UWO<td Patients with risk factors for coronary artery disease and new symptoms such as angina or exertional dyspnea should be assessed for the presence of obstructive coronary disease by means of a stress test. For patients with a normal resting ECG who are able to exercise, an exercise stress test is preferred. For those unable to exercise or with abnormal ECGs, a pharmacologic nuclear stress test is frequently used  In patients with suspected ACS who go on to have acute myocardial infarction, the initial ECG (ideally obtained within 10 min of emergency department arrival) is often normal or nondiagnostic. Regardless of cardiac biomarker (eg, troponin) levels, changes (eg, ST-segment elevation, depression) on subsequent ECGs can develop quickly and may significantly affect patient management. Relative frequency of selected presenting symptoms in acute coronary syndrome Acute Coronary Syndrome E] IYes ST elevation I l Chest pain 80%-85% Dyspnea 70%-75% ! Nausea 40%-55% Cardiac biomarkers Vomiting 15%-20% Epigastric pain 10%-15% INo ST elevation I I ST elevation Ml 11 Non-ST elevation Ml 11 Unstable angina I Atypical symptoms associated Figure 3.2: Acute Coronary Syndromes Diagnosis Algorithm with MI are more common in women, the elderly, and diabetics. CVS 200 https://t.me/usmleinnercircle Version 2 Stress Test Exercise stress testing Factors associated with increased risk of adverse cardiovascular events Exercise-induced angina at low workload Fall in systolic blood pressure from baseline Chronotropic incompetence >1 mm ST depression (flat or downsloping) ST depression at low workload ST elevation in leads without Q waves Ventricular arrhythmias le ECG variables Poor exercise capacity irc Clinical variables .. .. .. .. Should undergo coronary angiography to evaluate for revascularization (either stent or bypass) .. Exercise ECG test Mechanism Best for Not for rC Type of stress HR • Patients able to reach BP tHR (!HR = 85% of 220 • Pacemaker -age) • Patients unable to reach • LBBB tHR dipyridamole • Nonselective adenosine agonist • LBBB • Reactive airway disease • Pacemaker • Patients on ne Pharmacologic stress test with adenosine or • Dilates coronary arteries • Patients unable to reach without j HR or BP • B-1 agonist echocardiography • T HR± BP • Reactive airway disease In Dobutamine stress dipyridamole or tHR theophylline • Tachyarrhythmias • Patients unable to reach IHR LE Medicationsto withhold prior to cardiacstresstesting Beta blockers, calcium channel blockers, nitrates Hold for 48 hours prior to vasodilator stress test Dipyridamole Hold for 12 hours prior to vasodilator stress test Caffeine-containing food or drinks Continue angiotensin receptor blockers, digoxin, stat ins, U SM Hold for 48 hours Angiotensin-converting enzyme inhibitors, diuretics  What classes of medications 3 should be held for 48 hours prior to cardiac stress testing? beta blockers, calcium channel blockers, and nitrates exception: continue these medications in patients with known CAD undergoing stress testing to assess the efficacy of anti-anginal medication Perfusion Test CVS 201 https://t.me/usmleinnercircle Version 2 Reversible myocardial perfusion defect : ::F ltlE AH_ ' L.:.T ·S.CP ' BASE STRESS IRHC(G) * * '\ • \I * • 'I ' 'I ·s.UPI" ,I ·'.EP E L•T ,,I 'I nl n1 E:.:.·:.E REST _IRHC 'I perfusion (I tracer uptake) during stress testing (top row) but not at rest (bottom row) C)UWo,ld  Technetium-99-labeled (Tc-99m) perfusion agents (sestamibi or tetrofosmin) have a half-life of 6 hours, passively diffuse into perfused myocardial cells, have minimal redistribution afterward, and can provide an assessment of both myocardial function and perfusion Any differences between rest and stress images can help identify ischemia. • Patients with normal tracer uptake at both rest and exercise have a low likelihood of ischemia with an excellent prognosis and 1% annual risk of coronary artery disease CAD. • A decreased tracer uptake both at rest and with exercise (fixed defect) indicates likely scar tissue with decreased perfusion and CAD. • A decreased tracer uptake with stress but normal uptake at rest (reversible defect) indicates inducible ischemia and likely CAD.  What is preferred treatment to prevent coronary artery disease in a patient with inducible ischemia? Anti-platelet therapy (e.g. aspirin), BBlocker and risk factor modification Murmur & Valvular Defects CVS 202 https://t.me/usmleinnercircle Version 2 Splitting of S2 Pathologic Physiologic Pathologic Narrowed splitting Widened splitting ?f' P2 A2 P2 l?P A2 ±?f' P2 [ A2P2 l w I DelayedA2 • Aortic stenosis • Systemic HTN •LBBB, HCM irc Delayed P2 • Pulmonic stenosis • Pulmonary HTN •Aso·, RBBB Or early A2 •VSD f l Paradoxical splitting \/Vldened splitting Expiration A2P2 le A2 Inspiration = Split is audibly c:iscernible rC "v..1denedsplitting is fixed throughoutrespiratorycyde. ASD = atria/ septal defect; HCM = hypertropniccardiomyopathy;HTN = hypertension; LBBB = lett boodle-branchblock, RBBB = right bundle-b<anchblock; VSD = ventricular septal defect. C)UWofld ne Benign vs pathologic murmurs Benign .. . • Infants; poor weight gain, Asymptomatic Normal growth In History .. . No significant family history • Early or midsystolic Musical or vibratory LE Murmur characteristics* U SM Other findings Management Grade 1-2 intensity Decreases or disappears with standing & Valsalva maneuver** • Normal vital signs • Normal S1 & S2 • Symmetric pulses . Pathologic Reassurance respiratory distress, difficulty feeding • Older children: exertional fatigue, . • .. . .. . • . chest pain, syncope Family history of SCD or CHO Holosystolic or diastolic Harsh Grade ;,3 intensity Intensity persists with standing & Valsalva maneuver Central cyanosis Loud, fixed, or single S2 Weak femoral pulses Hepatomegaly ECG & echocardiography •Any single pathologicfeature warrants evaluation. **Venous hum is a benign murmur exception that increases with standing. CHD = congenital heart defect; SCD = sudden cardiac death.  Common innocent murmurs include the following: • Still's murmur: systolic, vibratory, best heard over left lower sternal border, ↑ intensity when supine • Pulmonic flow murmur: systolic ejection, best heard over left upper sternal border, may radiate to axilla CVS 203 https://t.me/usmleinnercircle Version 2 • Venous hum: continuous, best heard over the supra- or infraclavicular area, ↓ intensity with neck rotation In peds world, if musical qualities to murmur dont worry thats benign you don't need to investigate it  What is the next step in management for an asymptomatic adult with an early diastolic murmur at the left sternal border that is best heard with expiration? Transthoracic echocardiogram diastolic and continuous murmurs are usually due to an underlying pathologic cause and their presence should prompt further evaluation Extra (gallop) heart sounds Associated pathology• Features • Heard just after S2 • Heart failure with reduced EF Caused by reverberant sound as blood fills an enlarged LV • • High-output states (eg, thyrotoxicosis) cavity during passive diastolic filling • Mitral or aortic regurgitation S3 • Heard just before S1 • Caused by blood striking a stiff LV wall during atrial S4 contraction • Concentric LV hypertrophy • Restrictive cardiomyopathy • Acute myocardial infarction *S3 can be normal in children, young adults & during pregnancy. S4 can be nonmalin the elderly (eg, age >70). EF = ejection fraction; LV = left ventricular.  What abnormal heart sound is often heard during the acute phase of myocardial infarction? S4 (atrial gallop) due to left ventricular stiffening and ischemia-induced myocardial dysfunction • S4: often referred to as "ten-nes-see" a sound, which corresponds to S4S1S2 • S3 often referred to as "ken-tuc-ky" a sound, which corresponds to S1S2S3 CVS 204 https://t.me/usmleinnercircle Version 2 Normal 100 en :i:: E §. Aorta ::l 50 f a. .., 0 0 iii Mitra! valve closes _ .Left atrium Left ventricle ------~ le Time Aortic stenosis irc Aortic regurgitation 150 150 ci ci :i:: :i:: i[ i[ Aortic .., .., 0 0 rC E .§_100 E .§_100 Aorta 2 50 2 50 ID ne ID Time <0UWorld Left atrium Time <0UWorld Murmur max at max pressure gradient In Mitral regurgitation 100 C) :,: Mitra! stenosis E .§. f 100 LE Aorta =50 a .., g iii Left atrium Left ventncle U SM Time Cl :,: E .§. i!? =50 a .., 0 0 iii Antithrombotic therapy in patients with mechanical heart valves Warfarin (goal INR: 2.0-3.0*) Warfarin (goal INR: 2.5-3.5) Low-dose aspirin . .. . . Aortic valve replacement if no risk factors present** Mitral valve replacement Aortic valve replacement with ~1 risk factor Aortic valve replacement with an old-generation valve Only in patients with another strong indication (eg, severe CAD) *A lower target INR (eg, 1.5) may be acceptable with some new-generation mechanical aortic valves. **Risk factors include atrial fibrillation, left ventricular systolic dysfunction, prior thromboembolism & presence of hypercoagulable state. CAD = coronary artery disease. CVS 205 https://t.me/usmleinnercircle Version 2 Acute mitral regurgitation Ruptured mitral chordae tendineae from: Mitral valve prolapse Mitral Regurgitation Infective endocarditis  What is the most common cause of mitral regurgitation in developed countries?Rheumatic heart disease Trauma Mitral valve prolapse Papillary muscle displacement or rupture due to MI Clinical features usually causes mild MR with a mid-systolic click and mid-to-late systolic murmur but can Rapid onset of pulmonary edema develop into severe MR with a holosystolic murmur on examination Acute LV failure (also causing acute RV failure) Hypotension, cardiogenic shock Physical examination  What are the (3) causes of acute mitral regurgitation post-MI? • Ischemic papillary muscle displacement (immediate) Jugular venous distension, pulmonary crackles Hyperdynamic cardiac impulse Apical systolic murmur (often absent) Diaphoresis, cool extremities (if shock is present) Management • Papillary muscle rupture (days) • LV aneurysm (days-weeks) Hemodynamic changes in mitral regurgitation Acute MR Compensated Decompensated chronic MR chronic MR Preload ii i i Afterload ! No change i Contractile function No change No change ! Ejection fraction ii i ! Forward stroke volume ! No change ! Bedside echocardiography Emergency surgical intervention MR= mitral regurgitation. Surgical indications for severe chronic mitral valve regurgitation Primary MR Secondary MR .. . Surgery if LVEF 30%-60% (regardless of symptoms} Consider surgery if successful valve repair* is highly likely: 0 Asymptomatic & LVEF >60% 0 Symptomatic & LVEF <30% Medical management, valve surgery rarely indicated *When possible, durable valve repair is favored over replacement because replacement necessitates lifelong anticoagulation & repeat replacement is often neededafter-10 yr. LVEF= left ventricularejectionfraction:MR = mitralregurgitation. Surgical indications for chronic severe mitral valve regurgitation . Primary MR Secondary MR Valve surgery• indicated for: o Symptoms present (eg, dyspnea on exertion) . o Severe LV dilation or LVEF s60% (regardless of symptoms) Medical management; valve surgery rarely indicated "'Valve repair is favored over prosthetic valve replacement whenever possible. LV = left ventricular;LVEF = LV ejectionfraction: MR= mitral regurgitation. Mitral Stenosis CVS 206 https://t.me/usmleinnercircle Version 2 Mitra! stenosis in adults • Rheumatic heart disease (vast majority of cases) • Age-related calcification, radiation induced • Exertional dyspnea, orthopnea, paroxysmal nocturnal dyspnea, hemoptysis Clinical presentation • Pulmonary edema± right-sided heart failure (eg, lower extremity edema) Etiology Treatment Atrial fibrillation, j risk for systemic embolization Opening snap with middiastolic rumble at the apex Echocardiography: j transmitral flow velocity Percutaneous valvotomy or surgical repair/replacement • Dyspnea, orthopnea, PND, hemoptysis • Atrial fibrillation, systemic thromboembolism irc Mitral stenosis Clinical features I le Diagnosis . . • . • Voice hoarseness from recurrent laryngeal nerve compression due to LAE (Ortner syndrome) rC • Mitral facies (pinkish-purple patches on cheeks) Physical examination • Loud S1, loud P2 if pulmonary hypertension • Opening snap (high-frequency early diastolic sound) • Mid-diastolic rumble (best heard at cardiac apex) Diagnosis ne • CXR: Pulmonary blood flow redistribution to upper lobes, dilated pulmonary vessels, LAE, flattened left heart border • ECG: "P mitrale" (broad & notched P waves), atrial tachyarrhythmias, RVH (tall R waves in V1 & V2) In • TTE: MV thickening/calcification/ i mobility, coexisting MR  What is the likely diagnosis in a young patient from a developing country that presents with dyspnea, hemoptysis, and occasional palpitations? LE Mitral stenosis (from rheumatic heart disease) Aortic Regurgitation U SM  AR murmur due to Root Dilation: RUSB  AR murmur (vulvur): Left Sternal Border CVS 207 https://t.me/usmleinnercircle Version 2 Chronic aortic regurgitation • Congenital bicuspid aortic valve Common etiologies • Postinflammatory (eg, rheumatic heart disease, endocarditis) • Aortic root dilation (eg, Marfan s:i-:ndrome,syphilis) • Backflow from aorta into LV --+ t LV end-diastolic volume Pathophysiology Clinical findings • LV initially compensates with eccentric hypertrophy --+ j SV & CO • Eventual LV dysfunction --+ t SV & CO --+ heart failure • Decrescendo diastolic murmur • Widened pulse pressure (jSBP & tDBP) • Rapid rise-rapid fall ("water-hammer") pulsation • Abrupt carotid distension & collapse, "pistol-shot" femoral pulses CO = cardiacoutput; DBP = diastolic blood pressure;LV = left ventricle;SBP = systolic blood pressure;SV = stroke volume. Left lateral decubitus position brings the enlarged LV closer to the chest wall, increasing awareness of the heartbeat Pounding Heart) Prominent capillary pulsations in the fingertips or nail beds can be seen with aortic regurgitation AR as a result of widened pulse pressure. Aortic Stenosis  What is the likely diagnosis in a young patient with exertional chest pain, a systolic murmur at the first right intercostal space, and a palpable thrill in the suprasternal notch? Supravalvular aortic stenosis Usually refers to congenital LV outflow tract obstruction; LV hypertrophy develops overtime and increased myocardial O2 demand causing exertional angina. Can be associated with Williams Syndrome (hypersociability, elfin facies) Other findings: • Suprasternal notch thrills • Right arm > left arm BP (high pressure jet in ascending aorta), • Unequal carotid pulses • Can also have coronary artery stenosis as an associated anomaly. • Causes a systolic murmur similar to that seen with valvular AS; however, the murmur is usually best heard at the first right intercostal space, higher than where the valvular AS murmur is best heard. CVS 208 https://t.me/usmleinnercircle irc le Version 2 rC Discrete supravalvar aortic stenosis in a patient with Williams syndrome. This catheter Aortic stenosis • Calcific disease (most common, age >70) • Congenital bicuspid valve (younger patients) Causes • Rheumatic heart disease (common worldwide but rare in developed countries) ne • Dyspnea on exertion, decreased exercise tolerance • Angina pectoris (severe AS) Clinical manifestations • Syncope (severe AS) In • Heart failure (severe AS) • Crescendo-decrescendo systolic murmur best heard at RUSB with radiation to carotids & Physical examination • Aortic valve replacement for severe AS causing symptoms or LV systolic dysfunction LE Management axillae • Slow-rising (delayed) & weak carotid pulse AS = aortic stenosis; LV = left ventricular; RUSB = right upper sternal border. U SM Atenolol is a cardioselective -adrenergic blocker. It may exacerbate symptoms of heart failure in patients with aortic stenosis by reducing ejection fraction. Valve replacement in aortic stenosis • Aortic jet velocity 2:4.0 m/sec, or • Mean transvalvular pressure gradient 2:40 mm Hg Severe AS criteria • Valve area usually ::.1.0 cm2 but not required Severe AS & 2:1of the following: Indications for valve replacement • Onset of symptoms (eg, angina, syncope) • Left ventricular ejection fraction <50% • Undergoing other cardiac surgery (eg, CABG) AS = aortic stenosis;CABG = coronaryartery bypass grafting. • Physical examination findings suggestive of severe AS include: CVS 209 https://t.me/usmleinnercircle Version 2 • diminished and delayed carotid pulses ("pulsus parvus et tardus") • late-peaking, crescendo-decrescendo systolic murmur • soft and single S2 during inspiration D/D Angina d/t CAD but first rule out AS in old patients with Echo. Treadmill Test is C/I for Severe AS Physical examination features of LVOT obstruction due to aortic stenosis & hypertrophic cardiomyopathy Aortic stenosis Hypertrophic cardiomyopathy L Murmur intensity t Murmur intensity L Murmur intensity ! Murmur intensity 54 Often present Often present Ejection click* Usually present Absent Carotid pulses* Soft & delayed Brisk Decreased preload* (eg, Valsalva strain phase) Increased afterload (eg, handgrip) *Distinguishing feature. LVOT = left ventricular outflow tract. Transcatheter aortic valve implantation Positioning Expansion & placement Function For severe Aortic Stenosis Complications:  AR  Stroke (due to embolization of native valve calcification) Biscuspid Aortic Valve CVS 210 https://t.me/usmleinnercircle Version 2 .. . .. . .. Epidemiology Clinical manifestations Usually familial with autosomal dominant inheritance Affects -1 % of population, more common in males Strong association with Turner s:z:ndrome Accelerated valve calcification with early stenosis (eg, age >40) Aortic regurgitation can develop (less common than stenosis) Increased incidence of ascending aortic aneurysm Serial echo to monitor valve function & aneurysm development Echocardioaraohic screenina of first-dearee relatives le Management Bicuspid aortic valve irc  What is the likely underlying cause of an early decrescendo diastolic murmur in a young patient in the U.S. with a family history of heart disease? Congenital bicuspid aortic valve rC the murmur is typically best heard while the patient is sitting up, leaning forward, and holding a breath in full expiration  What is the likely underlying cause of a harsh systolic murmur (right second intercostal space) with radiation to the carotid arteries in a young patient in the U.S.? ne Bicuspid aortic valve bicuspid aortic valve is the cause of aortic stenosis in the majority of patients under 70 years old In In young patients, it can lead to Aortic Regurgitation and in older patients can lead to aortic stenosis • Balloon valvuloplasty is indicated in symptomatic and asymptomatic (if they plan to become • Aortic stenosis LE pregnant or participate in competitive sports) young adults when the following criteria are met: • No significant AV calcification or aortic regurgitation • Peak gradient 50 mm Hg U SM Pulmonary Regurgitation Etiologies Pathophysiology Clinical findings Treatment Chronic pulmonic regurgitation • Correction of pulmonic stenosis or RVOT obstruction (iatrogenic)* • Rheumatic heart disease, infective endocarditis • Progressive RV volume overload _, RV dilation & eventual failure • Decrescendo, diastolic murmur at LSB, t with inspiration • Soft (or sometimes absent) P2 • Dyspnea & prominent parasternal impulse (once RV dilation develops) • Serial echocardiography • Valve replacement once symptoms or RV dysfunction develops *Most common cause of severe pulmonic regurgitation. LSB = left sternal border,RV= right ventricular,RVOT = right ventricularoutflow tract. CVS 211 https://t.me/usmleinnercircle Version 2 Pulmonary Stenosis Pulmonic valve stenosis Etiology Clinical presentation Diagnosis Treatment .. .. .. . .. Congenital (usually isolated defect) Rarely acquired (eg, carcinoid) Severe: Right-sided heart failure in childhood Mild: Symptoms (eg, dyspnea) in early adulthood Crescendo-decrescendo murmur (j on inspiration) Systolic ejection click & widened split of S2 Echocard iography Percutaneous balloon valvulotomy (preferred) Surgical repair in some cases ECG Tracings AV nodal re-entry tachycardia (AVNRT) Atrial flutter (most common around tricuspid annulus) AV re-entry tachycardia (re-entry through accessory bundle) ECG JPEDIA.ORG CVS 212 https://t.me/usmleinnercircle Version 2 Estimating ventricular rate on ECG Method 1: large boxes between QRS complexes Start 300 150 100 75 60 50 VS 5 sec. Rate (beatS/min) = "Typical ECG strip is 10 seconds long (ie, 50 large boxes). ©UWorld In PSVT 10 sec. Number of QRS complexes x 6 (18 x 6 = 108 in this case) ne 1 sec. rC Method 2: QRS complexes per 10 seconds* irc 300 Rate (beats/min)= ----------------Number of large boxes between QRS complexes le ~--f--~--~---~--•--i • Paroxymal supraventricular tachycardia (PSVT) is a type of arrhythmia arising from a defect in the atrioventricular conduction that causes the heart to sporadically beat faster. LE • AVNRT = dysfunctional AV node that contains two electrical pathways ⟶ leads to circulating electrical impulses • AVRT = tachy caused by accessory pathway between atria and ventricles WPW U SM • Atrial tachy = atria respond to impulses from outside of SA node • AFib and Aflutter = types of supraventricular tachy CVS 213 https://t.me/usmleinnercircle Version 2 Atrioventricular nodal reentrant tachycardia t t t n Pathophysiology Key features Dual AV node conduction pathways (fast & slow) creating a reentrant circuit • Triggered by a premature atrial contraction 120-180 bpm Regular R-R intervals P waves buried within the QRS complexes Narrow QRS complexes T/t: Adenosine. Best distinguished from sinus tachycardia by a HR  150 • Paroxysmal Supraventricular Tachycardias (AVNRT, AVRT) can be treated in a hemodynamically stable patient with vagal maneuvers such as valsalva, carotid massage, and diving reflex (cold-head immersion). These work by increasing parasympathetic tone in the heart, leading to a slowing of conduction and increase in refractory period in the AV Node, leading to termination.  What is the likely diagnosis in a young patient with abrupt onset tachycardia and palpitations? Paroxysmal supraventricular tachycardia PSVT typically due to an atrioventricular nodal re-entrant tachycardia Atrial Fibrillation Atrial fibrillation with rapid ventricular response n f···+-+·+···i Pathophysiology Key features Chaotic electrical activity (fibrillatory waves) with irregular transmission to the ventricles • Aberrant foci commonly originate in pulmonary veins CVS >100 bpm Irregularly irregular R-R intervals Fibrillatory waves: no organized P waves 214 https://t.me/usmleinnercircle Version 2 Conditions associated with atrial fibrillation • Hypertensive heart disease (most common) • Coronary artery disease • Rheumatic/Valvular heart disease (eg, mitral stenosis, mitral regurgitation) Cardiac • Congestive heart failure • Hypertrophic cardiomyopathy • Congenital heart disease (eg, atrial septal defect) • Post cardiac surgery • Obstructive sleep apnea le • Pulmonary embolism Pulmonary • Chronic obstructive pulmonary disease • Acute hypoxia (eg, pneumonia) Miscellaneous irc • Obesity • Endocrine (eg, hyperthyroidism, diabetes) • Alcohol abuse rC • Drugs (eg, amphetamines, cocaine, theophylline) Pharmacologic rate control of atrial fibrillation Start beta blocker or nondihydropyridine (eg, verapamil, diltiazem) ne ccs· I Additional rate control needed? • In • Add another first-line drug of different class • Consider digoxin in patients with HFrEF I LE Adequate rate control still not achieved? • • Add amiodarone .. • Consider other measures (eg, ablation procedure) • Nondihydropyridine CCBs contraindicated in HFrEF •• Amiodarone relatively contraindicated in COPD & other chron,c lung disease U SM AV= atrioventricular, CCB = calcium chamel blocker, COPD = clYonlc obsll\Jcbve pulmonary disease; HFrEF = heart failure with reduced ejection fraction. CVS ©UWorld 215 https://t.me/usmleinnercircle Version 2 Comorbidities that encourage atrial fibrillation • Advanced age • Systemic hypertension Precipitants of atrial dilation &/or conduction remodeling • (Moot It>vf...-l-..,i:..) Mitral valve dysfunction • Left ventricular failure • Coronary artery disease & related factors (eg, OM, smoking) • Obesity & obstructive sleep apnea • Chronic hypoxic lung disease (eg, COPD) • H:r:eerth:r:roidism • Excessive alcohol use • Increased sympathetic tone Triggers of increased automaticity 0 Acute illness (eg, sepsis, PE, Ml) 0 Cardiac surgery • Sympathomimetic drugs (eg, cocaine) COPD = chronic obstructive pulmonary disease; DM = diabetes mellitus; Ml = myocardial infarction; PE= pulmonary embolism. Patients with new-onset AF should have TSH and free T4 levels measured. Long-term management of atrial fibrillation Rate-control strategy* • Atrioventricular node blockade 0 0 0 Beta blockers Nondihydropyridine Digoxin CCBs • Anticoagulation usually indicated Rhythm-control strategy* • Electrical cardioversion • Pharmacologic antiarrhythmics . 0 Flecainide, amiodarone Radiofrequency ablation • Anticoagulation often indicated •clinical outcomesfor each strategyare roughlyequivalent.Choiceof strategyis typicallybasedon case particulars(eg, durationof atrialfibrillation)& patient preference. CCBs = calciumchannelblockers.  What is the initial therapy for patients with atrial fibrillation due to hyperthyroidism? Beta blockers helps control heart rate and hyperadrenergic symptoms; should be continued until the patient becomes euthyroid with thionamides, radioiodine, and/or surgery CVS 216 https://t.me/usmleinnercircle Version 2 CHA 2DS2-VASc score for thromboembolic risk in nonvalvular atrial fibrillation Risk criteria Points Congestive heart failure 1 H Hypertension 1 A2 Age ?.75* 2 D Diabetes mellitus 1 S2 Stroke or TIA 2 V Vascular disease (eg, PAD, prior Ml) 1 A Age 65-74* Sc Sex category female le C 1 1 9 irc Maximum score Total score Female** 0 0 Low 1 2 Moderate ?.2 ?.3 High Antithrombotic therapy rC Generalized stroke risk Male None None or oral anticoagulant Oral anticoagulant factors are present (female patients cannot have a total score of 1). ne 'Patients are assigned to 1 of the 2 age categories. .. Different cutoffs are used for males & females. Female sex is considered a risk modifier that adds to the CHA2 DS2-VASc score only if other nonsex risk Ml; myocardial infarction; PAD; peripheral artery disease; TIA; transient ischemic attack. In Oral Anticoagulation: Warfarin or Dabigatran, Rivaroxaban, Apixaban PAC LE • Valvular Atrial Fibrillation: Warfarin; regardless of any score U SM Premature atrial contractions (PACs) Irregular rhythm PAC  Also known as a PAC or Atrial Premature Beat, these occur when part of the atria sends out a depolarisation wave a little too early. Clues include: 1. The P wave of the PAC looks different from the other P waves, since it starts at a diff location in the atria 2. The P wave and subsequent QRS complex come earlier than would be expected. CVS 217 https://t.me/usmleinnercircle Version 2 3. The QRS and T wave are followed by a relative pause before the SA node repolarises and initiates a normal beat. Hence, the Normal, Short, Long pattern.  What is the recommended management for a patient with a smoking/drinking history and atrial premature beats discovered on routine ECG (asymptomatic)? Avoid alcohol and tobacco this is a benign arrhythmia that doesn't require treatment, however reversible risk factors should be avoided If symptomatic, beta-blockers may be helpful. MAT • Multifocal atrial tachycardia MAT is a supraventricular tachyarrhythmia that likely occurs due to atrial conduction abnormalities triggered by disturbances such as right atrial enlargement, catecholamine surge (eg, sepsis), or electrolyte imbalance. It is most commonly seen in elderly (age 70 patients with an acute exacerbation of underlying pulmonary disease. The best treatment is appropriate management of the inciting disturbance; the administration of bronchodilators, systemic corticosteroids, and supplemental oxygen/ventilatory support for patient's acute exacerbation of COPD can lead to resolution of the MAT. Tachycardia itself is Asymptomatic) Multifocal atrial tachycardia n II Pathophysiology Key features • Ectopic atrial foci driven by acute illness • Most commonly seen in COPD exacerbation • Atrial rate > 100/min • 2:3different P wave morphologies • Irregular R-R intervals WPW  Acute treatment of AF in patients with WPW is aimed at prompt control of ventricular response and termination of AF as follows: • Hemodynamically unstable patients require immediate electrical cardioversion • For stable patients, rhythm control with anti-arrhythmic drugs such as intravenous ibutilide or procainamide is preferred • Curative: Catheter radiofrequency ablation • AV nodal blocking agents such as adenosine, beta blockers, CCB (especially verapamil), and digoxin should not be used for AF in WPW CVS 218 https://t.me/usmleinnercircle Version 2 may promote conduction across the accessory pathway and lead to degeneration of AF into VF. Ventricular PVC irc le Duration of ventricular diastole prior to each beat CIUWorld rC The beat marked by the letter Y in this patient is a premature ventricular contraction PVC, recognized by a wide QRS complex, opposite-facing T wave, and brief ensuing sinus pause (ie, delayed P wave). PVCs may result from increased ventricular automaticity leading to spontaneous ventricular depolarization. Occasional PVCs are normal and considered benign. ne When a PVC occurs, it interrupts diastolic filling of the left ventricle, resulting in decreased enddiastolic volume EDV compared to the previous normal beat (beat X  Y). The sinus pause that occurs after a PVC allows for longer-than-normal filling time before the next ventricular In contraction (ie, prolonged ventricular diastole), which creates increased EDV at the time of the post-PVC beat (beat Z  X). The greater-than-normal EDV causes a large-stroke volume ventricular ejection, which may be responsible for the palpitations that some patients experience LE with PVCs. Monomorphic Ventricular Tachycardia U SM Monomorphic nonsustained ventricular tachycardia Identification Predisposing conditions Precipitating factors • .:3 consecutive ventricular beats (wide QRS complex) • Rate >100/min & duration <30 sec • Heart failure with reduced ejection fraction • lschemic heart disease • Other cardiomyopathy (eg, HCM) • Serum electrolyte abnormalities (eg, l K, l Mg) • Hypoxemia or acute myocardial ischemia • Elevated sympathetic tone (eg, sepsis) Management • Address precipitating factors (eg, replace electrolytes) • Consider adding (or increasing dose of) beta blockade HCM = hypertrophiccardiomyopathy. CVS 219 https://t.me/usmleinnercircle Version 2 Types of beats in monomorphic ventricular tachycardia ECG morphology Physiology Ventricular beat • Ventricles fully depolarized by ventricular impulse • Upward transmission blocked by AV node • P waves created by sinoatrial rhythm are obscured Fusion beat (rare) • Ventricles depolarized partially by ventricular impulse & partially by sinoatrial impulse • Preceding P wave usually visible (arrow) • Confirms the presence of 2 separate rhythms Capture beat (rare) • Ventricles fully depolarized by sinoatrial node impulse • Preceding P wave usually visible (arrow) CUWorld Monomorphic nonsustained ventricular tachycardia ~--+-+--1 Pathophysiology Key features Underlying predisposing condition (eg, cardiomyopathy, ischemic disease) • Precipitating factor (eg, hypokalemia, increased sympathetic drive) ~3 ventricular beats (regular, wide-complex) Rate >100/min & duration <30 seconds Spontaneous return to baseline rhythm Monomorphic ventricular tachycardia Key features Pathophysiology • Aberrant conduction focus within the ventricles • Typically seen with cardiomyopathy or ischemia 100-240 bpm Regular R-R intervals P waves usually obscured by QRS complexes Wide QRS complexes (>120 ms/3 small boxes) Fusion beats may be occasionally seen Torsade de Pointes  What is the first-line therapy for conscious and stable patients with torsades de pointes? IV magnesium if patient is hemodynamically unstable, immediate defibrillation is indicated CVS 220 https://t.me/usmleinnercircle Version 2 Torsade de pointes (polymorphic ventricular tachycardia) n Key features Long QT interval creates a prolonged "vulnerable period" during which a PVC can trigger arrhythmia Ventricular depolarization varies in a cyclical fashion Rate 200-250/min Wide QRS complexes with cyclical variation in morphology P waves obscured by QRS complexes Irregular R-R intervals le Pathophysiology PVC = premature ventricularcontraction. disorders Bradyarrhythmias irc rC Tricyclic antidepressants Selective serotonin reuptake inhibitors (eg, citalopram) Antiarrhythmics (eg, amiodarone, sotalol, flecainide) Antianginal drugs (eg, ranolazine) Anti-infective drugs (eg, macrolides, fluoroquinolones, antifungals) Electrolyte imbalances (L potassium, L magnesium, L calcium) Starvation Hypothyroidism Sinus node dysfunction .. Atrioventricular block (2nd or 3rd degree) Hypothermia Myocardial ischemia/infarction lntracranial disease HIV infection • Jervell & Lange-Nielsen syndrome (autosomal recessive) U SM Inherited Antipsychotics (eg, haloperidol, quetiapine, risperidone) LE Others . .. . . .. Diuretics (due to electrolyte imbalances) Antiemetics (eg, ondansetron) ne Metabolic Causes of acquired long QT syndrome In Medications .. . .. . .. ©UWorld • Romano-Ward syndrome (autosomal dominant)  When possible, patients receiving intravenous haloperidol or other QT-prolonging medications, particularly individuals at increased risk for arrhythmia, should undergo ECG testing prior to administration. The medication must be withheld if the baseline-corrected QT interval is prolonged (eg, 450 msec). • Treatment for patients with prolonged QT intervals includes beta blocker therapy and pacemaker placement. beta blockers limit extertional heart rate and shorten the QT interval; additional management should also include avoidance of electrolyte derangements, vigorous exercise, and medications that block K channels. CVS 221 https://t.me/usmleinnercircle Version 2 Bradycardia  What is the initial treatment for symptomatic sinus bradycardia? IV atropine Management of symptomatic sinus bradycardia Pulse <SO/min & symptoms present l 7- Hemodynamically No I Yes Investigate & treat underlying cause (eg, sinus node dysfunction acute Ml, medication toxicity) Give atropine 1 mg IV (can repeat every 3-5 min p to 3 mg total) l '----P_e_rs_i_s_te_n_t_h_e_m_o_d..:.y_n_a_m_ic_i=n=s=ta=b=il=ity==--..J] - No I Yes ... Initiate transcutaneous or transvenous pacmg ·1v infusion of dopamine 5-20 mcg/kg/min or pmephnne 2-10 mcg/min may also be attemp ed prior to temporary pacing. IV= intravenous; Ml = myocardial infarction. eUWor1d Sinus bradycardia n_-v,.-- t t II Pathophysiology • Slow generation of impulses within the sinoatrial node • Common causes include sick sinus syndrome & medication adverse effect Key features • <60 bpm • Regular rate and rhythm • P wave precedes each QRS complex Tachyarrythemia and Cardiac Arrest CVS 222 https://t.me/usmleinnercircle Version 2 Sinus Tachycardia P waves usually before QRScomplex Ventricular rate;, 100 beats/min P waves are usually upright in leads I, II, aVF,V3-V6 Maximal heart rate in sinus tachycardia can be determined (beats/min= 220 - age) r1~rrri lM-u•Vl ~f1 I"' ,.,, .. I I 1 ltJn I n .LLLU 1 ne rC irc I le ;~""' U SM LE In © USMLEWorid, LLC CVS 223 https://t.me/usmleinnercircle Version 2 Management of adult tachycardia with a pulse (ACLS guidelines) Sustained rapid tachyarrhythmia l • Airway management & supplemental 0 2 as needed • Assess cardiac rhythm l Seek underlying cause & treat Sinus tachycardia? j-Yes----. . I No • Hemodynamic -Yes---. instability present?' Synchronized cardioversion I No • Vl/idened ORS complexes (>0.12 sec)? -i No Yes Regular rhythm? Regular rhythm? t i No • Likely Afib or Aflutt~ • Rate control agen~ I t 7 Yes • Non-Afib SVT (eg,AVNRT) • Vagal maneuvers &/or adenos1ne 1 i No Yes Afib with aberrancy, preexcjledAfjb. or PMVT • Expert consultation • Rhythm-specific therapy> Likely monomorphic VT • Expert consultation • Pharmacologic cardioversion' • • Hemodynamic instabillty usuaUyOCCU'S onlywithheartrate> 150/min. ' Beta blockeror nondihydropyMdine calcium channel blocker. 'Rate control for Afib with aberrancy, proca,namlde for preel«:lted A b, mag,esoum for PMVT • AmlOdarone, procainamlde, lidoca,ne, or sotalol ACLS = advanced cardiovascular life suppoM: Afib = atnal fibnllatlO<l;Aflutter= atnal Hutter; AVNRT = atnoventricular nodal reentranttachycardia;PMVT = polymorphic ventriculartachycardia;SVT = supraventncular tachycardia;VT= ventncular tachycardia. • Synchronized cardioversion Used for all wide- or narrow-based tachyarrhythmias --unstable AFib, AFlutter, atrial tachy, and SVT's Energy delivered is synchronized to the QRS complex. Synchronization avoids the delivery of a LOW ENERGY shock during cardiac repolarization (t-wave). If the shock occurs on the t-wave (during repolarization), there is a high likelihood that the shock can precipitate VF Ventricular Fibrillation) • Defibrillation (unsynchronized) is used when there is no coordinated activity in the heart (pulseless VT/VF) CVS 224 https://t.me/usmleinnercircle Version 2 Approach to adult cardiac arrest Start CPR, give oxygen & attach monitor/defibrillator ! i VF/pulseless VT PEA/asystole ! Defibrillation shock• L ! • Pulse & rhythm check every 2 min • Treat reversible causes ROSC ·Amkxtarone given after 3rd defibrillation shock. l rC j ! Shockable rhythm irc le • CPR x2 min • Airway, IV/10 access • Epinephrine every 3-5 min Unshockable rhythm 10 = intraosseous; IV= intravenous; PEA= pulse-lesselectrical activity; ROSC = return of spontaneous circulation; VF = ventricular fibrillation; VT = ventricular tachycardia. ne ©UWorld • Pulseless electrical activity PEA is the presence of an organized rhythm (eg, atrial fibrillation) on In cardiac monitoring without a palpable pulse (or measurable blood pressure) in a cardiac arrest patient. Reversible causes of asystole/pulseless electrical activity 5 Ts LE 5 Hs Ventricular fibrillation Chaotic, irregular waveforms of varying shapes & amplitude No identifiable P waves, QRS, or T waves Hypovolemia Tension pneumothorax Hypoxia Tamponade, cardiac Hydrogen ions (acidosis) Toxins (narcotics, benzodiazepines) Hypokalemia or hyperkalemia Thrombosis (pulmonary or coronary) Trauma U SM Hypothermia Factors associated with poor outcome after witnessed out-of-hospital sudden cardiac arrest .. . .. . • Time elapsed prior to effective resuscitation {delayed bystander CPR, delayed defibrillation) Initial rhythm of pulseless electrical activity or asystole Prolonged CPR (>5 min) • Absence of vital signs • Advanced age History of cardiac disease • ;,2 Chronic illnesses Persistent coma after CPR Need for intubation or vasopressors Pneumonia or renal failure after CPR • Sepsis, cerebrovascular accident, or class Ill or IV heart failure CPR; cardiopulmonary resuscitation. CVS 225 https://t.me/usmleinnercircle Version 2  American Heart Association guidelines recommend that untrained lay rescuers should perform compression-only CPR prior to arrival of emergency personnel. Heart Block Atrioventricular block Clinical presentation ECG features Management Asymptomatic PR interval prolongation Observation First degree Mobitz type I second degree (rarely PPM ORS complex placement) Mobitz type II second Fatigue, lightheadedness, Constant PR interval with randomly dropped ORS degree syncope complexes Fatigue, lightheadedness, Third degree (complete) Observation Progressive PR interval lengthening followed by dropped Usually asymptomatic Complete dissociation of P waves& ORS complexes syncope PPM placement PPM placement PPM = permanentpacemaker. Junctional rhythm n •·········+·········I vs t t t Pathophysiology Key features Usually sinoatrial node dysfunction or atrioventricular (AV) block Impulse initiated by AV node or His bundle after timely conduction not received from above Rate of 40•60 bpm Regular rhythm with narrow QRS complex No normal P waves preceding QRS Retrograde P waves may be visible adjacent to QRS complex* •oue to retrograde depolarization of the atria starting from the AV junction. ©UWorld 1st Degree First•degree atrioventricular n,~.. f·······l········I (AV) block f·······i········I -"-"-'1---"-"-'1-"-..A..J\.-""'-"'--'\.--A-f'--'~~ H H H .. -"-"-'\.-'~\-J'-A.--'l--"-"~l-"-..A..J Pathophysiology Key features • Slowed conduction through the AV node • Common causes include enhanced vagal tone and pharmacologic AV node blockade • Constant P-P intervals with P wave preceding each QRS • Prolonged PR interval >0.2 sec (one large box) • Regular rhythm with constant R-R interval  What is the recommended management for a patient with first-degree AV block and a normal QRS duration  120 msec)? Observation CVS 226 https://t.me/usmleinnercircle Version 2 likely due to delayed AV node conduction; prolonged QRS indicates delay below the AV node and warrants electrophysiology testing 2nd Degree Mobitz type II Level of block Usually AV node Below the level of AV node (eg, bundle of His) ECG findings Progressive prolonged PR interval leads to a nonconducted P wave ("group beating") PR interval remains constant with intermittent nonconducted P waves QRScompleK Narrow Narrow or wide Exercise or atropine Improves type I AV block Worsens type 11AV block Vagal maneuvers (carotid sinus massage) Worsens type I AV block Risk of complete heart block Low nsk rC irc Mobitz type I le Second-degree AV block: Distinguishing features of Mobitz type I & type II AV block Paradoxically improves type II AV block ne Higher nsk, 1nd1cat10n for pacemaker  In type 1, The block usually is located within the AV node, and causes include elevated vagal tone In (eg, highly trained athletes), excessive pharmacologic AV nodal blockade (eg, beta blockers), ischemia, and age-related degeneration of the conduction system LE T/t: Observation and correction of reversible causes Mobitz type I second degree AV block (Wenckebach) U SM l········l···-···+·······l H 1--~ 1---1 1---~ I·-··· Pathophysiology Key features Disrupted conduction, usually within the AV node Causes include enhanced vagal tone, pharmacologic AV node blockade, ischemia, age-related conduction degeneration Constant P-P interval Progressive PR interval lengthening followed by QRS ,01 Slight progressive RR interval shortening Usually narrow QRS complexes Complete Heart Block  What is the management in a stable patient with dizziness, weakness, and the ECG findings below? Complete heart block should be managed with a temporary pacemaker Manage with a temporary pacemaker while undergoing further evaluation to identify and correct reversible causes. Per AMBOSS, unstable patients should be given atropine. CVS 227 https://t.me/usmleinnercircle Version 2 a permanent pacemaker is indicated if no reversible causes of heart block are found on further evaluation Third degree (complete) AV block P wave-QRS complex dissociation t - - - f - - - - - - f - - - t--- f - - - t --- P waves (atrial rhythm) n~~·~•,--..J1..-,,J.________,, 1u - - - - - - - -t - - - - - - - - t - - - - - - - - l QRS complexes Ounctional escape rhythm) _J @UWorld EKG findings in complete AV block • There is a complete failure of atrial impulses (p waves) to capture the ventricles (ORS) • Unless an escape rhythm is initiated, ventricular asystole (syncope/death) will occur • The escape rhythm (ORS) can be narrow uunctional escape) or wide (ventricular escape) • The p waves have no relation to ORS complexes (complete AV dissociation) • Ventricular rate is always slower than the atrial rate and is usually < 50  Sudden onset results in asystole , which may lead to Stokes-Adams Attacks (sudden loss of consciousness) Sick Sinus Syndrome Clinical features ECG findings Treatment .. . .. . .. Sick sinus syndrome Elderly patients Bradycardia o Fatigue, dyspnea, dizziness, syncope Bradycardia-tachycardia syndrome o Atrial arrhythmias (eg, atrial fibrillation) 0 Palpitations Sinus bradycardia Sinus pauses (delayed P waves) Sinoatrial nodal exit block {dropped P waves) Pacemaker +/- Rate-control medication (if tachyarrhythmias) Heart Failure New York Heart Association heart failure classification Class I No symptomatic limitation of physical activity Class 11 Slight limitation of physical activity (eg, dyspnea with climbing stairs) Class 111 Marked limitation of physical activity (eg, dyspnea with house chores) Class IV Inability to perform physical activity without significant discomfort CVS 228 https://t.me/usmleinnercircle Version 2 • Cardiac resynchronization therapy with simultaneous pacing of the right and left ventricles (biventricular pacing) has been shown to improve exercise tolerance and NYHA functional class, and reduce the rates of recurrent hospitalization and overall mortality. Current guidelines recommend biventricular pacing devices for patients in sinus rhythm who meet all of the following criteria: • LV ejection fraction 35% • NYHA class II, III, or IV heart failure symptoms (ie, the presence of any symptoms) Indications for implantable cardioverter-defibrillator Secondary prevention .. Prior myocardial infarction & LVEF S30% NYHA class II or Ill symptoms & LVEF S35% irc .. Primary prevention placement le • Left bundle branch block with QRS duration 150 msec. Prior VF or unstable VT without reversible cause Prior sustained VT with underlying cardiomyopathy rC LVEF= left ventricularejectionfraction; NYHA = New York HeartAssociation;VF = ventricularfibrillation; VT= ventriculartachycardia. ne ICD placement is recommended in patients with symptomatic heart failure and LV ejection fraction 35%, regardless of MI history. In Systolic failure Clinical findings for heart failure Poor prognostic factors in systolic heart failure Dyspnea Serum BNP >100 pg/ml High Low High Moderate Moderate Moderate U SM Lower extremity edema LE Sensitivity Specificity Jugular venous distension Moderate Moderate Pulmonary crackles Moderate Moderate Orthopnea Moderate High Audible S3 Low High Clinical Laboratory • • • • • • • Higher NYHA functional class Resting tachycardia Presence of S3 gallop Elevated jugular venous pressure Low blood pressure(< 100/60 mm Hg) Moderate to severe mitral regurgitation Low maximal oxygen consumption (peak VO 2 ) • Hyponatremia • Elevated pro-BNP levels • Renal insufficiency Electrocardiography • QRS duration >120 msec • Left bundle branch block pattern Echocardiography • • • • BNP = brain natriuretic peptide. Associated conditions Severe LV dysfunction Concomitant diastolic dysfunction Reduced right ventricular function Pulmonary hypertension • Anemia • Atrial fibrillation • Diabetes mellitus BNP level can be falsely low in patients with heart failure and obesity because BNP undergoes increased clearance by fat cells CVS 229 https://t.me/usmleinnercircle Version 2 • Chronic hyponatremia is common in patients with HFrEF; it parallels disease severity and is an independent predictor of mortality. Patients with HFrEF and serum sodium 130 mEq/L have an estimated 1-year survival as low as 15%. It likely results primarily from ongoing and profound nonosmotic stimulation for ADH secretion in the setting of reduced renal perfusion. T/t: Fluid Restriction, DO NOT GIVE SALT TABLETS Initial management of acute decompensated heart failure Recognize clinical presentation • Oyspnea. orthopnea, S3, JVO, lung crackles, LE edema • Diagnostic studies: chest x-ray, BNP l j Evaluate clinlcal stability Improve symptoms (reduce preload) • Respiratory failure -+ supplemental 0 2• NIPPV. intubation • Cardiogenic shock .... inotropes (dobutamine. milrinone) • Intravenous diuretics (eg, furosemide) ± venodilator (eg, nitroglycerin) • Balanced vasodilator (eg, nitroprusside) if afterload reduction also needed l Investigate precipitating factors • ECG & troponin (ischemia. arrhythmia) • Bedside echocard1ogram (valvular dysfunction) BNP= brain natriurelic peptide;JVD = jugular venous distension;LE= lower extremity; NIPPV= noninvasive positive pressureventilation, CVS ©UW01ld 230 https://t.me/usmleinnercircle Version 2 Pharmacotherapy for chronic heart failure with reduced ejection fraction Indication . . . .. Angiotensin receptor-neprilysin inhibitor* NYHA classes I-IV with LVEF S40% Initial optimized Beta blocker therapy NYHA classes II-IV with volume Diuretics Step 3 of optimized therapy NYHA classes II-IV with LVEF Aldosterone antagonist S35% SGLT-2 inhibitor NYHA classes II-IV lsosorbide dinitrate + Angiotensin system blocker hydralazine intolerance Supplementary . . . . improves mortality Metoprolol succinate, carvedilol, or bisoprolol Reduces hospitalization & improves mortality Loop diuretic ± metolazone Improves symptoms & reduces hospitalization Reduces hospitalization & improves mortality irc therapy Slows disease progression & Reduces symptoms & improves mortality Improves symptoms & may improve rC Step 2 of optimized overload Notes le Medication agents Persistent symptoms despite Digoxin other therapy mortality Reduces hospitalization but no mortality benefit ne *Other angiotensinsystem blockers (ie, ACE inhibitor or angiotensinreceptor blocker) are alternatives. LVEF = left ventricularejection fraction; NYHA = New York Heart Association;SGLT-2= sodium-glucosecotransporter2. # SGLT2- are used in patients with HFrEF (both with diabetes mellitus type 2 and without) and persistent symptoms (ie, New York Heart Association class II-IV) despite otherwise optimized medical therapy. • Flash pulmonary edema is a form of acute decompensated heart failure in Treatment? Furosemide In which acute increases in LV diastolic pressure cause accumulation in the pulmonary interstitium. LE In acute decompensated heart failure, this is the most important intervention • Treatment of acute pulmonary congestion: LMNOP Lasix, morphine, nitrates, oxygen, upright position) U SM Do not use if patient is hypotensive or hypovolemic Intravenous vasodilators (eg, nitroglycerin) reduce intracardiac filling pressures and are recommended in patients with acute decompensated heart failure who have an inadequate response to initial diuretic therapy. In addition, they are indicated as initial therapy (prior to or instead of intravenous diuretics) in patients with "flash" pulmonary edema due to severe hypertension Inotropes only in patients with cardiogenic shock (ie, severe hypotension causing end-organ dysfunction CVS 231 https://t.me/usmleinnercircle Version 2 Clinical features of acute decompensated heart failure Clinical presentation Treatment • Acute dyspnea, orthopnea, paroxysmal nocturnal dyspnea • Hypertension common; hypotension suggests severe disease • Accessory muscle use, tachycardia, tachypnea • Diffuse crackles with possible wheezes (cardiac asthma) • Possible S3, jugular venous distention, peripheral edema Normal or elevated blood pressure with adequate end-organ perfusion • Supplemental oxygen • Intravenous loop diuretic (eg, furosemide) • Consider intravenous vasodilator (eg, nitroglycerin) Hypotension or signs of shock • Supplemental oxygen • Intravenous loop diuretic (eg, furosemide) as appropriate • Intravenous vasopressor (eg, norepinephrine)  What is the initial management for a patient with pulmonary hypertension that presents with JVD, peripheral edema, bibasilar crackles, and an ejection fraction of 30%? Loop diuretics and ACE inhibitors (or ARBs) the pulmonary hypertension is due to LV systolic dysfunction and thus treatment involves addressing the underlying cause; beta-blockers and occasionally aldosterone antagonists may be used as well Pulmonary interstitial edema Kerley B lines (peripheral interstitial thickening) ©UWorld CVS 232 https://t.me/usmleinnercircle Version 2 Patients with slowly progressive decompensation may have minimal or no pulmonary edema. Pulmonary lymphatics can gradually increase fluid outflow rate up to 10 times the baseline when needed, effectively offloading fluid from the pulmonary venous system to the central venous system and allowing evidence of elevated central venous pressure (eg, JVD, peripheral edema) to be present despite minimal or no pulmonary edema. irc le Chronic heart failure often manifests with mild interstitial edema (ie, Kerley B lines on chest x-ray) without alveolar edema (ie, no crackles on examination). . .. . . Heart failure with preserved ejection fraction Risk factors Management .. .. Causes of heart failure with preserved left ventricular function LV diastolic dysfunction due to impaired relaxation Chronic hypertension (concentric LV hypertrophy) Obesity & sedentary lifestyle (myocardial interstitial fibrosis) CAD & related risk factors (eg, diabetes mellitus) ne Etiology rC Diastolic Failure Specific therapies to reduce hoseitalization & eossibl~ mortali~: 0 MRAs (eg, spironolactone) 0 SGLT2 inhibitors (eg, dapagliflozin) Diastolic heart failure In Additional antihypertensives PRN to reduce afterload Treatment of exacerbating conditions (eg, CAD, OSA, A-fib) Exercise training/cardiac rehabilitation • Occult coronary artery disease Valvular heart disease Pericardia! disease A-fib = atrial fibrillation; CAD = coronary artery disease; LV = left ventricular; • Restrictive cardiomyopathy • Infiltrative cardiomyopathy (eg, sarcoidosis) • Hypertrophic cardiomyopathy 11 Loop diuretics as needed to treat volume overload • Hypertension with left ventricular hypertrophy • Aortic stenosis or regurgitation • Mitra! stenosis or regurgitation • Constrictive pericarditis • Cardiac tamponade MRAs = mineralocorticoidantagonists; OSA = obstructive sleep apnea; PRN = as needed; Systemic disorders (high-output cardiac conditions) U SM LE SGLT2 = sodium-glucose cotransporter 2. • Thyrotoxicosis • Severe anemia Physical fitness and the risk of developing heart failure Heart failure with reduced EF (HFrEF) Heart failure with preserved EF (HFpEF) A Physical activity level & fat mass reduction EF: 8jedionlradklo. CVS 233 https://t.me/usmleinnercircle Version 2 LV ejection fraction is normal in HFpEF, and diastolic dysfunction is not always detectable. Cor Pulmonale Pathophysiology . Cor pulmonale Right-sided heart failure due to primary pulmonary disorder • Absence of left-sided heart disease • Parenchymal disease/chronic hypoxia 0 COPD (most common) Common 0 Cystic fibrosis etiologies 0 Interstitial lung disease 0 Obstructive sleep apnea Symptoms Examination Diagnostic evaluation • Pulmonary vascular disease: pulmonary embolism • Dyspnea & fatigue on exertion • Exertional angina (due to j cardiac demand) . . Exertional syncope (due to t cardiac output) Jugular venous distension • Peripheral edema • Palpable RV heave, loud P2, tricuspid regurgitation murmur • Hepatomegaly with pulsatile liver • Electrocardiography . . 0 Incomplete/complete right bundle branch block 0 Right axis deviation, RV hypertrophy, RA enlargement Echocardiography 0 Pulmonary hypertension, RV dilation/dysfunction, tricuspid regurgitation Catheterization (gold standard) 0 Elevated filling pressures, decreased cardiac output, pulmonary hypertension COPD = chronic obstructivepulmonarydisease; RA= right atrial; RV= right ventricular. High Output Heart Failure Common mechanisms of high-output heart failure Increased quantity of peripheral vessels Bypass of systemic arteriolar resistance Vasodilation due to unmet metabolic demand in tissues • Morbid obesity • Paget disease • Acquired AV fistula (eg, trauma, HD) • Congenital AV malformation • Hyperthyroidism • Severe anemia • Thiamine deficiency (wet beriberi) AV= arteriovenous; HD = hemodialysis. CVS 234 https://t.me/usmleinnercircle Version 2 High-output heart failure . . Pathophysiology . SVR leads to cardiac output & j venous return Morbid obesity (most common) • Arteriovenous fistula (congenital or acquired) Clinical presentation Echocardiographic findings . . . . . Paget disease, thiamine deficienc;t Hyperdynamic circulation (j PP, warm extremities) Peripheral & pulmonary edema Laterally displaced PMI, systolic flow murmur Dilated LV, RV & inferior vena cava Elevated resting cardiac index le . H~:1ierth:troidism severe anemia advanced cirrhosis irc Causes LV = left ventricle; PMI = point of maximalimpulse; PP= pulse pressure;RV= right ventricle; SVR = systemicvascular resistance. rC  What is the likely diagnosis in a patient with a history of a stab wound that develops progressive weakness and exertional dyspnea with widened pulse pressure, tachycardia, and brisk carotid upstroke? ne High-output heart failure (secondary to AV fistula formation) results in increased preload, decreased afterload, and increased cardiac output; Cardiomyopathy In Doppler US is the preferred test for diagnosis and surgical therapy may be warranted LE  What is the mainstay of therapy to improve/normalize heart function in patients with alcoholic cardiomyopathy? Abstinence from alcohol U SM alcoholic cardiomyopathy is suggested by CHF in the presence of heavy alcohol consumption, macrocytic anemia, 21 ASTALT ratio, and no evidence of CAD/valvular defects  What is the initial treatment for tachycardia-mediated cardiomyopathy? Rate or rhythm control presents with progressive dyspnea, decreased exercise tolerance, and LV systolic dysfunction secondary to chronic tachycardia (e.g. chronic A-fib) An elevation in ventricular contraction rate (eg, 100/min) that is sustained for weeks or more can cause left ventricular dilation Peripartum Cardiomyopathy CVS 235 https://t.me/usmleinnercircle Version 2 Peripartum cardiomyopathy Risk factors Clinical features Management .. . . . . .. .. Maternal age >30 Multiple gestation Preeclampsia, eclampsia LVEF <45% Onset: 36 weeks gestation - 5 months postpartum No other cause of heart failure Deliver based on maternal hemodynamic stability Standard systolic heart failure regimen • Thromboembolism prophylaxis Recurrence risk LVEF <20% at diagnosis Persistent left ventricular systolic dysfunction LVEF = left ventricular ejection fraction. • Peripartum cardiomyopathy typically causes rapid-onset systolic heart failure at last month of pregnancy or within 5 months following delivery e.g. fatigue, dyspnea, cough, pedal edema Takotsubo cardiomyopathy Stress-induced (takotsubo) cardiomyopathy Risk factors Clinical features Diagnosis Treatment • Postmenopausal woman • Recent physical or emotional stressor • Chest pain mimicking myocardial infarction • Decompensated heart failure • Moderate troponin elevation • ECG: ischemic changes in precordial leads . Catheterization: no obstructive CAD • Echo: LV apical hypokinesis, basilar hyperkinesis • Resolves in several weeks with supportive care CAD = coronary artery disease; LV = left ventricular. HOCM CVS 236 https://t.me/usmleinnercircle Version 2 Hypertrophic cardiomyopathy • Genetic mutations affecting cardiac sarcomere proteins Pathophysiology • Autosomal dominant inheritance • Variable phenotypic penetrance • Many patients are asymptomatic • Exertional dyspnea, fatigue, angina, light-headedness, syncope Clinical features • Systolic ejection murmur accentuated by t LV blood volume • Diastolic dysfunction with audible S4 • f Risk for atrial fibrillation & ventricular tachycardia • ECG: left axis deviation, abnormalities of depolarization (eg, Q waves) or repolarization le (eg, inverted T waves) Diagnosis • Echocardiography: septal LV hypertrophy, dynamic LVOT obstruction, LA dilation • Beta blocker or nondihydropyridine CCB (facilitate f LV blood volume) • Avoidance of dehydration & vasodilators (avoid t LV blood volume) irc Management • ICD placement for increased risk for SCD • Septal ablation, cardiac transplantation CCB = calcium channel blocker; ICD = implantable cardiac defibrillator; LA= left atrial; LV = left ventricular; LVOT = left ventricular Normal heart rC outflow tract; SCD = sudden cardiac death. Hypertrophlc cardiomyopathy ne Management of hypertrophic cardiomyopathy I Asymptomatic --------------. l In LE High risk for SCD (eg, prior syncope or sustained VT, family history) ICD placement ] Atrial fibrillation Rate or rhythm control & anticoagulation obstructsleft ventricular outflow U SM ...... Significant LVOT obstruction Refractory symptoms -------Beta blocker or nondihydropyridine CCB Symptomatic (eg. dyspnea. angina) Thickenedsepll.lTIfurther Routine clinical monitoring __JI~---Nonobstructive Septal ablation or surgical myecotomy Cardiac transplantation CCB = calaum channel blocker. ICO = implantable cardiac defibrillator;LVOT = left ven1ricularoutflONtract; CU\Nofld SCO = sudden cardiac death, VT = ventricU1artadiycardia.  Syncope in HOCM is multifactorial and can be due to outflow obstruction (interventricular septal hypertrophy), arrhythmia, ischemia, or a ventricular baroreceptor response that inappropriately causes vasodilation. CVS 237 https://t.me/usmleinnercircle Version 2 Patients with hypertrophic cardiomyopathy are at increased risk for atrial fibrillation and ventricular tachycardia. Periodic ambulatory ECG monitoring is indicated to facilitate prompt recognition and management of these arrhythmias, especially in patients with suggestive symptoms (eg, palpitations). Distinguishing hypertrophic cardiomyopathy from athlete's heart Hypertrophic cardiomyopathy Athlete's heart Common Usually unremarkable LVH criteria + depolarization &/or repolarization LVH criteria without other abnormalities• abnormalities Enlarged Normal Usually decreased Slightly enlarged ;,1smm <15mm Yes No Impaired Normal Family history ECG findings Left atrial size LV cavity size LVwall thickness Focal septal hypertrophy LV diastolic function *Common depolarization abnormalities include prominent Q waves; common repolarization abnormalities include T-wave inversions. LV ; left ventricular;LVH ; left ventricular hypertrophy.  Additional ECG findings that can be seen include increased QRS voltage and J point elevation; isolated T-wave inversion in V1 is relatively common in the general population, including in athletes. vs T-wave inversions in 2 contiguous leads in HOCM. CVS 238 https://t.me/usmleinnercircle Version 2 Hypertrophiccardiomyopathy irc le Left ventricular hypertrophy: Tall R wave in aVL + Deep S wave in V3 (Cornell criteria) Repolarization changes in anterolateral leads (I, aVL, V4, VS, V6) rC Carditis ne Acute Pericarditis Acute pericarditis • Viral or idiopathic In 0 Early: peri-infarction pericarditis 0 Late: Dressler syndrome LE Etiology • Autoimmune disease (eg, SLE) • Uremia (acute or chronic renal failure) • Post myocardial infarction Clinical features U SM & diagnosis Treatment • Pleuritic chest pain U when sitting) ± fever • Pericardia! friction rub (highly specific) • ECG: diffuse ST-segment elevation & PR-segment depression • Echocardiography: pericardia! effusion • NSAIDs & colchicine for viral or idiopathic etiology • Variable for other etiologies NSAIDs = nonsteroidal anti-inflammatory drugs; SLE = systemic lupus erythematosus. Corticosteroids (eg, prednisone) are used in patients with a contraindication to NSAIDs (eg, renal insufficiency) or failure of combination NSAID/colchicine therapy.  Auscultation at the left sternal border typically indicates a triphasic pericardial friction rub (heard in atrial systole, ventricular systole, and early ventricular diastole).  Initial ECG typically shows subtle (diffuse) PR depression and diffuse ST elevation that eventually evolves to diffuse T wave inversion  What is the treatment of choice for peri-infarction pericarditis Post infarction fibrinous pericarditis)? Supportive (best prevented by early coronary reperfusion therapy) CVS 239 https://t.me/usmleinnercircle Version 2 NSAIDs are typically avoided (versus Dressler syndrome) for at least 7 days due to a possible increased risk of free wall rupture. Anticoagulation should be avoided in Dressler syndrome to prevent development of hemorrhagic pericardial effusion. All patients suspected of having PIP should undergo echocardiography, which allows for characterization of any associated pericardial effusion and helps rule out other post-MI complications (eg, free wall rupture). • Uremic pericarditis does not show typical ECG changes like ST-segment elevation (lack of inflammation invading the myocardium in uremic pericarditis) 50% of cases are accompanied by pericardial effusion, cardiac tamponade should be ruled out prior to dialysis initiation.  Young women with recent viral illness (sore throat, runny nose) who now presents with pleuritic chest pain but otherwise normal exam and labs most consistent with a diagnosis of viral pleurisy Pleurisy is basically analogous to a post-viral pericarditis, and therefore is also managed with an NSAID Key idea: Pleuritic chest pain ⟶ Pneumothorax, Pleurisy, Pericarditis, Pulmonary embolism Key idea: Recurrent ulcers + Pleurisy may also be pointing to a diagnosis of pleuritis in the setting of lupus • Peri-infarction pericarditis PIP is usually self-limited and some patients do not require pharmacologic treatment. However, for those with significant discomfort, high-dose aspirin (eg, 650 mg 3 times a day) is the treatment of choice for symptomatic relief. High-dose aspirin is believed to provide analgesia and anti-inflammatory effect while having a relatively small effect on myocardial healing and scar formation compared to other antiinflammatory drugs Constrictive Pericarditis CVS 240 https://t.me/usmleinnercircle Version 2 Features of constrictive pericarditis • Recurrent idiopathic or viral pericardilis • Cardiac surgery or radiation therapy Etiology • Tuberculous pericarditis (where endemic) • Fatigue, dyspnea on exertion • Peripheral edema, ascites, hepatic congestion ( ll'-IF sir) Clinical presentation • Pericardia! knock in early diastole • JVD with positive Kussmaul sign* & prominent y descent • ECG: low-voltage QRS complexes • Chest x-ray/CT: pericardia! calcification • Echocardiogram: biatrial enlargement, normal ventricular wall thickness & cavity size le Diagnostic findings *Increase in JVD with inspiration. irc JVD = jugular venous distension. Pericardial knock (mid-diastolic sound); occurs due to filling ventricles hitting thickened pericardium rC  What is the most common causes of constrictive pericarditis in the United States? Viral or idiopathic 40% ne other common causes include radiation therapy 30%, cardiac surgery 10%, uremia, and connective tissue disorders Tuberculosis common in developing countries( India, China) In Treatment: surgical removal of the stiff and fibrotic pericardium (ie, pericardiectomy) to restore ventricular diastolic function. U SM Myocarditis LE D/D Cardiac Amyloidosis: May manifest as Restrictive Cardiomyopathy, LV is thicked and will not have pericardial calcification Viral myocarditis • Relatively young adults (eg, age <55) Clinical presentation Diagnosis • Viral prodrome (eg, fever, malaise, myalgias) • Heart failure (eg, dyspnea, orthopnea, edema) • Chest pain • Sudden cardiac death • ECG: nonspecific • Echocardiography:4-chamber dilation • Cardiac MRI: late enhancement of the epicardium • Biopsy: lymphocytic infiltration, viral DNA or RNA • Medication (eg, diuretics, ACE inhibitor, beta blocker) Treatment • Temporary ventricular assist device, if needed • Heart transplant if no recovery  What is the likely diagnosis in a young adult with a month history of fever and malaise that develops symptoms of CHF with cardiomegaly on imaging? CVS 241 https://t.me/usmleinnercircle Version 2 Viral myocarditis Pediatric viral myocarditis Etiology Clinical presentation Coxsackie B virus Adenovirus Viral prodrome Heart failure: Dyspnea, syncope, tachycardia, nausea, vomiting, hepatomegaly Chest x-ray: Cardiomegaly Pulmonary edema Diagnostic studies ECG:Sinus tachycardia Echocardiogram: Decreased ejection fraction Diffuse hypokinesis Endomyocardial biopsy (gold standard): Inflammatory infiltrate of the myocardium with myocyte necrosis Mortality: Newborns:~ 75% Prognosis • Older infants/children:~ 25% Outcome of survivors: Full recovery within 2-3 months: ~66% Dilated cardiomyopathy/chronic heart failure: ~33% Cardiac Tamponade  What is the recommended treatment for a hemodynamically unstable patient with cardiac tamponade? Emergency pericardiocentesis • Unstable Pericardial fluid drainage (U/S-guided pericardiocentesis) and surveillance in ICU • Stable Can do pericardial window: incision in pericardium made to allow drainage from pericardial space into pleural cavity Cardiac tamponade Etiology Clinical signs Diagnosis • • Pericardia! effusion (eg, malignancy, infection, uremia) • Beck triad: hypotension, JVD, t heart sounds • Pulsus paradoxus: SBP t >10 mm Hg during inspiration • ECG: low-voltage QRS complex, electrical altemans • Chest x-ray: enlarged cardiac silhouette,* clear lungs Blood in pericardia! space (eg, LV rupture, cardiac surgery) • Echocardiography: right atrial & ventricular collapse, IVC plethora Treatment • Intravenous fluids to increase right-sided preload • Drainage via pericardiocentesis or pericardia! window •subacute but not acute tamponade. IVC = inferior vena cava; JVD = jugular venous distension; LV = left ventricular; SBP = systolic blood pressure. CVS 242 https://t.me/usmleinnercircle Version 2 • "Beck's triad"; symptoms are due to an exaggerated shift of the interventricular septum toward the left ventricular cavity (decreases LV preload, SV, and CO Characteristics of cardiac tamponade Acute Subacute Minutes to hours Days to weeks irc Rapidity of fluid accumulation le A "water-bottle" cardiac silhouette on CXR is seen in patients who have a large pericardial effusion. Also inability to palpate the point of maximal apical impulse Beck triad: hypotension, JVD, muffled heart sounds Pulsus paradoxus (>10 mm Hg decrease in SBP with inspiration) Clinical signs 100-200 ml Effusion volume 1-2 L rC Normal cardiac silhouette Enlarged, globular cardiac silhouette Chest x-ray findings JVD = jugularvenousdistension;SBP = systolicbloodpressure. Purulent pericardia! features • Risk factors: immunosuppression, hemodialysis, recent thoracic surgery/trauma • Organisms: Staphylococcus aureus (most common), Streptococcus pneumoniae, Salmonella, Candida • Acute presentation, patients often appear severely ill • Fevers, chills, chest pain (pleuritic or nonpleuritic) • Can be rapidly fatal In Clinical ne • Hematogenous or direct intrathoracic spread Etiology effusion • ECG: tachycardia, diffuse ST-segment elevation, ± low-voltage QRS complexes CXR: enlarged cardiac silhouette & clear lung fields Diagnosis • LE • Echocardiography: pericardia! effusion • Cytology: turbid fluid with i WBCs (neutrophil predominant), i protein, ! glucose Treatment • Intravenous antibiotics + pericardia! drainage U SM CXR = chest x-ray; WBCs = white blood cells. Etiology Clinical features Treatment .. .. .. .. Malignant pericardia! effusion Common primary tumors: lung, breast, GI tract, lymphoma, melanoma May be initial manifestation of malignancy or recurrence Progressive dyspnea, chest fullness, fatigue ECG: ! QRS voltage ± electrical altemans CXR: enlarged cardiac silhouette & clear lung fields Echo: large effusion± signs oftamponade (eg, right atrial collapse) Acute management: pericardiocentesis, cytologic fluid analysis Prevention of recurrence: prolonged drainage (eg, catheter, pericardia! window) CXR = chest x-ray; echo = echocardiography;GI = gastrointestinal. CVS 243 https://t.me/usmleinnercircle Version 2  What is the likely diagnosis in a patient with an aortic dissection that develops hypotension, JVD, and pulsus paradoxus? Cardiac tamponade Electrical alternans ©UWorld Electrical alterans with sinus tachycardia is highly specific for large pericardial effusion, which may result in cardiac tamponade. 1 week ago Today ©UW011d CVS 244 https://t.me/usmleinnercircle irc le Version 2 Appear on CXR as an enlarged and globular cardiac silhouette with clear lung fields rC  What is the likely diagnosis in a patient on post-op day 1 status-post CABG that presents with hypotension, tachycardia, and the findings below: Right atrium 20 mmHg ne Right ventricle 35/20 mmHg PCWP 20 mmHg Cardiac tamponade In equilibrated intracardiac diastolic pressures suggests cardiac tamponade LE Peripheral Vascular Diseases Ankle-brachia! index ABI = SBP of dorsal is eedis or eosterior tibial arte~ + SBP of brachia! artery Diagnostic of peripheral artery disease 0.91-1.3 Normal >1.3 Suggests calcified & uncompressible vessels* U SM S0.9 •other testing should be considered. ABI = ankle-brachia!index; SBP = systolic blood pressure.  Patients with PAD and intermittent claudication have an estimated 20% 5-year risk of non-fatal MI and stoke and a 1530% risk of death due to cardiovascular causes.  What is the recommended pharmaceutical therapy to reduce overall cardiovascular mortality in patients with peripheral arterial disease? Antiplatelet Agent (e.g. aspirin) and a Statin Statin recommended in all patients with ASCVD Risk factor management Step 1A Smoking cessation Blood pressure & diabetes mellitus control Antiplatelet & statin therapy Step 1B Supervised exercise therapy Step 2 Cilostazol (preferred over pentoxifylline) CVS Step 3. Revascularization for persistent symptoms https://t.me/usmleinnercircle Angioplasty ± stent placement Autogenous or synthetic bypass graft 245 Version 2  What is the best initial therapy for peripheral vascular disease? • Most important: Stop smoking and exercise (promotes circulation) • Aspirin, cilostazol Most effective) Percutaneous or surgical revascularization is generally reserved for patients who have persistent symptoms despite initial exercise and/or pharmacologic therapy. Cilostazol can also be considered with persistent claudication. Smoking cessation is a first-line intervention for peripheral arterial disease.  What is the most useful intervention to improve functional capacity and reduce symptomatic claudication in patients with peripheral artery disease? Supervised graded exercise program  What is the likely diagnosis in a hypotensive patient requiring IV fluids/pressors to maintain BP that develops cool extremities and the skin findings below? Pressor-induced vasospasm (e.g. norepinephrine) diagnosis is suggested by symmetric duskiness and coolness of all finger tips Can also negatively impact the intestines (causing mesenteric ischemia) and/or kidneys (causing renal failure). Venous Insufficiency  What is the most common cause of lower extremity edema? Venous insufficiency (valvular incompetence) classically worsens throughout the day and resolves overnight  What is the initial treatment for chronic venous insufficiency? Conservation measures (e.g. leg elevation, exercise, compression therapy) patients who do not respond to conservative measures should have diagnosis of CVI confirmed by identification of venous reflux in the deep venous system on duplex ultrasound CVS 246 https://t.me/usmleinnercircle Version 2 .. . .. Risk factors Clinical features Advanced age, family history Prolonged standing, obesity Smoking, prior deep vein thrombosis or trauma Leg discomfort/pain & heaviness, often improve with walking Varicose veins & telangiectasia, edema Stasis dermatitis (chronic): pruritus, scaling, brawny discoloration, woody induration Ulceration: painful, irregular ulcers with shallow bases, most often near medial malleoli Leg elevation, when possible Leg exercises (eg, ankle flexion, walking) to i calf strength & venous return Compression therapy (eg, graduated compression stockings) irc le Initial management .. .. . Chronic venous insufficiency Causes of lower extremity edema . . . Muscle strain Lymphedema Paralyzed limb Cellulitis Heart failure Venous insufficiency Medication adverse effect (eg, amlodipine) Postthrombotic syndrome rC Typically bilateral Deep vein thrombosis ne Typically unilateral .. . . . • Chronic venous insufficiency following acute DVT In Epidemiology • Most cases arise within 2 years of thrombus • Leg edema, fatigue, pain, superficial venous Manifestations dilation, venous stasis ulcers Stasis Dermatitis Treatment LE • Often worse at the end of the day • Exercise (eg, ankle flexion, walking) • Compression (eg, bandages, stockings) U SM DVT = deep vein thrombosis.  Approximately 50% of patients with acute DVT develop postthrombotic syndrome within 2 years due to venous hypertension distal to the site of previous thrombus. This is thought to arise due to venous valve damage from the release of inflammatory mediators and venous lumen stenosis due to wall remodeling. D/D Recurrent DVT  Do Venous Ultrasonography to rule it out first Acute Limb Ischemia CVS 247 https://t.me/usmleinnercircle Version 2 . Etiology Acute limb ischemia Cardiac/arterial embolus (eg, AF, LV thrombus, IE) • Arterial thrombosis (eg, PAD) • Iatrogenic/blunt trauma . 6 Ps of acute limb ischemia Pain • Pallor • Paresthesia • Pulselessness Clinical features . Poikilothermia (cool extremity) • Paralysis (late) Management • Anticoagulation (eg, heparin) • Thrombolysis vs surgery AF = atrial fibrillation; IE = infective endocarditis;LV = left ventricular;PAD = peripheralartery disease. Clinical features of acute limb ischemia Nonviable limb Viable limb Threatened limb (No tissue loss) (Risk of tissue loss) Mild Severe Variable None Mild/partial Severe/complete Capillary refill Intact Delayed Absent Arterial Doppler Audible Inaudible Inaudible Venous Doppler Audible Audible Inaudible Catheter-based or surgical Emergency surgical revascularization revascularization Pain Sensory/motor deficit Management (Permanent tissue damage) Amputation  What is the initial management for a patient with suspected acute limb ischemia? Anticoagulation (e.g. IV heparin) Should be initiated empirically, prior to further evaluation with other imaging studies • Diagnostics: Arterial and venous Doppler confirmed with Angiography (digital subtraction angio is the modality of choice) • Treatment: Non-threatened limb ⟶ angiography with revascularization. If limb is threatened, undergo emergent revascularization (catheter directed thrombosis is and/or thromboembolectomy) Non-viable = amputation Presence of gangrene requires amputation CVS 248 https://t.me/usmleinnercircle Version 2  What imaging study should be considered in a patient with acute limb ischemia after an MI? Transthoracic echocardiogram to screen for LV thrombus and evaluate LV function; patients also require immediate anticoagulation and vascular surgery consult  In patients with PAD, atherosclerosis of peripheral arteries (eg, popliteal artery) may be complicated by plaque disruption. This can lead to thrombosis and acute-on-chronic limb ischemia le Due to preexisting collateral circulation, acute limb ischemia in the setting of chronic peripheral artery disease PAD often presents less dramatically than in patients without PAD. irc Emergency intervention is still necessary. Anaphylactic Anaphylaxis rC Shock ne • Food (eg, nuts, shellfish) Triggers • Medications (eg, P-lactam antibiotics) • Insect stings • Cardiovascular Vasodilation ---> hypotension & tissue edema o Tachycardia In o • Respiratory U SM LE Clinical manifestations Treatment o Upper airway edema ---, strider & hoarseness o Bronchospasm --->wheezing • Cutaneous o Urticaria! rash, pruritus, flushing • Gastrointestinal o Nausea, vomiting, abdominal pain • Intramuscular epinephrine • Airway management & volume resuscitation • Adjunctive therapy (eg, antihistamines, glucocorticoids) IV epinephrine is indicated for patients with anaphylaxis who do not respond to initial IM epinephrine Nonclassic presentation Challenging clinical situations Medication effects Diagnostic challenges in anaphylaxis • No prior allergic reactions (ie, first episode) • Absence of hypotension, respiratory symptoms, or cutaneous signs • Protracted or biphasic presentation • Preexisting conditions with similar symptoms (eg, asthma, COPD) • Ongoing physiologic shifts (eg, childbirth, hemodialysis) • Impaired communication (eg, sedation, severe psychiatric illness) • Directly activate mast cells (eg, opiates, NSAIDs) • Interfere with epinephrine effect (eg, P-blockers, a-adrenergic blockers) COPD = chronic obstructive pulmonary disease; NSAIDs = nonsteroidalanti-inflammatorydrugs. CVS 249 https://t.me/usmleinnercircle Version 2 Diagnostic criteria for anaphylaxis Anaphylaxis is likely if there is rapid symptom onset & any 1 of the following criteria: 1 Skin/mucosa involvement (eg, hives, lip/tongue swelling) & either hypotension or respiratory distress Involvement of ~2 organ systems after exposure to a likely allergen 2 3 • Skin/mucosa (eg, hives, lip/tongue swelling) • Respiratory (eg, wheezing, strider, dyspnea) • Cardiovascular (eg, hypotension, tachycardia, syncope) • Gastrointestinal (eg, abdominal pain, vomiting, diarrhea) Hypotension after exposure to a known allergen Hypotension is not required to make the diagnosis. Management of anaphylaxis Immediate management Adjunct management • Epinephrine (most important): o IM preferred, may be repeated (eg, 3 doses) o IV in severe/refractory cases IV crystalloid & Trendelenburg positioning for hypotension • Albuterol for bronchospasm • Early intubation for upper airway obstruction . • • • • H1/H2 antihistamines Glucocorticoids Glucagon for patients on beta blockers (reversal) Hospital admission for severe initial presentation (eg, shock) or ongoing symptoms despite treatment IM = intramuscular;IV = intravenous.  Patients with anaphylaxis should be admitted to the hospital when symptoms are protracted or severe (eg, hypotension, upper airway edema, respiratory distress), or require multiple doses of epinephrine to resolve. These patients are at increased risk of biphasic anaphylaxis (ie, recurrence of symptoms after an initial period of resolution), which can be fatal. At the time of discharge, all patients should be prescribed epinephrine auto-injector and instructions on how to use it. Hymenoptera Venom Immunotherapy is recommended for patients who have severe systemic reactions to bee stings to decrease the risk of future reactions. Anti-venom IgG antibodies, or "blocking" antibodies are created, and upon re-exposure, block the interaction between the allergen and preformed lgE antibodies on the surface of mast cells and basophils to prevent degranulation. CVS 250 https://t.me/usmleinnercircle Version 2  When intravenous access cannot be obtained in emergency cases, intraosseous IO access should be attempted immediately. M/C Proximal Tibia Contraindications:  Infection (eg, cellulitis) overlying the access site,  Fracture or previous IO attempts in the chosen extremity, or  Bone fragility (eg, osteogenesis imperfecta). le Hypovolemic & Hemorrhagic Shock irc • Balanced resuscitation (aka, damage-control resuscitation) is used; it includes the following measures: • Limiting use of crystalloids (eg, 1 L), which dilute existing coagulation (eg, clotting) factors and rC platelets, thereby increasing coagulopathy • Replacing lost intravascular volume with blood products (rather than crystalloids), transfused in a ratio similar to that of whole blood (eg, 111 ratio of packed red blood cells/plasma/platelets) • Permitting hypotension (ie, permissive hypotension) to limit ongoing hemorrhage and/or prevent ne clot disruption and rebleeding With balanced resuscitation, blood products are administered only as needed to maintain a blood In pressure (eg, mean arterial pressure 65 mm Hg) sufficient for tissue perfusion, until definitive hemorrhage control (eg, surgical intervention) can be achieved. LE  Adjunct therapies for hemorrhagic shock include tranexamic acid (antifibrinolytic agent), which is effective in a subset of patients with acute life-threatening hemorrhage if administered within the first 3 hours of injury, and topical hemostatic agents (eg, kaolin-impregnated sponge, fibrin sealant U SM dressing), which are applied to control external hemorrhage. Control bleeding Give blood products early Limit use of crystalloids Management of hemorrhage due to trauma • Control bleeding from any compressible site • Early transfer to location (trauma center, operating room, angiography suite) for definitive care & . control of hemorrhage Transfuse blood products early in resuscitation • Massive transfusion protocol*: ratio of 1:1 :1 FFP/pRBCs/platelets to minimize risk of coagulopathy . • Limit to -1 L if patient is hypotensive Switch to blood products as soon as available *Massive transfusion protocol likely required in patients with <?2of the following: SBP S90 mm Hg, pulse <?120/min,positive FAST examination, and penetrating mechanism of injury. FAST = Focused Assessment with Sonography for Trauma; FFP = fresh frozen plasma; pRBCs = packed red blood cells; SBP = systolic blood pressure. In such situations, group O, Rh D-negative blood should be administered unless type-specific blood is immediately available. CVS 251 https://t.me/usmleinnercircle Version 2 Spl to women of childbearing age and to young girls. In men and in women past childbearing age, O Rh D-positive blood can be given.  What is the next step in management for a patient that needs emergency fluid resuscitation and peripheral IV access cannot be obtained? Attempt intraosseous access requires less skill and practice than central line placement and is safer and faster • Hemorrhagic shock is the most common type of shock in the trauma setting, particular attention is given during trauma survey to areas where large blood loss can occur ("blood on the floor and 4 more"): • External bleeding ("the floor"): up to the entire blood volume • Chest: up to 40% of the blood volume/hemithorax • Abdomen (ie, peritoneal cavity): up to the entire blood volume • Pelvis: up to the entire blood volume; blood loss often hidden within the retroperitoneum 0 Do pelvic X ray to rule out (retroperitoneal fluid can be missed in FAST • Thigh: up to 12 L/thigh Fluid Replacement • 25kg child with burns over 40% of her body. What is fluid of choice (and why? Lactated Ringer (closely resembles physiologic EC fluid and lactate reduces metabolic acidosis incidence) Do not use normal saline -- not suitable for fluid loss replacement in burn patients  Calculate fluid regimen for next 24 hours: • Parkland  Maintenance  Total • Parkland Formula: 4 mL x BSA x weight (kg) 0 4  40  25  4000 mL  4 L • Maintenance Fluid: 421 formula 0 4 ml/kg/hr for first 10kg  40 ml 0 2 ml/kg/hr for next 10kg  20 ml 0 1 ml/kg/hr for remaining  5 ml 0 65 ml/kg/hr  1560 ml per 24 hr = 1.5L • 4  1.5  5.5 L Lactated Ringer Adults only requires Parkland formula  What imaging modality should be used to confirm placement of a central venous catheter tip? CVS 252 https://t.me/usmleinnercircle Version 2 Chest X-ray this step may be omitted in the setting of an uncomplicated ultrasound-guided CVC placement  Ideal placement for a central venous catheter tip? rC irc le Lower superior vena cava ne  Tip placement in smaller veins (eg, subclavian, jugular, azygous) predisposes to venous perforation. In addition, inappropriately placed catheter tips may cause lung puncture, leading to pneumothorax, or myocardial perforation, leading to pericardial tamponade. In Adequate fluid resuscitation is an essential component of managing severe sepsis and septic shock. Patients with stopped. LE hypotension or evidence of impaired perfusion should receive immediate fluid resuscitation. If pt is on is on diuretics, they should be U SM Obstructive Shock  The expected hemodynamic parameters in obstructive shock include elevated central venous pressure CVP and low cardiac output; however, pulmonary capillary wedge pressure PCWP, an estimate of left atrial pressure and representation of left-sided preload, can vary depending on the location of the obstruction. • Prepulmonary obstructive shock (due to massive PE or tension pneumothorax) is most common and results from obstruction involving the right atrium, right ventricle, or pulmonary arteries. Because blood is unable to pass from the right side of the heart to the left side, PCWP is low or normal. • Postpulmonary obstructive shock typically involves obstruction within the left side of the heart or aorta; PCWP is elevated, making the hemodynamic parameters the same as those expected in cardiogenic shock. (e.g: Aortic Stenosis, Aortic Dissection) CVS 253 https://t.me/usmleinnercircle Version 2 CVS Drugs  What anti-hypertensive medication is commonly associated with peripheral edema as an adverse effect? Dihydropyridine Ca2+-channel blockers due to preferential dilation of precapillary vessels (arteriolar dilation), which increases capillary hydrostatic pressure  What drug classes are useful for reducing Ca2 channel blocker-induced peripheral edema? ACE inhibitors and ARBs the combination of CCB and ACE inhibitors was associated with a significantly lower risk of CCB-associated peripheral edema compared with CCB monotherapy.  The combination of atrial tachycardia with AV block is fairly specific for digitalis toxicity. digitalis causes both an increased ectopy in the atria or ventricles (atrial tachycardia) and an increased vagal tone AV block) Symptoms of digoxin toxicity Cardiac Gastrointestinal Neurologic . .. . .. .. Life-threatening arrhythmias Anorexia Nausea & vomiting Abdominal pain Fatigue Confusion Weakness Color vision alterations • Angioedema is s/e of both ACE inhibitor and ARNI not when ARB is used without Nephrilysin inhibitor / Anti-Arrhythmic  Enhanced effect at faster heart rates is known as use dependence. • Prolonged PR intervals at faster heart rates: Class IV CCBs) • Prolonged QRS durations at faster heart rates: Class I (especially class IC It is the primary mechanism behind their efficacy against supraventricular arrhythmias. CVS 254 https://t.me/usmleinnercircle Version 2 Thyroid effects of amiodarone Disorder Features Treatment None needed • t T4 • t T3 Decreased T4-T3 conversion • Normal/t TSH Inhibition of thyroid hormone synthesis Levothyroxine • t TSH • t T4 • t TSH AIT type 1 Antithyroid drugs • t T3 & T4 (iodine-induced increase • t RAIU in thyroid hormone synthesis) • Increased vascularity on ultrasound • t TSH Glucocorticoids • t T3 & T4 • Undetectable RAIU (destructive thyroiditis) Major side effects of amiodarone Endocrine Ocular Dermatologic pulmonary infiltrates) most common Hypothyroidism Hyperthyroidism Elevated transaminases, hepatitis • Corneal microdeposits • Optic neuropathy . . Blue-gray skin discoloration Peripheral neuropathy LE Neurologic Chronic interstitial pneumonitis (cough, fever, dyspnea, In Gastrointestinal/ . .. . ne Pulmonary de pointes rC • Sinus bradycardia, heart block • Risk of proarrhythmias: QT prolongation & risk of torsade Cardiac hepatic irc • Decreased vascularity on ultrasound AIT = amiodarone-induced thyrotoxicosis; RAIU = radioactive iodine uptake. le AIT type 2 U SM Miscellaneous Syncope  What is the most likely underlying etiology in a patient who passes out at home suddenly without any warning signs? Clonic jerks were noted while he was unconscious. His past medical history is significant for hypertension and previous myocardial infarction. Patient appears comfortable with no symptoms at time of examination. Arrhythmia • Clonic jerks can occur during any syncopal episode that is prolonged and associated with cerebral hypoxia, regardless of etiology. • Patients with arrhythmic cause of syncope usually have underlying structural heart disease and may not have any prodromal symptoms -- versus vasovagal or neurocardiogenic syncope, which have aura and notable triggers CVS 255 https://t.me/usmleinnercircle Version 2 • If this was a seizure, look for trigger stimuli (stress, lack of sleep, flashing lights) with prodromal aura, tongue laceration, and prolonged postictal phase. Seizure Vasovagalsyncope • Lack of sleep • Flashing light Triggers • Prolonged standing • Emotional stress • • Idiopathic • Preceding aura (eg, olfactory hallucinations) • Can occur with sleeping/sitting Alcohol withdrawal Clinical • Tonic/clonic movements clues • • Heat • Preceding lightheadedness (ie, presyncope) • Unlikely to occur while sleeping/sitting • Uncommon to have clonic jerks (can occur with prolonged cerebral Rapid, strong pulses • Tongue biting Sequelae • Physical/emotional stress hypoperfusion) • Weak, slow pulses • Incontinence • Pallor & diaphoresis • Delayed return to baseline (postictal drowsiness or Immediate return to baseline • confusion) Evidence of lateral tongue biting is the most reliable finding to differentiate epileptic seizure and syncope. It has low sensitivity 33% but high specificity 96% for epileptic seizure. Syncope Likely etiology Vasovagalor neurally mediated syncope Clinical clues to diagnosis Triggers: Prolonged standing or emotional distress, painful stimuli Prodromal symptoms: nausea, warmth, diaphoresis Situational syncope Triggers: Cough, micturition, defecation Orthostatic syncope Postural changes in heart rate/blood pressure after standing suddenly Aortic stenosis, HCM, anomalous coronary arteries Ventricular arrhythmias Syncope with exertion or du ring exercise Prior history of CAD, Ml, cardiomyopathy, or ! EF Sick sinus syndrome, bradyarrhythmias, atrioventricular Sinus pauses, f PR or f ORS duration block Torsades de polntes (acquired long QT syndrome) Congenital long QT syndrome Hypokalemia, hypomagnesemia, medications causing f QT interval Family history of sudden death, t QT interval, syncope with triggers (eg, exercise, startle, sleeping) • Continuous ECG monitoring can evaluate for transient arrhythmia (eg, ventricular tachycardia) that may not be detected on initial ECG. like Ambulatory ECG monitoring (eg, Holter monitor, insertable cardiac monitor) CVS 256 https://t.me/usmleinnercircle Version 2 Other vasodilation Volume depletion Sympathetic blockade Terazosin, prazosin, doxazosin Within 2-5 min of standing from a supine position . ~20 mm Hg i in systolic blood pressure OR ~10 mm Hg i in diastolic blood pressure Antipsychotics (eg, risperidone) Antihistamines, TCAs ACE inhibitors & ARBs Tilt-table testing can help distinguish between orthostatic and vasovagal syncope if the etiology is unclear (+ve Dihydropyridine CCBs Hydralazine, nitrates Phosphodiesterase inhibitors in Vasovagal) Diuretics SGLT-2 inhibitors le a1 blockade-mediated vasodilation .• . • .. . .. .. Examples Beta blockers Clonidine irc Mechanism . Diagnosis of orthostalic hypotension Medication-induced orthostasis ARBs = angiotensin II receptor blockers; CCBs = calcium channel blockers; rC SGLT = sodium-glucose cotransporter; TCAs = tricyclic antidepressants. Vasovagal & situational syncope Diagnosis Treatment .. ne Clinical presentation • Pain,* anxiety,* emotional stress, heat, prolonged standing • Situational: cough,* micturition,* defecation, eating, hair-combing* • Prodrome (eg, warmth, pallor, nausea, diaphoresis) • Rapid recovery of consciousness (eg, <1-2 min) Mainly based on clinical history of event Upright tilt table testing sometimes indicated in uncertain cases • Reassurance & avoidance of triggers • Counterpressure techniques for recurrent episodes In Triggers LE *Particularly common triggers in the pediatric population. Etiology U SM Aortic stenosis or HCM Ventricular tachycardia Sick sinus syndrome Advanced AV block Torsades de pointes Cardiac syncope .. • . .. .• .. . Clues to diagnosis Exertional syncope Systolic murmur on examination No preceding symptoms Cardiom~oeath~ or erevious Ml Preceding fatigue or dizziness Sinus pauses on ECG Bifascicular block or t PR interval on ECG Dropped QRS complexes on ECG No preceding symptoms Medications that prolong QT interval Hypokalemia or hypomagnesemia AV= atrioventricular:HCM = hypertrophiccardiomyopathy;Ml = myocardialinfarction. AV block can be intermittent and thus may not be present on ECG at the time of testing. CVS 257 https://t.me/usmleinnercircle Version 2 • Vasovagal Pathogenesis: Over-activation of vagus nerve (for whatever reason) leads to bradycardia (cardioinhibitory) and hypotension (vasodepressor) → fall in BP  syncope  For patients with recurrent syncope, management consists of advice to avoid triggers and to assume a supine position with leg raising at the onset of symptoms. Physical counterpressure maneuvers: Leg crossing with tensing of muscles, handgrip and tensing of arm muscles with clenched fists (improves venous return and cardiac output) aborting prodromal symptoms. Anomalous Aortic Origin of a Coronary Artery Anomalous aortic origin of a coronary artery Left main coronary Right coronary Tricuspid valve Normal Left main coronary Righi coronary Anomalous origin from right aortic sinus Anomalous origin from left aortic sinus L = leftaorticsinus:R = rightaorticsinus. •Anomalousarterymay get compressedbetweenaorta and pulmonaryarterialtrunkduringexertion. «lUWOl1d Anomalous aortic origin of a coronary artery is a common cause of sudden cardiac death in young athletes. Two types of AAOCA commonly associated with SCD are the left main coronary artery originating from the right aortic sinus and the right coronary artery originating from the left aortic sinus. These defects create sharp curvature of the anomalous artery, making it less amenable to high-volume flow. In addition, the anomalous artery passes between the aorta and the pulmonary artery, making it susceptible to external compression during exercise. Patients with AAOCA may experience exertional angina, lightheadedness, or syncope; however, some patients experience SCD without any premonitory symptoms. CVS 258 https://t.me/usmleinnercircle Version 2 Resting ECG is typically unremarkable. Transthoracic echocardiography can sometimes make the diagnosis, but it can also miss or inaccurately characterize AAOCA. CT coronary angiography or coronary magnetic resonance angiography provide the best visualization of coronary anatomy, and are the diagnostic tests of choice in patients with suspected AAOCA. le HOCM usually the most common cause but will have characteristic murmur unlike here. In - ne rC irc Pulsus Paradoxus inspiration U SM Stent LE Pulsus paradoxus Cardiac tamponade, Asthma, COPD): fall in systolic pressure  10 mmHg during  What is the likely cause of LAD artery occlusion V1V3 in a patient with recent stent placement in the LAD artery? Stent thrombosis (likely due to medication non-compliance) premature discontinuation of antiplatelet therapy is the strongest predictor of stent thrombosis after intracoronary stent implantation CVS 259 https://t.me/usmleinnercircle Version 2 Bare metal stent Shorter thrombotic risk penod Higher nsk of restenos1s t Early endothe/ializalion Drug-eluting stent Longer thrombotic nsk penod late endothellalization If stent was placed in LAD and now ST elevations are seen in lateral (ie, I, aVL, V5V6 or inferior (ie, II, III, aVF leads, this would suggest new atherosclerotic plaque rupture. (not stent thrombosis) Antiplatelet therapy after coronary stenting Recommended duration of therapy Perioperative management .. . • . • . DAPT for minimum of 6-12 months after BMS or DES placement DAPT for minimum of 4 weeks in select patients after BMS Continue DAPT for a total of 30 months if possible (eg, low bleeding risk) Continue aspirin indefinitely Elective surgery: defer procedure until after minimum DAPT duration Urgent surgery: continue P2Y 12 receptor blocker or hold for shortest duration possible Continue aspirin unless hi9h risk of severe sur9ical bleedin9 BMS= bare-metalstent; DAPT= dual antiplatelettherapy (aspirin+ P2Y12 receptor blocker); DES= drug-elutingstent. JVP CVS 260 https://t.me/usmleinnercircle Version 2 Estimating central venous pressure (CVP) CVP = Jugular venous pressure (JVP) + Depth to right atrium (5 cm) Elevated CVP (>8 cm H,0) irc le Normal CVP (6-8 cm H,O) rC • Cannon A waves are intermittent, prominent A waves caused by the surge in jugular venous pressure that occurs due to right atrial contraction against a closed tricuspid valve; these waves can be seen with any arrhythmia involving atrioventricular dissociation, such as ventricular tachycardia, which is characterized by self-propagation within the ventricles without communication with the atria. Other arrhythmias in which cannon A waves are commonly seen include complete atrioventricular block and frequent premature ventricular contractions. Patients may sometimes report symptoms ne associated with cannon A waves, including headache, jaw pain, and sensation of neck pulsation.  What physical exam manuever is useful for distinguishing between cardiac- and liver diseaserelated causes of lower extremity edema? In Hepatojugular reflux (distension of neck veins by pressure on liver with hand) U SM LE positive reflux is suggestive of cardiac causes (e.g. heart failure) CVS 261 https://t.me/usmleinnercircle Version 2 Hepatojugular reflux to differentiate between cardiac and liver disease Right ventricular failure (hepatojugular reflux present) Liver cirrhosis (hepatojugular reflux absent) t Hydrostatic pressure Lower extremity edema Lower extremity edema (due to hypoalbuminemia & iliac vein compression by ascites) RUQ = right upper quadrant. @UWorld Prosthetic Valve Prosthetic valve dysfunction . .. .. . . Types & causes Clinical manifestations Diagnosis Transvalvular regurgitation (cusp degeneration) Paravalvular leak (annular degeneration, IE) Valvular obstruction/stenosis (valve thrombus) New munmur (regurgitant or stenotic) Macroangiopathic hemolytic anemia Heart failure symptoms, thromboembolism Echocardiography IE = infective endocarditis. Prosthetic Valve Thrombosis Most common presentations of PVT include: • Thromboembolism: left-sided (ie, mitral or aortic) PVT can present as TIA, stroke, MI, bowel ischemia, and limb ischemia; by contrast, right-sided (eg, tricuspid) PVT may cause pulmonary embolism. • Prosthetic valve dysfunction: usually manifesting as obstruction (stenosis) or, rarely, regurgitation. Patients may have a new murmur or signs and symptoms of heart failure. Dx: Transthoracic echocardiography Mechanical prosthetic valves: are more thrombogenic and require anticoagulation. CVS 262 https://t.me/usmleinnercircle Version 2 Bioprosthetic valves: are less thrombogenic and typically require only aspirin therapy Hereditary Hemorrhagic Telangiectasia Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu) CNS Mucocutaneous Lung GI Sequelae Hemorrhagic CVA Brain abscess: l)aradoxical bacterial embolization across eulmona!l'. AVM Oral & cutaneous telangiectasia Recurrent epistaxis le .. .. .. .. Pulmonary AVM: hemoptysis PAH: right-sided heart failure Chronic GI bleed Liver: portal hypertension, high-output heart failure irc Vascular bed AVM = arteriovenousmalformation;CVA = cerebrovascularaccident;GI= gastrointestinal;PAH = pulmonaryarterial hypertension. rC  In patients with symptomatic, bleeding pulmonary AVMs (hemoptysis), management involves pulmonary angiography followed by embolization. ne Exercise Induced Postural Hypotension Exercise-associated postural hypotension . . Cessation of exercise results in sudden decrease in venous return to the heart (preload) In Pathophysiology Athlete collaeses immediatell after cessation of .. ---. exercise No loss of consciousness LE Manifestations . . Normal to minimally elevated core temperature Trendelenburg positioning (ie, feet inclined above the head) Oral hydration U SM Management Dizziness or lightheadedness Paeds CVS CVS 263 https://t.me/usmleinnercircle Version 2 Approach to neonatal cyanosis Neonatal cyanosis ] Distal extremities & lips Mucosa! or diffuse Normal Sp0 2 Low Sp0 2 Peripheral cyanosis: Central cyanosis: Reassurance Administer 0 2 l Sp0 2 unchanged Sp0 2 increased ] OR j Hemodynamic instability l Cardiac pathology: Administer prostaglandin Pulmonary pathology:• Provide respiratory support (eg, intubation, ventilation) •CoJ,Sfder persistentpulmonaryhypertension of the newborn,partJCUarly if differential cyanosis1s present. Sp02 = oxygensaturation measuredby pulseoxlmetry. C>U'Norid Hyperoxia test for Central Cyanosis  What is the next step in management for a newborn with cyanosis that does not improve with 100% O2 and a continuous machine-like murmur on auscultation? Administer prostaglandin E1 cyanosis that fails to improve with 100% O2 is indicative of a possible congenital heart defect; maintaining the patency of the PDA can be life-saving This patient is cyanotic  PDA are not cyanotic at birth, thus do not give endomethacin! Congenital heart disease CVS Cause Clinical features Examples Left-to-right shunting • Tachypnea • Poor weight gain • Sweating with feeds • Ventricular septa! defect • Atrial septa! defect • Isolated patent ductus arteriosus Right-to-left shunting • Cyanosis • • • • • Interrupted left ventricular output • Pallor or shock • Severe acidosis • Coarctation of the aorta • Hypoplastic left heart syndrome Transposition of the great vessels Tetralogy of Fallot Tricuspid atresia Anomalous pulmonary venous return Truncus arteriosus 264 https://t.me/usmleinnercircle Version 2 Cyanotic heart disease in newborns X-ray findings Examination • Single S2 Transposition of the great vessels "Egg-on-a-string" heart (narrow mediastinum) • +/- VSD murmur • Harsh pulmonic stenosis murmur Tetralogy of Fallot "Boot-shaped" heart (right ventricular hypertrophy) • VSD murmur • Single S2 Tricuspid atresia Minimal pulmonary blood flow • VSD murmur • Single S2 Truncus arteriosus Total anomalous pulmonary venous return with obstruction Increased pulmonary blood flow, edema irc • Systolic ejection murmur (increased flow through truncal valve) le Diagnosis Pulmonary edema, "snowman" sign (enlarged supracardiac veins & SVC) • Severe cyanosis rC • Respiratory distress VSD Ventricular septal defect Chest x-ray ne . .. Asymptomatic Loud, harsh, holosystolic murmur at LLSB Normal . . .. Serial echocardiography Most spontaneously close at age <2 .. Moderate to large Respiratory distress, poor feeding, poor growth {due to pulmonary overcirculation & high-output LV failure) ± Holosystolic murmur at LLSB; loud S2 Cardiomegaly Increased pulmonary markings Diuretics Surgical closure LE Management .. In Clinical features Small LLSB = lower left sternal border; LV = left ventricular. U SM  What is the likely diagnosis in a young child that presents with failure to thrive and easy fatigability with a grade II holosystolic murmur best heard over the left sternal border with a diastolic rumble over the cardiac apex? Large ventricular septal defect VSD larger VSDs tend to be quieter (grade II due to less turbulence; the diastolic rumble is heard due to increased flow across the mitral valve Tricuspid Atresia CVS 265 https://t.me/usmleinnercircle Version 2 Tricuspid atresia Pulmonary ____ ,(artery ASD = atrial septal defect; PFO = patent foramen ovale; VSD = ventricular septal defect @UWorld  What is the likely diagnosis in a newborn that becomes cyanotic with left axis deviation on ECG and decreased pulmonary markings on CXR? Tricuspid valve atresia left axis deviation is seen as up-going R wave in lead I and down-going R wave in lead avF; lack of blood flow to the RV results in underdevelopment of the pulmonary valve and/or artery; ASD and VSD are necessary for survival (hence LVH and left axis deviation) Tricuspid atresia deviation Tall,peaked Pwaves .. CVS mu n\lL I I :r7Tn·~sl-vr·1·T·, Minimal R wave: JAA~ AA A,. AA 266 https://t.me/usmleinnercircle Version 2 • Absent tricuspid valve No flow from right atrium RA to right ventricle RV results in enlarged RA (ie, tall, peaked P waves) and hypoplastic RV Determine axis le 1. Look at the QRS complex in leads I and aVF. 2. If both are mainly positive, then the axis is normal. 3. If mainly positive in lead I and mainly negative in aVF, the axis is deviated lo the left. 4. If mainly negative in lead I and mainly positive in aVF, the axis is deviated to the right. 5. If mainly negative in both I and a VF, then there i.s extreme right axis deviation. irc ECG in a newborn normally shows physiologic right axis deviation. rC Persistent Pulmonary Hypertension of the Newborn Persistent pulmonary hypertension of the newborn • Abnormal persistence of elevated fetal pulmonary vascular resistance Pathogenesis • Right-to-left shunting across ductus arteriosus ne • Lung hypoplasia (eg, congenital diaphragmatic hernia) • Meconium aspiration syndrome Risk factors • Infection (eg, neonatal pneumonia) • L Postductal relative to preductal oxygen saturation • Respiratory distress & cyanosis In Examination • Prominent S2 • Oxygenation & ventilation • Inhaled nitric oxide (pulmonary vasodilator) LE Treatment also known as persistent fetal circulation U SM Hypoplastic Left Heart Syndrome Normal heart Hypoplastic left heart syndrome Narrow ascending aorta Atreticmitral Atrial septal defect Underdeveloped leftventricle <OUWorld CVS 267 https://t.me/usmleinnercircle Version 2  Present with tachypnea, cyanosis, no murmur, and a single S2; presentation is typically delayed until a few days after birth when the ductus arteriosus closes. CVS Surgery Primary survey in Advanced Trauma Life Support • Airway with cervical spine protection • • • • Breathing & ventilation Circulation with hemorrhage control Disability (basic neurologic assessment) Exposure & environmental control Local vascular complications of cardiac catheterization Hematoma Pseudoaneurysm Arteriovenousfistula • ± Mass • Nobruit • Bulging, pulsatile mass • Systolic bruit • No mass • Continuous bruit  Management of small AVFs involves observation (sometimes resulting in spontaneous closure) or ultrasound-guided compression. Large AVFs typically require surgical repair. • Femoral artery aneurysms often present with vascular claudication and a pulsatile groin mass. Complications include acute limb ischemia, rupture, or distal embolism. Risk factors include male sex, older age, tobacco use, hypertension, hyperlipidemia, and coronary artery disease. Diagnosis is most frequently made with duplex ultrasonography or CT angiography. Treatment of symptomatic or large  3 cm) aneurysms consists of surgical arterial repair. Small pseudoaneurysms can be treated with ultrasound-guided compression or thrombin injection into the pseudoaneurysm cavity CVS 268 https://t.me/usmleinnercircle Version 2 Inadequate post-procedural arterial compression is the strongest risk factor for pseudoaneurysm development. The diagnosis should be confirmed by ultrasound. CABG  Atrial fibrillation commonly (up to 15%40% occurs within a few days after CABG and is usually self-limited, with resolution in 24 hours. le Rate control with beta-blockers or amiodarone is best irc  Acute mediastinitis can occur following cardiac surgery and present with fever, chest pain, leukocytosis, and mediastinal widening on chest x-ray. It is a serious condition that requires drainage, surgical debridement, and prolonged antibiotic therapy. rC • Pleural effusions occur in almost half of patients who undergo coronary artery bypass graft surgery Post–cardiac surgery effusions that meet the following criteria can be managed conservatively ne with observation only: • Small to moderate in size and not enlarging • Early onset (postoperative day 1 or 2 In • Not associated with respiratory symptoms LE Postpericardiotomy Syndrome Post-cardiac injury syndrome U SM Causes Clinical • Myocardial infarction (ie, Dressler syndrome) • Cardiac surgery or trauma • Percutaneous coronary intervention • Latent period of several weeks to months • Pleuritic chest eain, fever, leukoc~osis • Chest x-ray: pleural effusion ± enlarged cardiac silhouette • Echocardiography: pericardia! effusion NSAID (usually high-dose aspirin)± colchicine Treatment • • Corticosteroids in refractory disease features Prognosis • Usually self-limited disease course • May cause chronic/recurrent disease leading to constrictive pericarditis NSAID = nonsteroidal anti-inftammatory drug.  What is the likely diagnosis in an infant that presents with distant heart sounds and hypotension with cardiomegaly on X-ray one week after having cardiac surgery? Postpericardiotomy syndrome (pericardial effusion after cardiac surgery) CVS 269 https://t.me/usmleinnercircle Version 2 It presents 16 weeks after cardiothoracy surgery with fever, chest pain, tachy, and pericardial friction rub, and is often associated with pericardial effusion (that may develop into cardiac tamponade) Sternal Dehiscence  Sternal dehiscence is a complication of cardiac surgery characterized by separation of the bony edges of the sternum. Patients may report mild pain or sensation of chest wall instability and "clicking" with chest movement.  The diagnosis can be made radiographically (eg, displaced sternal wire) or clinically; palpable rocking or clicking of the sternum confirms the diagnosis. Management involves urgent surgical exploration and repair. • Soft tissue dehiscence occurs when only the superficial tissues (eg, skin, muscle) separate. There are no signs of sternal instability or systemic illness; local wound care or debridement followed by primary closure is indicated.  A high-mortality complication of dehiscence is deep tissue infection (mediastinitis), due to either contiguous spread of superficial infection or intraoperative deep tissue contamination. Although it classically presents with systemic symptoms (eg, fever, tachycardia), chest pain, chest wall edema/crepitus, and purulent wound discharge, atypical presentations can occur; therefore, any patient with significant sternal wound drainage should be evaluated with chest and sternal imaging (eg, mediastinal fluid collections or pneumomediastinum on CT scan). Management includes emergency surgical debridement, tissue cultures, and empiric intravenous antibiotics. Preoperative Cardiac Revised Cardiac Risk Index (RCRI) (cardiovascular risk of noncardiac surgery) • High-risk surgery (eg, vascular, intrathoracic) • lschemic heart disease • History of congestive heart failure . 6 risk predictors History of cerebrovascular disease (stroke or TIA) • Diabetes mellitus treated with insulin • Preoperative creatinine >2 mg/dl Risk of cardiac death, nonfatal cardiac arrest, or nonfatal Ml risk* • 0-1 factor: low -• 2:2 factors: elevated risk *RCRI score of0-1 originally reported as :S1%and still accepted as low risk. Slightly higher event rates of later studies probably due to using troponins (T sensitivity) and including additional outcomes (eg, all-cause mortality). Ml = myocardial infarction; TIA = transient ischemic attack. CVS 270 https://t.me/usmleinnercircle Version 2 Perioperative cardiovascular complications, referred to as major adverse cardiovascular and cerebrovascular events MACCE are a significant cause of morbidity and mortality in noncardiac surgical cases. Anesthesia and surgery place the body under tremendous physiological stress. which can lead to myocardial ischemia, infarction and potential cardiac death. Intermediate Low • Eat, dress, use toilet Surgery type <4METs Aortic or other major vascular lntrathoracic Open intraperitoneal • Walk indoors in the house Do light housework (eg, vacuuming) • .. .. irc High .. . • . • • .• Head apd peck (eg, thyroidectomy) Orthopedic i!:4METs E=lale.. Bmasl..(eg, lumpectomy, mastectomy) Endoscopic procedure Cataract extraction Climb a flight of stairs Run a short distance Do yardwork (eg, raking leaves) Participate in golf, tennis, or dancing rC Risk level Estimated cardiac functional capacity by activity (METs*) le Cardiac risk* of selected noncardiac surgical procedures •1 MET (metabolicequivalent)=volumeof oxygen consumedat rest (3.5 ml 02 uptake/kg/min). ne •cardiac death, nonfatalcardiacarrest, or nonfatalmyocardialinfarction. Preoperative evaluation of ischemic heart disease for noncardiac surgery I No t ----Yes----• In Emergency procedure• Acute coronary syndrome .. ----Yes----• Proceed to surgery Postpone surgery: manage per guidelines I LE No t Low risk for MACE: S1 RCRI or <1% risk calculator U SM I No t Functional capacity 24 METs I No Proceed to surgery t Cardiac evaluation will impact management I Yes t Pharmacologic stress test ]--- Negative----------~ I Positive .. Coronary revascularization "Emergency procedure.<6 hoursto avOtdlossof llfe/limb. --•Acutecoronarysyndromeacuteor recent(<60 days)myocardialinfarction or unstableangina. MACE = majoractversecardiacevent MET = metabolicequivalent RCRI = Revised Cardiac Risk Index. CVS CU\Nocld 271 https://t.me/usmleinnercircle Version 2 Anticoagulation management of preoperative atrial fibrillation Valvular heart disease management before noncardiac surgery Valve dysfunction Mild J Surgical bleeding risk Moderate/severe ! Symptoms I Minimal No Surgical bleeding risk I Low/moderate/high Minimal Low/moderate/high i Yes i Thromboembolic risk• Surgical risk J Low Intermediate or high ' t Noncardiac surgery J Moderate/high • StopVKA 3--5 d prior • No bridge • StopVKA 3--5d prior • Bridge.. i Valve-specific criteria• (eg, normal EF & no significant PHTN) Yes Low • Stop DOAC day of surgery • No bridge •Stop DOAC 1-3 d prior • No bridge • Continue VKA • No bridge i •Thromboembolic risk: Low: C~DS,-VASc S4 & no prior stroke Moderate: CHA:PSrVASc 5-6 or prior stroke >3 months High: CHApS 2-VASc ~7. stroke <3 months, or mechanical valve No t Valve intervention before surgery 'ARJAS = normal EF, MS= no significant PHTN, MR= normal EF & no significant PHTN. AR= aortic regurgitation;AS= aortic stenosis; EF = ejection fracbon; MR= m,tral regurgitation; MS = mitral stenosis, PHTN = pulmonaryhypertension. ••Moderate= consider bndging; High= bridge 1.11less very high bleeding risk. DOAC = direct oral anticoagulant: VKA = vitamin K antagonist CUVVorld OUWo<ld Hemodynamic management of preoperative atrial fibrillation Hemodynamically stable & asymptomatic Hemodynamically stable with uncontrolled rate Hemodynamically unstable (eg, hypotension, syncope, CP, HF) l Continue prior rate or rhythm control medications Achieve rate control -Failure(BB or ND-CCB) Electrical cardioversion I Success 't Optimize electrolytes, blood pressure & volume status Proceed to surgery after addressing anticoagulation Dela sur ery to address underlying cause(s) & anticoagulation BB = beta blocker. CP = chest pain: HF = heart failure: ND-CCB = nondihydropyridine calcium channel blocker. ©UWotld Other Tests CVS 272 https://t.me/usmleinnercircle Version 2 Indications for select preoperative tests Chest radiograph Hemoglobin Coagulation & platelets Creatinine & electrolytes .. .. History of coronary artery disease or arrhythmia Asymptomatic patients with risk of MACE ~1% History of cardiopulmonary disease Undergoing upper abdominal/thoracic surgery History of anemia, significant expected blood loss Undergoing major surgery History of abnormal bleeding, anticoagulant use Liver disease, malignancy, planned spinal anesthesia History of kidney disease, cardiovascular risk calculation Predisposing medications (eg, diuretic, ACE inhibitor, ARB) ACE = angiotensin-converting enzyme; ARB = angiotensinreceptorblocker;MACE = major adverse Vascular Trauma .. .. .. .. . Hard signs rC Extremity vascular trauma irc cardiovascularevent. le .. .. .. ECG Observed pulsatile bleeding Presence of bruiUthrill over injury Expanding hematoma Signs of distal ischemia ne Clinical manifestations Soft signs History of hemorrhage Diminished pulses In Bony injury Neurologic abnormality LE If hard signs or hemodynamic instability Evaluation Surgical exploration Otherwise .• • Injured extremity index CT scan or conventional angiography Duplex Doppler ultrasonography U SM -  Signs of distal ischemia(eg, absent pulses, cool extremities) Blunt Cardiac Injury CVS 273 https://t.me/usmleinnercircle Version 2 Blunt chest trauma Hemodynamically stable Hemodynamically unstable l l Resuscitation and evaluation eFAST • Chest x-ray • ECG High-risk mechanism or seriousinjuryon examination +-----Yes----- I +/. stabilizing intervention (eg, chest tube) if indicated No l l Hemodynamic stability achieved/maintained? Abnormal findings on evaluation, chest x-ray, ECG I I No No l l OR thoracotomy Additional tests (eg, CT chest) Possible discharge or observation eFAST= extendedFocusedAssessmentwithSonography forTral.lTla,OR= operatingroom Pathophysiology Clinical spectrum Confirmatory testing . . .. . .• OUWo<ld Blunt cardiac injury Rapid deceleration or direct blow to the precordium - shearing, compression, abrupt pressure change Arrythmia ranging from asymptomatic (eg, PVCs) to fatal (eg, VFib) • Acute coronary syndrome from coronary dissection or thrombosis Myocardial dysfunction ("myocardial contusion") Ruptured valve, septum, or ventricular wall Cardiac tamponade ECG Echocardiogram PVCs = premature ventricular contractions;Vfib = ventricularfibrillation.  Patients with findings concerning for BCI require 2448 hours of continuous cardiac monitoring because most life-threatening arrythmias occur during that time. Blunt cardiac injury can lead to Obstructive Shock or Cardiogenic Shock Myocardial Contusion What is the likely diagnosis in a hemodynamically unstable patient that presents following a motor vehicle accident with an elevated PCWP that worsens after administration of IV fluids? Myocardial contusion An elevated PCWP at baseline should raise suspicion of myocardial contusion (hypovolemic shock would have a low PCWP; urgent echocardigram is required for further evaluation Cardiac contusion results in cardiac dysfunction with poor cardiac output Cardiac contusion: arrhythmia (palpitations), hypotension and tachycardia that do not respond to fluid resuscitation (hypotension and tachy in trauma pt due to hemorrhage, however contusion should be suspected if tachycardia persists despite fluid resuscitation) CVS 274 https://t.me/usmleinnercircle Version 2 U SM LE In ne rC irc le Might as well have mild increase in troponins CVS 275 https://t.me/usmleinnercircle Version 2 Endocrine Pituitary HyperPituitarism SIADH HypoPituitarism Thyroid Hyperthyroidism Gestational Transient Thyrotoxicosis Hypothyroidism Subclinical Hypothyroidism Euthyroid Sick Syndrome Parathyroid Gland Hyperparathyroidism Adrenal Gland Adrenal Excess Conn Syndrome Cushing Adrenal Insufficiency Pancreas Diabetes Screening DKA Treatment Diabetic Nephropathy Metabolic Syndrome Hypoglycemia Endocrine Tumor Thyroid VIPoma Zollinger Ellison Neuroblastoma MEN Pheochromocytoma Endocrine Drugs Miscellaneous Milk Alkali Syndrome Hypoglycemia associated autonomic failure Endocrine Paeds Neonatal Thyrotoxicosis Congenital hypothyroidism Congenital Adrenal Hyperplasias Endocrine 276 https://t.me/usmleinnercircle Version 2 Pituitary HyperPituitarism Treatment .. . . Premenopausal women: Oligo/amenorrhea, infertility, galactorrhea, hot flashes, decreased bone density Postmenopausal women: Mass effect symptoms (headache, visual field defects) Men: Infertility, decreased libido, impotence, gynecomastia Serum prolactin (often >200 ng/ml) le Laboratory/imaging . .. Rule out renal insufficiency (creatine) & hypothyroidism (thyroid stimulating hormone, thyroxine) MRI of the head irc Clinical features Prolactinoma overview Dopamine agonist (cabergoline) • Transsphenoidal surgery rC  Mild elevations 20200 ng/mL may also be due to a prolactinoma, but other causes such as: medications (eg, certain antipsychotics), nipple stimulation, ne pregnancy, hypothyroidism, and In stress should also be considered. U SM LE  A prolactin level > 200 ng/mL is virtually diagnostic of prolactinoma. Endocrine 277 https://t.me/usmleinnercircle Version 2 Management of hyperprolactinemia in premenopausal women • Detailed clinical evaluation • Rule out secondary causes (eg, pregnancy) MRI of pituitary gland ] Asymptomatic microprolactinoma (<10mm) l No treatment ] • Macroprolactinoma (:e10 mm) • Risk or presence of neurologic symptoms • Hypogonadism • Galactorrhea ! Dopamine agonists • Cabergoline • Bromocriptine ! • Persistent hyperprolactinemia • Persistent symptoms • No decrease in tumor size • Intolerance to medication ! Consider surgical resection CUWorld  What is the preferred imaging modality for evaluation of pituitary tumors? MRI recommended for patients with elevated prolactin, mass-effect symptoms, very low testosterone levels, or disruptions in other pituitary hormones Endocrine 278 https://t.me/usmleinnercircle Version 2 Clinical suspicrn of acromegaly IGF-1level Nor'7 Rules out acromegaly L ~evated Oral glucose suppression test AdecuateGH suppression lnadecuate GH suppression Evaluate for extrapituitary cause of acromegaly (eg, ectopic GH- & GH RHsecreting tumors) rC Surgical versus medical management Normal pituitary irc Pituitary mass le MRI of brain GH = growthhormone;GHRH = growthhormone-releasinghormone;KiF-1 = insulin-likegrowthfactor1. ©UWortd In ne As a general rule in endocrinology, imaging is performed after the biochemical diagnosis of a disorder is made. Clinical features of acromegaly Musculoskeletal Skin Cardiovascular Gigantism, frontal bossing, malocclusion of jaw, macrognathia, arthritis, carpal tunnel syndrome, enlargement of hands/feet Skin thickening, hyperhidrosis {odor), skin tags Cardiomyopathy, hypertension, heart failure Sleep apnea U SM Respiratory Headache, visual field defects, cranial nerve defects LE Local tumor effect Gastrointestinal Colon polyps/cancer, diverticulosis Endocrine Galactorrhea, hypogonadism, diabetes mellitus, hypertriglyceridemia Additional features Enlarged tongue, thyroid, salivary glands, liver, spleen, kidney, prostate SIADH Endocrine 279 https://t.me/usmleinnercircle Version 2 Syndrome of inappropriate antidiuretic hormone Etiologies Clinical features Laboratory findings Management .. . .. .. . .. .. .. CNS disturbance (eg, stroke, hemorrhage, trauma) Medications (eg, carbamazepine, SSRls, NSAIDs) Lung disease (eg, pneumonia) Ectopic ADH secretion (eg, small cell lung cancer) Pain &/or nausea Mild/moderate hyponatremia: nausea, forgetfulness Severe hyponatremia: seizures, coma Euvolemia (eg, moist mucous membranes, no edema, no JVD) Hyponatremia Serum osmolality <275 mOsm/kg H2O (hypotonic) Urine osmolality >100 mOsm/kg H2O Urine sodium >40 mEq/L Fluid restriction ± salt tablets Hypertonic (3%) saline for severe hyponatremia ADH = antidiuretichormone;JVD = jugular venous distension;NSAIDs= nonsteroidalantiinflammatorydrugs; SSRls = selectiveserotoninreuptakeinhibitors. Stimuli for secretion of antidiuretic hormone Osmotic • Serum osmolality > -285 mOsm/kg H 2O • Nausea Nonosmotic • Pain • Physical or emotional stress • Hypotension • Hypovolemia • Hypoxia • Hypoglycemia • Intranasal Desmopressin use for DI can cause Secondary SIADH. HypoPituitarism ADH-related causes of polyuria & polydipsia Defect Primary polydipsia Central DI Nephrogenic DI t Water intake I ADH release from pituitary ADH resistance in kidney • Antipsychotics Etiology Clinical features Endocrine • Anxious, middle-age women Low serum Na • Idiopathic • Trauma • Pituitary surgery • lschemic encephalopathy High serum Na • Chronic lithium use • Hypercalcemia • Hereditary (AVPR2 mutations) Normal serum Na 280 https://t.me/usmleinnercircle Version 2 Serum Na+  137 mEq/L with dilute urine favours primary polydipsia and excludes diabetes insipidus • Central DI usually has significant hypernatremia 150 mEq/L due to an impaired thirst mechanism. Patients with nephrogenic DI usually have an intact thirst mechanism and adequate water intake; they usually compensate for renal water loss and may have a normal sodium level. Evaluation of suspected polyuria j irc l Concentrated urine: Osmotic diuresis Increased solute excretion __J (glucose, ~• saline) ne Dilute urine: Water diuresis Primary polydipsia, diabetes insipidus Urine output < 3L: Not true polyuria; work up causes of urinary frequency rC Urine output> 3L: Polyuria present l le Complete 24-hour urine collection C)UWarid In Low Urine osmolality and specific gravity 1.006  DI LE High Urine osmolality and specific gravity 1.006  DM . Clinical features of hypopituitarism Pituitary causes U SM • Primary (eg, adenoma) or metastatic mass Etiology Clinical presentation Endocrine . . . . • Infiltration (eg, hemochromatosis, lymphocytic hypophysitis) • Hemorrhage (pituitary apoplexy) or infarction (Sheehan syndrome) Hypothalamic causes • Mass lesions • Radiation therapy • Infiltration (sarcoidosis) • Trauma to skull base • Infections (tuberculosis meningitis) ACTH deficiency (secondary adrenal insufficiency) o Postural hypotension, tachycardia, fatigue, weight loss, hypoglycemia, eosinophilia Hypothyroidism (central) o Fatigue, cold intolerance, constipation, dry skin, bradycardia, slowed deep tendon reflexes Gonadotropins o Women: Amenorrhea, infertility o Men: Infertility, loss of libido 281 https://t.me/usmleinnercircle Version 2  What is the likely diagnosis in a patient that presents with constipation, cold intolerance, low libido, and hypoglycemia with eosinophilia on laboratory exam? Hypopituitarism patient is experiencing secondary hypothyroidism, secondary adrenal insufficiency, and hypogonadotropic hypogonadism Eosinophilia secondary to central adrenal insufficiency Remember: Aldosterone levels will be Normal Thyroid  What is the diagnostic test for thyroglossal duct cyst and why is it done? • U/S to evaluate cyst and confirm location of thyroid, screen TSH; • surgical excision 0 Need to make sure this is not the patients only functioning thyroid tissue and need to prevent postop hypothyroidism Hyperthyroidism Evaluation of hyperthyroidism Measure TSH, free T3 & T4 ,-----=---~- ~-- Primary hyperthyroidism •TSHlow • Free T3 & T4 high Secondary hyperthyroidism •TSH high • Free T3 & T4 high Signs of Graves disease (goiter+ ophthalmopathy)? Yes MRI of pituitary gland No Radioactive iodine uptake(+ scan) Low High I i I Nodular pattern 1 1 Graves disease Endocrine i Diffuse pattern I • Toxic adenoma • Multinodulargoiter Measure serum thyroglobulin I • Thyroiditis • Iodide exposure ilow I Exogenous hormone I 282 https://t.me/usmleinnercircle Version 2 Management of postpartum thyroiditis Confirm diagnosis l T4 and T3; ! TSH l Thyroglobulin l RAIU (±)TPO antibody; 0TRAb l l Hypothyroid phase ! T4 and T3; l TSH l Euthyroid phase - Beta blocker for symptom control - • Breastfeeding or attempting pregnancy? Hypothyroid symptoms? TSH > 6 months _ -- Levothyroxine irc l T4 and T3; ! TSH le Hyperthyroid phase • Wean off Levothyroxine (as tolerated) • Annual TSH measurement rC Normal T4,T3, and TSH ne RAIU = radioiodine uptake; TPO = thyroid peroxidase; TRAb = thyrotropin receptor antibody. Evaluation of thyroid nodules l In [ TSH level & thyroid US I l Normal or elevated TSH Hyperthyroid bone disease I Excess thyroid hormone I I Osteoclast activity LowTSH LE CUWo'1d • t Booe resorption I Bone density I Fracture risk Radioactive iodine scintigraphy U SM Hypofunctional ("cold") or indeterminate nodule Consider FNA based on size & US findings FNA • fine-needle aspiration; us• Endocrine uttrasonography. I Conversion 25-0H-vitamin D to 1,25-0H-vitamin D t Catabolism 1,25-0H-vitamin D I Renal calcium reabsorption ~----.--~ Hyperfunctional ("hot") nodule Hypercalciuria Net calcium wasting I Gastrointestinal calcium absorption I Renal calcium reabsorption PTH= parathyroidhormone Treat hyperthyroidism Cuw""" 283 https://t.me/usmleinnercircle Version 2 Antithyroid drugs .. Choice of treatment in Graves disease Can induce remission in many patients May be used as pretreatment prior to radioactive iodine or thyroidectomy (eg, high-risk patient, severe hyperthyroidism) Radioactive iodine Thyroidectomy .. . • Acceptable option for moderate hyperthyroidism May initially worsen ophthalmopathy and hyperthyroid symptoms Leads to permanent hypothyroidism over time Preferred in patients with very large goiter, obstructive symptoms, or ophthalmopathy e,f!.. C.Arl c.FP- After initiating antithyroid pharmacologic therapy for Graves disease, frequent monitoring of thyroid hormones is necessary to ensure correct dosing. Once the thyroxine concentration has stabilized and the patient is euthyroid, the dose of propylthiouracil can be decreased by 3050%. If it is not decreased, secondary hypothyroidism may develop. Serum total T3 and free T4 levels are used to assess thyroid function during treatment with antithyroid drugs. TSH may remain suppressed for several months following initiation of therapy and does not reliably reflect thyroid functional status during this time. Choice of treatment in Graves hyperthyroidism Antithyroid drugs • Mild hyperthyroidism • Older age with limited life e~pectancy • Preparation for radioactive iodine or thyroidectomy Radioactive iodine • Moderate lo severe hyperthyroidism with/Without mild ophthalmopathy • Palient preference in mild hyperthyroidism Thyroidectomy • Very large goiter • Suspicion of thyroid cancer • Coexisting primary hyperparathyroidism • Pregnant patients who cannot tolerate thiooamides • Pregnancy (PTU in 1st trimester) • Severe 0phlhalmopathy • Retrostemal goiter with obstructive symploms PTU = p,opyllhiouracil. ©UWorld • Radioactive iodine Endocrine 284 https://t.me/usmleinnercircle Version 2 contraindicated in patients with severe ophthalmopathy (due to increased thyroid-stimulating immunoglobulin titers); glucocorticoids and antithyroid drugs may be given initially to minimize side effects of RAI in patients with mild ophthalmopathy  What is a common complication of radioactive iodine when treating Graves disease? Permanent hypothyroidism the dose needed for treatment, as well the diffuse uptake of iodine in Graves disease, results in permanent hypothyroidism in 90% of patients Clinical features of thyroid storm • Thyroid or nonthyroidal surgery Precipitating • Acute iodine load (eg, iodine contrast) • High fever rC • Acute illness (eg, trauma, infection), childbirth factors irc le In contrast, when RAI is used to treat toxic nodular goiter and toxic adenoma, the radioisotope is taken up only by the autonomous thyroid tissue, and the function of the remaining normal tissue is usually adequate to prevent permanent hypothyroidism. • Tachycardia, hypertension, congestive heart failure, cardiac arrhythmias (eg, atrial fibrillation) Clinical • Goiter, lid lag, tremor ne • Agitation, delirium, seizure, coma presentation • Nausea, vomiting, diarrhea, jaundice .. .. .. Thyrotoxicosis with ! RAIU Painless (silent) thyroiditis Subacute (de Quervain) thyroiditis • Amiodarone-induced thyroiditis Graves disease Toxic multi nodular goiter LE .. . In Thyrotoxicosis with normal or t RAIU Toxic nodule Excessive dose (or surreptitious intake) of levothyroxine Struma ovarii Iodine-induced Extensive thyroid cancer metastasis U SM RAIU = radioactive iodine uptake. Painless thyroiditis is associated with thyroid peroxidase autoantibodies and is considered a variant of chronic lymphocytic Hashimoto) thyroiditis. Clinical manifestations of Graves disease General Heat intolerance, weight loss, sweating Eyes Lid lag, proptosis, diplopia Skin Hair loss, infiltrative dermopathy (pretibial myxedema) Cardiovascular Nails Endocrine Tachycardia, hypertension, atrial fibrillation Onycholysis, clubbing (acropachy) Hyperglycemia, hypercalcemia, bone loss, menstrual irregularities Gastrointestinal Diarrhea Neurology Endocrine Tremors, hyperreflexia, proximal muscle weakness 285 https://t.me/usmleinnercircle Version 2 Clinical features of thyrotoxicosisin older patients • Atrial fibrillation • Tachycardia (may be absent due to conduction defects or beta blockers) Cardiovascular • Heart failure • Apathy, confusion Neurologic • Tremor (often absent) • Proximal muscle weakness/wasting Proptosis, lid lag, thyromegaly (often absent) Endocrine Gastrointestinal • Decreased appetite • Constipation -  What is the likely diagnosis in a patient that develops post-operative high fever, tachycardia, hypertension, and lid lag with no muscle rigidity? Serum creatinine kinase is slightly elevated. Thyroid storm typically triggered by a specific event in patients with undiagnosed or inadequately treated hyperthyroidism (e.g. surgery, trauma, infection, CT scan with contrast due to acute iodine load) Also on the differential is pheochromocytoma, but will have no fever; episodes of pheochromocytoma can be precipitated by medications, especially anesthetic agents, and surgical procedures Also malignant hyperthermia -- look for similar symptoms but with elevated CK, hyperkalemia, and muscle rigidity Gestational Transient Thyrotoxicosis Physiologic changes .. . . Management . Endocrine Thyroid changes in pregnancy Increased thyroid hormone demand Estrogen increases thyroxine-binding globulin --> increased total T4 & T3 levels hCG stimulates thyroid follicles for increased T4 & T3 production with feedback sueeression of TSH Use trimester-specific norms when interpreting TSH 0 First trimester: 0.1-2.5 µU/ml o Second trimester: 0.2-3.0 µU/ml o Third trimester: 0.3-3.0 µU/ml Check free T4 or total T4/T3 levels if TSH significantly suppressed (<0.1 µU/ml) 286 https://t.me/usmleinnercircle Version 2 Evaluation of hyperthyroidism in pregnancy starts with measuring Free T4 levels (it is the only reliable marker in pregnancy) • TSH is the recommended initial screening test in pregnant patients with suspected hyperthyroidism. If TSH is suppressed below trimester-specific norms, free or total T4 is used for confirmation. If TSH is suppressed but T4 is normal or equivocal, a total T3 level may be useful. • Hyperthyroidism: PTU 1st trimester), Methimazole 2nd/3rd trimester) 0 irc • Hypothyroidism: Levothyroxine le  What is the treatment of hypo/hyperthyroidism in pregnancy? Monitor q4 weeks as pregnancy ↑ TBG, which will increase L-thyroxine requirements rC Overtreatment of maternal hyperthyroidism can cause fetal hypothyroidism and goiter, so treatment should be titrated to maintain a mild hyperthyroid state. Prepregnancy management ne Feedback suppression of TSH j Circulating thyroxine-binding globulin j Total T3 & T4, slight increase in free T3 & T4 Optimize levothyroxine dose to maintain TSH in low-normal range j Levothyroxine dose 30% at time of positive pregnancy test Measure TSH every 4 weeks & adjust levothyroxine dose to trimester-specific TSH norms LE Pregnancy management . . .. Stimulation of TSH receptors by J3-hCG In Physiologic changes . .. Hypothyroidism in pregnancy  What is the recommended management for a woman with medically-managed hypothyroidism that desires to get pregnant in the near future? U SM Increase levothyroxine dose at the time pregnancy is detected  During pregnancy, hCG directly stimulates TSH receptors on thyroid follicles, increasing the release of thyroid hormone. Very high hCG levels (eg, multiple gestation, hyperemesis gravidarum) can cause gestational transient thyrotoxicosis. This condition is generally self-limited and resolves as hCG levels fall by 1416 weeks gestation Because the severity of hyperemesis correlates with hCG levels, continued clinical hyperthyroidism after the resolution of vomiting may warrant additional investigation. Endocrine 287 https://t.me/usmleinnercircle Version 2 Thyroid disease during pregnancy Normal changes TSH Hyperthyroidism Hypothyroidism ! (<0.1 mU/L) i (> 4.0 mU/L) Slight! (0.1-4.0 mU/L) Free T4 No change Total T4 i No change (subclinical) or No change (subclinical) or i (overt) ! (overt) i Variable Hypothyroidism Thyroiditis Chronic autoimmune thyroiditis (Hashimoto thyroiditis) Painless thyroiditis (silent thyroiditis) Subacute (granulomatous) thyroiditis (de Quervain thyroiditis) .. .. .. .. . Clinical features Predominant hypothyroid features Diffuse goiter Variant of chronic autoimmune thyroiditis Mild, brief hyperthyroid phase Small, nontender goiter Spontaneous recovery Likely postviral inflammatory process Prominent fever & hyperthyroid symptoms Painful/tender goiter Diagnostic testing .. Variable radioiodine uptake . Positive TPO antibody . .. Positive TPO antibody Low radioiodine uptake Elevated ESR & CRP Low radioiodine uptake CRP = C-reactive protein; ESR = erythrocyte sedimentation rate; TPO = thyroid peroxidase. Painless thyroiditis is often a self-limited hyperthyroid phase of Hashimoto thyroiditis (hence the anti-TPO AB What is the likely diagnosis in a patient with a long history of Hashimoto thyroiditis that develops a rapidly enlarging, firm goiter with dysphagia, hoarseness, and fever? Thyroid lymphoma may also have facial plethora and cyanosis when raising the arms above the head due to compression of the subclavian vein and IJV between the clavicle and thyroid Pemberton sign) Endocrine 288 https://t.me/usmleinnercircle Version 2 Levothyroxine replacement therapy for hypothyroidism Dose adjustments Maintenance therapy Conditions requiring higher doses .. . . .. .. Standard dose: 75-125 mcg/day Elderly, heart disease: 25-50 mcg/day Increase dose every 6 weeks until TSH is within normal range Monitor TSH every 6-12 months Malabsorption (eg, celiac disease) Drugs that interfere with absorption (eg, iron, calcium) Drugs that increase thyroxine metabolism (eg, phenytoin, carbamazepine, rifampin) Other: obesity, pregnancy, overt proteinuria le Initial dose Subclinical hypothyroidism Elevated TSH (verified on repeat measurement) Normal free T4 rC .. . Clinical features Mild symptoms may or may not be present • TSH 2:10µU/ml • TSH 7-9.9 µU/ml o Age <70: treat o Age .::70:treat if convincing hypothyroid symptoms ne Indications for treatment irc Subclinical Hypothyroidism • TSH upper limit of normal - 6.9 µU/ml o Age <70: treat if convincing hypothyroid symptoms, enlarging goiter, or elevated anti-TPO titer o Age .::70: do not treat (possible harm) In TPO = thyroid peroxidase. LE Subclinical hypothyroidism Subclinical hypothyroidism (increased TSH, normal T4) U SM TSH > 10 µU/ml) TSH < 10µU/ml but> upper limit of normal J ! ! Yes Treat with levothyroxine ] ..---- L Positive anti-thyroid peroxidase antibody Patient with the fo~o::ng: • Goiter • Symptomatic • Pregnancy • Ovulatory dysfunction wrth infertility • Hypercholesterolemia !No Routine monitoring J C,UWorld  What pregnancy complication is associated with subclinical hypothyroidism? Miscarriage Endocrine 289 https://t.me/usmleinnercircle Version 2 due to high titers of anti-thyroid peroxidase antibodies; increased risk in both euthyroid and hypothyroid women The American Thyroid Association ATA recommends all individuals over the age of 40 to be screened for thyroid dysfunction. The American College of Physicians recommends screening women over 50 years with findings suggestive of thyroid disease. Euthyroid Sick Syndrome Euthyroid sick syndrome Early/mild Prolonged/severe T3 t t T4 Normal t TSH Normal t Reverse T3 t t • Low T3 is thought to be the result of decreased peripheral conversion of T4 to T3. ESS represents a mild, transient central hypothyroid state that functions to minimize maladaptive catabolism in severe illness. Treatment is not recommended unless abnormal thyroid function persists after the patient has returned to baseline health. rT3 is primarily used in patients with low TSH to differentiate central hypothyroidism (low T4 leads to low rT3 from ESS.  Factors that suppress conversion of T4 to T3 include: • Caloric deprivation • Increased glucocorticoid and inflammatory cytokine (eg, tumor necrosis factor, interferon alpha) levels • Elevated free fatty acid levels Parathyroid Gland Endocrine 290 https://t.me/usmleinnercircle Version 2 Treatment of chronic hypoparathyroidism is with calcium and vitamin D, but calcium and phosphorus levels must be closely monitored. A calcium-phosphorus product (serum calcium × serum phosphorus) 55 increases the risk of soft tissue calcification. Calcification in the basal ganglia can cause extrapyramidal manifestations. Hyperparathyroidism le  What tests help localize the involved gland in hyperparathyroidism? Primary hyperparathyroidism irc U/S and nuclear imaging (99-m technetium sestamibi scan below) are most used • Parathyroid adenoma (most common), hyperplasia, Etiology carcinoma rC • Increased risk in MEN types 1 & 2A • Asymptomatic (most common) • Mild, nonspecific symptoms (eg, fatigue, constipation) Symptoms • Abdominal pain, renal stones, bone pain, neuropsychiatric symptoms • Hypercalcemia Diagnostic ne • Elevated or inappropriately normal PTH findings • Elevated 24-hour urinary calcium excretion • Age <50 • Symptomatic hypercalcemia • Complications: Osteoporosis (T-score <-2.5, fragility In Indications for fracture), nephrolithiasis/calcinosis, CKD (GFR <60 mUmin) parathyroidectomy • Elevated risk of complications: Calcium ::>:1mg/dl above normal, urinary calcium excretion >400 mg/day LE CKD = chronic kidney disease; GFR = glomerular filtration rate; MEN = multiple endocrine neoplasia; PTH = parathyroid hormone.  What is the likely diagnosis in a patient with an elevated serum PTH/Ca2 and normal urine Ca2/Cr U SM clearance ratio  0.02? Primary hyperparathyroidism differentiated from familial hypocalciuric hypercalcemia by increased urine Ca2 excretion  0.02  Newborn boy presents three days after delivery with tachypnea, tachycardia, spasms, seizures. Labs show ↓ PTH, ↓ Ca++. The remainder of exam shows no abnormalities. Most likely cause? Maternal familial hypocalciuric hypercalcemia • Asymptomatic so may go undiagnosed • Maternal hyperCa++ causes hyperCa++ in fetus → suppression of parathyroid glands • After delivery, fetus presents with hypoparathyroidism and hypocalcemia • Ddx: DiGeorge → will have CATCH features as well Endocrine 291 https://t.me/usmleinnercircle Version 2  In patients with long-standing CKD, especially those with end-stage renal disease ESRD, chronic parathyroid stimulation can lead to parathyroid hyperplasia. This is associated with autonomous PTH secretion (ie, not suppressed by rising calcium levels) due to downregulation of calcium-sensing receptor and vitamin D receptor in the parathyroid glands. The net effect, termed tertiary hyperparathyroidism, is characterized by: • Extremely high serum PTH levels • Hypercalcemia due to mobilization from bone • Hyperphosphatemia due to renal phosphate retention  Indications for parathyroidectomy in tertiary hyperparathyroidism include: • Persistently elevated calcium (eg, 10.5 mg/dL), phosphorus, or PTH (eg, 800 pg/mL) levels • Soft tissue calcification or calciphylaxis (vascular calcification with skin necrosis) • Intractable bone pain or pruritus  Parathyroidectomy is recommended for patients with primary hyperparathyroidism who have symptomatic hypercalcemia, complications (eg, osteoporosis, nephrolithiasis, chronic kidney disease), or increased risk for complications (eg, moderate to severe hypercalcemia). In addition, patients age 50 are likely to develop complications later in life and should undergo parathyroidectomy. Adrenal Gland Adrenal Excess Functional adrenal masses Cortisol-producing adenoma (most common) Aldosterone-producing adenoma Pheochromocytoma . .. . .. .. Clinical features Central obesity, moon facies, enlarged dorsocervical fat pad Hypertension Proximal muscle weakness Hyperglycemia, hypokalemia Hypertension Hypokalemia (muscle cramps, weakness) Paroxysmal tachycardia, headache, tremor Hypertension Hormone levels .. . . . . Elevated cortisol (urine, salivary) Decreased ACTH & DHEAS No suppression by dexamethasone Elevated plasma aldosterone-to-plasma renin activity ratio Elevated urine metanephrines & catecholamines Elevated plasma metanephrines DHEAS = dehydroepiandrosteronesulfate. Conn Syndrome Endocrine 292 https://t.me/usmleinnercircle Version 2 Primary hyperaldosteronism (Conn syndrome) Diagnosis Treatment Bilateral adrenal hyperplasia (-60%) or unilateral adenoma Difficult-to-control hypertension Hypokalemia & metabolic alkalosis* No extravascular volume overload (aldosterone escape) Elevated plasma aldosterone & low plasma renin (ratio >20) Absence of aldosterone suppression with oral saline load CT scan of adrenal glands to determine bilateral h~perplasia vs adenoma Bilateral adrenal hyperplasia: MRA (eg, spironolactone) Unilateral adenoma: surgical resection preferred *In some patients, the characteristic electrolyte abnormalities are apparent only after a thiazide is initiated for blood pressure management. Evaluation of suspected hyperaldosteronism rC Hypertension & hypokalemia irc MRA = mineralocorticoid receptor antagonist. le Clinical features .. .. .. . .. Measure plasma renin activity & ne plasma aldosterone concentration f PRA,f PAC, PAC/PRAratio -10 !PRA, f PAC, PAC/PRAratio .!:20 In AND PAC.!e15 ng/dl U SM LE Secondaryhyperaldosteronism Diuretic use Cirrhosis or congestive heart failure Renovascularhypertension Renin-secreting tumor Malignant hypertension Coarctation of aorta Primary hyperaldosteronism Adrenal computed tomography to determine etiology Other causesof hypertension& hypokalemia Congenital adrenal hyperplasia Glucocorticoid resistance Exogenous mineralocorticoid Cushing'ssyndrome Altered aldosterone metabolism ©UWortd What is the likely diagnosis in a young patient with hypertension that develops severe weakness and muscle cramps after beginning a low-dose thiazide diuretic? Primary hyperaldosteronism patients with mild primary hyperaldosteronism are prone to developing diuretic-induced hypokalemia; other findings include metabolic alkalosis and mild hypernatremia Endocrine 293 https://t.me/usmleinnercircle Version 2 Adrenocortical causes of hypertension Plasma aldosterone Plasma renin activity Aldosterone/ renin ratio Primary hyperaldosteronism t ! t Secondary hyperaldosteronism t t +-+ Nonaldosterone mediated ! ! Clinical examples .. .. . .. . +-+ Bilateral adrenal hyperplasia Adrenal adenoma Renovascular HTN Malignant HTN Renin-secreting tumor Cushing syndrome Exogenous mineralocorticoids (eg, fludrocortisone) Deoxycorticosterone excess* 'Includes deoxycorticosterone-producing adrenal tumors (rare) & relatively uncommon forms of congenital adrenal hyperplasia (eg, 11J3-hydroxylasedeficiency). HTN = hypertension. Hypertension & hypokalemia Plasmaaldosterone/renin ratio Evaluatefor othercauses Adrenal suppression tests Adrenal imaging Evaluatefor other causes Adrenal venous sampling Consider surgery Unilateral adenoma or hyperplasia Consider surgery Medical therapy fl)UWo,ld Adrenal suppression testing usually involves salt loading and documenting inability to suppress serum aldosterone. • Adrenal vein sampling: aldosterone:renin ratio be 4x ↑↑ in tumor unilateral adenoma on side of the No difference between two sides in patients with bilateral Endocrine 294 https://t.me/usmleinnercircle Version 2 Cushing Features of Cushing syndrome • Central obesity • Skin atrophy & wide, purplish striae • Proximal muscle weakness Clinical • Hypertension • Glucose intolerance manifestations • Skin hyperpigmentation (if due to ACTH excess) • 24-hour urinary cortisol excretion • Late-night salivary cortisol assay • Low-dose dexamethasone suppression test irc Diagnosis le • Depression, anxiety vs Morning cortisol levels check in Adrenal insufficiency rC Hyperandrogenism (e.g Hirsutism) can be seen in Cushing syndrome due to ACTH-induced adrenal androgen production or co-secretion of cortisol and testosterone by an adrenal tumor. ne Adrenal Insufficiency Primary adrenal insufficiency In  What is the likely diagnosis in a patient with tuberculosis who presents with hyponatremia, hyperkalemia, and eosinophilia? LE Most common cause world-wide is TB Primary adrenal insufficiency U SM Etiology Clinical features Laboratory findings Treatment .. . . • .. . .. .. Autoimmune adrenalitis (most common) Infection (eg, tuberculosis) Metastatic infiltration Fatigue, weakness, anorexia/weight loss Nausea, vomiting, abdominal pain Salt craving, postural hypotension Hyperpigmentation Acute adrenal crisis: confusion, hypotension/shock Hyponatremia, hyperkalemia, eosinophilia Low morning cortisol, high ACTH Glucocorticoids (eg, hydrocortisone, prednisone) Mineralocorticoids (eg, fludrocortisone) ACTH = adrenocorticotropichormone. • Peripheral eosinophilia, nonspecific finding sometimes seen with adrenal insufficiency, as cortisol normally facilitates eosinophil migration from the bloodstream into the tissues. Endocrine 295 https://t.me/usmleinnercircle Version 2 Primary adrenal insufficiency is associated with increased levels of ADH. in response to volume depletion and increased corticotropin-releasing hormone; leads to dilutional hyponatremia In women with AI, features of hypogonadism (eg, loss of libido, decreased pubic hair) can be seen due to decreased adrenal androgen production; however, men with AI do not develop these findings as androgens are primarily produced in the testes.  What anaesthetic is associated with adrenal insufficiency, especially in elderly and critically ill patients? Etomidate due to inhibition of 11β-hydroxylase Symptoms &/or signs of adrenal insufficiency 250 µg cosyntropin stimulation test with cortisol & ACTH levels Basal cortisol low, Basal cortisol low, ACTH high ACTH low Minimal cortisol Minimal or suboptimal >20 µg/dl response to cortisol response to cosyntropin cosyntropin 30-60 min after cosyntropin) Further testing to Primary adrenal Secondary or tertiary assesspituitary insufficiency adrenal insufficiency Indeterminate Normal response (cortisol level i Unlikely to be adrenal insufficiency; investigate other function causes ©UWorld  What is the most specific test of adrenal function? Cosyntropin test (ACTH stimulation test) Cosyntropin is synthetic ACTH analogue  Because the ACTH assay can take several days, an ACTH stimulation test (cosyntropin test) is usually performed concurrently to rapidly confirm the diagnosis. • Infusion of a 250µg bolus of cosyntropin normally triggers a rapid increase in serum cortisol. Endocrine 296 https://t.me/usmleinnercircle Version 2 • In contrast, patients with PAI will not have a significant rise. • In addition, most patients with central adrenal insufficiency have a blunted (minimal or suboptimal) response due to adrenal atrophy from chronically low ACTH. • If results are indeterminate, further pituitary function testing can be considered. Acute adrenal insufficiency (adrenal crisis) Treatment Adrenal hemorrhage or infarction Illness/injury/surgery in patient with chronic Al Pituitary apoplexy le Clinical features .. . .. . .. Hypotension & shock Nausea, vomiting, abdominal pain Fever, generalized weakness irc Etiology Hydrocortisone or dexamethasone Rapid intravenous volume repletion rC Al = adrenal insufficiency. In patients with underlying chronic adrenal insufficiency, acute stressors (eg, procedure, ne illness, trauma) can trigger adrenal crisis, which presents with hypoglycemia and severe hypotension often refractory to initial volume resuscitation. Pancreas U SM Diabetes LE In A standard "stress dose steroid" regimen for septic shock involves 200 mg/day of intravenous hydrocortisone. Endocrine 297 https://t.me/usmleinnercircle Version 2 Subtypes of diabetes mellitus Type 1 diabetes Pathophysiology Associated characteristics Presentation . deficiency .. . . . . Laboratory findings Absolute insulin . Children; young adults Normal weight Family history less common Acute/severe (polyuria, weight loss) Ketoacidosis common Undetectable Cpeptide Positive pancreatic . . . .. .. .. autoantibodies* Type 2 diabetes Relative insulin deficiency Insulin resistance Adults; less frequently, adolescents Often overweight Family historv £Q!!l!!!2!) Gradual/asymptomatic Ketoacidosis rare Elevated C-peptide Pancreatic autoantibodies absent Monogenic diabetes (formerly MODY) .. .. . .. .. Impaired insulin secretion Impaired 9lucose sensin9 Young adults (age <30) Normal weight Family history common (autosomal dominant inheritance) Mild hyperglycemia Ketoacidosis rare Detectable C-peptide Pancreatic autoantibodies absent *For example, glutamic acid decarboxylase 65 autoantibody. MODY = maturity-onset diabetes of the young. • Glucosuria is not sufficient to establish diagnosis of DM Clinicalfeaturesof diabeticautonomicneuropathy • Tachycardia, impaired exercise tolerance Cardiovascular • Postural hypotension with loss of diurnal blood pressure variation • Dry skin, pruritus, callus formation Peripheral nerves • Foot ulcers & poor wound healing • Charcot arthropathy (increased fracture risk with resultant secondary ulceration) • Gastroparesis with delayed gastric emptying Gastrointestinal • Esophageal dysmotility with possible dyspepsia • Intestinal involvement with possible diarrhea, constipation, or fecal incontinence • Erectile dysfunction & retrograde ejaculation in men, decreased libido & dyspareunia in women Genitourinary • Decreased ability to sense full bladder leading to incomplete emptying & decreased urination • Eventual recurrent urinary tract infections &/or overflow incontinence (eg, dribbling, poor urinary stream)  What is the likely diagnosis in an insulin-dependent diabetic patient with anorexia, nausea/vomiting, early satiety, and post-prandial hypoglycemia without heartburn or epigastric pain? Diabetic gastroparesis Endocrine 298 https://t.me/usmleinnercircle Version 2 hypoglycemic episodes due to insulin administration prior to meals followed by impaired gastric emptying and delayed absorption • Monofilament testing is useful for determing the presence of peripheral neuropathy. Monofilament le Tests pressure sensation with a 10g monofilament. Patients with neuropathy have a higher pressure threshold and loss of monofilament sensation, which are associated with increased risk of foot ulceration test irc Checked annually ne rC The tuning fork test is an easy and inexpensive way to assess for the loss of vibratory sense in patients with diabetic neuropathy. In Insulin resistance is a fundamental response to injury/stress and is a well-known phenomenon in the post-op and post-trauma patient. Key idea: If a young patient develops diabetes, do NOT assume they have type 1 diabetes because a lot of children (especially now with high rates of obesity) are at risk for developing U SM Screening LE type 2 diabetes Endocrine 299 https://t.me/usmleinnercircle Version 2 Indications for testing 124 1125 1 The indications listed below are consistent with the 2023 ADA guidelines. The 2021 USPSTFguideline recommends screening in adults aged 35-70 years with overweight or obesity. I;] [26]1241125] • 35 years of age • History of prediabetes or gestational diabetes • Patients < 35 years of age with both I;] o Overweight or obesity I;] o AND~ 1 additional risk factor for T2DM • Presence of risk-enhancing comorbidities, including: o HIV infection o Cystic fibrosis o Post organ transplantation • Consider in women who are planning pregnancy with any risk factor for T2DM (e.g., overweight or obesity). • See "Gestational diabetes" for testing indications during pregnancy. If results are normal, repeat testing in asymptomatic patients at least every three 0 years. Patients with prediabetes should be tested at least annually to detect progression to diabetes. 125] Screeningtests for diabetes mellltus Interpretation Test A,, • • • • Prelerred test m nonfasting state ~6.5% = Diabetes mellltus 5.7-6.4% = lncreasod risk tor diabetes <5.7% = Nonna! Fasting blood gluco&e • • • • N'o caloric intake for >8 hours .:126 mg/dL = Diabetes mellitus 100-12S mg/dl = Increased risk for diabetes <100 mg/dl = Normal Random gluoose levels • .:200 mg/dL with symptoms of hyperglycemia= Diabetes meUitus • 140-199 mgldl = lncmased nsk for d1abotos • <140 mg/dl = Normal Oral glucose tolerance test Endocrine • • • • • Most sensitive test 75 g,glucose load with glucose testing tor 2 hours .:200 mg/dL = Diabetes mellitus 140-199 mg/dl= lne<eased risk for diabetes <140 mg}dl = Normal 300 https://t.me/usmleinnercircle Version 2 Health maintenance in diabetes mellitus . . . .. .. . Nephropathy screening Retinopathy screening Neuropathy screening Cardiovascular risk reduction GoalA1c: 0 S7% (younger, otherwise healthy) 0 SB% (older, limited life expectancy, comorbidities) Annual random urine albumin/creatinine ratio• 0 Normal value: <30 mg/g Periodic serum creatinine level Dilated eye examination every 1-2 years• Visual inspection at each visit Annual comprehensive foot examination Address lifestyle factors (eg, tobacco, obesity, diet & exercise) Annual lipid & BP screening Aspirin and statin (depending on other risk factors) le Glycemic control Check A 1c every 3-6 months irc .. •Begin at time of diagnosisfor type 2 diabetesmellitus& 5 years after diagnosisfor type 1. rC BP; blood pressure.  What test should be used to screen for diabetes mellitus in patients with polycystic ovarian syndrome? ne Oral glucose tolerance test : more sensitive than fasting glucose and HbA1c in patients with PCOS DKA In Diabetic ketoacidosis • Young age Patient characteristics • Brittle type 1 diabetes mellitus • May be initial manifestation of diabetes LE • Acute to subacute onset Clinical symptoms U SM Diagnosis Treatment 0 Initial: polydipsia/polyuria, blurred vision, weight loss 0 Later: altered mentation, hyperventilation, abdominal pain I • j Glucose (typically 300-800 mg/dl) • Metabolic acidosis (bicarbonate <18 mEq/L) • j Anion gap • Positive serum ketones • High-flow IV fluids (normal saline) • IV insulin • Follow & replace potassium IV; intravenous. Endocrine 301 https://t.me/usmleinnercircle Version 2 Management of diabetic ketoacidosis .. Fluids Insulin Potassium Bicarbonate Initial: give isotonic intravenous fluids Subsequent: 0 .. . . . Continue isotonic fluids if serum Na+ <135 mEq/L 0 Switch to half normal saline if serum Na+ ~135 mEq/L 0 Add dextrose when serum glucose <200 mg/dl Reduce insulin infusion rate when serum glucose <200 mg/dl Hold insulin infusion if serum K+ <3.3 mEq/L Switch to subcutaneous insulin on DKA resolution* Give replacement when serum K+ <5.3 mEq/L** Consider replacement for severe DKA with blood pH S6.9 *Normalizationof anion gap & patient able to eat. **Due to total body potassiumdepletion and risk of insulin-inducedhypokalemia. DKA = diabetic ketoacidosis. Because there is a slight delay in the absorption of insulin when it is given SQ, the insulin infusion should be continued for 12 hours after the administration of SQ insulin. This bridging process ensures sufficient time for SQ insulin to take effect and prevents rebound ketoacidosis. In contrast to diabetic ketoacidosis (DKA), which typically develops rapidly over hours, HHS develops over a few days to weeks.  Would you use nitroprusside or direct assay of beta-hydroxybutyrate to monitor resolution of DKA Direct assay of beta-hydroxybutyrate (nitroprusside picks up acetoacetate and acetone only) Urine dipstick doesn't pick up beta-hydroxybutyrate; can be used if urine ketones are negative but still suspicion for DKA  What else can be used to monitor resolution of DKA? Serum anion gap  What is the most important initial step in management of a patient with hyperosmolar hyperglycemia state? Fluid replacement with normal saline may switch to 0.45% saline after a few hours if corrected Na+ levels are high; IV insulin +/ K are important as well, but not as important initially Endocrine 302 https://t.me/usmleinnercircle Version 2 Diabetic ketoacidosis in children Clinical features . . Laboratory findings Polyuria/nocturia Polydipsia, polyphagia Vomiting, abdominal pain Weight loss, fatigue Kussmaul respirations (deep, rapid breathing) Dehydration Glucose >200 mg/dl Bicarbonate <15 mEq/L pH <7.3 • Anion gap >14 . Complications 10 mUkg isotonic fluid bolus over 1 hour irc .. Management Serum/urine ketones le .. .. .. .. Insulin infusion + isotonic fluids with potassium Cerebral edema rC Treatment ne Progressive therapeutic intensification in type 2 diabetes mellitus In Metformin (preferred initial therapy) LE Metforrnin + oral antidiabetic agents, GLP-1 analogue, or basal insulin U SM Basal-bolus insulin therapy ©UWorld  What type of insulin therapy is indicated in type 2 diabetic patients with persistently elevated A1C levels despite oral antidiabetic regimen ( i.e. failed dual therapy ? Basal supported oral therapy (long-acting insulin i.e. glargine Lantus) before bed time combined with oral antidiabetic drug)  What is the next step in management for a diabetic patient taking metformin and nightly insulin glargine that presents with an elevated hemoglobin A1c despite normal fasting glucose levels? Add rapid-acting mealtime insulin this patient likely has postprandial hyperglycemia — pt. with elevated HbA1c despite good control of fasting glucose may have post-prandial hyperglycemia. A combined regimen Endocrine 303 https://t.me/usmleinnercircle Version 2 of long-acting basal insulin (glargine) to control baseline fasting glucose and rapid-acting mealtime insulin (aspart) to control postprandial glucose excursions may be needed to provide optimal control  The indications for insulin therapy in diabetes is a newly diagnosed patient with an A1C 8.5% or symptomatic diabetes, pregestational and gestational diabetes, and end-stage renal failure. Also, if patient has insufficient glycemic control (target A1c not reached) over a 3-month period with metform + other antidiabetic drug ⟶ initial basal insulin Oral antidiabetic drugs are contraindicated in renal failure. Should also be avoided in patients undergoing surgery or with severe illness. What should be used instead? Insulin therapy • Recommended add-on therapy (ie, in addition to metformin, which remains the first-line agent for type 2 diabetes mellitus) for patients with established cardiovascular disease, includes the following: • SGLT2 inhibitors (eg, canagliflozin, empagliflozin) cause increased urinary glucose excretion. These agents induce weight loss (due to urinary glucose loss) and decrease the risk for atherosclerotic cardiovascular disease– and heart failure–related mortalities. They also slow progression of albuminuria in diabetic nephropathy. • Glucagon-like peptide-1 GLP1 receptor agonists (eg, semaglutide, liraglutide) regulate glucose by slowing gastric emptying, suppressing glucagon secretion, and increasing glucose-dependent insulin release, making them less likely to cause hypoglycemia. Similar to SGLT2 inhibitors, GLP1 agonists induce weight loss and reduce mortality associated with atherosclerotic cardiovascular disease. Type 2 diabetes and comorbid cardiovascular disease Lifestyle Cardioprotective antidiabetic agents Lipid/antiplatelet therapy Blood pressure control • Smoking cessation, regular exercise • Reduced saturated fat, refined sugar intake • GLP-1 receptor agonists (eg, liraglutide) • SGLT-2 inhibitors (eg, empagliflozin) • Stalins • Low-dose aspirin • Goal BP <130-140/80-90 mm Hg • ACE inhibitor/ARB preferred ACE= angiotensin converting enzyme; ARB= angiotensin receptor blocker; BP= blood pressure; GLP-1 = glucagon-like peptide-1; SGLT-2 = sodium-glucose cotransporter-2. Endocrine 304 https://t.me/usmleinnercircle Version 2 SGLT-2 inhibitors* Mechanism of action • Increased urinary glucose excretion (block proximal tubule glucose reabsorption) • Reduced progression of nephropathy & albuminuria Possible benefits • Reduced cardiovascular morbidity & mortality • Reduced hospitalizations for heart failure • Weight loss Adverse effects • Euglycemic ketoacidosis • Increased risk of genitourinary infections • History of OKA • Impaired renal function (eGFR <30-45 mUmin/1. 73 m2) irc *For example, canagliflozin & empagliflozin. le • Type 1 OM Contraindications DKA = diabetic ketoacidosis; DM = diabetes mellitus; eGFR = estimated glomerular filtration rate; SGLT-2 = rC sodium-glucose cotransporter 2. Morning glucose is dependent on how much long acting insulin you gave at night. ne  Giving a basal-bolus regimen involves giving basal insulin 12 times daily + a bolus injection 3045 minutes before meals In Bolus injection needs to be adjusted to preprandial blood glucose measurements LE 1 unit of insulin lowers the blood glucose by 3040 mg/dL. Problems: early-morning hyperglycemia U SM • Dawn phenomenon • A common problem (especially in young type 1 diabetic patients) • Definition: early-morning hyperglycemia occurs because of the physiological increase of growth hormone levels in the early morning hours, which stimulates hepatic gluconeogenesis. The subsequent increase in insulin demand cannot be met in diabetic insulin-dependent patients, resulting in elevated blood glucose levels in the morning. • Treatment: measurement of nocturnal blood glucose levels before initiating insulin therapy. The long-acting insulin dose may be given later (around 11 p.m.) or increased under careful glycemic control. Treatment with an insulin pump may be considered in children. • Somogyi effect • Rare • Definition: early-morning hyperglycemia i;:l because of a counterregulatory secretion of hormones i;:l that is triggered by nocturnal hypoglycemia secondary to an evening insulin injection • Treatment: reduction of the evening dose of the long-acting insulin  What drug should be discontinued in an acutely ill patient with sepsis and acute renal failure taking low-dose aspirin, atorvastatin, metformin, and sitagliptin? Metformin : Contraindicated in CHF, CKD, and liver disease (risk of lactic acidosis) due to less metformin excretion; not recommended with creatinine 1.4 Endocrine 305 https://t.me/usmleinnercircle Version 2 Nephrotoxins, such as NSAIDs and metformin, should NOT be given to patients with acute renal failure or sepsis; low-dose aspirin has not been shown to precipitate or worsen renal failure  How should patients with T1DM manage their insulin when exercising? • Exercise increases insulin sensitivity • Decrease insulin dosage by 12 units per 2030 minutes of physical activity • Monitor glucose before, during, after exercise • Eat slowly absorbed carbs after exercise to prevent delayed hypoglycemia • For brief exercise (ie, 60 min) occurring within 3 hours after a meal, short-acting insulin given prior to that meal should be reduced. In addition, the patient should have a slow-releasing carbohydrate snack following exercise to prevent delayed hypoglycemia (eg, 48 hr after exercise). • For exercise that occurs before breakfast (ie, fasting state) or lasts throughout the day (eg, camp, tournament), basal insulin (eg, given the night before or morning of), in addition to the appropriate premeal insulin dose, should be reduced. Management of hyperglycemia & diabetes mellitus in the hospital setting Step . 1 2 3 4 . . . . Management Classify diabetes: a Type 1 (do not stop basal insulin) a Type 2 0 Stress-induced hyperglycemia Determine dietary status (eating normally, decreased oral intake, not eating) Determine preadmission glycemic control & antidiabetic treatment regimen In general, discontinue oral drugs due to unpredictable response & risk of complications Determine insulin regimen: 0 Basal-bolus regimen Patients wit~ Patients with type 2 treated before admission with basal bolus regimen Patients with type 2 inadequately controlled by sliding-scale-only regimen Patients with newly diagnosed diabetes mellitus & high glucose levels 5 0 Insulin sliding scale only Appropriate initially in patients whose type 2 is well controlled with diet &/or oral medications before admission; addition of basal insulin if blood glucose is suboptimally controlled on sliding scale only 0 Insulin infusion (may require transfer to intensive care unit) Patients with type 1 who are not eating & with glucose levels suboptimally controlled with subcutaneous insulin Patients with type 1 perioperatively or during labor Hyperglycemic emergencies  Hospitalized patients often eat less than normal and are at risk for hypoglycemia. Therefore, the dose of basal insulin should generally be decreased (eg, 25%50% from the prehospital dose, especially in patients who have tight glycemic control at baseline or a glucose level below targets at the time of admission. Endocrine 306 https://t.me/usmleinnercircle Version 2 Characteristics of common insulins Insulin Peak effect Duration of (hr) effect (hr) Short-acting Regular 1.5-3.5 8 1-3 4-6 . . . Analogues • Lispro Aspart Characteristics Slow onset & offset Peak does not coincide with food peak Fast onset & offset . Peak coincides with food peak le • Glulisine Intermediate-acting --- • Peak effect more likely to cause hypoglycemia 12 Long-acting Detemir . Sometimes requires twice-daily administration . Higher dose has a longer duration of action 6-24 3-9 None 20-24 rC . Glargine U-100 irc 4-6 • NPH Peakless effect less likely to cause hypoglycemia . Higher concentration of glargine U-300 has duration of action >30 hr . Peakless effect less likely to cause ne None Degludec >24 hypoglycemia U SM LE Diabetic Nephropathy In When insulin is needed, basal insulin is typically added first. Long-acting insulin is generally preferred over NPH due to its lower risk for hypoglycemia Endocrine 307 https://t.me/usmleinnercircle Version 2 Diabetic kidney disease • Persistent albuminuria (>3 months apart) Clinical findings • Initial hyperfiltration followed by progressive decline in GFR • Hypertension usually present Evaluation • Screen at the time of diagnosis in type 2 DM • Screen 5 years after the diagnosis in type 1 DM • Serum creatinine • Urine spot albumin to creatinine ratio (or 24-hour urine protein) • Urinalysis/urine microscopy (to exclude other causes) • Intensive glycemic control • Management/prevention • 0 Target hemoglobin A1c S7% (for most patients) 0 SGLT2 inhibitor preferred; GLP-1 agonist Blood pressure control 0 Target blood pressure <130/80 mm Hg 0 ACE inhibitor preferred (or angiotensin II receptor blocker) General cardiovascular risk management 0 Smoking cessation 0 Lipid management OM= diabetes mellitus; GFR = glomerular filtration rate; GLP-1 = glucagon-like peptide-1; SGLT2 = sodium-glucose cotransporter-2. Random urine albumin-to-creatinine ratio testing is the most sensitive test to detect early elevations in albumin excretion. Perform annually.  What is the most beneficial therapy for reducing the progression of diabetic nephropathy? Strict blood pressure control Intensive glycemic control HbA1c  7 is important, but intensive BP control is the primary intervention proven to slow the decline in GFR A patient's diabetes is considered well controlled if the HbA1c is < 7.0 % • Tight glycemic control in patients with diabetes decreases the risk of micro-vascular complications such as retinopathy and nephropathy. Has uncertain effect on macrovascular complications MI, stroke) and all-cause mortality. ACCORD trial showed that very intensive glycemic targets (ie, A1c between 6.0% and 6.5%) appear to increase risk of all-cause and cardiac mortality. Endocrine 308 https://t.me/usmleinnercircle Version 2 Effect of intensive glycemic control in type 2 diabetes Macrovascular complications (eg, acute myocardial infarction, stroke) No change (short-term) Microvascular complications Improve (eg, nephropathy, retinopathy) Mortality No change or increased Metabolic Syndrome le Metabolic syndrome is diagnosed when at least 3 of the 5 following criteria are met: 2. Fasting glucose  100110 mg/dL 3. Blood pressure  130/80 mmHg) 4. Triglycerides  150 mg/dL rC 5. HDL cholesterol  40 mg/dL in men,  50 mg/dL in women) irc 1. Abdominal obesity (waist circumference  40 inches in males,  35 inches in females) A major pathogenic factor underlying metabolic syndrome is insulin resistance. ne Hypoglycemia Hypoglycemia in patients without diabetes mellitus In • Drugs: quinolones, quinine, beta blockers • Alcohol (usually with prolonged starvation) • Sepsis/critical illness • Adrenal insufficiency LE • lnsulinoma • Surreptitious insulin, sulfonylurea, or meglitinide use • Depleted glycogen stores (anorexia nervosa) U SM • Severe hepatic failure Hypoglycemia in insulin therapy Risk factors . Manifestations • Mild to moderate (neurogenic symptoms): anxiety, tremor, palpitations, sweating Management strategies Long-standing type 1 diabetes • Pancreatogenic diabetes . . • . Severe (neuroglycopenic symptoms): confusion, seizure, loss of consciousness Query patients regarding hypoglycemic symptoms Individualized glycemic targets Flexible insulin dosing regimen • Emergency glucagon kits  It can occur in those with sepsis or other critical illnesses due to increased use of glucose in the tissues, which is promoted by inflammatory cytokines. Sepsis may also lead to suppression of gluconeogenesis in the liver. Endocrine 309 https://t.me/usmleinnercircle Version 2 Evaluation of hypoglycemia Serum insulin C-peptide Hypoglycemic drug assay Exogenous insulin Normal/increased Low Negative Oral hypoglycemic agents Normal/increased Normal/elevated Positive lnsulinoma Normal/increased Normal/elevated Negative The following principles should guide the evaluation of hypoglycemia:  Whipple's triad must be fulfilled before a hypoglycemia workup is initiated. Many normal individuals may have blood glucose in the nominal hypoglycemic range without symptoms of hypoglycemia.  Low blood glucose in normal individuals will suppress production of insulin, C-peptide, and proinsulin. In the presence of significant hypoglycemia, normal levels of these hormones indicate an abnormal response.  Fingerstick blood glucose measurement is unreliable in the hypoglycemic range. Low blood glucose recorded by a bedside glucometer must be confirmed by a standard laboratory assay.  Blood samples for biochemical testing must be taken when the patient is hypoglycemic and before glucose administration. Endocrine Tumor Thyroid • Ultrasound of the neck and cervical lymph nodes is the primary modality for initial staging of thyroid cancer. Patients with a small 1 cm) papillary thyroid tumor may be treated with thyroid lobectomy. Total thyroidectomy is recommended for tumors 1 cm in diameter, tumor extension outside of the thyroid, distant metastases, and in patients with a history of head or neck radiation exposure. Levothyroxine treatment for differentiated epithelial (papillary & follicular) thyroid cancer Small, low-risk tumors • TargelTSH 0.1-0.5 µU/ml for 6-12 months, then low normal range Intermediate risk tumors • Target TSH 0.1-0.5 µU/ml Large, aggressive tumors • TargelTSH <0.1 µU/ml; continue for several years ----· • Post-operative adjuvant therapies for papillary thyroid carcinoma may include radioiodine ablation and/or suppressive doses of thyroid hormone. Endocrine 310 https://t.me/usmleinnercircle Version 2 thyroid hormone suppresses TSH, which can stimulate growth of occult residual or metastatic disease; adjuvant therapy is usually for patients with increased risk of recurrence Serum thyroglobulin measurements are used as a tumor marker once the normally functioning thyroid tissue is removed. • Medullary thyroid cancer arises from the calcitonin-secreting parafollicular C cells. VIPoma Diagnosis • • • • Hypokalemia (jintestinal potassium secretion) Hypercalcemia (increased bone resorption) Hyperglycemia due to increased glycogenolysis Stool studies show secretory diarrhea with j sodium & osmolal gap <50 mOsm/kg ne Laboratory findings • Watery diarrhea • Hypo- or achlorhydria due to ! gastric acid secretion • Associated flushing, lethargy, nausea, vomiting, muscle weakness/cramps • Watery diarrhea with VIP level >75 pg/ml • Abdominal CT or MRI to localize tumor in pancreas (usually in pancreatic tail) In Clinical presentation rC Clinical features of VI Poma irc le Serum calcitonin levels correlate with the risk of metastasis and recurrence, and are measured serially following surgery. LE watery diarrhea (can be tea coloured and odourless), • Facial flushing present in 20% Treatment: U SM  IV volume repletion,  octreotide to decrease diarrhea,  and possible hepatic resection in patients with metastasis to the liver Carcinoid syndrome can cause flushing, diarrhea, and bronchospasm, but occurs in the small intestine (not the pancreas) Zollinger Ellison Endocrine 311 https://t.me/usmleinnercircle Version 2 Epidemiology Clinical features Diagnosis .. .. . . .. Workup Zollinger-Ellison syndrome Age 20-50 80% Sporadic/20% MEN1 Multiple & refractory peptic ulcers Ulcers distal to duodenum Chronic diarrhea Markedly elevated serum gastrin (>1,000 pg/ml) in the presence of normal gastric acid (pH <4) Endoscopy CT scan/MRI & somatostatin receptor scintigraphy for tumor localization MEN1 = multipleendocrineneoplasiatype 1.  Why can Zollinger-Ellison Syndrome present with diarrhea and steatorrhea? Increased gastric acid secretion decreases the pH of intestinal contents, inactivating pancreatic enzymes Interferes with emulsfication of fat by bile acids and damages epithelial cells and villi ⟶ malabsorption Suspected gastrinoma Endoscopy shows multiple stomach ulcers & thickened gastric folds Check serum gastrin level off PPI therapy for 1 week No gastrinoma Check gastric pH off PPI therapy for 1 week 110-1000 pg/ml Negative Positive Secretin stimulation test Further testing to localize gastrinoma No gastrinoma ©UWorld  Ulcers distal to the duodenum in the jejunum suggest excess gastric acid that cannot be fully neutralized in the duodenum Neuroblastoma Endocrine 312 https://t.me/usmleinnercircle Version 2 .. . .. . .. . Pathogenesis Neuroblastoma Neural crest origin Involves adrenal medulla, stmeathetic chain Median age <2 • Abdominal mass Periorbital ecchymoses (orbital metastases) Spinal cord compression from epidural invasion ("dumbbell tumor") O12soclonus-m:ioclonusS:indrome Clinical features Diagnostic findings Elevated catecholamine metabolites Small, round blue cells on histology N-myc gene amplification le Raccoon Eyes irc • Calcifications and/or hemorrhages may be seen on X-ray • Tumor involvement in the cervical paravertebral sympathetic chain can lead to ipsilateral Horner Syndrome rC MEN Multiple endocrine neoplasia type 1 Clinical features ne Manifestation • Secretion of prolactin, growth hormone, ACTH (or "nonfunctioning" Pituitary adenomas tumors) (10%-20%) • Mass effects (eg, headache, visual field defects) (>90%) • Multiple parathyroid adenomas or parathyroid hyperplasia In Primary hyperparathyroidism • Hypercalcemia (eg, polyuria, kidney stones, decreased bone density) • Gastrinoma - recurrent peptic ulcers LE Pancreatic/gastrointestinal • lnsulinoma - hypoglycemia • VIPoma - secretory diarrhea, hypokalemia, hypochlorhydria (60%-70%) • Glucagonoma - weight loss, necrolytic migratory erythema, U SM neuroendocrine tumors hyperglycemia Pheochromocytoma Indications for testing Diagnostic approach Notable features Management .. Pheochromocytoma Classic triad: episodic headache, sweating & tachycardia Resistant HTN or HTN accompanied by unexplained j glucose • Family history or familial syndrome (eg, MEN2, NF1, VHL) • Urine or plasma metanephrine levels • Confirmatory abdominal imaging for j metanephrines . • . 10% bilateral, 10% extraadrenal, 10% malignant Preoperative alpha blockade prior to beta blockade Laparoscopic or open surgical resection HTN = hypertension; MEN2 = multiple endocrine neoplasia type 2; NF1 = neurofibromatosis type 1; VHL = von Hippel-Lindau syndrome. Endocrine 313 https://t.me/usmleinnercircle Version 2 High index of suspicion for pheochromocytoma • 24-hour urine fractionated metanephrines and catecholamines Normal • Plasma fractionated metanephrines j _, High (2-3 x upper limits of normal) Recheck during spell CT scan or MRI of abdomen l l Negative Positive Consider further imaging: Surgical evaluation - MIBG scan - Octreotide scan - Whole-body MRI - PETscan Genetic testing a and 13blockade prior to surgery MIBG scan if tumor >5 cm and suspicion of extra-adrenaldisease MIBG = 1231-metaiodobenzylguanidine; PET= positron emission tomography. Surgical complications of adrenalectomy for pheochromocytoma Complication Hypertensive crisis Hypotension . . . Mechanism Catecholamine release due to endotracheal intubation & adrenal gland manipulation Serum norepinephrine with larger tumors (>4 cm diameter) Catecholamines after tumor removal • Persistent alpha blockade from preoperative long-acting alpha blocker (eg, phenoxybenzamine) Hypoglycemia Cardiac tachyarrhythmias Treatment Intravenous nitroprusside, phenlolamine, or nicardipine Normal saline bolus, pressors if unreseonsive Insulin secretion following tumor removal (catecholamines suppress insulin secretion) Intravenous dextrose infusion Catecholamine release from adrenal gland handling Intravenous lidocaine oresmolol Endocrine Drugs What should be suspected in a patient taking PTU or methimazole that presents with fever and sore throat? Agranulocytosis Endocrine 314 https://t.me/usmleinnercircle Version 2 warrants discontinuation of the drug and WBC count measurement  A total WBC count less than 1,000/cubic mm warrants permanent discontinuation of the drug. If the total WBC count is more than 1,500 per cubic mm, antithyroid drug toxicity is unlikely to be the cause of the sore throat and fever. Major drug interactions of levothyroxine TBG concentration ! TBG concentration Miscellaneous Estrogen (oral), tamoxifen, raloxifene Heroin, methadone Androgens, glucocorticoids Anabolic steroids Slow-release nicotinic acid Rifampin Phenytoin Carbamazepine LE Milk Alkali Syndrome Proton pump inhibitors, sucralfate In TBG = thyroxine-binding globulin. Iron, calcium, aluminum hydroxide ne t Thyroid hormone metabolism . . . . . . . . Bile acid binding agents (eg, cholestyramine) irc ! Levothyroxine absorption rC . . . le Routine monitoring of the granulocyte count is not cost effective and not advocated U SM Milk-alkali syndrome Pathophysiology Symptoms Laboratory findings • Excessive intake of calcium & absorbable alkali • Renal vasoconstriction & decreased GFR • Renal loss of sodium & water, reabsorption of bicarbonate • Nausea, vomiting, constipation • Polyuria, polydipsia • Neuropsychiatric symptoms • Hypercalcemia • Metabolic alkalosis • Acute kidney injury • Suppressed PTH Treatment • Discontinuation of causative agent • Isotonic saline followed by furosemide GFR = glomerular filtration rate; PTH = parathyroidhormone. Endocrine 315 https://t.me/usmleinnercircle Version 2 Hypoglycemia associated autonomic failure Hypoglycemia-associated autonomic failure Normal response Long-standing diabetes Hypoglycemia Hypoglycemia l ' Epinephrine release Epinephrine release ' Hypoglycemic awareness Hypoglycemic awareness l Hepatic glucose production Behavioral response 'f Hepatic glucose production ' Behavioral response ' ·------------,------------· Correction of hypoglycemia ' Risk for severe hypoglycemia C\/Wor1d Clinical presentation Risk factors Management .. . . . .. Hypoglycemia-associated autonomic failure Reduced neurogenic hypoglycemic symptoms (eg, tremor, arousal, sweating) Increased risk for neuroglycopenic symptoms (eg, confusion, loss of consciousness) Long-standing type 1 diabetes Recurrent or severe hypoglycemia Strict avoidance of hypoglycemia Careful/reduced insulin dosing Less stringent glycemic targets  Hypoglycemia normally prompts a catecholamine (eg, epinephrine) surge, which increases hepatic glucose production and triggers hypoglycemic symptoms. Recurrent or severe hypoglycemia in patients with long-standing diabetes reduces the glucose-raising effects of epinephrine and suppresses the symptoms related to the catecholamine surge, increasing the risk for progressively worsening hypoglycemic episodes.  Those with long-standing diabetes (ie, 5 yr) often also have alpha cell failure, which can lead to decreased glucagon secretion and exacerbate hypoglycemia. Endocrine Paeds • Fetal goiters can be caused by inborn errors of thyroid hormone metabolism, developmental abnormalities of the thyroid, iodine excess or deficiency, transplacental passage of maternal Endocrine 316 https://t.me/usmleinnercircle Version 2 thyroid-stimulating antibodies, or transplacental passage of antithyroid drugs such as propylthiouracil. Large goiters can compress the esophagus, leading to polyhydramnios, or the airway, leading to respiratory distress at birth. Neonatal Thyrotoxicosis Diagnosis Treatment . . .. le .. . Transplacental passage of maternal anti-TSH receptor antibodies Antibodies bind to infant's TSH receptors & cause excessive thyroid hormone release Warm, moist skin Tachycardia Poor feeding, irritability, poor weight gain Low birth weight or preterm birth Maternal anti-TSH receptor antibodies ~500% normal irc Clinical features .. Self resolves within 3 months (disappearance of maternal antibody) Methimazole plus J3blocker Congenital hypothyroidism rC Pathophysiology Neonatal thyrotoxicosis ne Neonate with umbilical hernia, jaundice, hypotonia, decreased activity, poor feeding, macroglossia Infants usually appear normal due to presence of maternal hormones In Cretinism -- impaired development of brain, skeleton Congenital hypothyroidism LE • Usually asymptomatic at birth (rarely causes delayed meconium passage) • After maternal thyroxine wanes (weeks to months) o Lethargy, poor feeding Clinical manifestations o Enlarged fontanelle o Protruding tongue, puffy face, umbilical hernia U SM o Constipation o Prolonged jaundice o Dry skin Diagnosis Treatment Prognosis • i TSH & L free thyroxine levels • Newborn screening • Levothyroxine* • No deficits if treatment started in neonatal period • Untreated disease is associated with neurocognitive dysfunction (eg, L intelligence quotient) *Avoid coadministrationwith soy products,iron, or calcium. Endocrine 317 https://t.me/usmleinnercircle Version 2 Management Prognosis .. . . . Confirm TSH, T4 Start levothyroxine immediately Order ultrasound of the thyroid Refer to endocrinology Excellent with treatment • At risk for permanent neurological defects without treatment Infants with congenital hypothyroidism may experience permanent intellectual disability if hormone replacement is not initiated by age 2 weeks.  What is the most common cause of congenital hypothyroidism worldwide? Thyroid dysgenesis e.g. aplasia, hypoplasia, ectopic gland T/t: Levothyroxine (necessary to prevent neurodevelopmental injury) Delayed passage of meconium (first stool at 48 hr of life)  CF  Hirchsprung  Congenital Hypothyroidism Congenital Adrenal Hyperplasias All receive glucocorticoid therapy 17⍺: Spironolactone, ↓ Na+ intake, estrogen therapy for females 21⍺: lifelong fludrocortisone (aldosterone substitute) 11B Spironolactone, ↓ Na+ intake Classic congenital Pathogenesis Clinical • Autosomal recessive • 21-Hydroxylase deficiency .. Pathophysiology Ambiguous genitalia in girls • Normal gluco- & mineralocorticoids • Early pubic/axillary hair growth o Affects most girls & boys .. .. . • ! 21-hydroxylase activity • f Androgens Salt-wasting syndrome* 0 Treatment Nonclassic congenital adrenal hyperplasia hyperplasia • Autosomal recessive presentation Laboratory findings adrenal Hypotension, dehydration & vomiting • Severe acne Clinical features • f Growth velocity & bone age • f 17-hydroxyprogesteronelevel ! Sodium, f potassium, ! glucose f 17-Hydroxyprogesterone Treatment Glucocorticoids & mineralocorticoids High-salt diet Psychosocial support • Hirsutism & oligomenorrhea in girls • Hydrocortisone Partial deficiency of 21-hydroxylase. Basically similar to 21 alpha deficiency but with normal gluco and *Clinicalsymptoms& electrolyteabnormalitiesdevelopat age 1-2 mineralocorticoids weeks. Endocrine 318 https://t.me/usmleinnercircle Version 2 Adrenal steroids and congenital adrenal hyperplasias Ketoconazole /blocksseveralsteps,n stero1dogenes1s) fr-=- V Cholesterol (v,aSIAR') Anastrozole, tetrozole,exemestane -1 ry 1 7 1110 ,l,: ;,a,.h;;Y;;;d r,.o;;;xv.,la_se,Rr't· _117~-b]hy19d![!ro~xyVjpl[!reeiql.!!n~en3>0!]llo2J]l:ne Dehydroepiandrosterone IDHEA A , \It l hVdroaen rp •n l l 17a hvdroxvlase 17,20-lvas 17-hydroxyprogesterone Progesterone m~~:.__,_L--~L-_J 11-deoxycorticosterone Testosterone 1-0- Metyrapone Cortisol Corticosterone rC Aldosterone Cortisone ZONAGLOMERULOSA Mineralocorticoids ZONAFASCICULATA Glucocorticoids Finasteride ne ZONARETICULARIS Androgens Peripheral tissue U SM LE In Adrenalcortex V Dihydrotestosterone IDHTI 11-0- Glycyrrhetinic acid Angiotensin II ! Estradiol irc 11-deoxycortisol le l'I\ Endocrine 319 https://t.me/usmleinnercircle Version 2 Gynae & Reproductive Health Gynecology Physiology Physiologic Leukorrhea PMS Dysmenorrhea Menopause Infertility Ovarian Hyperstimulation Syndrome Amenorrhea Craniopharyngioma vs Kallmann's Hematocolpos Hirsutism Hyperandrogenism Ovarian Hyperthecosis Abdominal Pain PID Mid Cycle Pain PCOS Obesity and Anovulation Breast Nipple Discharge Lactation Uterus Prolapse Fibroid Endometriosis Abnormal Menstrual Bleeding Adenomyosis Uterine Polyp Endometrial Cancer Cervix Cervicitis Pap Smear CIN Cervical Cancer Ovary Cyst Torsion Ovarian Cancer Vulva Labial Adhesion Gynae & Reproductive Health 320 https://t.me/usmleinnercircle Version 2 Bartholin Cyst Lichen Sclerosus Vulvar Lichen Planus Vulval Cancer Vagina Vaginismus Cancer Miscellaneous Urethral Diverticulum Disorders of Sex Development le Turner Syndrome Aromatase Deficiency Mullerian Agenesis irc 5Alpha Reductase Male Gynecomastia Male Breast Cancer rC Hypogonadism Urethritis Scrotum Varicocele ne Hydrocele Prostate Prostatitis Erectile Dysfunction Balanitis Peyronie Disease Phimosis Testes Cryptorchidism U SM Cancer LE Penis Fracture In Prostate Cancer & BPH Penis Repro Paeds Precocious Puberty Neonatal Withdrawal Bleeding Growth Delay Circumcision Drugs Progesterone OCP & IUD Emergency Contraceptive SERM PDE5 Inhibitors Gynecology Gynae & Reproductive Health 321 https://t.me/usmleinnercircle Version 2 Physiology Normal pubertal growth in girls I I Peak heignt ~elocity 1r I-----+-- U= - \ 1-'-i Bre:1st ment de~ M I, 10 11 13 12 14 15 16 17 16 Age (years)  What is typically the first sign of puberty in girls? Breast development (thelarche) remembered with the mnemonic "boobs, pubes, grow, flow" Physiologic Leukorrhea • Cervical mucus just prior to ovulation is profuse, clear, thin, and corresponds with an LH surge. Not an indication of infection. Physiologic leukorrhea is a white, odourless, cervical discharge composed of cervical mucus, normal vaginal flora, and vaginal squamous epithelium. No signs of infection like pruritis, pain, fever, few PMN cells Occurs mid cycle due to Estrogen surge PMS Gynae & Reproductive Health 322 https://t.me/usmleinnercircle Version 2 Premenstrual syndrome and premenstrual dysphoric disorder Clinical features Evaluation Treatment • Symptoms occur during luteal phase • Physical: bloating, fatigue, headaches, hot flashes, breast tenderness • Affective: anxiety, irritability, mood swings, decreased interest; more severe in premenstrual dysphoric disorder • Symptom/menstrual diary • Selective serotonin reuptake inhibitor le Symptoms typically occur 12 weeks before menses (luteal phase) and resolve after menses (follicular phase) irc Do not recommend treatment without first confirming the diagnosis using a menstrual diary over 2 menstrual periods! rC  If the first SSRI is ineffective, another SSRI or combined estrogen/progestin oral contraceptive pills may be tried. Benzodiazepines and GnRH agonists such as leuprolide can also be effective but have more ne significant adverse effects. Patients with premenstrual syndrome or premenstrual dysphoric disorder are at increased risk of primary mood and anxiety disorders (eg, major depressive disorder). In Dysmenorrhea Primary dysmenorrhea Diagnosis LE Differential diagnosis of dysmenorrhea • Crampy lower abdomen &/ back pain during menses • Normalexamination Endometriosis • Pain peaks before menses • Oyspareunia • Infertility U SM Primary dysmenorrhea Fibroids Adenomyosis Pelvic congestion Etiology Clinical features • Age <30 • BMI <20 kg/m2 Risk factors • Tobacco use • Menarche at age <12 • Heavy/long menstrual periods • Sexual abuse • Pain first 2-3 days of menses • Heavy menses with clots • Constipation, urinary frequency, pelvic pain/heaviness • Enlarged uterus on examination Clinical features • Dysmenorrhea, pelvic pain • Menorrhagia • Bulky, globular & tender uterus • Excessive prostaglandin production Management • Dull & ill-defined pelvic ache that worsens with standing • Dyspareunia • Nausea, vomiting, diarrhea • Normal pelvic examination • Nonsteroidal anti-inflammatory drugs • Combination oral contraceptive pills vs pain throughout the cycle in endometriosis The features of primary dysmenorrhea includes pain before/during menstruation, most likely due to endometrial PGF2 (prostaglandin) production causing vasoconstriction/ischemia and stronger uterine contractions. Also causes Nausea, Vomiting, Diarrhea. The treatment includes NSAIDs (first-line) and OCP's (refractory). Gynae & Reproductive Health 323 https://t.me/usmleinnercircle Version 2 OCP's decrease PG levels by suppressing ovulation.  Patients with any of the following clinical features should be evaluated for secondary causes of dysmenorrhea: • Symptom onset at age 25 • Unilateral (non-midline) pelvic pain • No systemic symptoms (eg, fatigue, nausea) during menses • Abnormal uterine bleeding (eg, intermenstrual bleeding, postcoital spotting) Menopause Ovarian reserve with aging Follicle number 1,000,000 Optimal fertility Decreased fertility End of fertility Irregular cycles 1,QQQ I ' ' I I ' • I 0 Birth ©UWorld 'I I' I I t'' 18 I I 37 Age (yrs) 41 45 51 Menopause  In women with regular menstrual cycles, infertility can occur due to diminished ovarian reserve, characterized by decreased oocyte number and quality. Regular menstrual periods still occur due to continuing ovulation, but fecundability (ie, conception rate) decreases due to diminished oocyte quality. Increased FSH, as more FSH is needed for ovulation of those last left eggs. Gynae & Reproductive Health 324 https://t.me/usmleinnercircle Version 2 Menopause Genitourinary syndrome of menopause • Oligomenorrhea/amenorrhea • Sleep disturbances Clinical features Symptoms • Decreased libido • Depression • Cognitive decline • Vaginal atrophy Treatment Physical examination • Clinical manifestations • i FSH • Topical vaginal estrogen Treatment • Systemic hormone replacement therapy Very high levels of FSH is characteristic of menopause. irc Dx Clinically; Estrogen/ FSH levels NOT measured rC Diagnosis • Vulvovaginal dryness, irritation, pruritus • Dyspareunia • Vaginal bleeding • Urinary incontinence, recurrent urinary tract infection • Pelvic pressure • Narrowed introitus • Pale mucosa, t elasticity, t rugae • Petechiae, fissures • Loss of labial volume • Vaginal moisturizer & lubricant • Topical vaginal estrogen le • Vasomotor symptoms Vaginal atrophy can also develop in postpartum females due to low estrogen levels secondary to Lactational Amennorhea ne • Hormone replacement therapy is only indicated for the treatment of vasomotor symptoms in women age  60 who have undergone menopause within the past 10 years. e.g. hot flashes, sleep disturbances; In not indicated for chronic disease prevention (e.g. osteoporosis, coronary heart disease) LE  Menopausal women with vasomotor symptoms and contraindications to HRT are typically managed with non-hormonal therapy, such as SSRIs. contraindications include • history of coronary heart disease, U SM • thromboembolism, • TIA/stroke, • breast cancer, or endometrial cancer  What is the effect of low estrogen levels on vaginal pH? Increased pH  5 thus increased pH with normal urinalysis may indicate a hypoestrogenic state (e.g. atrophic vaginitis)  What is the next step in diagnosis for a middle-aged woman that presents with "night sweats", insomnia, and irregular menses? Pregnancy test is negative. Measure serum TSH and FSH Gynae & Reproductive Health 325 https://t.me/usmleinnercircle Version 2 these symptoms could be due to menopause or hyperthyroidism, thus both should be evaluated Treatment of menopause Vasomotor symptoms MAerate/severe Behavioral modifications Contraindications to estrogen* Nonhormonal therapy (eg, SSRI) Intact uterus Estrogen & progestin Estrogen only ·Contraindications to estrogen:Breastcancer,coronaryheartdisease, endometrial cancer,liverdisease,thromboembohsm SSRI = setecbveserotoninreuptakeinhibitor CU'Mlltd • Bilateral salpingo-oophorectomy in postmenopausal women may result in re-emergence of menopausal symptoms due to an abrupt decrease in circulating androgens. while postmenopausal ovaries no longer produce estrogen, they continue to produce androgens, which are peripherally converted to estrone and estradiol Female sexual interest/arousal disorder is a sexual dysfunction commonly seen in postmenopausal women. It can cause significant distress and should not be assumed to be a normal consequence of aging. Substance-induced and medical causes, including vaginal atrophy and dyspareunia, must be ruled out when making the diagnosis. Infertility Gynae & Reproductive Health 326 https://t.me/usmleinnercircle Version 2 Infertility . . .. .. . . Male factor Ovulatory function Ovarian reserve Fallopian tube patency Uterine cavity evaluation Diagnostic test Semen analysis Midluteal phase (day 21) progesterone level Day 3 FSH & estradiol levels Clomiphene citrate challenge test Antral follicle count Antimullerian hormone Hysterosalpingogram Sonohysterogram le Etiology Evaluate Men first irc • Infertility in patients age  35 is defined as the failure to achieve pregnancy after 12 months of regular, unprotected sexual intercourse. rC for women age  35, infertility evaluation can begin after 6 months What is the likely diagnosis in a 38-year-old woman that presents with infertility despite a regular menstrual cycle and normal physical exam? Decreased ovarian reserve ne characterized by decreased oocyte number and quality; sharp decline in conception is notable after age 37 In Ovarian Hyperstimulation Syndrome Ovarian hyperstimulation syndrome • i hCG enhances ovarian vascular permeability LE Pathophysiology U SM Clinical features • Acute fluid shift to extravascular space • Ascites • Respiratory distress • Hemoconcentration • Hypercoagulability • Electrolyte imbalances • Multiorgan failure (eg, renal failure) • Disseminated intravascular coagulation • Fluid balance monitoring • Serial CBC, electrolytes Evaluation • Serum hCG • Pelvic ultrasound • Chest x-ray • Echocardiography • Correct electrolyte imbalances Management • Paracentesis &/or thoracentesis • Thromboembolism prophylaxis CBC = complete blood count. Rare but life-threatening complication of ovulation induction Gynae & Reproductive Health 327 https://t.me/usmleinnercircle Version 2 Amenorrhea Primary amenorrhea evaluation Uterus Absent Present Karyotype (46,XY [Al], 46,XX [mOllerian agenesis]) FSH I Low Normal Karyotype TSH, prolactin Fl",o -----~1-------~ lTSH r Prolactin Hypothyroidism Normal TSH r Prolactin Normal Prolactinoma lmperforate hymen Turner syndrome Al = androgen insensitivity;FHA= functionalhypothalamicamenorrhea, POI = primaryovarian insufficiency . First Step: Pelvic USG to check for Uterus • ↑ FSH → karyotyping Turner's) • ↓ FSH  MRI Craniopharyngioma, Kallmann's)  Lack of menses is considered normal at age  15 if development of secondary sex characteristics has been appropriate. typically occurs around Tanner stage 4 Primary amenorrhea is not diagnosed until age  15 with normal secondary sex characteristics (age  13 without) Gynae & Reproductive Health 328 https://t.me/usmleinnercircle Version 2 Diagnostic findings of amenorrhea Pathophysiology of functional hypothalamic amenorrhea Excessive physical training Very-low-calorie dieVstarvation Weight loss Chronic illness Stress & depression Anorexia nervosa FSH LH Prolactin TSH Ovarian failure i i Normal Normal Functional hypothalamic amenorrhea j j Normal Normal Normal Normal Normal Normal Prolactinoma j j i Normal Hypothyroidism j j i i Asherman syndrome 1 Ghrelin, NPY,CRH, GABA & ~endorphins Leptin Hypothalamus ) Clinical features LH, FSH Ovaries ) i .. . . . .. • Amenorrhea at age <40 • Pituitarygland ) ! J le Primary ovarian insufficiency J GnRH J Estrogen Hypoestrogenic symptoms (eg, hot flashes) i FSH J Estrogen irc i Idiopathic • Turner syndrome (45,XO) Amenorrhea, low estrogen state, bone loss) CRH= corticotropin-releasing hofmone; NPY = neu-opeptide Y Major causes Fragile X syndrome (FMR1 premutation) • Autoimmune oophoritis rC CUWuld • Anticancer drugs The pathogenesis of hypogonadotropic hypogonadism involves loss of pulsatile GnRH secretion. Management . Pelvic radiation Galactosemia Estrogen therapy (with progestin if intact uterus) ne FMR1 = fragile X mental retardation 1. In  Patients with FHA have decreased estrogen levels but no vasomotor symptoms (eg, hot flashes, night sweats) or vaginal atrophy due to low basal levels of estrogen produced by the normal (yet unstimulated) ovaries. LE Patients with amenorrhea, vasomotor symptoms, and vaginal atrophy are more likely to have primary ovarian insufficiency. U SM Secondary amenorrhea evaluation Secondary amenor'.'.'.::J ! "Ir.~ ll-5 I -------+ Positive Pregnancy Negative i Prolactin (TSH normal) J i FSH J Hormones normal Prior uterine procedure j Pituit;Jry adenoma i Primary ovarian insufficiency J Asherman syndrome t Testosterone J t~ j j PCOS j Hypothyroidism J PCOS= polycysticovary syndrome. C>UW0<1d Gynae & Reproductive Health 329 https://t.me/usmleinnercircle Version 2 AUB & secondary amenorrhea evaluation AUB I secondary amenorrhea• Age< 45 j Age;, 45 hCG + Routine evaluation with FSH, TSH, prolactin j thCG No routine evaluation indicated. TSH, prolactin in selectca~ Pregnant] AUB = abnormal uterine bleeding, •secondary amenorrhea= no menses>3 monthswith prior regular menses OR nomenses >6months withoriorirreou'ar menses  What is the next step in management for a 45-year-old woman that presents with insomnia, fatigue, weight gain, amenorrhea, and an enlarged uterus? Measure hCG level all women of reproductive age 12 to 49 with amenorrhea and signs of pregnancy should be evaluated with an hCG level  Progesterone Withdrawal Challenge Bleeding: Anovulation No Bleeding: 1. Low Estrogen Levels 2. Outflow Track Obstruction normally estrogen causes the endometrium to proliferate, which sloughs following withdrawal of progesterone Do E/P withdrawal test to differentiate between these two. Progesterone withdrawal test High estrogen ::l Q) Progesterone administered I Low estrogen ., ., Progesterone administered Q) .II I .II :5 :5 <ii <ii ·;:; ·;:; Q) Q) E E 0 No withdrawal bleeding 0 'O 'O w w C: C: Time Time CIUW011d  A patient with secondary amenorrhea with a negative progesterone challenge and negative E/P challenge is indicative of outflow tract obstruction/scarring (Asherman's). Next best step? Hysterosalpingogram to ID lesion Treatment? Hysteroscopic resection of adhesions Gynae & Reproductive Health 330 https://t.me/usmleinnercircle Version 2 With Asherman's, pt. will still have cyclic abdominal pain, but no bleeding because blood is obstructed. Look for history of D&C. Craniopharyngioma vs Kallmann's Path: • Kallmann's: loss of GnRH  ↓ FSH/LH  ↓ E/P 0 + anosmia • Craniopharyngioma: AP tumor → ↓ FSH/LH  ↓ E/P + bitemporal hemianopsia le 0 • Primary Amenorrhea •  Uterus Diagnosis? rC • ↓ secondary sex characteristics (breast, pubic hair) irc Presentation? • Cranial MRI → mass = craniopharyngioma ("calcified cyst on CT") ne • Low FSH/LH (Turner's = ↑ FSH/LH  karyotype) In Hematocolpos lmperforate hymen Pathogenesis • Incomplete degeneration of hymen • Cyclic lower abdominal pain • Bulk symptoms (defecatory & urinary dysfunction) • Primary amenorrhea • Suprapubic mass (uterus) • Blue-tinged vaginal mass • Hymenal incision & drainage LE U SM Clinical features Management The enlarging blood collection with each menstrual period causes increasing pressure on the surrounding pelvic organs, resulting in lower back pain, pelvic pressure, or defecatory rectal pain. Pelvic examination typically reveals a blue, bulging vaginal mass or membrane that swells with increased intra-abdominal pressure (eg, Valsalva). Hirsutism Gynae & Reproductive Health 331 https://t.me/usmleinnercircle Version 2 Causes of hirsutism in women Etiology Clinical features • Oligomenorrhea, hyperandrogenism, obesity Polycystic ovary syndrome • Associated with type 2 diabetes, dyslipidemia, hypertension • Normal menstruation Idiopathic hirsutism • Normal serum androgens Nonclassic 21-hydroxylase deficiency Androgen-secreting ovarian tumors, ovarian hyperthecosis Cushing syndrome • Similar to polycystic ovary syndrome • Elevated serum 17-hydroxyprogesterone • More common in postmenopausal women • Rapidly progressive hirsutism with virilization • Very high serum androgens • Obesity (usually of the face, neck, trunk, abdomen) • Increased libido, virilization, irregular menses Cushing syndrome may be similar in presentation to PCOS (obesity, hirsutism, amenorrhea), but PCOS does not cause muscle weakness nor easy bruisability Hyperandrogenism Causes of hyperandrogenism in women Diagnosis Clinical features Oligo-ovulation, clinical or biochemical PCOS hyperandrogenemia, polycystic ovaries on imaging, no evidence of another diagnosis Nonclassic CAH Ovarian/adrenal Oligo-ovulation, hyperandrogenemia, j 17-hydroxyprogesterone levels Older age, rapidly progressive symptoms, j androgen levels (>3 times upper limit of normal) tumors Hyperprolactinemia Amenorrhea, galactorrhea, j prolactin levels Cushingoid features, nonsuppressible Cushing syndrome dexamethasone suppression test, j 24-hour urinary free cortisol Acromegaly Excessive growth, j GH & IGF-1 levels CAH = congenital adrenal hyperplasia; GH = growth hormone; IGF-1 = insulin-like growth factor 1; PCOS = polycystic ovary syndrome. Rapid Hirsutism  1 Year  Tumor • Signs of Virilization: Deepening Voice, Cliteromegaly, Inc Muscle Bulk, Male Pattern baldness The primary ovarian androgens are testosterone, androstenedione, and dehydroepiandrosterone (DHEA). The adrenals also produce these androgens as well as DHEA sulfate DHEAS. Therefore, women with a suspected androgen-producing tumor should be evaluated with serum testosterone and DHEAS levels: Gynae & Reproductive Health 332 https://t.me/usmleinnercircle Version 2 • Elevated testosterone levels with normal DHEAS levels suggest an ovarian source (more common). • Elevated DHEAS levels suggest an adrenal tumor (far less common). Causes of hyperandrogenism in pregnancy Diagnosis • No ovarian mass aromatase • High maternal & fetal virilization risk deficiency • cf mali::malSl<!IIll:llCIIIS after delivery • Solid, unilateral/bilateral ovarian masses Luteoma • Moderate maternal virilization risk; high fetal virilization risk cyst Sertoli-Leydig tumor • Cystic, bilateral ovarian masses • Moderate maternal virilization risk; low fetal virilization risk • Spontaneous regression of masses after delivery • Solid unilateral ovarian mass rC Theca lutein irc • Spontaneous regression of masses after delivery le Placental Clinical features • High maternal & fetal virilization risk • Surgery required (2nd trimester or postpartum) Pregnancy luteomas are rare, nonneoplastic lesions of the ovary thought to be caused by the hormonal effects of ne pregnancy In  What is the recommended management for a pregnant woman with a suspected theca lutein cyst secondary to a complete molar pregnancy? Suction curettage of the hydatidiform mole theca luteum cysts typically resolve following removal of the hydatidiform mole LE  What is the recommended management for a pregnant woman with a suspected luteoma? Observation and expectant management U SM masses typically regress spontaneously after delivery; luteomas are occasionally complicated by ovarian torsion or symptoms related to mass effect (e.g. hydronephrosis)  What is the likely diagnosis in an African-American woman at 20 weeks gestation that presents with new-onset hirsutism and acne? Pelvic ultrasound reveals an intrauterine gestation consistent with dates and bilateral 8-cm solid ovarian masses. Luteoma versus theca luteum cysts, which typically arise due to high hCG levels (e.g. molar pregnancy or multiple gestation)  If surgical removal of an ovarian tumor is indicated during pregnancy, when should surgery take place? After 10 weeks • Secretion of progesterone by corpus luteum is essential to maintain pregnancy Gynae & Reproductive Health 333 https://t.me/usmleinnercircle Version 2 • Placenta takes over this function by 10th week Ovarian Hyperthecosis  Ovarian hyperthecosis is a cause of virilization but is typically diagnosed in postmenopausal women. Patients with this condition also typically have signs of insulin resistance (eg, hyperglycemia, acanthosis nigricans) and low/normal LH and FSH levels. Typical ultrasound findings are solid-appearing, enlarged ovaries. Abdominal Pain Acute abdominal/pelvic pain in women Diagnosis Mittelschmerz Ectopic pregnancy Ovarian torsion Ruptured ovarian Clinical presentation • Recurrent mild & unilateral mid-cycle pain prior to ovulation • Pain lasts hours to da:z'.S • Amenorrhea, abdominal/pelvic pain & vaginal bleeding • Positive ~-hCG • Sudden-onset, severe, unilateral lower abdominal pain; nausea & vomiting • Unilateral, tender adnexal mass on examination • Sudden-onset, severe, unilateral lower abdominal pain immediately following strenuous or sexual activity cyst Pelvic inflammatory • Fever/chills, vaginal discharge, lower abdominal pain & cervical motion disease tenderness Ultrasound findings Not indicated No intrauterine pregnancy Enlarged ovary with decreased or absent blood flow Pelvic free fluid ± Tuboovarian abscess Ovarian Torsion: laparoscopic cystectomy and detorsion Absence of free pelvic fluid helps differentiate ovarian torsion from a ruptured cyst  What is the recommended treatment for a hemodynamically stable patient with a ruptured ovarian cyst? Supportive (e.g. analgesics) hemodynamically unstable patients require urgent surgical intervention Cyst could be Follicular Unruptured mature follicle) or Corpus Luteum Cyst (these can be hemorrhagic)  What is the next step in management for a young woman that presents with diffuse periumbilical abdominal pain that then localizes to the right lower quadrant? Her LMP was 25 days ago. Pregnancy test Gynae & Reproductive Health 334 https://t.me/usmleinnercircle Version 2 should be administered to any woman of childbearing age before performing any diagnostic tests (e.g. X-ray, CT scan) PID Pelvic inflammatory disease pelvic inflammatory disease • Lower abdominal pain • Abnormal bleeding • Pregnancy • Cervical motion tenderness Physical examination • Failed outpatient treatment • Fever >38.3 C (>100.9 F) • Mucopurulent cervical discharge • Noncompliant with therapy • Third-generation cephalosporin • Severe presentation (eg, high fever, vomiting) plus • Complications (eg, tuba-ovarian abscess, perihepatitis) irc Treatment • Inability to tolerate oral medications • Azithromycin or doxycycline • Tuba-ovarian abscess • Infertility • Ectopic pregnancy rC Complications le Symptoms Indications for hospitalization for • Perihepatitis PID  Fitz-Hugh-Curtis (perihepatitis) ne  25 y.o. sexually active female with cervical motion tenderness, fever, and pleuritic RUQ pain. inflammation of the liver capsule with adhesion formation resulting in right upper quadrant pain Hysterosalpingogram In  What is the recommended initial test/imaging study to evaluate infertility in a patient with a history of pelvic inflammatory disease? LE minimally invasive way to detect fallopian tube patency and/or uterine cavity anomalies U SM Tubal occlusion occurs causing infertility due to immune response to the infection, despite antibiotic treatment..  What is the likely diagnosis in a woman that presents with fever and RLQ abdominal pain with a large multiloculated adnexal mass on ultrasound? CA125 is elevated. Tubo-ovarian abscess severe complication of pelvic inflammatory disease Fever, leukocytosis, and elevated CA125 suggest infection rather than malignancy Mid Cycle Pain • Mittelschmerz pain, caused by peritoneal inflammation from ovarian follicle rupture ( physiologic corpus luteum cyst ), occurs during ovulation, approximately 2 weeks prior to menses. Gynae & Reproductive Health 335 https://t.me/usmleinnercircle Version 2 diagnosis can be confirmed by ultrasound, which typically reveals a simple-appearing ovarian cyst (a large cyst with several smaller cysts) with normal Doppler flow. Treatment: NSAIDʼs, Observe & Repeat Examination in 6 weeks PCOS Polycystic ovary syndrome • Androgen excess (eg, acne, male pattern baldness, hirsutism) Clinical features Pathophysiology Comorbidities Treatment options • Oligoovulalion or anovulalion (eg, menstrual irregularities) • Obesity • Polycystic ovaries on ultrasound • i Testosterone levels • j Estrogen levels • LH/FSH imbalance • Metabolic syndrome (eg, diabetes, hypertension) • Obstructive sleep apnea • Nonalcoholic steatohepatitis • Endometrial hyperplasia/cancer • Weight loss (first-line) • Oral contraceptives for menstrual regulation • Letrozole for ovulation induction  Ovulation Induction Now Letrozole is used, earlier Clomiphene was used.  Although many patients with PCOS have biochemical evidence of hyperandrogenism with elevated serum levels of total testosterone, these laboratory values may be normal in some due to decreased levels of sex hormone-binding globulin, with elevated free testosterone levels instead. Therefore, the diagnosis requires either clinical or biochemical evidence of hyperandrogenism. (Elevated LH/FSH are NOT required for diagnosis! Obesity and Anovulation Excess adipose tissue affects the hypothalamic-pituitary-ovarian axis by 2 major mechanisms: • Obesity causes increased insulin resistance and hyperglycemia, which decrease the production of sex hormone–binding globulin, causing elevated free androgen (eg, androstenedione) levels. • The increased free androgens are aromatized in the adipose tissue to estrone (a type of estrogen), which leads to persistently elevated estrone levels. High estrone levels affect GnRH pulses → lack of LH surge  Anovulation Overall LH/FSH concentration change is minimal Gynae & Reproductive Health 336 https://t.me/usmleinnercircle Version 2 Obesity & anovulation Hypothalamus (IGnRH) Aromatase Androstenedione _.. Estrone -.. Ovary ( I Estradiol) Anteriorpituitary (I FSH, LH) l Anovulation Amenorrhea ClUWo'1d le Breast Palpable breast mass irc Management of breast pain Breast pain /\ Age <:30 l l Ultrasonogram ±mammogram Mammogram ± ultrasonogram rC Age <30 Cyclic, bilateral, diffuse l Image-guided core biopsy l Suspicious for malignancy Mass ne l Needle aspiration (if patient desires) Complex cysVmass (solid mass) l Imaging Core biopsy In Simple cyst The diagnosis of a palpable breast mass can only LE be confirmed with a biopsy. Observe No mass Mass Biopsy, referral to breast surgeon Imaging Abnormal Biopsy Normal Observe tr\l 1Wnrlr4 Patients with cyclical pain and no masses: Supportive Bra, NSAIDS. U SM USG in 35y women acc to NBME. No mass Noncyclic, unilateral, focal In an adolescent with a suspected fibroadenoma, reassurance and reexamination after the next menstrual cycle may be appropriate. Gynae & Reproductive Health 337 https://t.me/usmleinnercircle Version 2 Breast cyst management Breast cyst Complex Simple Asymptomat,c~Tender Observe Non bloody aspirate C I Iv ys reso es/ No additional management Biopsy FNA "" Bloody aspirate PersistenV recurrent Biopsy, additional imaging FNA= fine.r-.eedleaspirabon. IOUWo,\d Breast cancer, the most common non-dermatologic malignancy in women, may present with a palpable breast mass. In patients over age 30 years mammography should be the first step in diagnosis. Regardless of imaging findings, biopsy should occur if the physical examination is highly concerning for breast cancer (specially in older women like in 50ʼs or 60ʼs where incidence of breast cancer is very high). Fine-needle aspiration is an appropriate initial biopsy technique, but it is associated with a low sensitivity. Those patients with suspicious masses but normal fine-needle aspiration results should therefore undergo excisional biopsy to confirm the diagnosis.  What is the next step in management for an afebrile woman that experiences relief of symptoms following aspiration of clear fluid from a simple breast cyst? Repeat breast examination in 2  4 months cystic fluid can re-accumulate; if no signs of recurrence, annual screening can be resumed Gynae & Reproductive Health 338 https://t.me/usmleinnercircle Version 2 Benign breast disease Fibroadenoma Age <30 Diagnosis Clinical features Epidemiology Breast cyst • Solitary, well-circumscribed, mobile mass • +/- Tenderness Clinical features • Single, unilateral, mobile, well-circumscribed mass Pain &/or size prior to menses Fibrocystic changes • Multiple, diffuse nodulocystic masses • Cyclic premenstrual tenderness Management • Observation & reassurance (adolescent) • Ultrasound for a persistent mass or older patient • Post-trauma/surgery • Firm, irregular mass • +/- Ecchymosis, skin/nipple retraction lntraductal papilloma Pathology Clinical features Breast cyst are SOFT vs Fibroadenoma which is HARD Management le Fat necrosis • Solitary, well-circumscribed, mobile mass • Cyclic premenstrual tenderness • Benign papillary tumor arising from breast duct lining .• .• Unilateral bloody nipple discharge (can be nonbloody) No associated breast mass or llm12hadeno12athl irc Fibroadenoma . Mammography & ultrasound Biopsy ± excision rC  What is the likely diagnosis in a young woman that presents with bilateral, non-focal breast tenderness and diffuse, cord-like thickening of the breasts with no discharge? Her last menstrual period was three weeks ago. ne Fibrocystic change classically causes diffusely nodular breasts with non-focal tenderness that fluctuates with menstruation In T/t: Supportive Bra, NSAIDʼs, Reexamination post menses Should show decrease in size of cysts), Maybe Cysts aspiration if it is persistent LE The size/tenderness of fibroadenomas 30y) and breast cysts 30y) increase with exposure to estrogen. U SM e.g. with pregnancy, prior to menstruation • Fat Necrosis: the presence of calcifications on mammography and a fixed mass on physical exam can mimic breast cancer Excise mass and reassure Gynae & Reproductive Health 339 https://t.me/usmleinnercircle Version 2 Breast cancer warning signs Clinical finding Pathophysiology Nipple retraction Invasion of lactiferous ducts Nipple scaling or ulceration Epidermal infiltration by neoplastic cells Nipple discharge lntraductal tumor growth ± necrosis Skin retraction Invasion of suspensory (Cooper) ligaments Peau d'orange Obstruction of dermal lymphatics Fixed breast mass Invasion into adjacent breast tissue Axillary lymphadenopathy Lymphatic spread to regional lymph nodes Inflammatory breast cancer Breast cancer risk factors • Hormone replacement therapy Modifiable • Nulliparity • Increased age at first live birth • Alcohol consumption • Genetic mutation or breast cancer in first-degree relatives Nonmodifiable • White race • Increasing age • Early menarche or later menopause screening mammography beginning at age > 50 every 2 years • Alcohol consumption is a dose-dependent risk factor for breast cancer. Retractednipple due to invasion of lymphatic spaces The risk of developing breast cancer is directly correlated with lifetime exposure to estrogen. The most common risk factor is increasing age, primarily due to increasing probability for spontaneous genetic mutations  What is the single most important prognostic consideration in the treatment of patients with breast cancer? Tumor burden (based on TNM staging) Gynae & Reproductive Health 340 https://t.me/usmleinnercircle Version 2  What is the likely diagnosis in an afebrile middle-aged woman that presents with unilateral breast warmth, erythema, and swelling refractory to antibiotics? Inflammatory breast carcinoma lack of fever and no response to antibiotics help distinguish inflammatory breast cancer from mastitis; other distinguishing features include axillary lymphadenopathy and a peau d'orange appearance irc Management of nipple discharge le Nipple Discharge Physical examination ] Palpable mass rC Normal Age-based imaging• & biopsy ! ! Abnormal Percutaneous biopsy LE Possible duct excision for symptom relief Galactorrhea work-up Routine mammogram if 2:40 In Age-based~] Normal Physiologic discharge Bilateral • Multiductal • Expressed only with manipulation ne Pathologic discharge Unilateral Bloody Spontaneous U SM • Age ?:40:mammogram + ultrasound Age 30-39. mammogram• utrasound Age <30.ultrasound t mammogram Breasl MRI WlnlUalImagingIs negaUve IC)UWo,ld For a patient with pathologic discharge and negative initial imaging, MRI of the breast should be performed for additional evaluation. I Pathologic ! l l Age <30 Age 30-39 Age 2:40 Ultrasound ± mammogram Mammogram ± ultrasound Mammogram + ultrasound  The most common cause of physiologic galactorrhea is hyperprolactinemia. Gynae & Reproductive Health 341 https://t.me/usmleinnercircle Version 2 Discharge can be milky, yellow, brown, gray, or green! Lactation • Nipple pain that worsens and persists between feedings is commonly due to nipple injury caused by poor infant positioning and improper latch-on technique. On examination, patients can have open, linear areolar abrasions that cause a bloody-appearing nipple discharge; bruising, cracking, and blistering may also be present. Breast engorgement, with bilateral, diffusely tender, and engorged breasts, can also develop because nipple pain limits breastfeeding. Initial management is with the observation of breastfeeding and patient education. Proper breastfeeding latch Common problems related to lactation Diagnosis Clinical features Engorgement Bilateral, symmetric fullness, tenderness & warmth Nipple injury Abrasion, bruising, cracking &/or blistering from poor latch Plugged duct Focal tenderness & firmness &/or erythema; no fever Galactocele Subareolar, mobile, well-circumscribed, nontender mass; no fever Mastitis Tenderness/erythema + fever Abscess Symptoms of mastitis + fluctuant mass Galactocele can be both painful and painless •Breast engorgement common 35 days after delivery when colostrum is replaced by milk; improves with breastfeeding  Methods for lactation suppression include a supportive bra, avoidance of nipple stimulation, application of ice packs to the breast, and administration of NSAIDs. NSAIDs are used to reduce pain and inflammation; medications are not recommended to suppress lactation (e.g. dopamine agonists) Gynae & Reproductive Health 342 https://t.me/usmleinnercircle Version 2 Lactational mastitis Pathogenesis Breast abscess • Maternal age >30 • Skin flora (eg, Staphylococcus aureus) enters ducts through nipple & multiplies in stagnant milk Risk factors • Engorgement & inadequate milk drainage due to: o o Sudden increase in sleep duration Clinical features Replacing nursing with formula or pumped breast milk Diagnosis o Weaning o Pressure on the duct (tight bra or clothing, prone Management o Cracked or clogged nipple pore o Poor latch • Fever Clinical presentation • Firm, red, tender, swollen quadrant of unilateral breast • ± Myalgia, chills, malaise • Analgesia Treatment • Antibiotics • Drainage le sleeping) • Fever • Focal inflammation • Fluctuant, tender mass • Breast ultrasound • Frequent breastfeeding or pumping rC • Antibiotics irc Risk factors • First pregnancy • Tobacco use • History of mastitis  What is the likely diagnosis in a breastfeeding woman that presents with fever and localized ne breast erythema/tenderness with a palpable, fluctuant mass on physical exam? Breast abscess In persistent mastitis can result in a collection of pus, thus causing an abscess  What is the likely diagnosis in a breastfeeding mother with bilateral red, shiny, painful nipples? Candidiasis of the nipple Uterus U SM Prolapse LE mastitis is not typically associated with intense nipple pain Gynae & Reproductive Health 343 https://t.me/usmleinnercircle Version 2 Pelvic organ prolapse .. .. . Definitions Risk factors Clinical presentation Management .. .. .. .. .. . .. . . Cystocele: bladder Rectocele: rectum Enterocele: small intestine Procidentia Apical prolapse: uterus, vaginal vault Obesity Multiparity Hysterectomy Postmenopausal age Pelvic pressure Obstructed voiding Urinary retention Urinary incontinence Constipation Fecal urgency, incontinence Sexual dysfunction Weight loss Pelvic floor exercises Vaginal pessary Surgical repair  Patients with severe, prolonged prolapse can develop vaginal or cervical erosions if the cervix and vaginal apex protrude through the introitus, rub on clothing, and become inflamed and denuded. These erosions often cause abnormal vaginal bleeding (eg, postcoital, postmenopausal). Treatment of prolapse is via pessary or surgical correction; erosions can be treated with vaginal estrogen.  What is the recommended treatment for an elderly patient with severe pelvic organ prolapse? Colpocleisis i.e. closure of the vagina; used for elderly women who are not good surgical candidates and no longer sexually active (colpocleisis can be performed without general anesthesia) Fibroid Gynae & Reproductive Health 344 https://t.me/usmleinnercircle Version 2 Uterine fibroids irc le Prolapsing uterine fibroid rC Dx: USG CUWond  Patients with symptomatic submucosal fibroids who desire future fertility are treated with ne hysteroscopic myomectomy  What type(s) of fibroids are most associated with heavy menstrual bleeding and/or recurrent pregnancy loss? In Submucosal and Intracavitary fibroids (leiomyomata)  What type(s) of fibroids are most associated with bulk-related symptoms and irregular uterine enlargement? LE Subserosal and Pedunculated fibroids (leiomyomata) e.g. constipation, incomplete voiding, pelvic pressure U SM  What is the likely diagnosis in a woman that presents with "labor pains" despite recently having her period? Pelvic examination reveals an irregularly enlarged uterus and a dilated cervix with a firm mass visible through the external os. Prolapsing uterine fibroid typically occur when a submucosal or intracavitary fibroid prolapses through the cervical os, resulting in labor pains and cervical dilation Uterine Sarcoma is Indistinguishable from fibroid but it should be suspected in Postmenopausal women. Usually solitary mass with Necrosis and Haemorrhage)  What medical therapy may be used for short-term treatment of symptomatic fibroids refractory to OCPs and NSAIDs? GnRH agonists (e.g. leuprolide) Gynae & Reproductive Health 345 https://t.me/usmleinnercircle Version 2 not recommended for more than 3  6 months; typically used before a hysterectomy or to bridge a woman close to menopause  Leiomyomata uteri can outgrow their blood supply and degenerate during pregnancy because myometrial blood flow shifts toward the developing fetus and placenta. An infarcted, degenerating uterine fibroid can cause severe abdominal pain; uterine tenderness; a palpable, firm, and tender mass; and signs of inflammation (eg, leukocytosis). A highly sensitive test for the evaluation of submucosal fibroids is sonohysterography, a form of transvaginal ultrasonography that infuses saline into the uterine cavity for improved detection of intrauterine pathology (eg, fibroid, polyp). Endometriosis Pathogenesis Clinical features Physical examination Diagnosis Treatment . ... ... .. .. . .. Endometriosis Ectopic implantation of endometrial glands Dyspareunia Dysmenorrhea Chronic pelvic pain Infertility Dyschezia Cyclic dysuria, hematuria Immobile uterus Cervical motion tenderness Adnexal mass Rectovaginal septum, posterior cul-de-sac, uterosacral ligament nodules Direct visualization & surgical biopsy Medical (oral contraceptives, NSAIDs) Surgical resection NSAIDs = nonsteroidalanti-inflammatorydrugs. The "three D's" of endometriosis are dysmenorrhea, deep dyspareunia, and dyschezia. Gynae & Reproductive Health 346 https://t.me/usmleinnercircle Version 2 Management of endometriosis Suspected endometriosis Chronic pelvic pain Dysmenorrhea Deep dyspareunia Dyschezia I NSAIDs +/oral contraceptives irc Yes le Contraindications to medical therapy? Need for definitive diagnosis? History of infertility? Concern for malignancy or adnexal mass? I rC Laparoscopy NSAIDs = nonsteroidal anti-inflammatcxy drugs. laparoscopy is reserved for treatment failure, adnexal mass, or infertility ne  What is the recommended management for an asymptomatic patient with incidentally discovered endometriosis during an unrelated surgery? Observation In intraoperative findings may include adhesions, powder-burn lesions, and "chocolate cysts"; asymptomatic patients do not require any treatment LE  What is the definitive treatment for endometriosis? Hysterectomy and oophorectomy typically done in symptomatic women who have completed childbearing U SM  What is the likely diagnosis in a young woman that presents with chronic pelvic pain, especially with exercise, and a homogenous cystic ovarian mass on ultrasound? Ovarian endometrioma (secondary to endometriosis) Patients with uterine or pelvic sidewall implants may have worsening pain with exercise or intercourse (ie, dyspareunia), as well as adhesion formation that can cause tubal infertility. Gynae & Reproductive Health 347 https://t.me/usmleinnercircle Version 2 Endometriomas on pelvic ultrasound homogenous, hypoechoic ovarian cyst with a ground-glass appearance. Abnormal Menstrual Bleeding  What is the pathogenesis of abnormal uterine bleeding in an adolescent? Immature HPA axis (leads to heavy, irregular bleeding)  What is the treatment for AUB in a hemodynamically stable adolescent • Progesterone only pills or Combined Oral Contraceptives • Estrogen triggers rapid growth of endometrium ⟶ stops sudden, heavy bleeding from uterine surface • Progestins stabilize endometrial lining  For hemodynamically unstable patient: Dilatation & Curettage can be done to quickly scrape thick endometrial lining causing acute bleeding along with Packed red cell transfusion. Gynae & Reproductive Health 348 https://t.me/usmleinnercircle Version 2 Causes of heavy menstrual bleeding Endometriosis Uterine leiomyomas (fibroids) Coagulopathy (eg, von Willebrand disease) Dysmenorrhea, pelvic pain Uniformly enlarged (globular), tender uterus Irregular, intermenstrual, heavy, or postmenopausal bleeding History of unopposed estrogen (eg, obesity, nulliparity, chronic anovulation) Nontender uterus (± enlarged) Uncommon cause of heavy menses Dysmenorrhea, pelvic pain, dyspareunia Fixed uterus, adnexal mass (endometrioma), rectovaginal nodularity Heavy, regular menses Bulk symptoms (eg, pelvic pressure/pain, constipation) Irregularly enlarged uterus with uneven contour Heavy, regular menses Bruising, mucocutaneous bleeding (eg, gums) Normal uterus Approach to postmenopausal bleeding ne Postmenopausal bleeding / >4mm Endometrial biopsy In TYUS endometrium le .. . .. . .. . .. . Endometrial cancer/hyperplasia Heavy, regular menses irc .. . Adenomyosis Clinical features rC Diagnosis Ben~/ "" :typia ~eoplas1a Progeslins, surgery LE Observation TVUS = transvagtnal ultrasound. CU_,d U SM Adenomyosis Pathogenesis Risk factors Clinical features Adenomyosis • Abnormal endometrial tissue within the uterine myometrium .. • Age >40 Multiparity Prior uterine surgery (eg, myomectomy) • Dysmenorrhea • Heavy menstrual bleeding • Chronic pelvic pain • Diffuse uterine enlargement (eg, globular uterus) • ± Uterine tenderness . • Clinical presentation Diagnosis MRI & ultrasound: Thickened myometrium • Confirmation via pathology Treatment Gynae & Reproductive Health • Hysterectomy 349 https://t.me/usmleinnercircle Version 2 • Adenomyosis typically causes dysmenorrhea and heavy menstrual bleeding in multiparous women > 40 years of age. The continued accumulation of endometrial tissue within the myometrium causes an increase in the endometrial cavity surface area (resulting in heavy menstrual bleeding) and progression to chronic pelvic pain. new-onset dysmenorrhea in later reproductive years is classic Uterine Polyp Endometrial polyps  Endometrial polyps are common, well-vascularized, hyperplastic endometrial gland growths that extend into the uterine cavity  Develop around 3040 age  Symptoms: Intermenstrual AUB with normal regular monthly painless menses • Intracavitary therefore, small, mobile, non-tender uterus  T/t: Polypectomy Endometrial Cancer Gynae & Reproductive Health 350 https://t.me/usmleinnercircle Version 2 Endometrial hyperplasia/cancer Excess estrogen • Obesity • Chronic anovulation/PCOS Risk factors • Nulliparity • Early menarche or late menopause • Tamoxifen use • Heavy, prolonged, intermenstrual &/or postmenopausal Clinical features bleeding • Pelvic ultrasound (postmenopausal women) • Hyperplasia: progestin therapy or hysterectomy Treatment • Cancer: hysterectomy irc PCOS = polycystic ovary syndrome. • Old + obese or HRT/SERM • Young  PCOS ne • Granulosa-Theca rC  What is the typical presentation? Primarily postmenopausal with vaginal bleeding le • Endometrial biopsy (gold standard) Evaluation In • HNPCC Lynch) LE Endometrial biopsy is indicated to evaluate for endometrial cancer in women with postmenopausal bleeding and an endometrial lining 4 mm on ultrasound. U SM If the Biopsy is non Diagnostic, Dilatation & Curettage or Hysteroscopy can be done. Age~45 Age <45 Age~35 .. .. . . Endometrial biopsy indications Abnormal uterine bleeding Postmenopausal bleeding Abnormal uterine bleeding PLUS: Unopposed estrogen (obesity, anovulation) Failed medical management Lynch syndrome (hereditary nonpolyposis colorectal cancer) Atypical glandular cells on Pap test Cervix Cervicitis Gynae & Reproductive Health 351 https://t.me/usmleinnercircle Version 2 Cervicitis Etiology Clinical presentation Evaluation Management .. .. .. .. . Acute cervicitis Infectious: Chlamydia trachomatis, Neisseria gonorrhoeae Noninfectious: foreign object, latex, douching Asymptomatic Mucopurulent discharge Postcoitallintermenstrual bleeding Friable cervix Nucleic acid amplification testing Wet mount microscopy Empiric treatment: ceflriaxone + doxycycline• *Ceftriaxone + azithromycin in pregnancy.  What is the likely diagnosis in a sexually active woman that presents with postcoital bleeding and mucopurulent discharge with a friable cervix on pelvic examination? Acute cervicitis less common symptoms include dysuria, dyspareunia, and vulvovaginal pruritus Pregnant patients with cervicitis often have no cervical motion tenderness (a sign of ascending upper reproductive tract infection) because the gravid uterus is sealed from the lower reproductive tract to maintain uterine sterility via the mucus plug. Fever is important differential for PID, which will have fever PID Fever Cervicitis No fever  What is the most likely diagnosis in a patient who presents in the third trimester with pelvic pain? She states the she recently began having sex and examination shows cervical bruising and tenderness with no signs of active bleeding. Cervical trauma (cervix becomes hyper-vascular during pregnancy) Pap Smear  Evaluation of atypical glandular cells on Pap test in women  35 years old includes colposcopy, endocervical curettage, and endometrial biopsy.  What is the next step in management for a pregnant patient with high-grade squamous intraepithelial lesion discovered on Pap testing? Colposcopy and biopsy (safe during pregnancy) Gynae & Reproductive Health 352 https://t.me/usmleinnercircle Version 2 le Colposcopy irc Acetic acid is used to help visualize abnormal cervical tissue (e.g. aceto-white changes) rC  Pap smears without HPV co-testing are repeated every 3 years beginning at age 21. begin at age 21 regardless of age of onset of sexual activity in immunocompetent patients; ne Pap smears with HPV co-testing are repeated every 5 years beginning at age 30. Cervical cancer screening . • . • . Age 21-29 Age >65 Cytology every 3 years Cytology every 3 years OR Cytology plus H PV testing every 5 years OR Primary HPV testing every 5 years LE Age 30-65 No screening In • Age <21 Hysterectomy No screening if negative prior screens & low risk • No screening if negative prior screens & low risk (with cervix removed) U SM . HIV Onsetof sexual intercourse or time of HIV diagnosis (whichever is first) • Annually until ~3 normal results, then routine testing • Onset of sexual intercourse lmmunosuppressed • Annual Pap test with HPV cotesting (eg, SLE, organ transplant) HPV = humanpapillomavirus;SLE = systemiclupuserythematosus. When to stop Pap testing • Age 65 or hysterectomy PLUS • No history of cervical intraepithelial neoplasia 2 or higher AND • 3 consecutive negative Pap tests OR • 2 consecutive negative co-testing results Gynae & Reproductive Health 353 https://t.me/usmleinnercircle Version 2 • Patients with cervical cancer risk factors (e.g. immunosuppression, tobacco use, DES exposure, high-risk sexual activity) may need continued Pap testing  If a patient has a history of CIN 2 or higher, Pap testing should continue for another 20 years after detection. Pap smear results requiring endometrial evaluation Group requiring endometrial sampling Result • Premenopausal women with: o Abnormal uterine bleeding OR Benign-appearing endometrial cells 0 • Risk for endometrial h;r:eerelasia Postmenopausal women • Women age ~35 OR at risk for endometrial hyperplasia Atypical glandular cells Atypical glandular cells, favor neoplastic • All women • In women age 45, endometrial cells are not reported on Pap test results because this is a common, benign finding, particularly during the first 10 days of the menstrual cycle. • In women age 45, endometrial cells are reported because this finding is more concerning for endometrial hyperplasia or cancer, CIN Cervical conization Management of CIN 3 CIN3 i Not currently pregnant ] Indications Cervical intraepithelial neoplasia grades 2 & 3* Complications • Cervical stenosis • Preterm birth • Preterm premature rupture of membranes • Second trimester pregnancy loss *Observation preferred for cervical intraepithelial neoplasia 2 in young women. Excisional procedure (eg, LEEP, cold knife conization) Cervical Stenosis: HPV-based testing at 6 months postprocedure l Negative result  Infertility d/t impaired sperm entry Annual HPV-based testing x3 ) CIN J = cervical intraepithelial necplasia 3; LEEP = loop electmsurgical excision procedure; HPV = human papillomavirus. Gynae & Reproductive Health  May impede menstrual flow & cause sec amenorrhea or dysmenorrhea ClUWorid 354 https://t.me/usmleinnercircle Version 2 LEEP cervical biopsy rC irc le Cervical cone biopsy ne Cervical Cancer Risk factors for cervical cancer Infection with high-risk HPV strains (eg, 16, 18) LE .. .. In Cervical lesions suspicious for cancer require cervical biopsy, which can be performed safely during pregnancy History of sexually transmitted diseases U SM • Early onset of sexual activity Multiple or high-risk sexual partners lmmunosuppression • Oral contraceptive use • Low socioeconomic status • Tobacco use HPV= human papillomavirus. Patients with invasive lesions must undergo Hysterectomy Ovary Cyst  What is the next step in management in asymptomatic, young patient with small, smooth walled ovarian cyst? Gynae & Reproductive Health 355 https://t.me/usmleinnercircle Version 2 Follow up exam to monitor growth Torsion . Ovarian torsion Ovarian mass • Women of reproductive age Risk factors • Infertility treatment with ovulation induction • Sudden-onset unilateral pelvic pain Clinical presentation . Nausea & vomiting • ± Palpable adnexal mass Ultrasound • Adnexal mass with absent Doppler flow to ovary • Laparoscopy with detorsion • Ovarian cystectomy Treatment • Oophorectomy if necrosis or malignancy Pain in unilateral and NOT radiating  Patients may initially have partial torsion, which occurs due to oscillation between the twisted and untwisted positions (ie, causing pain, nausea, and vomiting when twisted and relief when untwisted).  Can also cause Vaginal Bleeding from adnexal edema and necrosis • Complete torsion usually triggered by physical activity like walking. Remember: it is a CLINICAL DIAGNOSIS & medical emergency; Doppler USG can support the Dx but is NOT required Ovarian Cancer BRCA mutations& ovarian cancer Genetics • BRCA 1 & BRCA2 mutations • Autosomal dominant inheritance • Ashkenazi Jewish ancestry Cancer risks • • • • • Premenopausal epithelial ovarian cancer Fallopian tube cancer Primary peritoneal cancer Breast cancer at age <50 Breast cancer in male Risk modification • • • • • Bilateral salpingo-oophorectomy Oral contraceptive use Age <30 at first live birth Breastfeeding Tubal ligation • BRCA-positive individuals are recommended to have a prophylactic bilateral salpingooophorectomy to decrease incidence of ovarian cancer. recommended as soon as childbearing is complete Gynae & Reproductive Health 356 https://t.me/usmleinnercircle Version 2  What is the recommended screening for asymptomatic patients at average-risk of ovarian cancer? No screening recommended • Ovarian cancer typically presents with vague symptoms and is usually discovered at an advanced stage. The first-line therapy recommendation is surgical resection followed by Family history ]-Low/average risk- I No screening indicated rC High risk: 2'1 of the following l irc Risk-based ovarian cancer screening & management le systemic chemotherapy, preferably with a platinum-based agent. ne • Multiple members on 1 side of the family with breast or ovarian cancer • Male breast cancer • Bilateral breast cancer • Breast cancer diagnosed age <50 • Ashkenazi Jewish ancestry • ;,3 relatives with a Lynch syndrome-associated cancer l In Hereditary cancer syndrome testing (eg, BRCA 1, BRCA2, Lynch syndrome) !Positive LE • Concurrent TVUS and CA-125 every 6 months beginning at age 30 • rrBSO upon completion of childbearing 7 Negative • No screening • OCPs for pre-menopausal women • rrBSO in postmenopausal women, particularly at time of hysterectomy for benign reasons OCP = oral contraceptive pill; rrBSO = risk reducing bilateral sa1pingo-oophorectomy, U SM TVUS = transvaglnal l.Jtreso1S1d. CUWorld Lynch syndrome genetic screening criteria Patient-based criteria Family history-based criteria • Colorectal cancer • Endometrial cancer age <60 • First-degree relative with MMR/EPCAM gene mutation • 2'3 relatives with Lynch syndrome-associated cancer* • Involves 2'2 family generations • :>1 relatives diagnosed age <50 *Colorectal, small bowel, endometrial, transitional cell cancer; familial adenomatous polyposis excluded. Gynae & Reproductive Health 357 https://t.me/usmleinnercircle Version 2 Premenopausal adnexal mass evaluation Postmenopausal adnexal mass evaluation Adnexal mass Adnexal mass ] j ! l Malignant features Pregnancy test & pelvic ultrasound on ultrasound } Additional imaging & surgical management io CA-125]-Elevat~ Malignant features• Yes_LNo I Rule out ectopic pregnancy 7 -------~ Normal i Observation & serial ultrasounds CUWorld Conservative management: • NSAIDs • +/- narcotics l Repeat ultrasound ~every 6 weeks until resolution •Complex solidcomponentsseptationscalciftcatlons increasedvasc;ularity. 0UWorld  What is the next step in management for a postmenopausal woman with an incidentally discovered ovarian cyst on ultrasound? Measure CA125 levels an elevated CA125 level in a postmenopausal patient is suspicious for malignancy, even if the ultrasound findings seem benign  Postmenopausal women with ultrasound findings suspicious for ovarian cancer and/or elevated CA125 should undergo further imaging (e.g. CT, MRI prior to surgical exploration. helps assess for the presence of metastatic disease which can guide surgical exploration  What is the first step in the work-up of an adnexal mass? Transvaginal U/S  What features indicate a malignant ovarian mass? Irregularly thickened septa, indistinct borders  What work up is absolutely contraindicated? Fine needle aspiration cytology → risk of spreading tumor cells to peritoneum Gynae & Reproductive Health 358 https://t.me/usmleinnercircle Version 2 Epithelial ovarian carcinoma • Acute: shortness of breath, obstipation/constipation presentation Laboratory findings Ultrasound findings Management with vomiting, abdominal distension • Subacute: pelvic/abdominal pain, bloating, early satiety • Asymptomatic adnexal mass • i CA-125 • Solid mass • Thick septations • Ascites • Exploratory laparotomy le Clinical irc Biopsy is Contradicted Epithelial ovarian carcinoma refers to a malignancy involving the ovary, fallopian tube, and rC peritoneum. Histologically, abnormalities can begin at any of these sites and present with the ne hallmark large ovarian mass and widespread pelvic and abdominal metastasis regardless of primary origin. Sertoli-Leydig cell tumor Clinical features Voice deepening 0 Male-pattern balding 0 Increased muscle mass 0 Clitoromegaly • Sex cord-stromal tumor • i Estradiol • i lnhibin . Complex ovarian mass • Juvenile subtype 0 Clinical features Oligomenorrhea Breast tenderness o Abnormal uterine bleeding Surgery (tumor staging) Testosterone inhibits hypothalamic GnRH and pituitary FSH/LH release, Precocious puberty • Adult subtype 0 Unilateral, solid adnexal mass U SM Management .. . 0 Pathogenesis In • Sex cord-stromal tumor • i Testosterone • Rapid-onset virilization LE Pathogenesis Granulosa cell tumor 0 Histopathology Management Postmenopausal bleeding • Call-Exner bodies (cells in rosette pattern) • Endometrial biopsy (endometrial cancer) • Surgery (tumor staging) resulting in low estrogen  What is the likely diagnosis in a young girl with breast development and a white, odorless vaginal discharge with an ovarian mass on ultrasound? Granulosa cell tumor White, odorless vaginal discharge is indicative of estrogen stimulation Gynae & Reproductive Health 359 https://t.me/usmleinnercircle Version 2  What is the recommended treatment for advanced ovarian cancer? Exploratory laparotomy followed by chemotherapy (e.g. cisplatin + paclitaxel) the ovaries, uterus, omentum, and any visible cancerous lesion are removed and pelvic/aortic lymph nodes are dissected Vulva Labial Adhesion Normal Labial adhesion  Labial adhesions, or fused labia minora, typically affect prepubertal girls (with a peak incidence at age 23 due to low estrogen production. Can also lead to Pruritis T/t: Observation; Estrogen cream if symptomatic Bartholin Cyst Bartholin gland cysUabscess Urethral meatus Labia minora Labia majora --Normal Bartholin gland • Idiopathic or sec to Trauma, Edema. Gynae & Reproductive Health 360 https://t.me/usmleinnercircle Version 2 • 4 o clock or 8 o clock position vs Skene Gland cyst which are lateral to Urethral meatus  Asymptomatic Cyst: Observation only; self resolve  Symptomatic Cyst/Abscess: Incision & Drainage  Word Catheter to reduce recurrence  Recurrent Cyst: Marsupialization procedure, which creates another point of drainage for the Bartholin gland. Word catheter le Bartholin gland marsupialization 2. Evert edges of cyst or abcess & suture to mucosal edge U SM Lichen Sclerosus LE In ne rC irc 1. Incise & drain cyst or abscess Epidemiology Clinical features Workup Treatment Vulvar lichen sclerosus • Prepubertal girls & perimenopausal or postmenopausal women • Thin, white, wrinkled skin over the labia majora/minora; atrophic changes that may extend over the perineum & around the anus • Excoriations, erosions, fissures from severe pruritus • Dysuria, dyspareunia, painful defecation • Punch biopsy of adult-onset lesions to exclude malignancy • Superpotent corticosteroid ointment  In severe cases, normal anatomic structures may be obliterated or atrophied, leading to loss of the labia minora and clitoral hood retraction, which can result in dyspareunia. Gynae & Reproductive Health 361 https://t.me/usmleinnercircle Version 2 Does NOT affect the vaginal mucosa, which is an important distinguishing feature from atrophic vaginitis and Lichen Planus • Biopsy is recommended only in ADULTS not children Vulvar lichen sclerosus Vulvar Lichen Planus Clinical features . • • . Vulvar lichen planus Women age 50-60 Vulvar pain or pruritus Dyspareunia Erosive variant (most common): 0 Erosive, glazed lesions with white border 0 Vaginal involvement± stenosis 0 Associated oral ulcers • Papulosquamous variant: 0 Small pruritic papules with purple hue Diagnosis Vulvar biopsy Treatment High-potency topical corticosteroids Acute vaginal inflammation that causes friable vaginal mucosa and a serosanguinous vaginal discharge; chronic inflammation can eventually result in stenosis of the vaginal introitus. Vulval Cancer Gynae & Reproductive Health 362 https://t.me/usmleinnercircle Version 2 Vulvar cancer • Persistent HPV infection • Chronic inflammation • Tobacco use Risk factors • Vulvar lichen sclerosus • Immunodeficiency • Prior cervical cancer • Vulvar/cervical intraepithelial neoplasia • Vulvar pruritus Clinical features • Vulvar plaque/ulcer • Abnormal bleeding Diagnosis • Biopsy irc HPV = human papillomavirus. le Etiology Vagina rC  Patients with cytology results that show bacterial vaginosis on Pap testing should be asked about symptoms (eg, malodorous vaginal discharge). Symptomatic patients are screened (eg, wet mount microscopy, potassium hydroxide whiff testing) and treated if the diagnosis is confirmed. MND or clindamycin ne Asymptomatic patients do not require treatment. In Differential diagnosis of prepubertal vaginal bleeding Etiology .. .. .. . . Labial bruising, abrasion (eg, history of fall, saddle injury) Can be a sign of sexual abuse (eg, perinea! tear) LE Trauma Findings Foreign body U SM Infection Malignancy (eg, rhabdomyosarcoma) Precocious puberty Chronic, yellow, malodorous vaginal discharge No external abnormalities on examination Group A Streptococcus: well-demarcated perinea! erythema ± purulent discharge Shigella: vu Ivar erythema, often recent history of diarrhea Soft nodules protruding through vagina in young child (age <3) Earl~ (age <8) breast development, pubic/axillary hair Vaginal foreign bodies in children Epidemiology • Usually prepubertal girls • Toilet paper most commonly retained object • Vaginal spotting Clinical features Management • Malodorous vaginal discharge • No signs of trauma (eg, lacerations) • Topical anesthetic & warmed fluid irrigation • If necessary, vaQinoscopy under anesthesia • Speculum examination should not be performed in a prepubertal girl. Gynae & Reproductive Health 363 https://t.me/usmleinnercircle Version 2 It can result in significant discomfort and trauma due to the narrow vaginal introitus and sensitive hymenal tissue from a low estrogen level at this age. Even when under anesthesia, prepubertal girls should be evaluated with vaginoscopy, not speculum examination. • Vulvovaginitis in a prepubertal child presents with vaginal itching and erythema, as well as foul odor or vaginal discharge based on the cause. Causes include poor hygiene, foreign bodies, occlusive clothing, exposure to irritants, and infection. Children presenting with vulvovaginitis should be carefully evaluated for signs and symptoms of physical or sexual abuse. Treatment of nonspecific vulvovaginitis is initially conservative, with recommendations to improve hygiene and limit exposure to irritants such as bubble baths or synthetic materials. Vaginismus Genito-pelvic pain/penetration disorder • Sexual trauma Risk factors . • Lack of sexual knowledge History of abuse • Pain with vaginal penetration Clinical features • Distress/anxiety over symptoms • No other medical cause Treatment • Desensitization therapy • Kegel exercises  What is the likely diagnosis in a premenopausal woman with aversion to sexual intercourse due to muscle spasm/pain with penetration? External pelvic examination is unremarkable. Genito-pelvic pain/penetration disorder (vaginismus) treatment is aimed at relaxing the vaginal muscles (e.g. desensitization therapy, Kegel exercises); Differentiated from vulvodynia by absence of pain to superficial touch of the vaginal vestibule Cancer Gynae & Reproductive Health 364 https://t.me/usmleinnercircle Version 2 Sarcoma botryoides Vaginal cancer Age> 60 Age <20 Risk factors •HPV16or18 • History of cervical dysplasia or cancer • Cigarette use In utero exposure to diethylstilbestrol Location of cancer Upper 1/3 of the posterior vaginal wall Upper 1/3 of anterior vaginal wall Clinical features • Malodorous vaginal discharge • Postmenopausal or postcoital vaginal bleeding • Irregular mass, plaque, or ulcer on vagina Diagnosis Biopsy irc Epidemiology le Clear cell adenocarcinoma Squamous cell rC Type  The most significant risk factors for vaginal squamous cell carcinoma are smoking and HPV infection. ne similar to the risk factors for cervical cancer Urethral Diverticulum In Miscellaneous Urethral diverticulum Definition LE Urethral diverticulum • Urethral mucosa herniated into surrounding tissue U SM • Dysuria Clinical features • Postvoid dribbling • Dyspareunia • Anterior vaginal wall mass • Urinalysis Urine culture Diagnostic testing • • MRI of the pelvis • Transvaginal ultrasound • Urethral diverticulum, an abnormal localized outpouching of the urethral mucosa into surrounding tissues. Gynae & Reproductive Health 365 https://t.me/usmleinnercircle Version 2 A urethral diverticulum typically arises from recurrent periurethral gland infections, which can develop into an abscess that can eventually breach the urethral mucosa. The persistent infection, inflammation, and increased tissue tension in the area causes a tender anterior vaginal wall mass that may present as dyspareunia or a palpable mass on pelvic examination. In addition, the diverticulum may collect urine and debris, resulting in a purulent discharge, dysuria, or postvoid dribbling. MRI is used to confirm the diagnosis of a urethral diverticulum; patients are treated via surgical excision. Urethral prolapse Occurs in Prepubertal girls Disorders of Sex Development Gynae & Reproductive Health 366 https://t.me/usmleinnercircle Version 2 Disorders of sexual development X-linked mutation of androgen receptor Axillary Breast development Reproductive organs & pubic Yes Absent uterus & upper vagina; cryptorchid testes Minimal to absent 46,XY Yes Absent or rudimentary uterus & upper vagina; normal ovaries Normal 46,XX Normal uterus, abnormal vagina; normal ovaries Hypoplastic or absent miillerian ductal system Transverse vaginal septum Malformation of urogenital sinus & MOllerian ducts Yes Turner syndrome Complete/partial absence of 1 X chromosome Variable (depending on ovarian function) Normal Normal uterus & vagina; streak ovaries Normal 46,XX 45,X ne (MayerRokitanskyKusterHauser syndrome) rC Miillerian agenesis Karyotype hair le Complete androgen insensitivity syndrome Cause irc Diagnosis Turner Syndrome .. . . . .. .. Congenital heart defects•: 4-extremity BP, ECG, echocardiogram Aortic dilatation/dissection**: TTE or cardiac MRI Metabolic syndrome: BP, HbA1c, lipid panel, LFT LE Cardiovascular In Comorbidity screening in Turner syndrome Renal Musculoskeletal U SM Vision & hearing Autoimmune Horseshoe kidney: renal ultrasound Osteoporosis: 25-hydroxyvitamin D level, DXA scan Strabismus. myopia: ophthalmology evaluation Recurrent otitis media, hearing loss: audiology testing Celiac disease: tissue transglutaminase antibodies Hypothyroidism: TSH, free T4 *Aortic coarctation, bicuspid aortic valve. .. High risk in pregnancy. BP= blood pressure; HbA1c = glycosylated hemoglobin; LFT = liver function test; TTE = transthoracicechocardiogram. Gynae & Reproductive Health 367 https://t.me/usmleinnercircle Version 2 Turner syndrome Low hairline-----:-1'1. Webbed neck-----.1 Coarctationof aorta Broad chest with widely spaced nipples~-----+--~ Bicuspidaorticvalve Horseshoe kidney Aorticdissection Streak ovaries (amenorrhea, infertility) Congenital lymphedema  All patients with Turners require screening for cardiac disease with 4-extremity blood pressures, ECG, and imaging (eg, echocardiography, cardiac MRI. If aortic dilatation is detected, beta blockers and aggressive blood pressure control are indicated; surgical repair may be considered based on aortic size and risk factors. Aromatase Deficiency Aromatase deficiency • Karyotype: 46 XX or 46 XY (normal sex development) • Pathophysiology: Mutations in the CYP19Al gene which encodes for the enzyme aromatase i;;::l -+ ! serum estrogen and t serum testosterone • Clinical features • Females (46 XX) _Birth:Ambiguous genitalia despite normal internal genital organs _Puberty Impaired maturation of _secondarysexual characteristics Primary amenorrhea i;;::l ~[~i_l)~_i!!l<?_rl_ (e.g., -~l~~~_tJ~_n_,_, severe _acne) • Both males and females Childhood Tall stature Osteoporosis (e.g., fractures following minimal trauma) • Mothers of affected children may experience virilization during pregnancy (may start at 12 weeks' gestation and typically disappear after delivery) • Treatment • Estrogen and progesterone replacement therapy • Calcium and vitamin D supplementation • Surgical correction of ambiguous genitalia  What is the likely diagnosis in an adolescent girl with delayed puberty, clitoromegaly, and osteoporosis? Laboratory exam reveals undetectable estrogen and elevated testosterone levels. Aromatase deficiency Gynae & Reproductive Health 368 https://t.me/usmleinnercircle Version 2 Mullerian Agenesis Mullerian agenesis Pathogenesis • Mullerian duct system defect • Abnormal development of uterus, cervix & upper third of vagina • Primary amenorrhea • Normal female external genitalia Clinical features • Blind vaginal pouch • Absent or rudimentary uterus le • Vaginal dilation (surgical or nonsurgical) 5-Alpha Reductase • 46,XY genotype rC 5-a reductase deficiency irc Management • Bilateral functioning ovaries (normal FSH) • Evaluate for renal tract abnormalities (eg, renal ultrasonography) • Impaired conversion of testosterone to DHT • Impaired virilization during embryogenesis • Normal male testosterone & estrogen levels • Male internal genitalia (eg, testes, vas deferens) • Female external genitalia (eg, blind-ending vagina) • Phenotypically female at birth ne Pathogenesis • Virilization at puberty (t testosterone) In Clinical features LE 0 Clitoromegaly 0 Increased muscle mass 0 Male pattern hair development 0 Nodulocystic acne DHT = dihydrotestosterone. U SM Dx: elevated testosterone/DHT ratio. Male Gynecomastia Gynae & Reproductive Health 369 https://t.me/usmleinnercircle Version 2 Male breast enlargement Gynecomastia Bilateral (usually) Pseudogynecomastia Bilateral Malignancy Unilateral Evaluationof gynecomastia Enlargement of breast tissue in a male patient 1 Discrete enlargement of glandular breast tissue Indistinct fatty enlargement, obese habitus Rule out toxic exposure (herbal supplements, anabolic sterotds, cannabinoids) Nadiscrete glandular tjssye Pseudogynecomastia Measure hormone levels (hCG, LH, testosterone, estradiol) tLH thCG .i.or normal LH .i.Testosterone .i.Testosterone tLH t Testosterone t Estradiol Testicular or gonadal germ-cell tumor Central hypogonadism (eg, pituitary tumor) Primary hypogonadism (eg, Klinefelter syndrome) Possible thyrotoxicosis Testicular or adrenal tumor hCG = Human chorionicgonadotropin,LH = Luteinizinghormone ©uworld Common pathological causes of gynecomastia • Testicular, adrenal, or human chorionic gonadotropin-secreting tumors Increased estrogen production or peripheral conversion • Cirrhosis or malnutrition • Thyrotoxicosis • Congenital excessive aromatase activity • Androgen use • Drugs (eg, spironolactone, cimetidine), herbal products (eg, tea tree oil, lavender oil) Androgen deficiency • Primary or secondary male hypogonadism (eg, Klinefelter syndrome, testicular damage) • Hyperprolactinemia • Renal failure Gynae & Reproductive Health 370 https://t.me/usmleinnercircle Version 2  What should you tell a pubertal boy 1214 years) with gynecomastia? Reassurance Persistence will require work up Occurs in 2/3 of boys and is normal, Due to conversion of testosterone to estrogen Pubertal gynecomastia features Management (Tanner stages 3-4) • Small (<4 cm) firm, unilateral or bilateral, subareolar mass • No pathologic features (eg, nipple discharge, axillary lymphadenopathy, systemic illness) • Reassurance & observation le Clinical • Imbalance of estrogens & androgens during midpuberty irc Etiology • Resolution within a year rC • Physiologic gynecomastia is a benign glandular proliferation of male breast tissue due to an imbalance between levels of estrogen and androgens. It is common in older men due to decreased testicular testosterone production and increased Male Breast Cancer ne conversion of testosterone to estrogen in adipose tissue. Male breast cancer Presentation • Family history; BRCA 1/2 In Risk factors • Abnormal estrogen/androgen ratio: Klinefelter s:tndrome, obesity, cirrhosis, marijuana use • Subareolar mass • Skin & nipple dimpling, induration, ulceration LE • Often detected at advanced stage • Mammography • Biopsy: invasive ductal carcinoma (hormone receptor-positive) most common U SM Diagnosis • Gynecomastia can have variable density but is typically symmetric, is centrally located with respect to the nipple, and may have an indistinct margin with surrounding fat. • Breast cancer is more likely eccentric to the nipple, has well-defined or spiculated margins, and may contain calcifications. Hypogonadism Gynae & Reproductive Health 371 https://t.me/usmleinnercircle Version 2 Male secondary hypogonadism • Fatigue, decreased libido • Testicular atrophy Clinical features • Laboratory: low testosterone, low/normal LH • Pituitary tumors, hyperprolactinemia • Medications: opioids, glucocorticoids, exogenous androgens (withdrawal phase) Common causes • Infiltrative disease (eg, hemochromatosis) • Chronic/severe illness • Eating disorders, severe weight loss Breast enlargement in secondary hypogonadism is less prominent. (vs Primary) • Opioids suppress GnRH and LH secretion, leading to reduced Leydig cell testosterone synthesis, decreased spermatogenesis, and testicular atrophy. Urethritis Infectious urethritis in men • Neisseria gonorrhoeae Etiology • Chlamydia trachomatis • Mycoplasma genitalium • Trichomonas vagina/is Acute epididymitis Age <35: sexually transmitted (chlamydia, gonorrhea) Manifestations Diagnosis • Dysuria • Discharge • Gram stain & culture • Nucleic acid amplification testing • GU alone: ceftriaxone Treatment • GU + Chlamydia*: ceftriaxone + doxycycline • Chlamydia/Mycoplasma: azithromycin • Trichomonas: metronidazole 'GU + uncertainChlamydia statusis also treatedwith ceftriaxone+ doxycycline. GU = gonococcalurethritis. First-line empiric treatment awaiting results: Single dose of intramuscular ceftriaxone plus a 7-day course of oral doxycycline  The diagnosis of infectious urethritis is made when 1 of the following are present: • Purulent or mucopurulent penile discharge • 2 leukocytes per field on urethral swab Gram stain • Positive leukocytes on urine dipstick or 10 leukocytes/hpf on urinalysis Trichomoniasis is usually asymptomatic in men and is an uncommon cause of urethritis. Scrotum Gynae & Reproductive Health 372 https://t.me/usmleinnercircle Version 2 Varicocele Varicocele Primary Secondary • Compression of left renal vein between SMA and • Extrinsic compression (renal or retrooeritoneal mass) of aorta IVC • • "Bag of worms" mass Incompetent venous valves • • • Initial management • "Bag of worms" mass • Prepubertal onset Pubertal onset • Right-sided Left-sided Decompresses when supine • Persists when supine • Reassurance and observation • Abdominal ultrasound irc Clinical features • Venous thrombus • Clinical findings Treatment 0 ! In supine position 0 t With standingNalsalva maneuvers • Subfertility • Testicular atrophy . Retrograde venous flow • Tortuous, anechoic tubules adjacent to testis • Dilation of pampiniform plexus veins • Gonadal vein ligation (boys & young men with In Ultrasound Soft scrotal mass ("bag of worms") ne presentation rC IVC = inferior vena cava; SMA = superior mesenteric artery. Varicocele le Pathophysiology testicular atrophy) • Scrotal support & NSAIDs (older men who do not desire additional children) LE NSAIDs = nonsteroidalanti-inflammatorydrugs. U SM Left sided and Bilateral are common; Right sided should raise the suspicion for Malignancy Hydrocele Ouctus deferens Normal anatomy Noncommunicating hydrocele Abdominal /cavity Communicating hydrocele Patent processus vaginalis Closed vaginalis Gynae & Reproductive Health 373 https://t.me/usmleinnercircle Version 2 • Transillumination helps differentiate scrotal masses, as hydroceles are cystic and transmit light whereas inguinal hernias contain bowel that does not typically transilluminate. Most hydroceles, both communicating and noncommunicating, should spontaneously resolve by age 1 year and can be safely observed during that period. Prostate Text Prostatitis Overview of prostatitis Acute Clinical presentation . flu like symptoms . . . . Fever, chills, malaise, myalgia Pelvic pain, cloudy urine Pyuria, tender prostate Diagnosis Urine culture positive (usually Escherichia colt) Treatment TMP-SMX or fiuoroquinolones . . . . . . Chronic Dysuria and urinary frequency No symptoms of acute prostatitis Recurrent urinary tract infection Pyuria with possible tender prostate Urine culture positive (usuallyEschen·chiacoli) Fluoroquinolones • Cystoscopy and urethral catheterization should generally be avoided in patients with Acute Bacterial Prostatitis because passage of instruments through the urethra can cause septic shock (due to release of bacteria from prostate) or prostatic rupture. Patients who develop acute urinary retention due to prostatic swelling from ABP often require suprapubic (not urethral) catheterization. Chronic prostatitis/chronic pelvic pain syndrome Symptoms • Pain in pelvis, perineum, genitalia • Irritative voiding symptoms (eg, urgency, hesitancy) • Hematospermia, pain with ejaculation Diagnosis • No or mild prostate tenderness • Sterile urine culture • Alpha blockers (eg, tamsulosin) Management • Antibiotics (eg, ciprofloxacin), especially if history of UTI • 5-alpha-reductase inhibitors (eg, finasteride) UTI = urinary tract infection. Prostatic abcess- dx by fluctuance, USG guided drainage or surgery Gynae & Reproductive Health 374 https://t.me/usmleinnercircle Version 2 Chronic bacterial prostatitis Pathogens Presentation • Young & middle-aged men • j Risk with diabetes, smoking, urinary tract procedure • Coliforms enter from urethra via intraprostatic reflux • Escherichia coli causes >75% of cases • Recurrent urinary tract infection (with the same organism) • +/- Prostatic tenderness & swelling (often absent) • Pain with ejaculation • History of antibiotic treatment -+ transient improvement • Pyuria and bacteriuria on urinalysis • Bacteria in prostatic fluid > bacteria in urine Treatment • Fluoroquinolones (eg, ciprofloxacin) for 6 weeks irc Diagnosis le Epidemiology  What is the most likely diagnosis in an elderly male with 3-month history of perinatal and scrotal pain, pain during ejaculation, and a mildly tender prostate? Vitals are within normal limits. rC Urine analysis shows: Positive WBC, Urine Culture Negative. Chronic Pelvic Pain Syndrome complains, and painful ejaculation Prostate Cancer & BPH ne Diagnosis of exclusion. Patients present with persistent pelvic pain, irritation urological In  What is the most appropriate pain management for a patient with advanced prostate cancer with bony metastasis, status-post orchiectomy? Radiation therapy LE bisphosphonates are useful for controlling chronic pain, but radiation is more acute; anti-androgen therapy is not needed in patients that have undergone orchiectomy  What are the current recommendations regarding PSA screening in asymptomatic patients? U SM Controversial; should be determined on a case-by-case basis by the physician and patient  What is the best next step in a male with nocturia, increased urinary urgency, and frequency? Vitals are normal. Rectal exam shows a diffusely enlarged and non-tender prostate with a smooth surface. Urine analysis Patients with lower urinary tract symptoms should have a urine analysis to evaluate for hematuria (bladder cancer, stones) and infection. Kava kava - anxiety and insomnia- risk of severe liver toxicity. St. John's wort -depression-inconsistent evidence its efficacy. Hypercholesterolemia - garlic preparations- limited data regarding its efficacy. Fish oil supplementation - refractory hypertriglyceridemia. Osteoarthritis - glucosamine and chondroitin- safe,their clinical benefit are limited. Gynae & Reproductive Health 375 https://t.me/usmleinnercircle Saw palmetto is a popular herbal preparation most often used by men to treat benign prostatic hyperplasia. Its use has not been shown to significantly improve urinary symptoms or flow measures. In addition, saw palmetto does not appear to affect prostate-specific antigen levels or prostate size. Version 2 Comparison of benign prostatic hyperplasia & prostate cancer BPH Risk factors Affected part Examination . . . . Prostate cancer Age >50 Central portion (transitional zone) Symmetrically enlarged & smooth prostate Can have elevated PSA . . . . Age >40, African American & family history Usually peripheral zone of prostate but can be anywhere Asymmetrically enlarged, nodules & firm prostate Markedly elevated PSA Medical therapy for benign prostatic hyperplasia PSA increased due acute obstruction d/t drug or infection recheck after 2-6 week Alpha-adrenergic antagonists (eg, terazosin, tamsulosin) 5-alpha-reductase inhibitors (eg, finasteride, dutasteride) Antimuscarinics (eg, tolterodine) • Relax smooth muscle in bladder neck, prostate capsule & prostatic urethra • Usual first-line therapy • Side effects: Orthostatic hypotension, dizziness • • • • Inhibit conversion of testosterone to dihydrotestosterone Reduce prostate gland size Effectiveness may take 6-12 months Side effects: Decreased libido, erectile dysfunction • Used to treat overactive bladder (urinary frequency, urgency & incontinence) • Side effects: Urine retention, dry mouth  What is the preferred initial treatment for uncomplicated benign prostatic hyperplasia BPH? Alpha-1 blockers (e.g. terazosin, tamsulosin) 5-alpha-reductase inhibitors can be used as an alternative or in addition to alpha blockers but have a slower onset of action  Patients can have re-growth of BPH after TURP. Penis Hypospadias Normal urethral opening Gynae & Reproductive Health Hypospadias 376 https://t.me/usmleinnercircle Version 2  Which anti-depressant medication is associated with priapism? Trazodone however the most common drug that causes priapism is prazosin  Postprocedural bleeding is a common complication of circumcision and typically resolves with direct pressure (eg, compressive elastic dressing). Erectile Dysfunction Clinical features Endocrine Medications rC Psychogenic • Gradual onset, loss of bulbocavernosus reflex • Sudden onset • Situational (eg, ED with partner, normal erection during masturbation) • Normal nonsexual nocturnal erections ne Neurologic • Cardiovascular risk factors (eg, hypertension, smoking, diabetes) • Abnormal vascular examination (eg, bruits, decreased pulses) • Neurologic comorbidity (eg, diabetic neuropathy, multiple sclerosis, spinal injury/surgery) • Additional symptoms due to underlying disorder • Abnormal hormone levels (eg, TSH, prolactin) • Onset related to starting medication • Antihypertensives, SSRls, antiandrogenic medications In Vascular irc Common causes of erectile dysfunction le A compressive dressing should be used for only a short time and must be removed prior to discharge to prevent necrosis. • Gradual onset • Decreased libido, gynecomastia, testicular atrophy • Low serum testosterone LE Hypogonadism ED = erectile dysfunction; SSRls = selective serotonin reuptake inhibitors. U SM  Lack of nocturnal erections suggests an organic etiology of erectile dysfunction, such as neurogenic or vascular. Normal testing suggests psychogenic etiology. Balanitis Gynae & Reproductive Health 377 https://t.me/usmleinnercircle Version 2 Balanitis & balanoposthitis Definition Etiologies Evaluation Management • Balanitis: inflammation of the glans penis • Balanoposthitis: inflammation of the glans penis + foreskin • Irritation (eg, poor hygiene, contact dermatitis) • Infection (eg, Candida albicans, bacterial flora, STls) • Trauma (eg, aggressive foreskin retraction) • Consider KOH microscopy for suspected Candida infection (eg, thick, white discharge)* • STI screen if urethral discharge 12resent • Foreskin hygiene, sitz baths • Topical treatment (ie, antifungal or antibiotic) • DM screen for Candida infection without risk factor (eg, diaeer dermatitis, recent antibiotic use) •Empiric treatment appropriate with concurrent diaper dermatitis. DM = diabetes mellitus; KOH = potassium hydroxide; STI = sexually transmitted infection. Penis Fracture Clinical features • Cracking, snapping, or popping sound caused by rupturing of the tunica albuginea and soft tissue • Immediate detumescence (loss of erection) • Pain; varies in intensity depending on severity of the injury • Pronounced soft tissue swelling, curving, and hematoma of the penis ("eggplant" appearance) i;:J • Blood at the urethral meatus in concomitant urethral injury References:[ 11 » Feedbac , .. Your notes Diagnostics Differential diagnoses Treatment • Surgical procedure i;:J • Exploration of the penis with evacuation of hematoma • Repair of possible urethral injury with insertion of a catheter for stenting purposes • Suture of rupture in the tunica albuginea • Postoperative measures • Pain medication; prophylactic oral antibiotics • Light compression dressing • 4 weeks of sexual abstinence (suppression of spontaneous erections with diazepam or stilboestrol may be considered) • Follow-up with retrograde urethrography in patients with urethral reconstruction upon removal of the urethral catheter after 2 weeks I Penile fracture is a urological emergency and requires immediate surgical treatment to restore functionality and minimize risk of long-term complications!  Are penile fractures typically managed medically or surgically? Surgically medical management of PF has a higher rate of complications Peyronie Disease Arises due to repetitive blunt trauma to the penis during sexual intercourse with subsequent aberrant wound healing. It is characterized by the formation of fibrous plaques (due to transforming growth Gynae & Reproductive Health 378 https://t.me/usmleinnercircle Version 2 factor-1 up-regulation) in the tunica albuginea.  Manifestations generally include penile pain, curvature, and dorsal nodules or plaques. Dx: Clinical or USG T/t: Spontaneous resolution or progressive PD often require NSAIDs drugs for pain; pentoxifylline to reduce fibrosis; and/or intralesional injections of collagenase. Surgery may be indicated in refractory cases. le Phimosis Uncircumcised ne rC irc Phimosis penis Phimosis Physiologic in children In CIUWorid Paraphimosis Urologic emergency Phimosis, or the inability to retract the prepuce (foreskin) in an uncircumcised male patient, is usually considered physiologic in infants and young children. Forced manipulation of the prepuce may result in paraphimosis, the retraction and entrapment of the prepuce. Due to decreased lymphatic and venous blood flow to the distal penis, the presentation of paraphimosis includes edema and significant tenderness of the glans penis and prepuce. Initially, the glans may appear pink and soft. Without intervention, however, arterial blood flow may be compromised, leading LE to ischemia and penile tissue firmness and darkening (blue to black). Management of paraphimosis is emergency reduction, either manually or surgically, to maintain vascular flow to the glans penis. Circumcision is a definitive treatment option for preventing the recurrence of paraphimosis but may be unnecessary for an isolated episode that is easily resolved U SM by reduction. • Pathologic phimosis is the inability to retract the foreskin due to scarring and fibrosis (eg, secondary to balanoposthitis) of the prepuce. Topical corticosteroids are used in these cases Testes Cryptorchidism Gynae & Reproductive Health 379 https://t.me/usmleinnercircle Version 2 Management of cryptorchidism in infants Undescended testicle (testicle not present in scrotum) l • Bilateral nonpalpable testicles • Hypospadias ~_l___::,=t ..,., Age <6 months Age ~6 months l l sex development" • Karyotype • Pelvic ultrasound • Adrenal/gonadal hormones Refer for orchiopexy Monitor for spontaneous descent •eg, congenital adrenal hyperplasia, hypogonadotrophic hypogonadism, androgen insenslbvity, gonadal dysgenesis iC)UWorld Cancer Testicular cancer Epidemiology • Age 15-35 • Risk factors: Family history, cryptorchidism • Unilateral, painlesstesticularnodule Manifestations • Dull lower abdominal ache • Metastatic symptoms (eg, dyspnea, neck mass, low back pain) • Examination: Firm, ovoid mass Diagnosis • Tumor markers (AFP, 13-hCG) • Scrotalultrasound • Screening imaging (CT scan, chest x-ray) • Radical orchiectomy Treatment • Chemotherapy • Cure rate -95% Seminoma Germ cell (95%) Nonseminoma Leydig Stromal (5%) Sertoli .. .. .. .. Malignant testicular neoplasms Retain features of spermatogenesis 13-hCG,AFP usually negative ~1 partially differentiated cells: yolk sac, embryonal carcinoma, teratoma, and/or choriocarcinoma 13-hCG,AFP usually positive Often produces excessive estrogen (gynecomastia) or testosterone (acne) Can cause precocious puberty Rare Occasionally associated with excessive estrogen secretion (eg, gynecomastia) AFP = alpha-fetoprotein. Gynae & Reproductive Health 380 https://t.me/usmleinnercircle Version 2  Patients with testicular cancer may present with low back pain due to para-aortic (retroperitoneal) lymphadenopathy. • Scrotal ultrasound solid, hypoechoic lesion (seminoma) or a lesion with cystic areas and calcifications (nonseminomatous germ cell tumor NSGCT GERM CELL Seminoma YOLK SAC AFP EMBRYONAL AFP + p-HCG TERATOMA No marker I I ------------1-------TERATOCARCINO MA AFP + p-HCG If AFP is elevated, it excludes seminoma Elevated MP levels Elevated BCG levels Radical inguinal orchiectomy Radiation therapy Chemotherapy Retroperitoneal lymph node disseclion No Rare Yes Radiosensitive Yes No onseminoma Lesscommon Common le CHORIOCARCINOMA p-HCG Most common irc 1-------------1--------1----------1-------I Incidence Common Yes ot radiosensitive Yes Yes (many patients) rC I SE MINOMA--------------+-------NON-SEM INOMA p-HCG• I ne • What is the next best step in a patient with a hard mass in the left testicle and ultrasound evidence likely for a testicular tumor? Inguinal Radical Orchiectomy In Do not cut the scrotum, may spread the disease. Likewise, do not perform needle biopsy. There is a high cure rate, even if there is metastasis. Kill first, investigate later— biopsy can be done with the orchiectomy. LE FNAC and trans royal biopsy are contraindicated because of risk of spillage of cancer cells. U SM Repro Paeds  What is thelarche? Onset of breast development as a result of release of estrogen • Adrenarche? Onset of pubic or axillary hair development in female as a result of release of adrenal androgens Gynae & Reproductive Health 381 https://t.me/usmleinnercircle Version 2 • Breast buds are usually the first sign of puberty in females 15% is pubic hair). Firm, Retroareolar Mass, might be slightly Tender  What is the first sign of puberty in a male? Testicular enlargement Tanner stages Tanner 1 Tanner 2 (age 10-11.5) Tanner 3 (age 11.5-13) Tanner 4 (age 13-15) Tanner 5 (age >15) Precocious Puberty  What is the treatment of premature thelarche and premature adrenarche? None - Very common and benign in 1st 2 years of life Gynae & Reproductive Health 382 https://t.me/usmleinnercircle Version 2 Evaluation of precocious puberty Early secondary sexual development* Normal bone age Advanced bone age GnRH stimulation test c= '"j "''"'" j Peripheral precocious puberty Central precocious puberty II Isolated breast development Isolated pubic hair development j j Premature thelarche Premature adrenarche rC •Secondary sexual development in gir1s age <8 or boys age <9. le High basal LH irc Low basal LH Central: Idiopathic, Tumor; Peripheral: CAH, Exogenous, McCune Albright  What are the (4) components of the work-up for central precocious puberty? ne 1. FSH, LH, sex steroids 2. GnRH stimulation test (inc. in LH) 4. Thyroid (r/o hypothyroidism) In 3. MRI (r/o CNS lesions) - prior to starting GnRH agonist therapy  What is the mechanism of treatment of central precocious puberty? LE GnRH agonists (leuprolide)  What is the likely diagnosis in an obese young girl that presents with pubic hair, acne, and normal bone age? U SM Premature adrenarche adiposity can trigger excess insulin production, which stimulates the adrenal glands to produce sex hormones; associated with an increased risk of PCOS in adolescence  What is the likely diagnosis in a young girl that presents with precocious puberty with an advanced bone age, high FSH/LH levels, and a normal MRI? Idiopathic precocious puberty t/t: Continuous GnRH agonist therapy (e.g. leuprolide) prevents premature epiphyseal plate fusion and maximizes adult height potential  What is the likely diagnosis in a young boy that presents with advanced bone age, coarse pubic hair, and severe cystic acne with low basal LH levels and normal testicular exam? Late-onset (non-classic) congenital adrenal hyperplasia Gynae & Reproductive Health 383 https://t.me/usmleinnercircle Version 2 due to 21-hydroxylase deficiency; excessive adrenal androgen production leads to peripheral precocious puberty McCune-Albright syndrome • Mutation GNAS gene Pathogenesis • Constant G protein activation • Hormone overproduction • Peripheral precocious puberty Clinical features • Irregular cafe-au-lait macules • Polyostotic fibrous dysplasia • Thyrotoxicosis Complications • Acromegaly • Cushing syndrome girls typically have premature vaginal bleeding and breast development Neonatal Withdrawal Bleeding Neonatal withdrawal bleeding Physiology • In utero: maternal estrogen stimulates fetal endometrial proliferation • After delivery: withdrawal of maternal hormones _, endometrial sloughing in neonate • Light vaginal bleeding in first 2 weeks of life • ± Additional signs of maternal estrogen exposure: Clinical features 0 Physiologic leukorrhea 0 Labial swelling 0 Breast hypertrophy ± galactorrhea • Clinical diagnosis Diagnosis & management I • Reassurance for parents • Resolves on its own within days  What are consequences of intrauterine estrogen exposure? • Breast bud development is normal in infants of either gender • Newborn girls have bloody or mucoid vaginal discharge (most common cause of vaginal bleeding in neonate) Growth Delay Gynae & Reproductive Health 384 https://t.me/usmleinnercircle Version 2 Delayed puberty in boys .. .. Clinical features Initial workup 0 Klinefelter syndrome Secondary hypogonadism 0 Constitutional, chronic illness, malnutrition 0 Hypothyroidism, hyperprolactinemia 0 Kallmann syndrome 0 Craniopharyngioma Absent testicular enlargement by age 14 Delayed growth spurt FSH, LH, testosterone, TSH, prolactin Bone age radiograph Constitutional delay of growth and puberty Prognosis Short stature; normal growth velocity Delayed bone age rC Management Family history of "late bloomers" Delayed puberty Reassurance; watchful waiting ± Hormone therapy Puberty onset correlates with family members ne Clinical features .. . . .. . . le Causes Primary hypogonadism irc . . Normal expected adult height In  If psychosocial concerns are severe, or if puberty is significantly delayed, short-term testosterone therapy in boys, or estrogen therapy in girls, may be considered. Circumcision LE Pharmacotherapy, however, should be used only in boys age 14 or girls age 12. Health benefits associated with neonatal circumcision include the following: U SM • Significant decrease 10-fold) in urinary tract infections in the first year of life • Prevention of pathologic phimosis (ie, foreskin constriction preventing retraction) and modest reduction in the risk of penile cancer (likely due to reduction in phimosis, which is a risk factor). • Reduction in certain penile inflammatory disorders (eg, balanitis). • Reduction in the risk of acquiring some sexually transmitted infections STIs) (eg, human papillomavirus) and HIV through penile-vaginal sex, especially in countries where HIV is endemic. Drugs • GnRH agonists are not recommended for adolescents due to decreased bone mineral density caused by prolonged use. Gynae & Reproductive Health 385 https://t.me/usmleinnercircle Version 2 Clinical features of androgen abuse • Exogenous (eg, testosterone replacement therapy) Types of androgens • Synthetic (eg, stanozolol, nandrolone) • Androgen precursors (eg, DHEA) • Reproductive 0 Men: decreased testicular function & sperm production, gynecomastia o Women: acne, hirsutism, voice deepening, menstrual irregularities Side effects/ clinical presentation • Cardiovascular: left ventricular hypertrophy, possible t HDL & t LDL • Psychiatric: aggressive behavior (men), mood disturbances • Hematologic: polycythemia, possible hypercoagulability DHEA = dehydroepiandrostenedione. Contraception options Type Typical use efficacy Intrauterine device >99% Contraception methods & adverse effects Method Combined hormonal contraceptive Implant >99% Female sterilization >99% Male sterilization >99% Injection 94% CHCs 91% Condom 82% Withdrawal 78% pill, patch, or ring .. . . .. . . . .. Adverse effects Breakthrough bleeding Breast tenderness Nausea Initial irregular bleeding • Amenorrhea Depot medroxyprogesterone Progestin subdermal implant CHCs = combinedhormonalcontraceptives(eg, pills, patch, ring). Progestin intrauterine device Copper intrauterine device Reversible bone loss Delayed return to fertility ± Weight gain Irregular bleeding Irregular bleeding • Amenorrhea Heavy menses Dysmenorrhea Progesterone  Adolescents with irregular menstrual bleeding due to anovulatory cycles may benefit from progesterone therapy. progesterone, which is normally secreted by the corpus luteum during ovulatory cycles, causes differentiation of the proliferative endometrium into secretory endometrium; withdrawal causes menstruation  Common side effects of the injectable medroxyprogesterone contraceptive are vaginal bleeding/spotting and weight gain.  The progestin-releasing subdermal implant is a long-acting (ie, up to 3 years), reversible contraceptive that reduces menstrual bleeding in 50% of patients and causes amenorrhea in 20% of patients Useful for patients where estrogen is contraindicated Gynae & Reproductive Health 386 https://t.me/usmleinnercircle Version 2 OCP & IUD Combined estrogen/progestin oral contraceptive pills • Irregular, unscheduled bleeding • Breast tenderness, nausea, bloating • Amenorrhea Adverse effects • Hypertension (most due to estrogen component) • Menstrual cycle regulation Benefits le • Venous thromboembolic disease • Liver disorders (eg, hepatic adenoma) • j Serum triglycerides • Pregnancy prevention irc • ! Dysmenorrhea • ! Hyperandrogenism (eg, acne, hirsutism) • ! Risk of ovarian & endometrial cancer (if ever used) • j Risk of cervical cancer (only if currently or recently used) Risk augmentation - rC Should not be used in women age  35 that use tobacco due to risk of myocardial infarction and stroke Side effects & risks of combination oral contraceptives ne • Hypertension: due to increased angiotensinogen synthesis by estrogen during first-pass metabolism; discontinuing use can correct the hypertension in most patients Absolute contraindications to combined hormonal contraceptives • Breakthrough bleeding • Breast tenderness, nausea, bloating • Migraine with aura • Hypertension • Venous thromboembolic disease In • Amenorrhea • 2'..15cigarettes/day PLUS age 2'..35 • Hypertension 2'..160/100mm Hg • Decreased risk of ovarian & endometrial cancer • Heart disease • Increased risk of cervical cancer • Diabetes mellitus with end-organ damage LE • Liver disorders (eg, hepatic adenoma) U SM • Increased triglycerides (due to estrogen component) • History of thromboembolic disease • Antiphospholipid-antibody syndrome • History of stroke • Breast cancer • Cirrhosis & liver cancer • Major surgery with prolonged immobilization • Use <3 weeks postpartum Weight gain is NOT a side effect of OCP  Progestin and estrogen also affect the endometrial lining, which can cause breakthrough bleeding. This occurs because progestins create a thin, atrophic endometrium that can shed erratically, particularly with OCPs that have low doses of estrogen, which normally acts to thicken and stabilize the endometrium. Gynae & Reproductive Health 387 https://t.me/usmleinnercircle Version 2 Therefore, breakthrough bleeding may be treated by prescribing an OCP with a higher dose of estrogen.  What is the preferred method of contraception for patients with breast cancer? Copper IUD all hormone-containing contraceptives are contraindicated in patients with breast cancer (both estrogen and progesterone may have a proliferative effect on breast tissue)  What is the recommended contraceptive for a woman with a history of anemia and medication noncompliance? Levonorgestrel IUD typically causes amenorrhea, which is beneficial in patients with anemia (versus the copper IUD, which can cause heavy menstrual bleeding); Also the progestin-releasing IUD does not affect thromboembolic risk. less common side effects include mood changes, breast tenderness, and headache Contraindications to IUD placement • Pregnancy Copper IUD & • Endometrial or cervical cancer • Unexplained vaginal bleeding • Gestational trophoblastic disease • Severe uterine cavity distortion • Active pelvic infection (eg, PID, cervicitis) Active liver disease Progestin IUD • progestin IUD • Current breast cancer Copper IUD • Wilson disease IUD = intrauterine device; PID = pelvic inflammatorydisease. Age and parity are not contraindications to IUD insertion  In postpartum patients who are breastfeeding, progestin-only contraception methods (eg, subdermal progestin-releasing implant, progestin-releasing IUD) are preferred. Estrogen containing contraceptive avoided due to risk of thromboembolism Emergency Contraceptive Gynae & Reproductive Health 388 https://t.me/usmleinnercircle Version 2 Emergency contraception Timing after intercourse Copper-containing intrauterine device Progestin-releasing intrauterine device Efficacy 0-120 hr >99% 0-120 hr >99% Contraindications • Wilson disease • Active pelvic infection • Severe uterine cavity distortion • Breast cancer . Active pelvic infection • Severe uterine cavity distortion • None Ulipristal 0-120 hr 98%-99% Oral levonorgestrel 0-72 hr Oral contraceptives* 0-72 hr • None 75%-89% • None ---- 92%-98% •IUD contraindicated if the patient has acute cervicitis or PID rC SERM irc •combined estrogen/progestinoral contraceptivepills containinglevonorgestrelor norgestrel. le Method Selective estrogen receptor modulators Indications In Mechanism of action • Tamoxifen • Raloxifene • Competitive inhibitor of estrogen binding • Mixed agonist/antagonist action • Prevention of breast cancer in high-risk patients • Tamoxifen: adjuvant treatment of breast cancer ne Drugs • Raloxifene: postmenopausal osteoporosis • Hot flashes • Venous thromboembolism LE Adverse effects • Endometrial hyperplasia & carcinoma (tamoxifen only) • Uterine sarcoma (tamoxifen only) U SM • Selective estrogen receptor modulators increase the risk for venous thromboembolism by increasing protein C resistance. due to estrogen agonist activity In addition, they increase 2/7/8/10, decrease antithrombin 3, and decrease protein S  What is the most common side effect of tamoxifen? Hot flashes 80% due to anti-estrogenic activity in the CNS which causes thermoregulatory dysfunction in the anterior hypothalamus Tamoxifen decreases the blood cholesterol level and may protect against coronary artery disease. Gynae & Reproductive Health 389 https://t.me/usmleinnercircle Version 2  Tamoxifen increases the risk for endometrial hyperplasia/cancer and uterine sarcoma in postmenopausal women. However, asymptomatic patients on tamoxifen do not require routine screening for these complications. Evaluation via ultrasonography or endometrial biopsy is indicated only for symptomatic patients.` PDE-5 Inhibitors Adverse effects of phosphodiesterase-5 inhibitors Cardiovascular Ocular Genitourinary other • Hypotension (especially with nitrates, alpha blockers) • Blue discoloration of vision • Nonarteritic anterior ischemic optic neuropathy • Priapism • Flushing • Headache • Hearing loss  What is the first-line treatment for most patients with erectile dysfunction? PDE5 inhibitors (e.g. sildenafil) If patient has CAD and is on beta-blockers, which may be associated with ED, do not stop these medications unless patients are completely intolerant or experience severe side effects  PDE5 inhibitors are contraindicated in patients who take nitrates and used cautiously in patients who take alpha blockers due to the risk of hypotension. use minimum doses of alpha blockers and sildenafil at least 4 hours apart Gynae & Reproductive Health 390 https://t.me/usmleinnercircle Version 2 Obs Physiology Physiologic Hydronephrosis of Pregnancy Exercise le Vaccines RhD & ABO incompatibility Amniotic Fluids irc Polyhydramnios Oligohydramnios Fetus Gestational Age rC Fetal Surveillance Fetal Heart Rate Prenatal Testing & Screening Congenital Infections ne Group B Strep Syphilis Rubella HSV In Hepatitis HIV IUGR Demise Hydrops Fetalis Hypertension Preeclampsia U SM Eclampsia LE Twins HELLP Gestational Diabetes Pregnancy Preterm Labour Labour Breech External Cephalic Version Internal Podalic Version Shoulder Dystocia Post Term 3rd Trimester Bleeding Placenta Previa Vasa Previa Uterine Rupture Obs 391 https://t.me/usmleinnercircle Version 2 Placental Abruption PROM Chorioamnionitis Miscarriage/ Abortion Recurrent Pregnancy Loss Ectopic Pregnancy Hydatidiform Mole Choriocarcinoma Vomiting Hyperemesis Gravidarum Postpartum PPH Uterine Inversion Placenta Accreta Sheehan Syndrome Perineal Laceration Fistula Pubic Symphysis Diastasis Septic Pelvic Thrombophlebitis Postpartum Endometritis Urinary Retention Miscellaneous Back Pain Round Ligament Pain Liver Disorders of Pregnancy Pseudocyesis Drugs Uterotonic Physiology Obs 392 https://t.me/usmleinnercircle Version 2 Normal physiological changes during pregnancy Renal/Urinary Clinical finding Mechanism t Glomerular filtration rate & f Cardiac output & renal blood renal size, l blood urea nitrogen & serum creatinine flow due to progesterone, Urinary frequency, nocturia t Urine output & sodium excretion with t renal excretion Hormones reset threshold to Mild hyponatremia Dilutional anemia t ADH releasefrom pituitary f Plasmavolume & red blood cell mass le System Hormone-mediated l in total protein S Cardiovascular t Cardiac output & heart rate Pulmonary Chronic respiratory alkalosis with metabolic compensation, t Pao, & l Paco, antigen & activity; t in fibrinogen & coagulation factors irc Prothrombotic state t Blood volume, l systemic vascular resistance Progesterone directly stimulates central rC Heme respiratory centers tot tidal volume & minute ventilation Increase in TV and Minute ventilation Decrese in RV and FRC Normal VC and FEV1 no change in Resp rate ne Gestational thrombocytopenia • Asymptomatic .. .. . • 2nd-3rd trimester of pregnancy Platelet count 70,000-150,000/mm 3 No history of thrombocytopenia In Diagnosis LE Pathophysiology Resolution after delivery Hemodilution • Accelerated destruction of platelets . • Serial complete blood counts Repeat evaluation postpartum to ensure resolution U SM Management No associated fetal thrombocytopenia • Fall in sodium concentration during pregnancy closely correlates with inc. production of hCG Fall below 130 should prompt evaluation for pathologic causes of hyponatremia ↑ fibrinogen → low/normal fibrinogen in pregnancy may be sign of DIC  When should folic acid supplementation ideally begin for pregnancy? 4 weeks prior to pregnancy Consequences of deficiency? Neural tube defects (anencephaly, meningomyelocele) (look for inc. AFP If patient with previous NTD pregnancy is on prenatal vitamins already, she still requires extra folic acid! Obs 393 https://t.me/usmleinnercircle Version 2 Weight gain in pregnancy Prepregnancy BMI (kg/m 2) <18.5 Ideal weight gain Complications 12.7-18.1 kg (28-40 lb) Inadequate weight gain . 18.5-24.9 11.3-15.9 kg (25-35 lb) • Low birth weight 25-29.9 6.8-11.3 kg (15-25 lb) Excessive weight gain 2:30 Definition Complications 5-9 kg (11-20 lb) . .. .. Preterm delivery • Gestational diabetes mellitus • Fetal macrosomia • Cesarean delivery Short interpregnancy interval <6-18 months from delivery to next pregnancy Maternal anemia PPROM Preterm delivery Low birth weight PPROM = preterm prelabor rupture of membranes. Physiologic Hydronephrosis of Pregnancy Due to  Increase blood volume → Increased filtration  High Progesterone → Ureteral Dilatation  Uterine Compression → Dilatation of Proximal Ureters and Bilateral Hydronephrosis  No management needed • Renal pelvis and ureter dilation during pregnancy occurs more commonly on the right side due to cushioning provided by the sigmoid colon on the left Exercise  What exercise regimen is recommended for healthy women with uncomplicated pregnancies? 20  30 minutes of moderate-intensity exercise on most or all days of the week contact sports and activities with high fall risk should be avoided; women with risk for preterm delivery, preeclampsia, or severe cardiopulmonary disease should also avoid exercise Obs 394 https://t.me/usmleinnercircle Version 2 Pregnancy& exercise • Amniotic fluid leak • Cervical incompetence • Multiple gestation Absolute contraindications • Placenta abruption or previa • Premature labor • Preeclampsia/gestational hypertension • Severe heart or lung disease Unsafe • High fall risk (eg, downhill skiing, gymnastics, horseback riding) activities • Hot yoga rC Benefits . .. .. 30 minutes of moderately intense exercise most days of the week l Gestational diabetes mellitus risk l Preeclampsia risk l Cesarean delivery risk Shorter postpartum recovery ne Goal Exercise during pregnancy irc • Scuba diving le • Contact sports (eg, basketball, ice hockey, soccer) • Weight management . • Walking/running .. .. .. Cycling In • Yoga Swimming Light-weight strength training Scuba diving LE Recommended Contact sports Exercise with falling risk Skydiving U SM Not recommended Prior cesarean delivery is NOT a contraindication to exercise. Vaccines Obs 395 https://t.me/usmleinnercircle Version 2 Vaccines during pregnancy • Tdap@ 27 - 36 weeks • Inactivated influenza in all trimesters Recommended • Rho(D) immunoglobulin@ 28- 32 weeks and postpartm • Hepatitis B • Hepatitis A Indicated for high-risk patients ABVP HM • Pneumococcus • Haemophi/us influenzae • Meningococcus • Varicella-zoster immunoglobulin • HPV • MMR Contraindicated • Live attenuated influenza • Varicella • Tdap: Given 26 weeks (in every pregnancy) If there is wound injury, give Tdap regardless of gestational age on the basis of standard wound management guidelines (e.g if not received in last 10 years)  What is the recommended management for a pregnant woman that is rubella-nonimmune? Immediate postpartum vaccination (e.g. MMR contraindicated during pregnancy but safe during breastfeeding The inactivated influenza vaccine is safe during every trimester of pregnancy and while breastfeeding. RhD & ABO incompatibility Indications for prophylactic administration of anti-D immune globulin for Rh(D)-negative patients· Hydrops fetalis • At 28-32 weeks gestation • <72 hours after delivery of Rh(D)-positive infant • <72 hours after spontaneous abortion • Ectopic pregnancy • Threatened abortion • Hydatidiform mole • Chorionic villus sampling, amniocentesis • Abdominal trauma • 2nd- & 3rd-trimester bleeding • External cephalic version *Antepartum prophylaxis is not indicated if the father is Rh(D) negative.  What screening test is used to determine if an Rh(D)- pregnant woman has already alloimmunized? Obs 396 https://t.me/usmleinnercircle Version 2 Antibody screen (indirect Coombs test) detects the presence of any RBC antibodies; Rh(D)- women with a negative antibody screen should still receive anti-D immune globulin at 28  32 weeks gestation Rh alloimmunization irc le Pregnancy 1 -r ne antib~ rC Matemal anli-Rh(D) Hemolytic disease of the fetus/newborn RBCs = red bloodcells. LE In IgM at first exposure, cant cross placenta. IgG seroconversion by the time of second pregnancy Rh Antibody Screening --------------- c____,......._____J ------------.. __ U SM Rh negative / ----'-- ,/'----' Sensitized Antibody titer _::c._ __ _ Rh positive '--.. '-,. ~-=------, I Unsensitized I No further screening i Further Monitoring Repeat at 28 weeks and give Rhogam as indicated  If unsensitized → re-screen at 28 weeks and delivery and give RhoGAM as indicated Administration of RhD immune globulin is ineffective in a patient who has already developed RhD antibodies Obs 397 https://t.me/usmleinnercircle Version 2 In patients with elevated titer levels 18, serial ultrasounds are performed to monitor for signs of anemia and hydrops fetalis. Fetal anemia can be treated with intrauterine fetal RBC transfusion in an attempt to prevent the development of hydrops fetalis.  What does "unsensitised" mean? Rh negative mother with no antibodies to Rh Next step? Give RhoGAM (anti-D Rh Ig) if: • Any maternal-fetal mixing (any gyn operation, delivery, abortion, C/S • Unsensitized at 28 weeks • Within 72 hours of Delivery if baby is Rh-positive  The Kleihauer-Betke test may be used to determine if a higher dose of anti-D immune globulin is needed after a procedure or delivery. the standard dose 300 mg) is enough to neutralize 30 mL of fetal blood; this test is typically used when there is an increased risk of fetal blood cells entering the maternal circulation (e.g. placental abruption, amniocentesis) ABO hemolytic disease Risk factors • Infants with blood types A or B born to a mother with blood type 0 • Jaundice within 24 hours of birth • Anemia Clinical features • i Reticulocyte count • Hyperbilirubinemia • Positive Coombs test Management • Serial bilirubin levels, oral hydration & phototherapy for most neonates • Exchange transfusion for severe anemia/hyperbilirubinemia  Antibodies against A and B are hemolytic. However, in addition to red blood cells, A and B antigens are present on the cells of all other fetal tissues. The reaction of the anti-A and anti-B antibodies with the antigens on these other cells neutralizes the antibody response, leading to a much milder form of hemolytic disease.  Non-invasive diagnosis of fetal anemia may be made by middle cerebral artery peak systolic velocity on Doppler ultrasound useful for fetuses at risk of anemia due to red cell alloimmunization Amniotic Fluids Obs 398 https://t.me/usmleinnercircle Version 2 • Polyhydramnios is characterized by an amniotic fluid index > 24 cm or a single deepest pocket  8 cm. • Oligohydramnios is characterized by an amniotic fluid index < 5 cm or a single deepest pocket  2 cm. Amniotic fluid index Oligohydramnios (AFI <5 cm) Complications • Preeclampsia • Esophageal/duodenal atresia • Abruptio placentae • Anencephaly le • Uteroplacental insufficiency • Multiple gestation • Renal anomalies • Congenital infection • NSAIDs • Diabetes mellitus • Fetal malpresentation • Meconium aspiration Umbilical cord prolapse • • Preterm delive0£ Preterm labor • Umbilical cord compression •• Preterm prelabor rupture of membranes rC AFI = amniotic fluid index; NSAIDs = nonsteroidalanti-inflammatorydrugs. irc Causes Polyhydramnios (AFI i!:24cm) Polyhydramnios ne Management is based on severity, maternal symptoms, and gestational age: • Patients with severe or symptomatic polyhydramnios at preterm gestation are at increased risk for obstetric complications, including preterm labor and preterm prelabor rupture of membranes. Therefore, these patients may benefit from amnioreduction (ie, amniotic fluid removal by In amniocentesis) • Patients with mild, asymptomatic polyhydramnios at term gestation, can undergo expectant Oligohydramnios LE management because obstetric outcomes are unchanged by increased antenatal fetal surveillance or intervention. U SM The etiology of oligohydramnios can vary with gestational age: • Early-gestation oligohydramnios is concerning for fetal etiologies (eg, aneuploidy, renal agenesis, posterior urethral valves) because amniotic fluid volume is dependent on normal fetal urine production. • Second- and third-trimester causes of oligohydramnios are typically due to uteroplacental insufficiency (with concomitant fetal growth restriction) or maternal causes, such as dehydration or rupture of membranes (with normal fetal growth). Fetus Gestational Age Obs 399 https://t.me/usmleinnercircle Version 2 Measuring fundal height Ultrasound assessment of gestational age Ultrasound parameter Gestational age (weeks) Accuracy (days) Gestational sac diameter 4.5-6 +/. 5-7 Crown-rump length Bi parietal diameter, head circumference, femur length AP. ... ~"----r-rXiphoid process 7-10 +/. 3 11-14 +/. 5 14-20 +/. 7 21-30 +/-14 >30 +/. 21-28 Umbilicus 'l'"f"~~r:i,;;~212:'j~---1,.. Symphysis pubis OlMlorld  What is the most accurate way to determine estimated gestational age? Ultrasound crown-rump measurement in the first trimester last menstrual period may be used to estimate gestational age if the patient has normal menses and a reliable LMP After 20 weeks gestation, fundal height in centimeters should directly correlate to gestational age in weeks with a small variation (eg, 23 cm) Fetal Surveillance Obs 400 https://t.me/usmleinnercircle Version 2 Antepartum fetal surveillance Description Test Normal result Abnormal result • Nonreactive: Nonstress test <2 accelerations • Reactive: External fetal heart rate monitoring for 20-40 2:2accelerations minutes • Recurrent variable or late decelerations • 2 points • O points • Nonstress test plus ultrasound assessment of the following: Amniotic fluid volume o Fetal breathing movement • Equivocal: 6 points ° Fetal movement o le Biophysical profile o • Abnormal: 0, 2, or 4 points 8 or 10 points Fetal tone • Oligohydramnios (maximum 10/10) • 0 points per category if abnormal test External fetal heart rate monitoring during No late or spontaneous or induced (eg, oxytocin, nipple recurrent variable stimulation) uterine contractions decelerations Evaluation of umbilical artery flow in fetal intrauterine growth restriction only Late decelerations with >50% of contractions High-velocity diastolic Decreased, absent, or flow in umbilical artery reversed end-diastolic flow ne Doppler sonography of the umbilical artery rC Contraction stress irc • 2 points per category if normal Biophysical profile* Component Normal finding Reactive fetal heart rate monitoring In 1. Nonstress test Single fluid pocket ~2 x 1 cm or amniotic fluid index >5 3. Fetal movements ;,,3general body movements 4. Fetal tone ;,,1 episodes of flexion/extension of fetal limbs or spine 5. Fetal breathing movements ;,,1 breathing episode for ;,,30 seconds U SM LE 2. Amniotic fluid volume Maximumscore= 10; 0 = abnormal;2 = normalfor each component •Performedcontinuous observationfor minutes  Management? • 04 fetal hypoxia ⟶ urgent delivery • 6: repeat in 24 hours • 810 normal and rules out fetal hypoxia  What is the recommended management for a pregnant woman at 32 weeks gestation with gestational hypertension and a biophysical profile score of 6 at today's visit? Repeat biophysical profile in 24 hours Obs 401 https://t.me/usmleinnercircle Version 2 pregnant women with gestational hypertension need weekly BPPs starting at 32 weeks gestation; a BPP of 6/10 is equivocal and should be repeated in 24 hours  What is the next step in management for a pregnant woman at 34 weeks gestation that presents with decreased fetal movement? Non-stress testing reveals a normal FHR but is non-reactive despite vibroacoustic stimulation. Biophysical profile or Contraction stress test choice of test depends on resources available and contraindications (e.g. contraction stress test is contraindicated if there are contraindications to labor, such as placenta previa or prior myomectomy) Normal fetal movements: 10 movements in 2 hours  What is the next step in management for a pregnant woman at 32 weeks gestation that presents with decreased fetal movement? Heart tones are heard by Doppler. Non-stress test i.e. recording the fetal heart rate while monitoring for spontaneous perceived fetal movements  What is the most common cause of a non-reactive non-stress test? Fetal sleep cycle vibroacoustic stimulation may be used to awaken the fetus; sleep cycles can last as long as 40 minutes therefore a non-reactive test should be extended to 40  120 minutes  What is a normal (reactive) nonstress test? Nonstress test • Baseline of 110-160/min Reactive • Moderate variability (6-25/min) • 2:2 accelerations in 20 minutes, each peaking 2:15/min above baseline & lasting 2:15 seconds Nonreactive • Does not meet criteria for reactivity Due to fetal sympathetic nervous system activity, which develops around 26  28 weeks (no reactivity before 28 weeks) NST → Non-reactive → Vibroacoustic Test/BPP → Still Non reactive/ equivoval or low score → Contraction Stress Test  Can a reactive non-stress test effectively rule out fetal acidemia? Obs 402 https://t.me/usmleinnercircle Version 2 Yes a reactive NST has a high negative predictive value for fetal acidemia Non-reactive NST has a low positive predictive value for fetal acidemia (high rate of false positives) • Late- and post-term pregnancies require antenatal fetal surveillance beginning at 41 weeks with a biophysical profile to screen for fetal hypoxia. late- and post-term pregnancies are at risk for uteroplacental insufficiency (e.g. oligohydramnios, late decelerations) irc le aging placentas may have decreased fetal perfusion, which causes decreased renal perfusion and urinary output Late-termand postterm pregnancycomplications Fetal Maternal • Cesarean delivery • Meconium aspiration • Infection • Stillbirth • Postpartum hemorrhage • Macrosomia • Perinea! trauma ne • Convulsions rC • Oligohydramnios U SM LE In Fetal Heart Rate Obs 403 https://t.me/usmleinnercircle Version 2 lntrapartum fetal heart rate monitoring Relationship to contraction • Symmetric to contraction • Nadir of deceleration corresponds to peak of contraction • Gradual (,:30 sec from onset to nadir) Early Etiology • Fetal head compression • Can be normal fetal tracing Relationship to contraction • Delayed compared to contraction Late Umbilical cord prolapse Onset : I~! Recovery + sec i l: l ... J Nadir .. !j Recovery Onset I~ J +sec; • Gradual (,:30 sec from onset to nadir) :: t j Nadir • Uteroplacental insufficiency : Contraction j • Nadir of deceleration occurs after peak of contraction Etiology i . : Con~ction .:: j : <30 : Recovery sec!/ • Abrupt (<30 sec from onset to nadir) Variable • Decrease ,:15/min; duration ,:15 sec but <2 min ~a~ir Etiology • Cord compression ,.F-F---- Ruptured membranes j _, Relationship to contraction • Can be but not necessarily associated with contractions cord Contra'ctioh • Oligohydramnios Occurs when the umbilical cord delivers through the cervix ahead of the presenting fetal part. Risk factors:  Malpresentation  Polyhydramnios • Cord prolapse  Cord Compression: Variable deceleration & return to baseline Dec in amniotic fluid after rupture of membranes cause cord compression and decompression) • Cord Prolapse: Abrupt, prolonged deceleration with no return to baseline  Which type(s) of decelerations may be indicative of fetal hypoxia and/or acidosis? late and recurrent variable decelerations Obs 404 https://t.me/usmleinnercircle Version 2 Fetal heart rate variability Clinical significance Absent: undetectable amplitude Abnormal or intermediate pattern Etiology: CNS depressants (narcotics, alcohol, recreational drugs) Minimal: ~5 bpm Temporary fetal sleep Fetal hypoxia irc Moderate: 6-25 bpm le Prematurity Normal pattern rC Marked: >25 bpm ne Unclear significance CNS = centralnervoussystem. • Cord compression/prolapse • Treatment: • Late decelerations (onset occurs after peaks of uterine contractions) Treatment: Intrauterine resuscitation (O2, reposition mom) → if failure → amnioinfusion or C-section Recurrent 50% contractions): Intrauterine resuscitation (O2, reposition mom) U SM 0 Intermittent: 50% contrations → no treatment LE 0 In •Variable decelerations → delivery if fails Cord Prolapse - Urgent C Section  What is the first-line intervention for a woman in first stage of labor with recurrent variable decelerations and moderate variability on fetal heart tracing? Maternal repositioning (e.g. left lateral decubitus, all fours) amnioinfusion (instillation of saline in intrauterine cavity) is a possible second-line intervention; assisted vaginal delivery could be indicated if the patient was fully dilated Obs 405 https://t.me/usmleinnercircle Version 2 Amnioinfusion is contraindicated in a patient with a history of uterine surgery. • Fetal scalp stimulation is performed to evaluate fetal acidosis in patients who have no accelerations on FHR monitoring Not performed in patients with decelerations Baseline fetal heart rate • Maternal fever (eg, intraamniotic infection) Fetal tachycardia (>160/min) Fetal bradycardia (<110/min) • Medication side effect (eg, beta agonists) • Fetal hyperthyroidism • Fetal tachyarrhythmia • Maternal hypothermia • Medication side effect (eg, beta blockers) • Fetal hypothyroidism • Fetal heart block (eg, anti-Ro/SSA, anti-La/SSB) Fetal heart rate tracing patterns Category I Requires all the following criteria: • Baseline 110-160/min • Moderate variability (6-25/min) • No late/variable decelerations • ± Early decelerations • ± Accelerations Category II • Not category I or Ill (indeterminate pattern) ~1 of the following characteristics: Category Ill • Absent variability + recurrent late decelerations • Absent variability + recurrent variable decelerations • Absent variability + bradycardia • Sinusoidal pattern Cesarean Section for category 3 Sinusoidal fetal heart rate Contractions u...o.....,Hg tJAO,.,,.,1-1g Fetal anemia typically presents with a sinusoidal fetal heart tracing Prenatal Testing & Screening Obs 406 https://t.me/usmleinnercircle Version 2 Routine prenatal laboratory tests • Rh(D) type & antibody screen • Hemoglobin/hematocrit, MCV, ferritin • HIV, VDRURPR, HBsAg, anti-HGV Ab • Rubella & varicella immunity Initial prenatal visit • Urine culture • Urine dipstick for protein • Chlamydia PCR (if risk factors are present) • Pap test (if screening indicated) • Hemoglobin/hematocrit 24-28 weeks • Antibody screen if Rh(D)-negative 36-38 weeks le • 1-hr 50-g GCT • Group B Streptococcus rectovaginal culture polymerasechain reaction;RPR = rapid plasmareagin. rC 100g 3-hour OGT to confirm ! irc anti-HCV Ab = hepatitisC antibody;GCT = glucosechallengetest; HBsAg = hepatitisB surfaceantigen;MCV = mean corpuscularvolume; PCR = An indirect antiglobulin Coombs) test can be used to determine whether an Rh-negative ne patient has developed anti-Rh antibodies and is an important component of early prenatal care, especially in patients with previous pregnancies. LE In Prenatal visits are recommended every 4 weeks until 28 weeks of gestation, every 2 weeks from 28 to 36 weeks of gestation and then every week until delivery. High-risk sexually transmitted infection screening in pregnancy U SM High-risk patients Required screening • Age <25 • Prior sexually transmitted infection • High-risk sexual activity (eg, multiple partners, commercial sex work) • Performed at initial prenatal visit & 3rd trimester: 0 HIV 0 Syphilis 0 Hepatitis B & C viruses 0 Gonorrhea 0 Chlamydia A negative Chlamydia trachomatis nucleic acid amplification test at the initial prenatal visit followed by a positive test in the third trimester most likely indicates a newly acquired infection during pregnancy.  Patients treated for a sexually transmitted infection during pregnancy require retesting a month after completion of treatment to ensure response to therapy (ie, test of cure). Patients with a sexually Obs 407 https://t.me/usmleinnercircle Version 2 transmitted infection diagnosed earlier during pregnancy are also retested in the third trimester, prior to delivery. Prenatal testing Test First-trimester combined test* Cell-free fetal DNA Chorionic villus sampling Second-trimester quadruple screen** Amniocentesis Second-trimester ultrasound Timing Advantages (weeks) Disadvantages 9-13 Early screening Not diagnostic ?.10 High sensitivity & specificity for aneuploidy Not diagnostic 10-13 Definitive karyotypic diagnosis Invasive; risk of spontaneous abortion 15-22 15-20 18-20 Screens for neural tube defects & aneuploidy Definitive karyotypic diagnosis Not diagnostic Invasive; risk of membrane rupture, fetal injury & pregnancy loss Measures fetal growth, evaluates fetal Cannot identify all abnormalities; some anatomy, confirms placenta position findings are of uncertain significance *Pregnancy-associated plasma protein, Jl-hCG, nuchal translucency. ••Maternal serum a-fetoprotein, estriol, Jl-hCG, inhibin A.  When is cell-free DNA testing (cffDNA indicated? Test used in 10 weeks gestation onwards to identify chromosomal aberrations (high specificity and sensitivity for aneuplodies); not diagnostic (must confirm with CVS or amniocentesis) Cell-free fetal DNA testing Indications Applications • • • • • Maternal age ~35 Abnormal maternal serum screening test Sonographic findings associated with fetal aneuploidy Previous pregnancy with fetal aneuploidy Parental-balanced Robertsonian translocation • Screening for trisomy 21, 18, 13 & sex-chromosome aneuploidies Fetal sex determination . 1st trimester screening Trisomy Jl-hCG PAPP-A 21 I j 18 j j 13 j j 2nd trimester screening Trisomy Jl-hCG lnhibin A Estriol 21 I I .!- ±.. 18 J.- -or__!. I_ j... 13 -- - - - - - AFP - Cell free DNA is also used to screen for heritable disorders. In white patients these include Cystic Fibrosis, Tay Sachs, Spinal Muscular Atrophy. Obs 408 https://t.me/usmleinnercircle Version 2  What is the next step in management for a pregnant woman at 12 weeks gestation with an abnormally elevated beta-hCG level and increased nuchal thickness on ultrasound? Chorionic villous sampling CVS this patient has a positive first-trimester combined test; diagnosis is made via CVS or amniocentesis depending on gestational age Congenital Infections Toxoplasmosis • Diffuse intracerebral calcifications • Severe chorioretinitis Syphilis • Rhinorrhea • Abnormal long-bone radiographs • Desquamating or bullous rash Rubella • Cataracts • Heart defects (eg, PDA) irc • Periventricular calcifications rC Cytomegalovirus ne All • Intrauterine growth restriction • Hepatosplenomegaly • Jaundice • Blueberry muffin spots le Clinical findings of congenital infections CMV periventricular calcifications VZV limb hypoplasia, cataracts and skin scars In Chorioretinitis • Zika: Intracranial calcifications  Microcephaly  Toxoplasma: Intracranial calcifications  Microcephaly/ U SM LE Macrocephaly (hydrocephalous) Congenital CMV infection is the most common cause of nonhereditary sensorineural hearing loss in children. The infection is frequently asymptomatic in infants but can cause progressive or delayed-onset, unilateral or bilateral hearing loss later in childhood. Group B Strep Obs 409 https://t.me/usmleinnercircle Version 2 Preventing neonatal group B Streptococcus infection • Rectovaginal culture at 36-38 weeks gestation Antenatal screening • GBS bacteriuria or GBS urinary tract infection in current pregnancy (regardless of treatment) • GBS-positive rectovaginal culture in current pregnancy • Unknown GBS status PLUS any of the following: Indications for intrapartum prophylaxis o <37 weeks gestation 0 lntrapartum fever 0 Rupture of membranes for ;?18 hours • Prior infant with early-onset neonatal GBS infection • Intravenous penicillin lntrapartum prophylaxis GBS = group B Streptococcus. Intrapartum = During Labour !! Screen early but give penicillin only during labour ! Pt has h/o treated GBS infection but vaginal delivery at home now came to hospital observe for 36 hrs Intrapartum antibiotic prophylaxis IAP is not necessary for planned cesarean delivery without labor or rupture of membranes because GBS transmission is low in such settings.  In patients with mild Penicillin allergy(e.g just rash) → Cephalosporin Pathogenesis . • . . Neonatal group B Streptococcus infection Most common cause of neonatal sepsis, pneumonia & meningitis Early onset (age <1 week) 0 0 • Prevention . Usually exposure to colonized contacts Sepsis, pneumonia, meningitis: 0 Clinical findings Usually exposure to bacteria in maternal genital tract Late onset (age >1 week) Temperature instability (eg, fever, hypothermia) 0 Irritability, lethargy, poor feeding 0 Tachycardia, hypotension 0 Respiratory distress (eg, tachypnea, grunting) Focal infection (late onset): 0 Septic arthritis 0 Cellulitis lntrapartum antibiotic prophylaxis for mothers with positive screening GBS culture at 36 weeks GBS = group B Streptococcus. Syphilis  Which TORCH infections are routinely screened for in pregnant women during their first prenatal visit? Rubella and Syphilis other routinely screened infections include HIV, Hepatitis B, and Chlamydia trachomatis Obs 410 https://t.me/usmleinnercircle Version 2 Syphilis in pregnancy • Universal at first prenatal visit Screening • Third trimester & delivery (if high risk) • Nontreponemal (RPR, VDRL) Serologic tests • Treponemal (FTA-ABS) Treatment • Intramuscular benzathine penicillin G • Intrauterine fetal demise Pregnancy effects • Preterm labor • Hematologic {hemolytic anemia, ! platelets) Fetal effects • Musculoskeletal ( long bone abnormalities) irc • Failure to thrive le • Hepatic {hepatomegaly, jaundice) FTA-ABS = fluorescent treponemal antibody absorption; RPR = rapid plasma reagin. Desensitize if penicillin allergic .. . . . .. . Early* features Snuffles: copious clear, purulent, or serosanguineous rhinorrhea Maculopapular rash 0 Palms, soles, buttocks, legs usually involved 0 Desquamation & hyperpigmentation ne Clinical Long bone abnormalities (eg, metaphyseal lucencies) Late Prevention .. Serologic testing: VDRL, RPR In Treatment Saddle nose, notched (Hutchinson) teeth, saber shins, sensorineural hearing loss Darkfield microscopy: Treponema pallidum in infectious material (eg, nasal discharge, skin lesions, placenta) Penicillin First-trimester maternal screening; repeat if high risk (eg, other prenatal STI) LE Diagnosis rC Congenital syphilis Prenatal penicillin *Nonspecificfindings:jaundice, hepatosplenomegaly,growth restriction,"blueberrymuffin" spots. U SM RPR = rapid plasma reagin; STI = sexuallytransmittedinfection. Obs 411 https://t.me/usmleinnercircle Version 2 Hutchinson teeth Saber shins Rubella  What is the treatment for a mother with rubella who is at 21 weeks gestation? Reassurance Infection from 111 weeks is associated wtih 90% risk After 20 weeks there is very low risk • Diagnosis • Newborn and pregnant women PCR for rubella RNA (throat swab, CSF) Serology (lgM antibodies, lgG antibodies in abnormally high or persistent concentrations) Viral culture (nasopharynx, blood) • Fetus lgM antibody serology (chorionic villi, amniotic fluid) PCRfor rubella RNA (chorionic villi, amniotic fluid) • Treatment • Intrauterine rubella infection < 16 weeks: counsel to terminate pregnancy > 16weeks: reassurance • Congenital rubella syndrome: supportive care (based on individual disease manifestations) and surveillance (including monitoring for late-term complications) • Prevention • Immunization of seronegative women before pregnancy • Nationally notifiable condition: Suspected congenital rubella syndrome should be reported to the local or state health department. JliMTriad congenital rubella syndrome: CCC = Cataracts, Cochlear defect, \ Obs ( Cardiac abnormality 412 https://t.me/usmleinnercircle Version 2 Triad is NOT always present. HSV Primary maternal infection Longer duration of rupture of membranes Vaginal delivery with active lesions Impaired skin barrier (eg, fetal scalp electrode) Preterm birth irc • • • • • le Risk factors for neonatal herpes simplex virus infection Pregnancy management in patients with HSV Routine prenatal care ne Antiviral suppression beginning at 36 weeks rC Prior HSV infection j In Lesion/prodromal symptoms during labor LE Vaginal delivery Cesarean delivery HSV = herpessunptexVirus. CUVVo~d U SM Hepatitis Epidemiology Risk factors Transmission Perinatal hepatitis B virus infection .. • 90% risk of vertical transmission without prophylaxis <2% risk after prophylaxis Chronic infection in 90% of perinatally infected infants • High maternal viral load • Maternal HBeAg+ • Perinatal exposure to genital secretions (most common) . • Transplacental (rare) Not transmitted by breastfeeding • HBV vaccine (active immunization) Prevention AND • HBIG (passive immunization) HBIG = hepatitis B immune globulin; HBV = hepatitis B vaccine. Continue routine HBV Vaccine i.e 0, 2, 6 months and then do serology after 3 months to check for infectivity. Obs 413 https://t.me/usmleinnercircle Version 2 Hepatitis C in pregnancy Potential complications • Gestational diabetes • Cholestasis of pregnancy • Preterm delivery • Ribavirin is teratogenic & should be avoided Maternal management • No indication for barrier protection in serodiscordant, monogamous couples • Hepatitis A & B vaccination • Vertical transmission strongly associated with maternal viral load Prevention of vertical transmission • Cesarean delivery not protective • Scalp electrodes should be avoided • Breastfeeding should be encouraged unless maternal blood present (eg, nipple injury)  What is the risk of a neonate developing chronic hepatitis B if mother has active Hep B infection? 90% Treatment of newborn? HB vaccine  HBIG These should be administered within 12 hours of birth, regardless of the infant's birth weight or clinical condition. HIV HIV management during pregnancy • HIV RNA viral load at initial visit, every 2-4 weeks after initiation or change of therapy, monthly until undetectable, then every 3 months Antepartum • CD4 cell count every 3-6 months • Resistance testing if not previously performed • ART initiation as early as possible • Avoid amniocentesis unless viral load :51,000 copies/ml • Avoid artificial ROM, fetal scalp electrode, operative vaginal delivery lntrapartum • Viral load :51,000copies/ml: ART + vaginal delivery • Viral load >1,000 copies/ml: ART + zidovudine + cesarean delivery • Mother: Continue ART Postpartum • Infant (maternal viral load :51,000copies/ml): Zidovudine • Infant (maternal viral load >1,000 copies/ml): Multi-drug ART ART= antiretroviral therapy; ROM = rupture of membranes.  What is the recommended treatment for an infant born to a mother with an HIV viral load  1000 copies/mL? Multi-drug ART for  6 weeks  What is the recommended antepartum treatment for a pregnant woman with HIV? Combined anti-retroviral therapy (cART Obs 414 https://t.me/usmleinnercircle Version 2 e.g. two NRTIs and an NNRTI or protease inhibitor How can you reduce risk of transmission of HIV in pregnancy? • cART (combined antiretroviral therapy) is recommended throughout pregnancy • Cesarean delivery if viral load  1,000 • HIV post-exposure ppx (neonatal zidovudine) • Avoid breastfeeding le • Risk of transmission w/ treatment = 2% HIV in infancy • High maternal viral load (eg, insufficient prenatal care, lack of antiretroviral therapy) • Breastfeeding by infected mother • Failure to thrive Clinical features • Chronic diarrhea rC Risk factors irc • Viral load 1,000  cART -- may consider vaginal delivery  HIV post-exposure PPX • Lymphadenopathy, hepatosplenomegaly • Pneumocystis pneumonia • Prolonged/refractory candidiasis • HIV DNA or RNA PCR Treatment • Combination antiretroviral therapy ne Diagnosis In Selective loss of CD4 T lymphocytes, however absolute lymphocyte count is normal (versus SCID); diagnosis is confirmed with PCR reaction testing LE Antibody testing is not performed at age 18 months as maternal antibodies may cause false positives U SM  Infants have high baseline levels of CD4 cells due to amplified thymic activity in the months after birth. Therefore, they frequently develop immunocompromised state from HIV at relatively high CD4 counts (eg, 1,0001,200/mm3) D/D SCID Decreased absolute lymphocyte count and absence of lympoid tissue (vs Lympadenopathy in HIV Chlamydia Obs 415 https://t.me/usmleinnercircle Version 2 Chlamydia in pregnancy Screening Risk factors Obstetric complications Fetal complications Treatment .. .. .. .. . .. . Universal screening at first prenatal visit High risk: repeat screening in 3rd trimester Age <25 History of sexually transmitted infection Recent new partner History of multiple partners Preterm prelabor rupture of membranes Preterm labor Postpartum endometritis Neonatal conjunctivitis Neonatal pneumonia Azithromycin IUGR Fetal growth restriction Definition • Weight <10th percentile for gestational age • Maternal hypertension • Pregestational diabetes mellitus Risk factors • Genetic abnormalities • Congenital infection • Large anterior fontanel • Thin umbilical cord Appearance • Loose, peeling skin • Minimal subcutaneous fat • Placenta histopathology Evaluation • Consider karyotype, urine toxicology, serology • Polycythemia • Hypoglycemia Neonatal complications • Hypocalcemia • Poor thermoregulation Fetal growth restriction Symmetric Definition Onset Etiology Clinical features Asymmetric • Ultrasound estimated fetal weight <10th percentile for gestational age • 1st trimester • 2nd/3rd trimester • Chromosomal abnormalities • Utero-placental insufficiency • Congenital infection • Maternal malnutrition • Global growth lag • "Head-sparing" growth lag • Weekly biophysical profiles Management • Serial umbilical artery Doppler sonography • Serial growth ultrasounds Obs 416 https://t.me/usmleinnercircle Version 2 • Monitor/treat complications (eg, hypoglycemia, hypothermia, polycythemia) Management • Hypoglycemia: frequent screening and frequent feedings • Hypothermia: skin to skin with mother, examinations in incubator • Polycythemia and hypocalcemia: screen if symptoms develop (eg, poor feeding, vomiting, jitteriness)  FGR can be a complication of maternal vascular disease (eg, type 1 diabetes mellitus, chronic hypertension), which causes chronic placental vasoconstriction and ischemia with resultant uteroplacental insufficiency Patients with FGR have a high risk for intrauterine fetal demise and require an immediate umbilical le artery Doppler ultrasound to assess placental perfusion rC irc Bicornuate uterus with two separate endometrial cavities has less room for appropriate egg implantation, placental development, and fetal growth. Patients with this abnormality are also at increased risk for preterm delivery, fetal growth restriction, and malpresentation. Fetal constitutional smallness is associated with short maternal stature, low prepregnancy weight, and fetal female sex. Constitutional smallness presents with an ultrasound-estimated fetal weight 10% for gestational age, normal umbilical artery Doppler evaluation, and ne appropriate interval growth. In Constitutionally small infants have normal long-term outcomes. U SM LE Twins Obs 417 https://t.me/usmleinnercircle Version 2 Twin pregnancy • Monochorionic, monoamniotic o 1 placenta, 1 amniotic sac • Monochorionic, diamniotic Types o 1 placenta, 2 amniotic sacs o "T-sign" at intertwin membrane • Dichorionic, diamniotic o 2 placentas, 2 amniotic sacs o "Lambda sign" at intertwin membrane • Hyperemesis gravidarum Maternal complications • Preeclampsia • Gestational diabetes mellitus • Iron-deficiency anemia • Congenital anomalies • Fetal growth restriction • Preterm delivery Fetal complications (r'IC.) • Malpresentation (eg, breech) • Monochorionic twins o Twin-twin transfusion syndrome • Monoamniotic twins o Conjoined twins o Cord entanglement  The risk of preterm labor during a multiple gestation pregnancy may be reduced with adequate early weight gain T sign for twins Lambda sign for twins Monochorionic diamniotic Dichorionic diamniotic Two placentas Single placenta Obs 418 https://t.me/usmleinnercircle irc le Version 2 rC • Monochorionic monoamniotic twins are typically managed inpatient beginning at 28 weeks gestation with frequent fetal monitoring (eg, nonstress test) and antenatal corticosteroid administration. ne Patients are delivered preterm (3234 weeks gestation) via cesarean delivery. Demise Definition In Intrauterine fetal demise • Absent fetal cardiac activity at ~20 weeks gestation .. . • Aneuploidy LE Fetal or placental anomalies Risk factors Fetal growth restriction Congenital infection • Substance use (eg, tobacco, cocaine) • Maternal conditions (eg, hypertension, diabetes U SM mellitus) Fetal • Autopsy • Gross & microscopic examination of placenta & umbilical cord Evaluation • Karyotype/genetic studies Maternal When an autopsy is legally mandated* Suspicion of crime Suspicion of suicide Unexplained death in an otherwise healthy person Infectious disease or other public health concern Inmate death Suspicion of medical malpractice *Laws vary by state. • Kleihauer-Betke test • Antiphospholipid antibodies • Coagulation studies• Kleihauer-Betke: A maternal blood smear is exposed to acid, and stained afterwards. Adult hemoglobin is removed by the acid, whereas fetal hemoglobin HbF is not, resulting in pink color cells that indicate fetal hemoglobin on a positive test. Obs 419 https://t.me/usmleinnercircle Version 2 Women with unexplained late term still birth , should undergo weekly non-stress testing in the third trimester starting at 32 weeks gestation Even after thorough evaluation, up to half of IUFDs have no identifiable cause. Therefore, patients should be counselled in advance that testing may be negative. Intrauterinefetal demise Delivery planning for a nonviable fetus • Anencephaly Definition Fetal death at ;,20 weeks Diagnosis Absence of fetal cardiac activity on ultrasound Fetal diagnosis 20-23weeks • Dilation & evacuation Management • Holoprosencephaly • Acardia • Thanatophoric dwarfism OR • Intrauterine fetal demise • Vaginal delivery• ;,24 weeks • Vaginal delivery• Complication • Bilateral renal agenesis Obstetric management Coagulopathy after several weeks of fetal retention "Cesarean delivery by maternal choice if history of prior classical cesareanfmyomectomy Neonatal management • Vaginal delivery • No fetal monitoring • Palliative care if not stillborn Induce vaginal delivery when mother is ready Development of DIC requires urgent delivery of the fetus, via vaginal or cesarean delivery.  What is the recommended management for a pregnant woman at 28 weeks gestation that presents in labor for a fetus diagnosed with anhydramnios and anencephaly? The fetus is in breech presentation. Allow spontaneous vaginal delivery the priority for patients with lethal fetal anomalies (e.g. anecephaly) is to minimize maternal morbidity/mortality; C-section has an increased risk of maternal complications A breech presentation in lethal anomalies does not require a C-section (vaginal delivery preferred due to increased risk of maternal complications associated with C-section)  What is the next step in management for a pregnant woman at 25 weeks gestation that presents after feeling no fetal movement for the past two days? Fetal heart sounds are not heard on Doppler. Transabdominal ultrasound the patient likely has intrauterine fetal demise (fetal death at  20 weeks), which must be confirmed by the absence of fetal cardiac activity on ultrasound  Because parents may blame themselves for the baby's death, it is important for the physician to respond with a clear statement that it is not their fault and there is nothing they could have done to Obs 420 https://t.me/usmleinnercircle Version 2 prevent the loss. The physician should also avoid prematurely changing the focus of discussion to medical management and reassurance about future pregnancies. Referring to the fetus as their "baby" and avoiding medical terminology (eg, fetal demise) are helpful. Hydrops Fetalis Etiology le irc t cardiac output demand causing heart failure t fluid movement into interstitial spaces (third spacing) Pericardia! effusion Pleural effusion Ascites Skin edema Placental edema rC Clinical features .. .. .. .. . . Polyhydramnios Immune 0 Rh(D) alloimmunization Non immune 0 Parvovirus B19 infection 0 Fetal aneuploidy 0 Cardiovascular abnormalities 0 Thalassemia (eg, hemoglobin Barts) In High output heart failure ne Pathogenesis Hydrops fetalis Fetal hydrops U SM LE Hypertension Obs 421 https://t.me/usmleinnercircle Version 2 Hypertensive disorders of pregnancy Chronic hypertension Gestational hypertension • Systolic pressure ~140 mm Hg &/or diastolic pressure ~90 mm Hg prior to conception or 20 weeks gestation • New-onset elevated blood pressure at ~20 weeks gestation • No proteinuria or end-organ damage • New-onset elevated blood pressure at ~20 weeks gestation Preeclampsia AND • Proteinuria OR signs of end-organ damage • Preeclampsia Eclampsia AND • New-onset grand mal seizures Chronic hypertension with superimposed preeclampsia Chronic hypertension AND 1 of the following: • New-onset proteinuria or worsening of existing proteinuria at ~20 weeks gestation • Sudden worsening of hypertension • Signs of end-organ damage Elevated blood pressure must be seen on 2 separate measurements taken at least 4 hours apart Obstetric complications of hypertension Antihypertensive medications in pregnancy • Superimposed preeclampsia First-line (safe) Second-line Contraindicated • Cesarean delivery Maternal • Abruptio placentae • Postpartum hemorrhage • Maternal mortality • Fetal growth restriction ± oligohydramnios Fetal • Methyldopa • Thiazide diuretics • ACE inhibitors • Beta blockers (labetalol) • Clonidine • Angiotensin receptor blockers • Hydralazine • Aldosterone blockers • Calcium channel blockers (nifedipine) • Furosemide • Direct renin inhibitors • Preterm delivery • Intrauterine fetal demise • Perinatal mortality Preeclampsia Obs 422 https://t.me/usmleinnercircle Version 2 Preeclampsia • Nulliparity Preexisting medical condition (eg, SLE, chronic hypertension) Multiple gestation Advanced maternal age • New-onset hypertension• (SBP 2'140 or DBP 2'90 mm Hg) at 2'20 weeks Definition AND • Proteinuria OR signs/symptoms of other end-organ damage • Severe-range hypertension (SBP 2'160 or DBP 2'110 mm Hg) • Platelets <100,000/mm 3 Severe features • Creatinine >1.1 mg/dl or 2x normal . . . Elevated transaminases >2x upper limit of normal Pulmonary edema Vision or cerebral symptoms (eg, headache) • <37 weeks & no severe features: expectant le AST/ALT > 2x ULN BP > 160/ 110 Cr> 1.1 Disturbance in vision orheadache Epigastric or RUQ pain Pulmonary edema Platelets< 1lakh Obesity irc Mnemonic for SEVERE features ABCDE PP . . . . Risk factors • ;,,37 weeks (or ;,,34 weeks with severe features): delivery • Severe-range blood pressure: IV labetalol, IV hydralazine, PO nifedipine rC Management • Seizure prophylaxis: magnesium sulfate *On 2 measurements ~4 hr apart. ne DBP = diastolicblood pressure;IV= intravenous;PO= by mouth; SBP = systolic blood pressure; SLE = systemiclupus erythematosus. Preeclampsia prevention New-onset hypertension & proteinuria &/or In . . Definition end-organ damage at >20 weeks gestation Prior preeclampsia LE • Chronic kidney disease U SM High risk Moderate risk Prevention • Chronic hypertension . Diabetes mellitus • Multiple gestation • Autoimmune disease . . Obesity • Advanced maternal age Nulliparity • Low-dose aspirin at 12 weeks gestation  High-risk patients also require a 24-hour urine collection for total protein at the initial prenatal visit to establish a baseline (prepregnancy) proteinuria assessment.  What level of proteinuria is required to make a diagnosis of preeclampsia?  300 mg/day (or a protein/creatinine ratio  0.3  Patients with preeclampsia are at increased risk of hemorrhagic and ischemic stroke due to acute elevations in cerebral perfusion pressure and intracerebral vessel rupture (hemorrhagic), as well as Obs 423 https://t.me/usmleinnercircle Version 2 preeclampsia-mediated vascular endothelial damage and microthrombi formation (ischemic). NOT Subarachnoid Haemorrhage Preeclampsia can present upto 6 weeks after delivery.  What is the likely diagnosis in a pregnant woman with preeclampsia that develops suddenonset dyspnea, hypoxia, and crackles? Pulmonary edema rare but life-threatening complication of severe preeclampsia Preeclamptic patients have generalized arterial vasospasm leading to increased systemic vascular resistance and high cardiac afterload -- the heart becomes hyperdynamic to try to overcome the systemic hypertension. Pathophysiology of pulmonary edema In preeclampsia/eclampsia Treatment of preeclampsia Drug Indication Hydralazine IV, labetalol IV, or nifedipine PO Lower blood pressure acutely to decrease stroke risk Magnesium sulfate IV or IM Prevent or treat eclamptic seizures Generalized arterial vasospasm(systemic hypertension) t Afterload against which the heart is pumping t Pulmonary capillary pressure I Pulmonary edema I+-j Renal function t Vascular permeability C)USMlEWorld.l1C j  In patients with Myasthenia Gravis and Preeclampsia, magnesium sulfate is contraindicated because it may trigger a myasthenic crisis. In these patients, seizure prophylaxis is with valproic acid.  What are the first-line agents 3 for maternal hypertensive crisis? nifedipine, hydralazine, or labetalol Be careful with labetalol -- if patient has bradycardia(<60 bpm) or asthma, do not give! Nifedipine is oral, thus patient may not be able to tolerate if they have emesis! Eclampsia Obs 424 https://t.me/usmleinnercircle Version 2 Eclampsia (preeclampsia + new-onset seizure) • Hypertension, typically severe (ie, SBP ~160 or DBP ~110 mm Hg) • Seizure, typically tonic-clonic with postictal phase Clinical features • Severe headache • Visual disturbances (ie, scotoma) • Hyperreflexia • Mainly clinical • Bilateral frontal lobe edema on CT scan of the head • Magnesium sulfate infusion Diagnosis Management • Antihypertensive agent for severe hypertension DBP = diastolic blood pressure;SBP = systolicblood pressure. rC HELLP irc • Delivery le • Proteinuria HELLP syndrome • Preeclampsia • Nausea/vomiting • Right upper quadrant abdominal pain Laboratory findings • Microangiopathic hemolytic anemia • Elevated liver enzymes • Low platelet count In ne Clinical features • Delivery • Magnesium for seizure prophylaxis • Antihypertensive drugs LE Treatment  Delivery should occur at  34 weeks gestation or with deteriorating maternal/fetal status U SM Gestational Diabetes Obs 425 https://t.me/usmleinnercircle Version 2 Gestational diabetes mellitus • Human placental lactogen secretion • 24-28 weeks gestation • 1-hr 50-g GCT • 3-hr 100-g GTT • 1st line: diet • 2nd line: insulin, glyburide, metformin • Fasting: S95 mg/dl Pathophysiology Screening Management Target blood glucose goals . 1-hr postprandial: S140 mg/dl • • Fasting glucose at 24-72 hr • 2-hr 75-g GTT at 6- to 12-week visit 2-hr postprandial: s120 mg/dl Postpartum management GCT = glucose challenge test; GTT = glucose tolerance test. 2-step approach for screening & diagnosing gestational diabetes mellitus 24-28 weeks gestation Step1 • Administer 50-g oral glucose load • Check serum glucose 1 hr later Blood glucose <140 mg/dl Gestational diabetes mellitus unlikely No further testing Blood glucose 2:140mg/dl Step2 • Check fasting serum glucose • Administer 100-g oral glucose load • Check serum glucose each hour afterward for 3 hr Diagnosis of gestational diabetes mellitus (2:2abnormal values) Blood glucose level Carpenter & Coustan NDDG Fasting ;,95 mg/dL (5.3 mmol/L) ;,105 mg/dL (5.8 mmol/L) 1 hr ,e180mg/dL (10 mmol/L) ;,190 mg/dL (10.6 mmol/L) 2 hr ;,155 mg/dL (8.6 mmol/L) ,e165mg/dl (9.2 mmol/L) 3 hr ;,140 mg/dL (7.8 mmol/L) ;,145 mg/dL (8 mmol/L) NDDG = National Diabetes Data Group criteria. ©UWorld Patients with risk factors (e.g. obesity, previous GDM) should be screened earlier and re-screened at 2428 weeks if negative Obs 426 https://t.me/usmleinnercircle Version 2 Prenatal diabetes screening High-risk• patient I No J Nonnal Third-trimester screen Normal Yes ! Abnonnal l First-trimester screen I Abnonnal NoGDM GDM I l 1- to 3-year glucose screen First-trimester screen Abnonnal l irc I Nonnal J le Postpartum screen Type 2 diabetes mellitus ] "Obesllyplus 2:1of the following: priorGDM, priormacrosom,c 1nfanl, familyhistory(firstdegree). paycysticovarysyn<rome,age oi?:40 G0M • gestat!Onal diabetes melhtus rC CUWorld ne All patients at risk for type 2 DM (eg, obesity, affected first-degree relative) require diabetic screening at the initial prenatal visit (eg, hemoglobin A1c, glucose challenge test). In Infant of mother with diabetes mellitus: complications Maternal hyperglycemia LE First trimester Fetal hyperglycemia & hyperinsulinemia U SM Congenital heart disease Neural tube defects Small left colon syndrome Spontaneous abortion Second & third trimesters f Metabolic demand Macrosomia 1 Glycogen Fetal hypoxemia j synthesis Shoulder dystocia Glycogen deposition in interventricular septum ! ! f Erythropoiesis Polycythemia Organomegaly Neonatal hypoglycemia l Brachial plexopathy Clavicle fracture Perinatal asphyxia l l Hypertrophic cardiomyopathy OUWorld The fetus cannot produce insulin in the 1st trimester, thus hyperglycemia can disrupt organogenesis Obs 427 https://t.me/usmleinnercircle Version 2 Neonatal complications of diabetes during pregnancy • Maternal hyperglycemia ---+fetal hyperglycemia ---+P-cell hyperplasia & Pathogenesis Associated hyperinsulinemia • t Fetal fat & glycogen stores • t Fetal metabolic demand • Prematurity • Congenital anomalies (eg, caudal regression syndrome) • Macrosomia & associated complications (eg, brachia! plexus injury, clavicle fracture) risks • Respiratory distress syndrome • Hypertrophic cardiomyopathy • Hypoglycemia • Polycythemia, low iron • Hypocalcemia & hypomagnesemia • Hyperbilirubinemia Laboratory findings Hypertrophic cardiomyopathy in infants of diabetic mothers Pathogenesis .. t .. . .. . . Maternal hyperglycemia ---+fetal hyperglycemia & hyperinsulinemia Glycogen & fat deposition in interventricular septum ---+dynamic LVOT obstruction Often asymptomatic Clinical findings May have respiratory distress and/or hypotension Systolic ejection murmur Imaging Treatment Prognosis Chest x-ray: cardiomegaly Echocardiogram: t thickness of interventricular septum, ! LV chamber size Intravenous fluids & beta blockers to increase LV blood volume Spontaneous regression by age 1 LVOT = left ventricularoutflow tract.  The pathophysiology of hypocalcemia in infants of diabetic mothera relates to maternal hypomagnesemia, which is caused by osmotic diuresis in the setting of poorly controlled gestational diabetes. Low fetal magnesium concentrations, which reflect maternal levels, lead to PTH suppression and low calcium levels in the neonate. Symptomatic hypocalcemia can present with jitteriness  What maternal pathology is associated with increased risk for neonatal respiratory distress syndrome? Maternal diabetes maternal diabetes results in fetal hyperinsulinism, which antagonizes cortisol and blocks maturation of sphingomyelin. other risk factors include Prematurity, male sex, perinatal asphyxia and C-section Obs 428 https://t.me/usmleinnercircle Version 2 Pregnancy Absolute contraindications to pregnancy • Pulmonary arterial hypertension • Peripartum cardiomyopathy with residual LV dysfunction VSD———> Eisenmenger syndrome • Heart failure with LVEF <30% Conditions in which pregnancy is contraindicated • Severe coarctation • Severe mitral stenosis • Severe symptomatic aortic stenosis le • Severe aortic dilation (eg, Marfan syndrome) irc • Recommend against pregnancy at preconception counseling visit • If pregnant, discuss abortion; if abortion declined, regular cardiology follow-up Management LV = left ventricle; LVEF = left ventricular ejection fraction. Preterm Labour Cervical cone biopsy Preterm labor Prior spontaneous preterm delivery Multiple gestation Short cervical length Cervical surgery (eg, cold knife conization) Cigarette use ne Screening & prevention • • • • • • Cervical length measurement by TVUS • Progesterone administration • Cerclage placement In Risk factors rC Pregnancy contraindicated UNLESS the condition is fixed first. U SM LE Strongest: preterm delivery in a prior pregnancy Risk factor for Cervical insufficiency A negative fetal fibronectin test prior to term is an indicator of low risk for preterm delivery. high negative predictive value; fetal fibronectin levels are high until 20 weeks gestation and thus are not a reliable marker of preterm delivery in early pregnancy Obs 429 https://t.me/usmleinnercircle Version 2 Preterm birth prevention Prior preterm delivery? i·----No----~----Yes---~i Prior preterm delivery associated with painful contractions? 2nd-trimester TVUS for cervical length r-No_J_ Yes 7 Normal cervical length Incidental short cervix (S2.5 cm at <24 weeks) Cervical insufficiency Prior preterm labor• Routinecare • Vaginalprogesterone Prophylactic cerdage Progesterone (IM or vaginal) •Pretenn labor= regular contractions causing cervical change at <37 weeks gestation with intact membranes. IM = intramuscular; TVUS = transvaginal ultrasound. CUWorld  What is the first step in evaluating risk of preterm delivery in a patient with a history of cervical surgery (e.g. cold knife conization)? Transvaginal ultrasound measurement of cervical length in the 2nd trimester  What is the recommended management for a pregnant patient with no history of preterm labor and a short cervix  2 cm) on TVUS? Vaginal progesterone progesterone maintains uterine quiescence and protects against premature rupture of membranes Cerclage Cervical insufficiency Risk factors Normal cervix Cerclage • Collagen defects • Uterine abnormalitiesSeptate uterus • Cervical conization • Obstetric injury 1 ~2 prior painless, 2nd-trimester losses Clinical features • 2• Painless cervical dilation or Management Incompetent cervix • Cerclage placement Long, closed cervix Short, dilated cervix Amniotic sac bulging at os Sutures visible cervical dilation and no uterine contractions or 3. USGbased: asymptomatic cervical shortening* at <2.5 cms @ <24 weeks gestation In patients who have bulging or prolapsing amniotic membranes, a rescue cerclage is not typically recommended due to the high risk of membrane rupture, complications (eg, intraamniotic infection, cervical trauma), and likely imminent delivery. Obs 430 https://t.me/usmleinnercircle Version 2 Preterm labor management Preterm labor· Maternal instability, intrauterine infection, fetal distress/demise - Yes - Immediate delivery j I No 34-37 weeks le 32-34weeks (moderate preterm) • Antenatal corticosteroids • Penicillin if GBS+ or unknown • Tocolysis: nifedipine (late preterm) • ± Antenatal corticosteroids • Penicillin if GBS+ or unknown irc <32 weeks (very preterm) • Antenatal corticosteroids • Penicillin if GBS+ or unknown • Tocolysis: indomethacin • Magnesium sulfate 'Preterm labor= regular contractions causing cervical change at <37 weeks gestation with intact memb<anes. GBS = group B Streptococcus. rC Magnesium sulfate given 32 weeks for neuroprotection if birth is indicated. (Decreases risk for Cerebral Palsy) ne Tocolytics: Indomethacin (preferred if given with magnesium), nifedipine, Terbutaline. • Indomethacin C/I after 32 weeks due to risk of premature Fetal Ductus Arteriosus Closure. In  It can also cause Decreased renal perfusion and fetal oliguria can result in oligohydramnios. Hence not given for more than 48 hours. LE  30 year old female that has gone into preterm labor 32 weeks). The patient was given a drug to attempt to slow down the labor WHILE she is given appropriate pharmacotherapy to promote fetal lung development. An hour to 2 hours later the heart rate of the woman increases significantly. Mechanism? U SM Nifedipine causing arterial dilation ⟶ reflex tachycardia Labour Labour has three stages: • The first stage is when the neck of the womb (cervix) opens to 10cm dilated. • The second stage is when the baby moves down through the vagina and is born. • The third stage is when the placenta (afterbirth) is delivered. Stages of Normal Labor • Stage 1 Latent: 06 cm; 20 hr (nulli), 14 hr (multi) • Stage 1 Active: 610cm • Stage II: 10cm → delivery; 3 hr (n), 2 hr (m) Obs 431 https://t.me/usmleinnercircle Version 2 • Stage III: Fetus → placental expulsion;  30 min False labor versus latent labor Contractions False labor Latent labor Timing Irregular, infrequent Regular, increasing frequency Strength Weak Increasing intensity Pain None to mild Yes Cervical change No Yes •False labor: characterized by mild, irregular contractions (Braxton Hicks contractions) that cause no cervical change  What is the recommended management for patients that present in false labor? Expectant management Disorders of the active phase of labor Diagnosis Clinical features Treatment Protraction • Cervical change slower than expected • ± Inadequate contractions Oxytocin • No cervical change for M hours with adequate contractions Arrest OR • Cesarean delivery No cervical change for ~6 hours with inadequate contractions • The active stage of labor typically begins at  6 cm dilation ( 1 cm dilation every 2 hour) Protraction: 1 cm dilation in 2 hours Adequate contractions are defined as generation of > 200 Montevideo units MVUs) in 10 minutes. measured via an intrauterine pressure catheter Obs 432 https://t.me/usmleinnercircle Version 2 Second stage arrest of labor Insufficient fetal descent after pushing for: • ~3 hours if nulliparous Definition • ~2 hours if multiparous • Maternal obesity • Excessive pregnancy weight gain Risk factors • Diabetes mellitus • Cephalopelvic disproportion • Malposition Etiology le • Inadequate contractions • Maternal exhaustion • Cesarean delivery irc • Operative vaginal delivery Management In order to Operative Vaginal delivery to occur, fetal head must be fully engaged i.e at 0 rC station ne Neuraxial anesthesia (eg, epidural) can lengthen the second stage of labor NOT first. Left occiput transverse position In  What is the most common cause of second stage arrest of labor? Fetal malposition (e.g. occiput transverse) LE the optimal fetal position is Occiput anterior Occiput posterior position U SM Occiput anterior position CUWMd Obs 433 https://t.me/usmleinnercircle Version 2 Occiput anterior positions Sagittal suture Pubic symphysis Left occiput anterior Occiput anterior L Right occiput anterior Rupture of membranes when there is no palpable presenting fetal body part or there is fetal malpresentation carries a high risk for umbilical cord prolapse, which can lead to cord compression and fetal hypoxia.  What is the recommended management for a patient at 28 weeks gestation with the fetus in transverse lie position? The patient desires a vaginal delivery. Expectant management most fetuses in transverse lie spontaneously convert to vertex presentation prior to term; persistent malpresentation may require external cephalic version or C-section Transverse lie C>UW<Xld Uterine surgical history & vaginal birth Obs Surgery Trial of labor contraindicated? Low transverse cesarean delivery (horizontal incision) No Classical cesarean delivery (vertical incision) Yes Abdominal myomectomy with uterine cavity entry Yes Abdominal myomectomy without uterine cavity entry No 434 https://t.me/usmleinnercircle Version 2 • Digital cervical examination is used to determine cervical dilation and fetal presentation (ie, the fetal part [eg, head, buttocks] directly overlying the pelvic inlet) If fetal presentation (eg, cephalic, breech) is uncertain on digital cervical examination, a transabdominal ultrasound should be performed to confirm fetal presentation and determine the safest route of delivery. le Amniotomy / Iatrogenic Rupture of Membranes are NO longer done. The most commonly injured nerves in the lithotomy position (with feet in stirrups) are the rC irc femoral nerve and the peroneal nerve. The femoral nerve can be compressed under the inguinal ligament when the hip is flexed greater than 90 degrees, which can lead to numbness of the anterior and medial aspects of the thigh, as well as weakness of the quadriceps muscle. Breech ne Peroneal nerve injuries typically are caused by compression at the fibular head against the stirrup, leading to foot drop and numbness over the dorsal foot and lateral lower extremity Breech presentation In . Breech U SM LE types Obs Risk factors Management Frank: hips flexed & knees extended (buttock presenting) • Complete: hips & knees flexed • Incomplete: 1 or both hips not flexed (feet presenting) • Advanced maternal age (~35) • Multiparity .. • • .. . Uterine didelphys, septate uterus Uterine leiomyomas Fetal anomalies (eg, anencephaly) Preterm (<37 weeks gestation) Oligohydramnios/polyhydramnios Placenta previa • External cephalic version Cesarean delivery 435 https://t.me/usmleinnercircle Version 2 Breech Presentation Types Frank Incomplete Complete Single Footling Double Footling • Frank, Complete: Can try Vaginal  Incomplete, Footling: Vaginal Contraindicated  What is the recommended management for a pregnant woman at 35 weeks gestation that presents in preterm labor with the fetus in breech presentation on ultrasound? Cesarean delivery betamethasone +/- penicillin may be administered as well  If vertex presentation on ultrasound? Expectant management Active labor is a contraindication for external cephalic version  What is the management of Breech presentation/Transverse Lie: •  37 weeks = no intervention as most fetuses spontaneously convert to cephalic • External cephalic version = 37 weeks, no active labor, no fetal distress → C-section if fails External Cephalic Version External cephalic version Procedure Indications Absolute 0 Prior classical cesarean delivery contraindications 0 Prior extensive uterine myomectomy 0 Placenta previa Complications Obs Contraindications to external cephalic version • Manual rotation of fetus to cephalic presentation • Decreases cesarean delivery rate • Breech/transverse presentation • 2'37 weeks gestation • Contraindication to vaginal delivery • Indications for cesarean delivery regardless of fetal lie (eg, failure to progress during labor, non-reassuring fetal status) • Placental abnormalities (eg, placenta previa or abruption) • Oligohydramnios • Ruptured membranes • Hyperextended fetal head • Fetal or uterine anomaly • Multiple gestation • Abruptio placentae • Intrauterine fetal demise Active Labour 436 https://t.me/usmleinnercircle Version 2 External cephalic version Forward roll le  What is the next step in management for a healthy pregnant woman at 37 weeks gestation that desires a vaginal delivery? Ultrasound reveals the fetus is in a frank breech presentation. irc External cephalic version can be attempted in women with breech pregnancies at  37 weeks of gestational age if there's rC no contraindications to vaginal delivery and the fetus is in good health Internal Podalic Version LE In ne Breech extraction of the second twin Place hand in uterus, grab fetal foot Deliver baby, feet first CUWMI U SM Shoulder Dystocia Obs 437 https://t.me/usmleinnercircle Version 2 Shoulder dystocia Definition Risk factors Warning signs Shoulder dystocia • Failure of usual obstetric maneuvers to deliver fetal shoulders 1. McRoberts maneuver Legs flexed onto abdomen causes rotation of pelvis, alignment of sacrum & opening of birth canal 2. Suprapublc pressure Applied to fetal anterior shoulder • Fetal macrosomia • Maternal obesity • Excessive pregnancy weight gain • Gestational diabetes • Postterm pregnancy >42 Weeks • Protracted labor • Retraction of fetal head into the perineum after delivery (turtle sign) Fractured clavicle Fractured humerus .. . .. . .. Erb-Duchenne palsy . . Klumpke palsy .. Complications of shoulder dystocia Clavicular crepitus/bony irregularity t Moro reflex due to pain on affected side Intact biceps & grasp reflexes Upper arm crepitus/bony irregularity t Moro reflex due to pain on affected side Intact biceps & grasp reflexes t Moro & biceps reflexes on affected side Waiter's tip 0 Extended elbow 0 Pronated forearm 0 Flexed wrist & fingers Intact grasp reflex Clawhand 0 Extended wrist 0 Hyperextended metacarpophalangeal joints 0 Flexed interphalangeal joints o Absent grasp reflex Horner syndrome (ptosis, miosis) Intact Moro & bice12sreflexes • Variable presentation depending on duration of hypoxia Perinatal asphyxia • Altered mental status (eg, irritability, lethargy), respiratory or feeding difficulties, poor tone, seizure  In Erb & Klumpke Palsy, Management involves observation alone because most infants recover arm function spontaneously within a few months. Obs 438 https://t.me/usmleinnercircle Version 2 Management of shoulder dystocia (BE CALM) B Breathe; do not push E Elevate legs & flex hips, thighs against abdomen (McRoberts) C Call for help A Apply suprapubic pressure L Enlarge vaginal opening with episiotomy Maneuvers: • Deliver posterior arm • Rotate posterior shoulder (Woods screw) - apply pressure to anterior aspect of the posterior shoulder • Adduct posterior fetal shoulder (Rubin) - apply pressure to the posterior aspect of the posterior shoulder le M • Mother on hands & knees - "all fours" (Gaskin) Nuchal cord reduction irc • Replace fetal head into pelvis for cesarean delivery (Zavanelli) loop of umbilical cord wrapped around neck U SM Post Term LE In ne I rC Reducing the nuchal cord Definition Risk factors .. .. .. . . Late-term: :2:41weeks gestation Post-term: :2:42weeks gestation Prior post-term pregnancy Nulliparity Obesity Age :2:35 Fetal anomalies (eg, anencephaly) Fetal/neonatal 0 Complications Management Obs Late- & post-term pregnancy . .. Macrosomia 0 Dysmaturity syndrome 0 Oligohydramnios 0 Demise Maternal 0 Severe obstetric laceration 0 Cesarean delivery 0 Postpartum hemorrhage Frequent fetal monitoring (eg, nonstress test) Delivery prior to 43 weeks gestation 439 https://t.me/usmleinnercircle Version 2  If you find signs of UteroPlacental insufficiency or fetal distress, immediate delivery. Placental sulfatase deficiency is a risk factor for post-term delivery. other risk factors include fetal adrenal hypoplasia and anencephaly 3rd Trimester Bleeding Placenta Previa Placenta previa • Prior placenta previa Risk factors • Prior cesarean section or other uterine surgery • Multiparity • Advanced maternal age Clinical features Diagnosis/management • Painless 3rd-trimester bleeding • Transabdominal followed by transvaginal sonography • NO intercourse or digital vaginal examination T/t:  Stable with mild bleed: IV fluids & Observation  Unstable or acti: Immediate C/S Patients who present with vaginal bleeding after 20 weeks gestation should not undergo a Digital Cervical examination until USG has been performed to determine the exact location of placenta. Risk of causing massive haemorrhage if it is placenta previa)  What recommendations should you make for diagnosis of placenta previa? Pelvic rest, abstinence from intercourse Treatment? Schedule C/S at 36/37 weeks  The majority (~90%) of cases resolve spontaneously due to lower uterine segment lengthening and/or placental growth toward the fundus; therefore, initial management is with routine obstetric care. Vasa Previa Obs 440 https://t.me/usmleinnercircle Version 2 Vasa previa • • Placenta previa • Multiple gestations • In vitro fertilization • Succenturiate placental lobe Definition Fetal vessels overlying the cervix Risk factors Clinical presentation Management .. Painless vaginal bleeding with ROM or contractions FHR abnormalities (eg, bradycardia, sinusoidal pattern) • Fetal exsanguination & demise • Emergency cesarean delivery  What is most important risk factor for uterine rupture? irc Uterine Rupture le FHR = fetal heart rate; ROM = rupture of membranes. Presentation? rC Previous C-section or myomectomy (scar becomes weakest point of uterus) • Painful 3rd trimester bleeding, sudden abdominal pain during labour • Palpable fetal parts on abdominal exam • Fetal heart decels (bradycardia) ne • Loss of fetal station (presenting fetal part retracts) In Laboring patients at high risk of uterine rupture require urgent laparotomy and delivery U SM LE Uterine rupture Progressively decreasing contraction amplitude: Staircase Sign Placental Abruption Obs 441 https://t.me/usmleinnercircle Version 2 Abruptio placentae Definition • Placental detachment from the uterus before fetal delivery • Hypertension, preeclampsia . Risk factors Abdominal trauma • Prior abruptio placentae • Cocaine & tobacco use • Sudden-onset vaginal bleeding Clinical presentation • Abdominal or back pain • High-frequency, low-intensity contractions • Rigid, tender uterus Diagnosis Complications • Clinical • Ultrasound: ± Retroplacental hematoma • Fetal hypoxia, preterm birth, mortality • Maternal hemorrhage, disseminated intravascular coagulation Painful Bleeding which may be concealed • Regular, High frequency contractions (vs irregular and decreasing in Uterine Rupture) Occurs after 20 weeks gestation. Uterine tachysystole (eg, 5 contractions in a 10 minute period).  What is the initial management for a hemodynamically unstable pregnant patient that presents with abruptio placentae following a motor vehicle accident? IV fluid resuscitation and left lateral decubitus positioning left lateral decubitus position displaces the uterus off the aortocaval vessels and maximizes cardiac output PROM Obs 442 https://t.me/usmleinnercircle Version 2 Preterm prelabor rupture of membranes (PPROM) Definition Risk factors • Membrane rupture at <37 weeks prior to labor onset • PriorPPROM • Genitourinary infection (eg, ASB, BV) • Antepartum bleeding • Vaginal pooling or fluid from cervix Management irc Complications • Nitrazine-posilive (blue) fluid • Ferning on microscopy • <34 weeks (reassuring): latency antibiotics, corticosteroids • <34 weeks (nonreassuring): delivery • <!:34weeks: delivery • Preterm labor • lntraamniotic infection • Placental abruption • Umbilical cord prolapse le Diagnosis rC ASB = asymptomatic bacteriuria; BV = bacterial vaginosis; PPROM = preterm prelabor rupture of membranes.  Rupture of membranes before onset of uterine contractions AND before 37 weeks ne Management of preterm prelabor ROM Rupture of membranes In <34 weeks I Uncomplicated LE l • Expectant management • Latency antibiotics (eg, ampicillin & azithromycin) Corticosteroids • Fetal surveillance U SM Ampicillin covers group B Streptococcus, aerobic gram-negative bacilli, and anaerobes; Azithromycin covers Ureaplasma. Infection, fetal/maternal compromise l 34 to <37 weeks l Delivery GBS prophylaxis (eg, penicillin G) ± corticosteroids Delivery IAI treatment (eg, ampicillin & gentamicin) Corticosteroids Magnesium if <32 weeks GBS = group B streptococcal;IAI = intraamnioticinfection;ROM = rupll.-e of membranes. CUWorld Tocolysis is NOT indicated in PROM. It is only used to preterm labour if membranes have not yet ruptured.  What are the risk factors for premature and premature/preterm rupture of membranes? • Ascending infection GBS • Smoking • Multiple Pregnancies Obs 443 https://t.me/usmleinnercircle Version 2 • Previous PPROM Although membrane rupture typically presents as a sudden gush of fluid, patients can have a subclinical presentation with slow leakage occurring over days, with increased clear vaginal discharge.  What is the likely diagnosis in an obese, multiparous pregnant woman at 34 weeks gestation that presents with intermittent leakage of clear fluid and a negative nitrazine/fern test? Stress urinary incontinence A small pool of urine in the posterior vagina may be seen on speculum examination because urine can become trapped in the vagina (ie, retrograde vaginal voiding) during pregnancy differentiated from rupture of membranes by negative nitrazine/fern tests and absence of vaginal pooling Test • Nitrazine test Fluid from vaginal exam placed on strip of nitrazine paper Paper turns blue in presence of alkaline (pH> 7.1) amniotic fluid • Fern test Fluid from vaginal exam placed on slide and allowed to dry Amniotic fluid narrow fern vs. cervical mucus broad fern Chorioamnionitis lntraamniotic infection (chorioamnionitis) . • Prolon9ed rueture of membranes (>18 hours) Risk factors Preterm prelabor rupture of membranes • Prolonged labor • Internal fetal/uterine monitoring devices • Reeetitive va9inal examinations • Presence of 9enital tract eatho9ens Maternal fever PLUS 2:1 of the following: Diagnosis Management Complications • Fetal tachycardia (>160/min) • Maternal leukocytosis • Purulent amniotic fluid • Broad-spectrum antibiotics • Delivery • Maternal: postpartum hemorrhage, endometritis • Neonatal: preterm birth, pneumonia, encephalopathy Deliver regardless of age Obs 444 https://t.me/usmleinnercircle Version 2 A low amniotic fluid glucose is an indicator of intra-amniotic infection  Management? • IV ampicillin + gentamicin (+ Clindamycin for C/S • Immediate delivery via augmentation of labour • C/S generally not indicated unless of obstetrical complications (non-reassuring tracings, breech) Induction of labor irc le  What is the next step in management for a pregnant woman at 38 weeks gestation with chorioamnionitis after administration of antibiotics? Fetal heart tracing reveals tachycardia but is otherwise reassuring. cesarean delivery is reserved for standard obstetric indications (e.g. prior uterine surgeries, rC breech presentation, non-reassuring fetal heart tracing)  What is the likely diagnosis in an intrapartum patient that presents after 18 hours of membrane ne rupture and prolonged labor with fever and tachycardia? Fetal heart tracing demonstrates fetal tachycardia. Chorioamnionitis (intra-amniotic infection) LE Miscarriage/ Abortion In intra-amniotic infection caused by migration of vaginal or enteric flora through the cervix, typically in a patient with premature or prolonged rupture of membranes; intrapartum fever is a distinguishing feature • Spontaneous abortion = loss  20 weeks U SM • Stillbirth (intrauterine fetal demise) = loss after 20 weeks Obs 445 https://t.me/usmleinnercircle Version 2 Types of Miscarriages Missed No vaginal bleeding Threatened Vaginal bleeding Inevitable Vaginal bleeding Closed cervical os No fetal cardiac activity or empty sac Closed cervical os Fetal cardiac activity Dilated cervical os Products of conception may be seen or felt at or above cervical os Incomplete Vaginal bleeding Dilated cervical os Complete Vaginal bleeding Closed cervical os Some products of conception Products of conception expelled & some remain completely expelled • Missed Abortion Patients are typically asymptomatic or have loss of pregnancy symptoms (e.g. nausea, breast tenderness) with decreasing beta-hCG levels USG findings: No Cardiac activity, empty gestational sac without a fetal pole  Complete Abortion other findings include Unilateral Ovarian Cyst (Corpus luteum) and some fluid in the pelvis. BHCG can take 6 weeks to go undetectable Obs 446 https://t.me/usmleinnercircle Version 2 Spontaneous abortion Definition • Pregnancy loss <20 weeks • Advanced maternal age Risk factors • Previous spontaneous abortion • Substance use disorder options • Expectant • Medical induction (misoprostol) • Suction curettage if infection or hemodynamic instability Additional • Rho(D) immunoglobulin management • Pathology examination le Treatment • Hemorrhage irc • Retained products of conception Complications • Septic abortion • Uterine perforation rC • Intrauterine adhesions Spontaneous abortion is most often due to chromosomal trisomies, especially trisomy 16 ne Septic abortion Retained POC from: • Elective abortion with nonsterile technigue • Missed or incomplete abortion (rare) • Fever, chills, abdominal pain Sanguinopurulent vaginal discharge Clinical presentation • • Boggy, tender uterus; dilated cervix • Pelvic ultrasound: retained POC, thick endometrial stripe In Risk factors . • . LE Management Intravenous fluids Broad-spectrum antibiotics Suction curettage U SM POC = productsof conception.  What is the recommended treatment for patients with septic abortion? Broad-spectrum antibiotics and suction curettage medical emergency; urgent treatment required to reduce risk of sepsis Recurrent Pregnancy Loss Obs 447 https://t.me/usmleinnercircle Version 2 Causes of recurrent pregnancy loss Structural • Uterine: fibroids, adhesions, polyps • Cervical insufficiency • Aneuploidy Chromosomal Immunologic/ Hematologic • Translocations/rearrangements • Mosaicism • Hypercoagulable disorders (eg, antiphospholipid syndrome) • Alloimmune intolerance • Thyroid disease Endocrine • Polycystic ovary syndrome • Diabetes mellitus • Hyperprolactinemia • Advancing maternal age Other • Defective endometrial receptivity • Decreased ovarian reserve • Celiac disease Ectopic Pregnancy Ectopic pregnancy locations Ectopic pregnancy Tubal (mostcommon) • Previous ectopic pregnancy Risk factors • Previous pelvic/tubal surgery • Pelvic inflammatory disease • Abdominal pain, amenorrhea, vaginal bleeding Clinical features • Hypovolemic shock in ruptured ectopic pregnancy • Cervical motion, adnexal &/or abdominal tenderness • ± Palpable adnexal mass Diagnosis Management • Positive hCG • Transvaginal ultrasound revealing adnexal mass, empty uterus • Stable: Methotrexate • Unstable: Surgery  What is the likely diagnosis in a hemodynamically unstable patient with a gestational sac in the uterine cornu and free fluid in the posterior cul-de-sac on transvaginal ultrasound? Ruptured ectopic pregnancy i.e. a cornual or interstitial ectopic pregnancy; often presents as abdominal pain and vaginal bleeding Obs 448 https://t.me/usmleinnercircle Version 2 Management of suspected ectopic pregnancy Positive urine hCG, lower abdominal pain, &for vaginal bleeding Hemodynamically unstable Hemodynamically stable Immediate surgical consultation Intrauterine pregnancy) Nondiagnostic ) i i Treat ectopic pregnancy Serum B-hCG level ! <3,500 IU/L ] ~3,500 IU/L ] Repeat B-hCG level + TYUS in 2 days : transvaginal ultrasound . Repeat B-hCG level in 2 days CUWo<ld ne TVUS i rC i irc Adnexal mass ) le i 1 Mx: Methotrexate LE In Methotrexate is contraindicated in patients with hepatic disease or renal disease (eg, diabetic nephropathy) due to decreased clearance and slower drug metabolism increasing the risk for methotrexate toxicity. • Early but viable intrauterine pregnancies : 35%50% rise in β-hCG every 48 hours. • Completed spontaneous abortions : β-hCG levels decrease U SM • Ectopic and nonviable intrauterine pregnancies : 35% rise  To distinguish between an ectopic pregnancy and nonviable intrauterine pregnancy, patients may undergo diagnostic dilation and curettage, a procedure that samples tissue within the endometrial cavity. The procedure confirms the abnormal pregnancy's location based on the postprocedure βhCG level: • A negative or decreased β-hCG level confirms that the patient had a nonviable intrauterine pregnancy; these patients require reassurance and observation only because the products of conception have been removed by the dilation and curettage. • A persistent rise in β-hCG level after dilation and curettage is diagnostic for an ectopic pregnancy (ie, the uterus has been evacuated but an extrauterine pregnancy continues to produce β-hCG. These patients require additional management. Methotrexate)  A progesterone level  5 ng/mL suggests an abnormal or extrauterine pregnancy Obs 449 https://t.me/usmleinnercircle Version 2 >25 ng/mL suggests a healthy intrauterine pregnancy Hydatidiform Mole Hydatidiform mole • Abnormal vaginal bleeding ± hydropic tissue • Uterine enlargement> gestational age • Abnormally elevated J3-hCGlevels Clinical presentation • Theca lutein ovarian cysts • Hyperemesis gravidarum • Preeclampsia with severe features • Hyperthyroidism • Extremes of maternal age Risk factors • History of hydatidiform mole • "Snowstorm" appearance on ultrasound Diagnosis • Quantitative serum J3-hCG • Histologic evaluation of uterine contents • Dilation & suction curettage Management • Serial serum J3-hCGpost evacuation • Contraception for 6 months Management of hydatidiform mole Suction curettage l Weekly J3-hCGlevels until undetectable Decreasing l Monthly J3-hCG levels x 6 months 13-hCG undetectable Increasing/ plateauing Diagnosis of gestational trophoblastic neoplasia ~hCG becomes detectable l Surveillance complete Can attempt pregnancy Choriocarcinoma Choriocarcinoma Obs 450 https://t.me/usmleinnercircle Version 2 Gestational trophoblastic neoplasia Evaluation Treatment Hydatidiform mole Maternal age >40 Vaginal bleeding Pelvic pain/pressure i 13-hCGlevel Metastasis (eg, vagina, lungs) Pelvic ultrasound Chest x-ray Thyroid function tests Heealic function tests Renal function tests le Clinical features .. .. .. .. .. . .. Chemotherapy Hysterectomy irc Risk factors rC  What is the likely diagnosis in a 6-month postpartum woman that presents with irregular vaginal bleeding, an enlarged uterus, and dyspnea with multiple infiltrates on CXR? Choriocarcinoma ne classically occurs after a complete hydatidiform mole, but can occur after normal pregnancy or spontaneous abortion Vomiting In • Nausea and vomiting during pregnancy affects many women in the first trimester and is relatively mild with no associated weight loss, hypovolemia, or electrolyte abnormalities. LE  What is the pharmacological treatment (1st and 2nd line) of nausea and vomiting in pregnancy ("morning sickness"? • Avoid triggers, consume fluids 30 minutes before or after solid meals • 1st line: ginger, pyridoxine B6 U SM • 2nd line: add doxylamine  Some cases of nausea and vomiting during pregnancy can mask an underlying eating disorder, with potential concerning findings, including: • Weight loss with an already relatively low BMI • Unpredictable appetite (suggesting dysregulated eating habits) with skipping meals (suggesting possible caloric restriction) followed by episodes of overeating (suggesting possible binge eating) • Distorted view of body weight and shape, evidenced by the perception of "looking pregnant" despite early gestational age (when the uterus is still below the pubic symphysis) and switch to maternity clothing despite weight loss • Evaluate for Disordered Eating behaviour Hyperemesis Gravidarum Obs 451 https://t.me/usmleinnercircle Version 2 Hyperemesis gravidarum . • • History of hyperemesis gravidarum Clinical features Laboratory abnormalities Treatment Hydatidiform mole Multifetal gestation Risk factors . • .. . • . • .. Severe, persistent vomiting >5% loss of prepregnancy weight Dehydration Orthostatic hypotension Ketonuria Hypochloremic metabolic alkalosis Hypokalemia Hemoconcentration Admission to hospital Antiemetics & intravenous fluids  Patients with HG can develop prolonged hypoglycemia due to inadequate oral intake; this can lead to ketoacidosis and ketones on urinalysis. Ketonuria suggests more severe disease and is an indication for hospital admission With appropriate treatment, there are rarely any adverse fetal effects. Postpartum Postpartum period • Transient rigors/chills • Peripheral edema • Lochia rubra findings • Uterine contraction & involution • Breast engorgement • Rooming-in/lactation support • Serial examination for uterine atony/bleeding Routine • Perinea! care care • Voiding trial • Pain management Normal Obs 452 https://t.me/usmleinnercircle Version 2 Normal postpartum lochia Expected duration Lochia rubra Lochia serosa Lochia alba . . Birth to 3-4 days postpartum 4th postpartum day to 10th or 14th postpartum day • 11th postpartum day to 6 weeks postpartum . . . Description Dark or bright red (blood); odor similar to that of menstrual blood; occasional small clots; quantity decreasing each day Serosanguineous (pink); brownish (old blood); quantity gradually decreasing in amount White/yellow; creamy; light quantity lochia may increase in quantity after breastfeeding (suckling releases oxytocin & causes uterus to contract) & 7-14 days postpartum, when scabbing le on the placental site sloughs off (heavier bleeding for <2 hr); lochia may also feel increased after lying down & then standing (due to blood pooling in irc vagina).  Lochia Rubra : upto 1 Week; Bright Red blood with small clots 1 pads per hour) rC  Patients often have concerns about postpartum bleeding because it is heavier and more prolonged than normal menses. These patients should be evaluated for features concerning for delayed postpartum hemorrhage, including the following: • Passage of large blood clots ne • Increased pad counts (eg, saturation of 1 pad/hr for 2 consecutive hours) • Signs and symptoms of anemia (eg, dizziness, chest pain) due to acute blood loss In PPH Postpartum hemorrhage • >500 ml after vaginal delivery • >1,000 ml after cesarean delivery LE Definition • Prolonged or induced labor • Chorioamnionitis U SM Risk factors • Multiple gestation • Polyhydramnios • Grand multiparity • Operative delivery • Uterine atony (most common) • Retained placenta Causes • Genital tract laceration • Uterine rupture • Coagulopathy • Bimanual uterine massage, oxytocin • Intravenous fluids, oxygen Treatment • Uterotonics (methylergonovine, carboprost, misoprostol) • Intrauterine balloon tamponade • Uterine artery embolization • Hysterectomy Oxytocin is a first-line agent; other agents, such as methylergonovine and carboprost may be administered if oxytocin fails Obs 453 https://t.me/usmleinnercircle Version 2  What are the (4) etiologies of postpartum hemorrhage? • 'Absent' Uterine inversion • 'Soft and boggy' Uterine atony (most common) • 'Firm' Placental retention or incomplete seperation • Normal/Firm: Trauma/laceration/DIC Management of postpartum hemorrhage due to uterine atony Postpartum hemorrhage• & soft, boggy uterus Step 1: Uterine massage & high-dose oxy1ocin Step 2: Tranexamic acid Step 3: Second-line uterotonic agents· • Methylergonovine (Cl: hypertension) • Carboprost tromethamine (Cl: asthma) • Misoprostol i Step 4: Intrauterine balloon tamponade ) i Step 5: Laparotomy "Estimatedblood loss >1,000 ml Of bleeding+ hypovolemia. Cl = contraindication. CUWor1d  Uterine atony unresponsive to medical management may respond to a B-lynch compression suture during exploratory laparotomy. Differential diagnosis of postpartum hemorrhage Risk factors Diagnosis • Prolonged labor Uterine atony • • Examination • Enlarged, boggy uterus Chorioamnionitis conception • Bimanual uterine massage • Uterotonic Uterine overdistension (multiples, fetal medications macrosomia, polyhydramnios) Retained products of Management • Succenturiate placenta • Manual extraction of placenta • History of previous uterine surgery • Enlarged, boggy uterus • Placenta missing cotyledons • Manual extraction • Retained placental fragments on ultrasound Genital tract trauma Inherited coagulopathy Obs • Operative vaginal delivery • History of abnormal bleeding in patient or family members • Laceration of cervix or vagina • Enlarging hematoma • Continued bleeding despite contracted uterus • Laceration repair • Correction of coagulopathy 454 https://t.me/usmleinnercircle Version 2 Vaginal hematoma Risk factors Clinical • Operative vaginal delivery • Infant ~4000 g (8.8 lb) • Nulliparity • Prolonged 2nd stage of labor • Vaginal mass • Rectal or vaginal pressure • ± hypovolemic shock Treatment • Nonexpanding: observation • Expanding: embolization, surgery le features irc Because the vagina is supplied by branches of the uterine artery (which receives 30% of rC maternal cardiac output at delivery), patients with a vaginal laceration/hematoma can have profuse bleeding. ne Vulval Hematoma caused by damage to Pudendal Artery. Secondary (late) postpartum hemorrhage Retained POCs Placental site subinvolution Clinical features • Heavy bleeding • ± Uterine atony Management • Dilation & curettage In Cause • Heavy bleeding • Uterine atony • Uterotonics (eg, oxytocin, methylergonovine, carboprost) • Fever • Uterine tenderness • Broad-spectrum IV antibiotics (eg, clindamycin & gentamicin) • Purulent lochia LE Postpartum endometritis U SM POCs = productsof conception;IV = intravenous. Obs Secondary = 24 hours after Delivery 455 https://t.me/usmleinnercircle Version 2 Delayed placental delivery occurs when the placenta does not deliver within 30 minutes. A common risk factor is extreme preterm delivery (eg, 26 weeks gestation) because these deliveries typically require prolonged oxytocin administration and are often complicated by intraamniotic infection, stillbirth, and placental disorders (eg, preeclampsia, placenta accreta). In addition, the prolonged oxytocin administration can cause a paradoxical decrease in myometrial contractility, thereby inhibiting placental expulsion. Intrauterine inflammation (eg, from intrauterine fetal demise) can also increase placental adhesion to the uterine wall. Patients with a retained placenta usually have profuse postpartum bleeding, which may be immediate or delayed. Initial management includes gentle downward cord traction and oxytocin administration to promote placental separation and expulsion. If the placenta does not deliver with these measures, manual placental extraction or dilation and curettage are indicated.  What is the next step in management for a mother with post-partum hemorrhage following a forceps-assisted vaginal delivery? The patient is afebrile and the uterus is normal-sized and firm. Genital tract inspection genital tract injury is a common cause of PPH after operative vaginal deliveries Operative vaginal delivery (vacuum/forceps) . Bimanual uterine massage • Protracted 2nd stage of labor Indications Fetal heart rate abnormalities • Maternal contraindications to pushing • Laceration .. • Cephalohematoma Fetal complications Facial nerve palsy lntracranial hemorrhage • Shoulder dystocia • Genitourinary tract injury Maternal complications . • Urinary retention Hemorrhage • Post-cesarean delivery patients with hemorrhagic shock and no signs of uterine atony most likely have intraabdominal bleeding from uterine artery injury. a rare but life-threatening cause of postpartum hemorrhage that typically presents with no incisional bleeding and minimal abdominal or back pain Obs 456 https://t.me/usmleinnercircle Version 2 Hemodynamically unstable patients with a suspected retroperitoneal hematoma require emergency laparotomy. Uterine Inversion Uterine inversion Pathophysiology • Excessive fundal pressure • Excessive umbilical cord traction • Lower abdominal pain • Round mass protruding through cervix . • Uterine fundus not palpable transabdominally le Presentation Hemorrhage shock . • Manual replacement of the uterus irc • Aggressive fluid replacement Management Placental removal & uterotonic drugs after uterine replacement • Neurogenic shock, due to the traction effect on the surrounding peritoneum, may also occur, rC resulting in a paradoxical bradycardia. Placenta Accreta ne Placenta accreta Definition . Risk factors • Placenta previa + prior uterine surgery (eg, cesarean delivery, D&C, m~omectom~) Management . . . Prenatal diagnosis: US with placenta previa, numerous placental lacunae, myometrial thinning In Clinical features Morbidly adherent placental attachment to the myometrium Postpartum diagnosis: adherent placenta, postpartum hemorrhage Cesarean h~sterectom~ with placenta in situ LE D&C; dilation & curettage; US ; ultrasound.  What is the likely diagnosis of a "firm uterus" in the setting of a post-partum hemorrhage? Placenta accreta U SM Most important risk factor? Previous C-section Presents with delayed placental detachment and massive PPH at the time of attempted manual separation of the placenta Antenatally diagnosed placenta accreta is delivered by planned cesarean hysterectomy. Sheehan Syndrome Obs 457 https://t.me/usmleinnercircle Version 2 Sheehan syndrome Pathogenesis • Obstetric hemorrhage complicated by hypotension • Postpartum pituitary infarction • Lactation failure(! prolactin) Clinical features • Amenorrhea, hot flashes, vaginal atrophy (! FSH, LH) • Fatigue, bradycardia (! TSH) • Anorexia, weight loss, hypotension (! ACTH) • Decreased lean body mass(! growth hormone) • T/t: Replace the hormones individually. Cant do anything else Perineal Laceration Perinea! lacerations First-degree Second-degree -~--,..., Third-degree Fourth-degree Fistula Rectovaginal fistula • Pelvic radiation • Obstetric trauma • Pelvic surgery Risk factors • Colon cancer • Diverticulitis • Crohn disease Clinical features • Uncontrollable passage of gas &/or feces from the vagina • Physical examination Fistulography Diagnostic studies • • Magnetic resonance imaging • Endosonography Obs 458 https://t.me/usmleinnercircle Version 2 • Rectovaginal fistula may occur after obstetric trauma and presents with incontinence of flatus and feces through the vagina. "Red, velvety (rectal) mucosa" may be seen on posterior vaginal wall  What is the likely diagnosis in a patient that presents with foul-smelling brown discharge from the posterior vaginal wall two weeks after a vaginal delivery complicated by third-degree laceration? Rectovaginal fistula Bladder dye test le Vesicovaginal fistula • Pelvic surgery • Pelvic irradiation • Prolonged labor/childbirth trauma • Genitourinary malignancy • Painless, continuous urine leakage from the vagina • Physical examination Diagnostic studies \ Catheter-- • Dye test Tampon • Cystourethroscopy rC Clinical features irc Risk factors -Syringe Pelvic examination typically shows vaginal pooling of Vesicovaginal fistula Blue dye on tampon ne urine, a visible defect, or an area of raised, red granulation tissue on the anterior vaginal wall. (with dye) In Takes some days to form and no bladder distention (vs Bladder atony sec to epidural) LE Pubic Symphysis Diastasis U SM Risk factors Pubic symphysis diastasis • Fetal macrosomia • Multiparity • Precipitous labor • Operative vaginal delivery • Difficulty ambulating Presentation • Radiating suprapubic pain • Pubic symphysis tenderness • Intact neurologic examination • Conservative Management • Nonsteroidal anti-inflammatory drugs • Physical therapy • Pelvic support typically resolves within the first 4 weeks postpartum Septic Pelvic Thrombophlebitis Obs 459 https://t.me/usmleinnercircle Version 2 Septic pelvic thrombophlebitis • Cesarean delivery • Pelvic surgery Risk factors Pathophysiology Presentation Treatment . • • . • • . • . • • . . Endometritis Pelvic inflammatory disease Pregnancy Malignancy Hypercoagulability Pelvic venous dilation Vascular trauma Infection Fever unresponsive to antibiotics No localizing signs/symptoms Negative infectious evaluation Diagnosis of exclusion Anticoagulation • Broad-spectrum antibiotics Diagnosis of exclusion; due to an infected thrombosis of the deep pelvic or ovarian veins Postpartum Endometritis Postpartum endometritis • Cesarean delivery • lntraamniotic infection Risk factors • Group B Streptococcus colonization • Prolonged rupture of membranes • Operative vaginal delivery • Fever >24 hr postpartum Clinical • Uterine fundal tenderness features • Purulent lochia Etiology • Polymicrobial infection Treatment • Clindamycin & gentamicin Infection of Endometrium lining • Neither blood nor endometrial cultures are required for diagnosis, but further evaluation is indicated if there is no clinical improvement after 48 hours of antibiotic therapy Clindamycin covers: Gram +ve and Anaerobes Gentamycin (Aminoglycoside) covers Gram • Postpartum endometritis is 510x more common in patients with Cesarean delivery Because of the risk for puerperal sepsis following cesarean delivery, women typically receive a prophylactic dose of antibiotics at the time of surgery. Obs 460 https://t.me/usmleinnercircle Version 2 Chronic endometritis shows Plasma Cells on biopsy. Urinary Retention  What is the most likely diagnosis in a post-partum patient unable to void since delivery and is experiencing urine dribbling? Overflow incontinence secondary to bladder atony le Suspect if patient hasn't voided in 6 hours after delivery 2/2 epidural) • Primiparity • Regional anesthesia Risk factors • Operative vaginal delivery • Perinea! injury rC • Cesarean delivery irc Postpartum urinary retention • Inability to void or small-volume voids Clinical • Incomplete bladder emptying features • Dribbling of urine • Self-limited condition • Intermittent catheterization ne Management Confirmed by  150 mL of urine upon urethral catheterization In  Postpartum urinary retention often occurs due to the following: • Perineal trauma from a prolonged second stage of labor and/or perineal laceration that results in a pudendal nerve injury. Damage to the pudendal nerve can result in a decreased voiding LE sensation, thereby promoting urinary retention, and cause external urethral sphincter dysfunction. • Reduced sensory and motor sacral spinal cord impulses from regional neuraxial anesthesia (eg, U SM epidural anesthesia), which can suppress the micturition reflex and decrease detrusor tone, resulting in bladder atony. Self limited: 1 week Miscellaneous Back Pain Obs 461 https://t.me/usmleinnercircle Version 2 Low back pain during pregnancy • Etiology Enlarged uterus --+ exaggerated lordosis • Joint/ligament laxity from j progesterone/relaxin • Weak abdominal muscles--+ decreased lumbar support Risk factors • Excessive weight gain • Chronic back pain . Back pain in prior pregnancy • Multi parity Imaging • Not indicated • Behavioral modifications Management • Heating pads • Analgesics  What is the recommended management for a woman in the third trimester of pregnancy that presents with lower back pain that radiates down the legs, especially with activity? Physical exam is benign. Reassurance and Conservative management The management includes: • Reassurance and behavioral modifications 0 Lifestyle interventions (wear-low heeled shoes, good back support, heat/cold/massage to the painful area) 0 Rest with hip flexion 0 Exercise 0 Medication: Short course analgesic → acetaminophen has best safety profile in pregnancy Round Ligament Pain  Round ligament pain develops when the ligament is stretched by the gravid uterus, usually in the late second trimester and the third trimester. It typically presents as a sharp unilateral pain that radiates to the vagina, is triggered by movement (ie, positional), and resolves without treatment. Liver Disorders of Pregnancy Obs 462 https://t.me/usmleinnercircle Version 2 Disorder Presentation Laboratory abnormalities Clinical features HELLP • Preeclampsia • Right upper-quadrant pain • Nausea/vomiting • Hemolysis • Moderately elevated liver aminotransferases • Thrombocytopenia • Malaise • Right upper-quadrant pain • Nausea/vomiting • Sequelae of liver failure Laboratory findings Management • Hypoglycemia • Mildly elevated liver aminotransferases • Elevated bilirubin • Possible disseminated intravascular coagulopathy Right upper quadrant/epigastric pain Fulminant liver failure Profound hypoglycemia j Aminotransferases (2-3• normal) j Bilirubin Thrombocytopenia Disseminated intravascular coagulopathy Immediate delivery Immediate delivery irrespective of gestational age • Normal BP (vs HELLP irc AFLP • Intense pruritus • Elevated bile acids • Elevated levels of liver aminotransferases • Diagnosis of exclusion Nausea, vomiting le ICP .. . .. .. . . Acute fatty liver of pregnancy Liver disorders unique to pregnancy rC AFLP is unique because it is an intrahepatic process due to microvesicular fatty infiltration of hepatocytes secondary to abnormal maternal-fetal fatty acid metabolism. Intrahepatic cholestasis of pregnancy ne  Pregnancy, pruritis, elevated total bile acids, +/- elevated aminotransferases In Treatment: ursodeoxycholic acid, typically resolves within weeks following delivery lntrahepatic cholestasis of pregnancy • Development in 3rd trimester Clinical LE features • Generalized pruritus • Pruritus worse on hands & feet Laboratory U SM abnormalities Obstetric risks Management I • No associated rash • Right upper quadrant pain • i Total bile acids (2:10 µmol/L) • j Liver transaminases (typically <2x normal, rarely >1000 U/L) • ± j Total & direct bilirubin • Intrauterine fetal demise • Preterm delivery • Meconium-stained amniotic fluid • Neonatal respiratory distress syndrome • Ursodeoxycholic acid • Antihistamines • Delivery at 37 weeks gestation The risk of intrauterine fetal demise IUFD is particularly high when serum bile acids are 100 µmol/L Obs 463 https://t.me/usmleinnercircle Version 2 Symptomatic cholelithiasis in pregnancy • t Biliary cholesterol excretion (estrogen) • t Gallbladder motility (progesterone) Recurrent, postprandial epigastric/RUQ pain Clinical features • Pathophysiology . Management RUQ ultrasound with echogenic foci (stones or sludge) • Conservative (eg, pain control) • Cholecystectomy (for complicated, recurrent cases) RUQ = right upper quadrant. Cholecystectomy usually delayed until postpartum Patients who develop biliary colic while pregnant are typically treated with intravenous fluids and pain control. If symptoms cannot be controlled with supportive care, cholecystectomy is often performed during the second trimester. Pregnancy-induced skin changes vs intrahepatic cholestasis of pregnancy Pregnancy-induced skin changes Presentation Etiology Laboratory abnormalities Obstetric risks Obstetrical management • Focal pruritus • No rash • Generalized pruritus • Hands & foot involvement • No rash • Pregnancy hormone changes • lntrahepatic cholestasis • None • t Bile acids • ± Mild transaminitis • Transaminitis • None • Intrauterine fetal demise • Exeectant • Delivery at 37 weeks • Oatmeal baths Treatment lntrahepatic cholestasis of pregnancy • Ultraviolet light • Antihistamines • Ursodeoxycholic acid • Antihistamines Pseudocyesis What is pseudocyesis? • "False pregnancy" • Women present with classic signs of pregnancy (e.g. morning sickness, amenorrhea, abdominal distention) despite it being ruled out with U/S and negative pregnancy test.  Pseudocyesis can occur when psychological pressures (eg, difficulty conceiving), social pressures, and the somatization of stress affect the hypothalamic-pituitary-ovarian axis, or when bodily changes (eg, weight gain, amenorrhea) are misinterpreted by the patient. Obs 464 https://t.me/usmleinnercircle Version 2 The end result is a deeply held, nondelusional belief of being pregnant that may be strong enough to cause a patient to misread a negative home pregnancy test result as positive Because pseudocyesis is a form of somatization, management requires psychiatric evaluation and treatment. Drugs Magnesium toxicity • Mild: nausea, flushing, headache, hyporeflexia Clinical Severe: respiratory paralysis, cardiac arrest Stop magnesium therapy Give IV calcium gluconate bolus rC Treatment . . . irc • Moderate: areflexia, hypocalcemia, somnolence features le  Inhaled B agonist and inhaled corticosteroids are safe to use in pregnancy. Mg becomes toxic at concentrations  8 mg/dL ne • Hypocalcemia due to temporary suppression of PTH Magnesium is solely excreted by the kidneys In thus patients with renal insufficiency are at increased risk for toxicity LE Illicit drug abuse in pregnancy U SM Risk factors Obstetric complications • Adolescent pregnancy • Late/noncompliant prenatal care • Inadequate pregnancy weight gain • Spontaneous abortion • Preterm birth • Preeclampsia • Abruptio placentae • Fetal growth restriction • Intrauterine fetal demise Uterotonic What are the (4) uterotonic agents? • Oxytocin • Carboprost tromethamine (Hemabate PGF2 analog 0 CI in asthma • Methylergonovine (Methergine Ergot alkaloid 0 Obs CI in htn 465 https://t.me/usmleinnercircle Version 2 • Misoprostol Prostaglandin 0 also used in abortions, cervical ripening, labor induction Oxytocin Indications • Induction or augmentation of labor • Prevention & management of postpartum hemorrhage Adverse effects • Hyponatremia • Hypotension • Tachysystole Oxytocin is not effective in stimulating uterine contractions or expelling retained products of conception during the first or second trimesters because few oxytocin receptors are in the uterus during early pregnancy.  What are the (3) complications of oxytocin toxicity? Released from posterior pituitary with ADH and both have similar structure • Hyponatremia (seizures) 0 Treat with hypertonic saline • Hypotension • Uterine Tachysystole (5 contractions in 10 minutes) Obs 466 https://t.me/usmleinnercircle Version 2 GIT Salivary Gland Sialadenosis Suppurative Parotitis le Esophagus Achalasia GERD irc Perforation Eosinophilic esophagitis Stomach rC Dyspepsia Vomiting & Diarrhea Factitious Diarrhea Chemotherapy-related diarrhea ne Bile Salt Diarrhea Constipation Gastroparesis Gastric Outlet Obstruction In Liver Jaundice Acute Liver Failure NAFLD LE Cirrhosis Hepatic Encephalopathy HepatoPulmonary Syndrome HepatoRenal Syndrome U SM Budd Chiari Liver Abscess Gall Bladder & Bile Duct Acalculous Cholecystitis Emphysematous Cholecystitis Acute Cholangitis Primary Sclerosing Cholangitis Primary Biliary Cholangitis Post-cholecystectomy syndrome Pancreas Severe Acute Pancreatitis Pancreatic Pseudocyst Pancreatic Cyst Chronic Pancreatitis Spleen GIT 467 https://t.me/usmleinnercircle Version 2 Small Intestine Malabsorption Syndrome DXylose Test IBS IBD Celiac Disease SIBO Appendicitis Infectious ileocecitis Pseudo-appendicitis) Obstruction Large Intestine & Rectum Hemorrhoids Diverticulitis & Diverticulosis Angiodysplasia Colovesical Fistula Toxic Megacolon Colonic Pseudo-obstruction Ogilvie Syndrome) Volvulus Ischemic Colitis Microscopic Colitis Proctalgia Fugax Proctitis Rectal Prolapse Anal Fissure Anorectal Fistula Anorectal Abscess Peritoneum & Mesentery Retroperitoneal Hematoma Mesenteric Ischemia Peritonitis Hernia Umbilical Hernia Incisional Hernia Rectus Abdominis Diastasis GIT Tumors Esophagus Stomach Pancreas Colon PJ Syndrome Liver MALToma Miscellaneous Abdominal Compartment Syndrome TPN Paeds GIT Tracheoesophageal Fistula Foreign Body Ingestion GIT 468 https://t.me/usmleinnercircle Version 2 Crying Infant Constipation Vomiting Cyclical Vomiting Syndrome Food Allergen Proctocolitis Breastfeeding Dehydration Midgut volvulus Pyloric Stenosis Neonatal Jaundice le Bilirubin induced Neurologic Dysfunction Biliary Atresia Biliary cyst irc Intestinal Atresia Hirschsprung Intussusception Meckel's Diverticulum rC NEC Reye Syndrome Malnutrition GIT Surgery ne Bariatric Surgery Gastric Bypass Billroth 2 Pancreaticoduodenectomy Abdominal Trauma Pancreatic Injury Splenic Rupture In Nissenʼs Fundoplication LE Duodenum Perforation Duodenal Hematoma Rectus Sheath Hematoma Wound Dehiscence U SM Pilonidal Sinus GI Bleed Variceal Bleeding Hemobilia Salivary Gland  What is the pharmacologic treatment of sialadenitis? Clindamycin or nafcillin + metronidazole Poor response to 48 hours of antibiotics or abscess -- proceed to surgical drainage and decompression  What bacteria is the most common cause of acute bacterial parotitis? Staphylococcus aureus GIT 469 https://t.me/usmleinnercircle Version 2  What preventive measures are useful for preventing post-operative acute bacterial parotitis? Adequate fluid hydration and Oral Hygiene Sialadenosis • Sialadenosis is a benign, non-inflammatory, painless enlargement of the salivary glands often seen in patients with dietary/nutritional disorders (diabetes, bulimia), chronic alcohol use or advanced liver disease. Suppurative Parotitis Risk factors Clinical presentation Management .. .. .. .. .. . . Suppurative parotitis Elderly, dehydrated. postsurgical Decreased oral intake (eg, NPO perioperatively) Medications (eg, anticholinergics) Obstruction (eg, calculi, neoplasm) Firm, erythematous pre/postauricular swelling Exquisite tenderness exacerbated by chewing and palpation Trismus, systemic findings (eg, fever, chills) Elevated serum amylase without pancreatitis Ultrasound or CT scan (eg, ductal obstruction, ~) Hydration, oral hygiene Antibiotics • Massage (ie, milking pus out of gland) Sialagogues Esophagus Globus sensation is a diagnosis of exclusion and is characterized by the sensation of a lump in the back of the throat. It is a functional disorder and does not cause any abnormalities on barium esophagram.  What is the likely diagnosis in a patient with GERD who complains of difficulty swallowing solid foods and has symmetric, circumferential narrowing on barium swallow? Esophageal stricture other causes of peptic strictures include radiation, systemic sclerosis, and caustic ingestion; biopsy is necessary to rule out malignancy (typically asymmetric narrowing) Strictures typically cause slowly progressive dysphagia to solid foods without anorexia or weight loss. As they progress, they can actually block reflux, leading to improvement of heartburn symptoms GIT 470 https://t.me/usmleinnercircle Version 2 Medication-induced esophagitis Drug class Drug Antibiotics Tetracyclines Aspirin & many nonsteroidal anti-inflammatory drugs Bisphosphonates Alendronate, risedronate others Potassium chloride, iron le Anti-inflammatory agents irc  What is the likely diagnosis in a patient with sudden-onset odynophagia and retrosternal pain with a discrete ulcer in the mid-esophagus? Pill esophagitis mid-esophagus most common due to compression by the aortic arch or an enlarged left atrium the site of the ring. T3,T4 ne Can also compress trachea in infants  Cyanosis rC • Vascular ring is a congenital anomaly of the aortic arch. Esophageal compression causes dysphagia, vomiting, and food impaction, but not abdominal pain. Endoscopy shows an indentation at In • Schatzki ring is a circumferential band of tissue that partially occludes the esophageal lumen and will appear as a thin, ringed defect on barium esophagography. U SM Achalasia LE Patients typically present with intermittent dysphagia to solids. GIT 471 https://t.me/usmleinnercircle Version 2 Evaluation of dysphagia History of difficulty initiating swallowing with cough, choking, or nasal regurgitation Likely oropharyngeal dysphagia Videofluoroscopic modified barium swallow No Likely esophageal dysphagia Dysphagia with solids progressing to liquids Dysphagia with solids & liquids at onset Mechanical obstruction History of prior radiation, caustic injury, complex stricture, or surgery for esophageal/laryngeal cancer Barium swallow followed by possible endoscopy Barium swallow followed by possible manometry Upper endoscopy ©USMLEWo,ld, UC Oropharyngeal dysphagia: Characterized by difficulty initiating a swallow; underlying etiologies include stroke, NMS disorders, Zenker diverticulum, Advanced Dementia Achalasia key points: • Ba swallow is the best initial test • Manometry follows here and offers more info • Endoscopy must be done to rule out cancer • treatment is preferred w/ Heller myotomy (balloon dilatation can cause perforation; botox is less effective) • Pseudoachalasia is a narrowing of the distal esophagus secondary to causes other than denevervation (achalasia), such as esophageal cancer. In this case, a endoscopy must be performed to exclude malignancy. Looks for clues such as weight loss, acute onset, and age 60 What test is most accurate for diagnosis of achalasia? Esophageal manometry findings include high LES resting pressure and incomplete LES relaxation; barium esophagram is sometimes used as an initial test GIT 472 https://t.me/usmleinnercircle Version 2  Structural lesions that lead to dysphagia in the pharynx and upper esophagus may be visualized with nasopharyngeal laryngoscopy. GERD Complications Initial treatment f Risk with obesity, pregnancy, smoking, alcohol intake Regurgitation of acidic material in mouth Heartburn Odynophagia (often indicates reflux esophagitis) le . .. .. .. .. Decreased tone or excessive transient relaxations of LES Anatomic disruption to gastroesophageal junction (eg, hiatal hernia) irc Manifestations .. Extraesophageal manifestations: cough, hoarseness, wheezing Esophageal: erosive esophagitis, Barrett esophagus, strictures Extraesophageal: asthma exacerbation, laryngitis Lifestyle (eg, weight loss) & dietary changes rC Pathophysiology Gastroesophageal reflux disease H2R blocker or PPI H2R = histamine 2 receptor; LES = lower esophageal sphincter; PPI = proton pump inhibitor. ne Symptoms consistent with GERD LE Refractory symptoms Men age >50 with symptoms for >5 years or cancer risk factors OR ;,larm symptoms" In Once daily PPIfor 2 months Perform endoscopy Switch to different PPIor increase PPIto twice daily U SM Symptoms controlled Persistent symptoms Continue present Consider endoscopy therapy or esophageal pH monitoring Consider further testing for following diagnoses: Treat ;,ccording to diagnosis: Pill esophagitis Achalasia Gastroparesis Autoimmune skin disease Zollinger-Ellison syndrome Nonacid reflux disease Eosinophilic esophagitis Nocturnal acid breakthrough Barrett's esophagus *Alarm symptoms Melena Persistent vomiting Hematemesis Esophageal manometry Impedance testing Weight loss Gastric scintigraphy Anemia Dysphagia/odynophagia GIT 473 https://t.me/usmleinnercircle Version 2 Management of gastroesophageal reflux disease (GERO) GERO symptoms (substemal burning, regurgitation) l Presence of alarm features• OR Multiple Barrett esophagus risk factors•· Yes-----~-- No~ Symptom severity Mild <2 days/wk Severe .?2days/wk Upper gastrointestinal endoscopy Antacids (eg, calcium carbonate) or H2RA + lifestyle changes PPI + lifestyle changes 'Alarm features • Oysphagia/odynophagia • Iron deficiency anemia • GI bleeding • Unexplained weight loss • Persistent vomiting • Family history of gastrointestinal cancer ••Barrett esophagus risk factors •Age >50 • Male sex • Smoking history • GERO ;,,5 years • Obesity • Family history • Vl/hite ethnicity • Hiatal hernia l GI = gastrointestinal;H2RA = H2 receptorantagonist;PPI = proton pump inhibitor. C)UWOrld  Patient with GERD and failed PPI therapy (greater than 6 weeks) ⟶ go to EGD  Person with a history of Barrett's Esophagus and symptoms worsening ⟶ go to EGD Clear EGD? Still can have GERD → 24-hour esophageal pH monitoring Gold standard) Complications of GERD 1. Adenocarcinoma 2. Strictures 3. Ulcers Perforation GIT 474 https://t.me/usmleinnercircle Version 2 Esophageal perforation • Instrumentation (eg, endoscopy), trauma • Effort rupture (Boerhaave syndrome) • Esophagitis (infectious/pills/caustic) • ChesUback &/or epigastric pain, systemic signs (eg, fever) Clinical presentation • Crepitus, Hamman sign (crunching sound on auscultation) • Pleural effusion with atypical (eg, green) fluid • Chest x-ray or CT scan: widened mediastinum, pneumomediastinum, pneumothorax, pleural effusion Diagnosis • CT scan: esophageal wall thickening, mediastinal fluid collection • Esophagography with water-soluble contrast: leak from perforation • NPO, IV antibiotics & proton pump inhibitors Management • Emergency surgical consultation irc le Etiology Pleural effusion is typically left-sided because the esophagus is positioned on the left anatomically What is the most common cause of esophageal rupture? rC Endoscopy Characteristics of gastroesophageal mural injury presentation Studies ne Submucosal venous or arterial plexus bleeding Hematemesis (bright red or coffee-ground) Possible hypovolemia treat persistent bleeding) .. Acid suppression Most heal spontaneously Transmural tear Spillage of esophageal air/fluid into surrounding tissues ChesUback/epigastric pain Crepitus, crunching sound (Hamman sign) Odynophagia, dyspnea, fever, sepsis • Chest x-ray: pneumothorax, pneumomediastinum, pleural Upper GI endoscopy confirms diagnosis (& can U SM Management Mucosal tear Epigastric/back pain . • . • . • Forceful retching Forceful retching In Clinical .. • . • . . Boerhaave syndrome LE Etiology Mallory-Weiss syndrome effusion • Esophagography or CT scan with water-soluble contrast .. confirms diagnosis Acid suppression, antibiotics, NPO Emergency surgical consultation GI = gastrointestinal.  What is the diagnosis of Boerhaave Syndrome in a stable and unstable patient? • Stable → Gastrografin swallow (water soluble) • Unstable → CT scan Do NOT start out with EGD (camera may go through perforation) Do NOT do barium swallow (non-water soluble barium enters mediastinum → mediastinitis) Eosinophilic esophagitis GIT 475 https://t.me/usmleinnercircle Version 2 Eosinophilic esophagitis Eosinophilic esophagitis in adults Pathogenesis & • Th2-mediated inflammatory response triggered primarily by food antigen exposure epidemiology • Comorbid atopic disease (asthma, eczema, food allergies, allergic rhinitis) common • Dysphagia Clinical • Heartburn & epigastric pain features • Regurgitation • Food impaction Diagnosis • Endoscopy & esophageal biopsy (eosinophils: 2'15/hpf) • Exclusion of alternate diagnoses (eg, achalasia, infection) Six Food Elimination Diet [SFED] 1. cow milk 2. Soy 3. wheat 4. egg 5. peanut 6. seafood/ shellfish • Elimination diet Six Food Elimination Diet [SFED] Treatment • Proton pump inhibitors • Topical glucocorticoids Th2 = T-helper cell type 2. First-line treatment is dietary modification to avoid potential food triggers  Does eosinophilic esophagitis respond to GERD therapy (e.g. PPIs)? No however, a 2-month trial of PPIs is part of the diagnostic evaluation; if there is no symptom improvement, an endoscopy with esophageal biopsy is warranted Stomach  What is the likely diagnosis in a patient with abdominal pain and hematemesis following recent use of alcohol, aspirin, and cocaine? Acute erosive gastropathy characterized by development of severe hemorrhagic lesions after exposure to various injurious agents Hallmark is development of hemorrhagic and erosive lesions shortly after exposure of gastric mucosa to various substances or reduction in mucosal blood flow. Things like NSAIDs and GIT 476 https://t.me/usmleinnercircle Version 2 alcohol (direct injury) or burns, sepsis, trauma (hypoxia of mucosa) cause loss of the protective barrier and permits acid and other substances to penetrate into the laminate propria. D/D Mallory Weiss Tear but will have several episodes of nausea and vomiting before hematemesis happens, wont be hematemesis from first bout of vomiting itself. Indications for stress ulcer prophylaxis • Coagulopathy: platelets <50,000/mm 3 , INR >1.5, ?!2factors PTT >2x normal control Mechanical ventilation >48 hr GI bleeding or ulceration in last 12 months Head trauma, spinal cord injury, major bum Glucocorticoid therapy >1 week ICU stay • Occult GI bleeding >6 days • Sepsis rC GI = gastrointestinal;ICU = intensivecare unit. le .. . .. irc Any 1 factor • Stress ulceration is common in patients in the ICU and can cause occult or gross gastrointestinal bleeding. Painless bleeding (vs Peptic Ulcer) In T/t: PPI ne Risk factors include shock, sepsis, coagulopathy, mechanical ventilation, traumatic spinal cord/brain injury, burns, and high-dose corticosteroids. LE Both NSAIDs and aspirin can cause gastritis and/or gastric ulcers leading to chronic gastrointestinal blood loss and depletion of iron stores IDA. U SM • Autoimmune atrophic gastritis is an autoimmune disorder resulting in the formation of antibodies toward parietal cells (resulting in hypochlorhydria and unchecked gastrin production) and intrinsic factor (resulting in B12 deficiency). Common manifestations include postprandial abdominal pain, bloating, elevated serum gastrin levels, and macrocytic anemia. Dyspepsia GIT 477 https://t.me/usmleinnercircle Version 2 Dyspepsia Symptoms • Epigastric pain or burning • Postprandial fullness, early satiety, bloating • ~1 months • Functional/idiopathic (75%) Etiology • Malignancy (eg, gastric, esophageal) • Peptic ulcer (eg, Helicobacter pylori infection, NSAIDs) • Drug-induced (eg, NSAIDs, bisphosphonate) • Low malignancy risk (eg, age <60 & no alarm symptoms*) Work-up 0 • 0 Treatment Testing & treatment for H pylori High malignancy risk (eg, age >60 or alarm symptoms) Gastroenterology referral/upper endoscopy • Treatment of underlying cause • Trial of proton pump inhibitor if no cause found *GI bleeding, weight loss, iron deficiency anemia, lymphadenopathy,dysphagia, odynophagia,persistentvomiting, palpable mass, family history of upper GI cancer. GERD = gastroesophagealreflux disease; GI = gastrointestinal;NSAIDs = nonsteroidalanti-inflammatorydrugs. Dyspepsia management Symptoms of epigastric pain/burning, postprandial fullness, early satiety High-risk features• No alarm features • Older age (>60) • Symptoms: odynophagia. dysphagia. I DA, weight loss, LAD • + family history of gastric cancer Noninvasive H pylori testing (eg, urea breath test, stool antigen test) l Consider upper endoscopy with endoscopic testing for H pylori ! l Positive Negative + + Treatment for H pylori Trial of PPI 'Higherriskfor seriouspathology,includingmalignancyor significantbleeding H pylori= Hellcobacterpylori; IDA= Irondeficiencyanemia,LAD= lymphadenopathy, PPI = proton pumpinhibitor. C)lMlorld Epigastric pain vs usually sub-sternal in GERD.  Most cases of dyspepsia are idiopathic (ie, functional dyspepsia), but other causes, including malignancy (eg, gastric adenocarcinoma), infection (eg, Helicobacter pylori), or medication effect (eg, chronic nonsteroidal anti-inflammatory drug therapy), should be investigated first. • Helicobacter pylori eradication should be confirmed for patients with the following: • persistent symptoms GIT 478 https://t.me/usmleinnercircle Version 2 • H pylori associated ulcer on endoscopy • evidence of an H pylori associated malignancy (eg, mucosa-associated lymphoid tissue lymphoma). Common symptoms of duodenal ulcers include nocturnal pain (due to circadian rhythm of gastric acid secretion), worsening of the pain with fasting, postprandial bloating, and nausea • Biopsy of the gastric antrum during endoscopy can confirm infection. irc le  In a patient without occult bleeding, noninvasive diagnostic testing, including stool antigen studies and urea breath testing, can be employed. rC Serology cannot distinguish between active and cleared infection and is not preferred. Activ,e infection ne Peptic ulcer disease can be complicated by perforation, which typically causes acute-onset, severe pain; a systemic inflammatory response (eg, fever, tachycardia); and peritonitis Guarding, diffuse abdominal pain) with H. pyk,ri In Are ANY of the fallowing present? • Prim· exposure to macrulides for any reason • Local darithmmycin resistance ,-at,es ;;a:15"%or eradication rates ~,iith darithromycin based triple therapy :s;85'% * I I Yes No U SM LE I I Yes Bismuth quadruple therapy1l + Pencillin allergy pre-sent? Yes No Metronida!,!;ole us,e ,-nthin the past few yeas? Clarithro mycin based triple therapy with amoxicillinll + No + Treat with any one of the following re<Jimens: • Clarithromycin based triple therapy metronidazole • Bismuth quadruple therap,y with Tripple Therapy: PPI + Clarithromycin + Amoxycillin/ Metronidazole Quadruple: PPI  Metronidazole  Tetracycline  Bismuth Vomiting & Diarrhea GIT 479 https://t.me/usmleinnercircle Version 2 Common causes of steatorrhea • Chronic pancreatitis due to alcohol abuse, cystic fibrosis, or autoimmune/hereditary pancreatitis Pancreatic insufficiency • Pancreatic cancer • Small-bowel Crohn disease • Bacterial overgrowth • Primary biliary cirrhosis Bile salt-related • Primary sclerosing cholangitis • Surgical resection of ileum (at least 60-100 cm) • Celiac disease Impaired intestinal surface epithelium • AIDS enteropathy • Giardiasis Other rare causes • Whipple disease • Zollinger-Ellison syndrome • Medication induced Vomiting and diarrhea are conditions that can lead to a decrease in insulin demand Vomiting and diarrhea lead to decreased glucose uptake, increasing the risk of hypoglycemia vs. acute stress reaction in illness and stress where there is increase in demand  Is diarrhea that occurs during fasting/sleeping characteristic of secretory or osmotic diarrhea? Secretory Etiologies include chronic infection, microscopic colitis, bile acid diarrhea Bowel Resection or Cholecystectomy), or a hormone-secreting tumor (eg, gastrinoma, VIPoma), Diabetes  A low stool osmotic gap is indicative of secretory diarrhea.  50 mOsm/kg; due to increased secretions of ions (e.g. bacterial/viral infection, congenital disorders of ion transport, postsurgical changes)  A high stool osmotic gap is indicative of osmotic diarrhea.  125 mOsm/kg; due to non-absorbed and unmeasured osmotically active agents in the GI tract (e.g. lactose intolerance) SOG " plasma osmolality- 2 x (stool sodium + stool potassium) • Chronic diarrhea can be due to either a functional disorder (eg, irritable bowel syndrome) or an organic disorder (eg, inflammatory bowel disease IBD, malabsorptive disease, chronic infection). An organic cause should be suspected when any of the following features are present: GIT 480 https://t.me/usmleinnercircle Version 2 • Age 50 • Nocturnal symptoms • Systemic symptoms (eg, fever, weight loss) • Rectal bleeding • Laboratory abnormalities (eg, elevated inflammatory markers) • Family history of colon cancer or IBD irc le If a patient has weight loss and episodes of diarrhea overnight, she requires further work-up for organic pathology, including malabsorptive conditions (eg, celiac disease) and chronic infections (usually parasitic eg, Giardia, Cryptosporidium) rC Diarrhea from small intestinal conditions is usually large volume, whereas that from colon pathologies is small volume but frequent. Factitious Diarrhea ne  What is the likely diagnosis in a female with frequent, watery, nocturnal diarrhea and melanosis coli on colonoscopy? Factitious diarrhea (laxative abuse) typically in a healthcare worker; diagnosis is supported by positive stool screen for laxatives In other points: Metabloc Alkalosis, Hypokalemia, U SM LE Check history of no weight loss (vs celiac disease which has weight loss) Melanosis coli  Melanosis coli can develop within a few months of the onset of regular anthraquinone(eg, senna) laxative abuse and can similarly disappear if laxative use is discontinued. If melanosis coli is not seen on gross inspection, histological examination may demonstrate the pigment in the macrophages of the lamina propria. GIT 481 https://t.me/usmleinnercircle Version 2 Depending on the laxative abused, the SOG can be high (eg, lactulose) or low (eg, senna).  Patients with factitious diarrhea purposely cause large, voluminous stools, most commonly by improper use of laxatives; however, they can also create the appearance of diarrhea by adding fluid to the stool. Therefore, helpful tests in evaluating factitious diarrhea include: • Stool osmolality: Stool osmolality is in equilibrium with plasma osmolality and typically remains constant (eg, 290 mOsm/kg) in organic gastrointestinal disease. Hypoosmolality suggests addition of water or other dilute fluid; hyperosmolality suggests addition of a concentrated fluid (eg, urine). • Stool electrolytes: Elevated stool magnesium or phosphate levels suggest overuse of saline osmotic (ie, magnesium- or phosphate-containing) laxatives. • Stool osmotic gap Osmotic laxatives (eg, lactulose, polyethylene glycol) cause a high osmotic gap diarrhea, whereas senna and bisacodyl produce a low osmotic gap secretory diarrhea. Chemotherapy-related diarrhea Secretory diarrhea, which is voluminous, watery, and persistent despite periods of fasting (eg, nocturnal diarrhea). Treatment: Loperamide, diphenoxylate-atropine Chemotherapy is a risk factor for infection (particularly with C difficile), all patients with persistent diarrhea should undergo laboratory evaluation (eg, complete blood count, serum chemistry) and stool testing (eg, C difficile stool studies, fecal occult blood) prior to a presumptive diagnosis of CRD. Bile Salt Diarrhea Bile acid diarrhea • Unresorbed bile acids spill into the colon, resulting in mucosal irritation Pathophysiology . Clinical features Treatment 0 Bile acid enters terminal ileum too rapidly & overwhelms resorptive capacity (eg, post cholecystectomy) 0 lleal disease imeairs bile abso!Etion (eg, Crohn disease, abdominal radiation damage) Secretory diarrhea (eg, fasting diarrhea, nocturnal episodes) • Bloating, abdominal cramps • Unremarkable serum & stool studies • Bile acid-binding resins (eg, cholestyramine, colestipol) Causes Fat soluble vitamins deficiency ! Constipation GIT 482 https://t.me/usmleinnercircle Version 2 Alarm features of constipation • Hematochezia • Weight loss ~10 lb (4.5 kg) • Family history of inflammatory bowel disease • Iron deficiency anemia • Family history of colorectal cancer • Positive fecal occult blood test • Recent onset without obvious cause (especially in older adults) irc le The presence of iron deficiency anemia in a patient with new-onset constipation suggests ongoing gastrointestinal blood loss and requires evaluation with diagnostic colonoscopy. Hemorrhoids are commonly seen in patients with constipation and may cause mild bleeding, but they are rarely expected to generate sufficient blood loss to produce iron deficiency rC anemia.  Fecal impaction is common in older patients with impaired mobility, inadequate fluid or dietary fiber ne intake, chronic constipation, or decreased sensation of stool in the rectal vault (eg, spinal cord injury, dementia). Obstruction of fecal flow in the rectum can cause backup of stool proximal to the impaction; passage of liquid stool around the impaction leads to incontinence. Urinary incontinence is also common due to pressure against the bladder. In  The diagnosis of fecal impaction is typically apparent on digital rectal examination, although impaction in the proximal rectum may be apparent only on abdominal x-ray. LE Initial management includes manual disimpaction to break up the hard stool followed by enemas (eg, tap water, mineral oil) to dislodge the fecal fragments. Mechanism lactulose t Osmotic gradient for water--> bowel distension --> t peristalsis Pharmacologic treatment of chronic constipation* Class Polyethylene glycol, U SM Osmotic laxatives Agent Saline laxatives Magnesium salts Stimulant laxatives Bisacodyl, senna (irritants) Enteric neuron stimulation --> t peristalsis + t fluid secretion Lubiprostone CFTR chloride channel stimulation --> t fluid secretion Methylnaltrexone µ Opioid receptor antagonism --> t peristalsis Chloride channel activators Opioid antagonists .. *<3 bowel movements/weekwith symptoms (eg, straining, hard stools) for at least 3-6 months. ••For chronic constipationinduced by opioids. CFTR = cystic fibrosis transmembraneconductanceregulator. • Polyethylene glycol is generally safe for daily use in older adults, making it an ideal choice for preventive therapy. GIT 483 https://t.me/usmleinnercircle Version 2 • Bisacodyl is a stimulant laxative that increases intestinal peristalsis and fluid secretion. Daily, longterm use is not recommended due to the risks of protein-losing enteropathy and electrolyte imbalances (eg, hypokalemia, salt loss). Gastroparesis Gastroparesis • Diabetes mellitus (autonomic neuropathy) • Medications (eg, opioids, anticholinergic drugs) Causes • Traumatic/postsurgical injury (ie, vagus nerve injury) • Neurologic (eg, multiple sclerosis, spinal cord injury) • ldiopathic/postviral • Nausea & vomiting, epigastric abdominal pain Clinical features • Early satiety, bloating, weight loss • Labile glucose (diabetes mellitus) • Epigastric distension & succussion splash Diagnosis • Exclude obstruction: upper endoscopy± CT/MR enterography • Assess motility: nuclear gastric-emptying study • Frequent small meals (low fat, soluble fiber only) Treatment • Promotility drugs (eg, metoclopramide, erythromycin) • Gastric electrical stimulation &/or jejunal feeding tube (refractory symptoms) Gastric Outlet Obstruction  Common causes of gastric outlet obstruction include gastric malignancy, peptic ulcer disease, Crohn disease, strictures (with pyloric stenosis) secondary to ingestion of caustic agents, and gastric bezoars.  The presence of an "abdominal succussion splash" suggests a diagnosis of gastric outlet obstruction. the physician places the stethoscope over the upper abdomen and rocks the patient back and fourth at the hips; definitive diagnosis requires endoscopy  What is the likely cause of gastric outlet obstruction in a patient with a history of acid ingestion? Pyloric stricture characterized by early satiety, nausea, non-bilious vomiting, weight loss, and abdominal succussion splash Liver Jaundice GIT 484 https://t.me/usmleinnercircle Version 2 Approach to hyperbilirubinemia in adults Possible causes Hyperbilirubinemia ---~----' Mainly conjugated Overproduction (eg, hemolysis) Mainly unconjugated Reduced uptake (eg, drugs, portosystemic shunt) • Conjugation defect (eg, Gilbert syndrome) Normal AST, ALT, alkaline phosphatase Predominantly elevated alkaline phosphatase • Viral hepatitis Dubin-Johnson syndrome Cholestasis of pregnancy • Autoimmune hepatitis Rotor syndrome Malignancy (eg, pancreas, ampullary) Toxin/drug-related hepatitis irc Predominantly elevated AST & ALT le Evaluate liver enzyme pattern Cholangiocarcinoma Primary biliary cholangitis rC Hemochromatosis Primary sclerosing cholangitis lschemic hepatitis Choledocholithiasis • Alcoholic hepatitis ne • Abdominal imaging (ultrasound or CT) ALT= alaninetransaminase:AST= aspartatetransaminase. CUWotlO • Antimitochondnal antibody In Presence of biliary dilatation is suggestive of extrahepatic cholestasis; absence of biliary dilatation suggests intrahepatic malignancy. LE • Endoscopic retrograde cholangiopancreatogram ERCP is usually performed in patients when initial ultrasonography or CT scan suggests the presence of obstruction due to cholelithiasis or U SM ERCP in these settings can be both diagnostic and therapeutic by relieving obstruction and facilitating biliary drainage. GIT 485 https://t.me/usmleinnercircle Version 2 .. . .. . Etiologies .. .. . Manifestations Diagnosis Malignant biliary obstruction Cholangiocarcinoma Pancreatic/hepatocellular carcinoma Metastasis (eg, colon, gastric) Jaundice, pruritus, acholic stools, dark urine Weight loss RUQ pain RUQ mass or hepatomegaly f Direct bilirubin, ALP, GGT Serum tumor markers (CEA, CA-19, AFP) Abdominal imaging (ultrasound, CT scan) EUS or ERCP for tissue diagnosis if unclear AFP = alpha-fetoprotein;ALP= alkaline phosphatase;CEA= carcinoembryonicantigen; EUS = endoscopicultrasound;ERCP = endoscopic GGT = gamma-glutamyltransferase;RUQ = right upper quadrant. retrogradecholangiopancreatography; Can cause painless jaundice  What is the likely diagnosis in an elderly patient with conjugated hyperbilirubinemia, elevated alkaline phosphatase, and painless jaundice? Malignant biliary obstruction Acute Liver Failure Acute liver failure Etiology • • • • • • Viral hepatitis (eg, HSV- CMV· hepatitis A B D & E) ~OT c Drug toxicity (eg, acetaminophen overdose, idiosyncratic) lschemia (eg, shock liver, Budd-Chiari syndrome) Autoimmune hepatitis Wilson disease Malignant infiltration Clinical presentation • • • • • • Generalized symptoms (eg, fatigue, lethargy, anorexia, nausea) Right upper quadrant abdominal pain Pruritus & jaundice due to hyperbilirubinemia Renal insufficiency Thrombocytopenia HypQglycemia Diagnostic requirements • Severe acute liver injury (ALT & AST often >1000 U/L) • Signs of hepatic encephalopathy (eg, confusion, asterixis) • Synthetic liver dysfunction (INR ~1.5) The presence of hepatic encephalopathy differentiates acute liver failure from acute hepatitis • Drug-induced liver disease can also be broadly categorized according to morphology:  Cholestasis (eg, anabolic steroids),  Fatty liver (eg, valproate),  Hepatitis (eg Isoniazid)  Toxic or fulminant liver (eg, acetaminophen)  Granulomatous (eg, allopurinol) GIT 486 https://t.me/usmleinnercircle Version 2 Increased concentrations of bilirubin, serum creatinine, and INR are associated with a worse prognosis. MELD SCORE  What is the likely diagnosis for a patient with an acute, massive increase in AST/ALT in the setting of hypotension? Ischemic hepatic injury (shock liver) le often accompanied by modest elevations in total bilirubin and alkaline phosphatase; and dramatic increase in ALT/AST irc  What is the next step in management for a patient with acetaminophen toxicity that develops worsening acute liver failure despite N-acetylcysteine treatment? Refer to liver transplant center rC this is an ethically complicated issue, however, if there is no history of psychiatric illness or previous suicide attempt, liver transplantation is typically pursued Chronic alcohol use is thought to potentiate acetaminophen hepatotoxicity by depleting ne glutathione levels and impairing the glucuronidation process.  What ASTALT ratio is typically seen in non-alcoholic fatty liver disease NAFLD? In  1 ASTALT useful distinguishing feature from alcoholic hepatitis, which is characterized by 21 ASTALT LE ratio. The absolute values of AST and ALT in alcoholic hepatitis are almost always  500 IU/L. Reye Syndrome will show micro-vesicular steatosis on liver biopsy. U SM What produces the opposite finding? Macrovesicular = alcoholic hepatitis, NASH Cirrhosis GIT 487 https://t.me/usmleinnercircle Version 2 Overview of cirrhosis management Treat underlying liver disease Provide preventive care • HCV, HBV: antiviral therapy • NASH: weight loss • Avoid alcohol & hepatotoxic medications • Vaccinate for HAV & HBV (unless already immune) • Screen for esophageal varices (endoscopy) Manage complications • Screen for HCC: ultrasound± serum AFP every 6-12 months • Frequent clinical assessment for ascites and encephalopathy (prophylactic treatment not recommended) AFP = alpha fetoprotein; HAV = hepatitis A virus; HBV = hepatitis B virus; HCC = hepatocellular carcinoma; HCV = hepatitis C virus; NASH = nonalcoholic steatohepatitis. Cirrhosis can have close to normal ALT, AST levels: No cells left to die Management of cirrhosis PeriodicsurveUlance of liVer fooctiontests (eg, INR, albllTiil, biirubin) Compensated • Ultrasound surveilance for hepatocenular carcinoma :!: alpha-fetoprotm every 6 months • EGD varicessurveilance Decompensated Assess complcations Variceal hemorrhage Start nonselecbve beta blockers, repeat EGD every year Ascites Dietary sodium restriction, diuretics, paracentesis, abstinence from alcohol Hepatic encephalopathy Identify undertyilg cause (eg, ilfection, gastrointestinal bleeding), lactulose therapy EGD = e.sophagogastroduodenoscopy. euw011d Liver also produces TBG, which binds thyroid hormones. Cirrhosis lowers total T3 and T4 but free T3 and T4 are unchanged, and thus TSH is normal. Most common infectious complication of cirrhosis? SBP Spontaneous Bacterial Peritonitis). Prevent by Paracentesis & Fluoroquinolones GIT 488 https://t.me/usmleinnercircle Version 2 In the United States, cirrhosis is most commonly due to chronic alcohol misuse, nonalcoholic steatohepatitis, and hepatitis C. Cirrhosis promotes the formation of ascites due to portal hypertension, which causes hemodynamic change (ie, splanchnic vasodilation) that leads to salt and water retention. Patients with ascites have increased abdominal girth and discomfort, as well as weight gain, dyspnea (from increased abdominal pressure), and early satiety. Physical examination shows shifting dullness and a fluid wave. irc Non-selective beta blockers (e.g. propanolol, nadolol) le  What pharmaceutical therapy is recommended for prophylactic treatment of non-bleeding esophageal varices? endoscopic variceal ligation can be used as an alternate primary prevention therapy in patients with contraindications to beta blockers rC Most cirrhotic patients should undergo diagnostic upper endoscopy to assess for varices and to determine their risk of hemorrhage. Those with (medium- or large-sized) varices, generally should be started on a nonselective beta blocker. ne Nonselective beta blockers (eg, propranolol, nadolol) are recommended to decrease progression to large varices and the risk of variceal hemorrhage. They are thought to act by decreasing adrenergic In tone in mesenteric arterioles, which results in unopposed alpha-mediated vasoconstriction and decreased portal venous flow.  How does baseline body temperature change in patients with cirrhosis? Decreased (hypothermic) LE thus any temperature  100°F 37.8°C warrants investigation Ascites fluid characteristics U SM • Bloody: trauma, malignancy, TB (rarely) Color • Milky: chylous • Turbid: possible infection • Straw color: likely more benign causes Neutrophils • 2:250/mm3 : peritonitis (secondary or spontaneous bacterial) • 2:2.5 g/dl (high-protein ascites) Total protein o CHF, constrictive pericarditis, peritoneal carcinomatosis, TB, Budd-Chiari syndrome, fungal • <2.5 g/dl (low-protein ascites) o Cirrhosis, nephrotic syndrome • 2:1.1 g/dl (indicates portal hypertension) SAAG o Cardiac ascites, cirrhosis, Budd-Chiari syndrome • <1.1 g/dl (absence of portal hypertension) o TB, peritoneal carcinomatosis, pancreatic ascites, nephrotic syndrome CHF = congestiveheart failure; SAAG = serum-ascitesalbumin gradient;TB = tuberculosis. SAAG 1.1 indicates hypoalbuminemia or malignancy (decreased oncotic pressure) GIT 489 https://t.me/usmleinnercircle Version 2  The ascites bilirubin level is elevated in the setting of a perforated biliary duct or bowel • Cytology should be performed for patients in whom there is a concern for underlying malignancy— usually those with persistently bloody ascites and cachexia. Management of ascites in cirrhosis • Imaging for confirmation (eg, abdominal ultrasound) Initial evaluation • Diagnostic paracentesis to confirm etiology & rule out infection 0 SAAG, cell count & differential, total protein • Spironolactone with furosemide Medical therapy • Alcohol abstinence, sodium restriction • Avoid ACE inhibitors, angiotensin receptor blockers, NSAIDs Refractory ascites • Large-volume paracentesis • Transjugular intrahepatic portosystemic shunt NSAIDs = nonsteroidal anti-inflammatory drugs; SAAG = serum-ascites albumin gradient. ACE inhibitors, ARBʼs and NSAIDʼs are Avoided in Cirrhosis. Blunt RAAS effect and lead to organ hypoperfusion in these patients IV albumin has been shown to decrease the incidence of renal failure and reduce mortality in patients with SBP. NAFLD Nonalcoholic fatty liver disease Definition • Hepatic steatosis on imaging or biopsy • Exclusion of other etiologies (eg, alcohol, hepatitis C, glucocorticoids) • Mostly asymptomatic Metabolic syndrome Clinical features • • AST/ALT ratio <1 --- • Hyperechoic texture on ultrasound examination Treatment Prognosis • Weight loss (eg, diet modification, exercise) • Consider bariatric surgery if BMI 2:35 • Hepatic fibrosis associated with increased risk for cirrhosis & liver-related death ALT= alanineaminotransferase;AST= aspartateaminotransferase. Hepatic Encephalopathy GIT 490 https://t.me/usmleinnercircle Version 2 Clinical presentation Treatment Drugs (eg, sedatives, narcotics) Hypovolemia (eg, diarrhea) Electrolyte changes (eg, hypokalemia) j Nitrogen load (eg, GI bleeding) Infection (eg, pneumonia, UTI, SBP) Portosystemic shunting (eg, TIPS) Sleep pattern changes Altered mental status Ataxia Asterixis Correct precipitating causes (eg, fluids, antibiotics) t Blood ammonia concentration (eg, lactulose, rifaximin) le Precipitating factors .. .. .. .. .. .. Hepatic encephalopathy irc GI= gastrointestinal; SBP = spontaneous bacterial peritonitis; TIPS= transjugular intrahepatic portosystemicshunt; UTI = urinary tract infection. Neomycin may be used to treat HE in patients unresponsive to lactulose and unable to tolerate rifaximin rC • Common causes of asterixis include hepatic encephalopathy, uremic encephalopathy, and hypercapnia. ne • Hypokalemia and metabolic alkalosis can precipitate hepatic encephalopathy, leading to lethargy, confusion, and asterixis. In 1. Be careful when giving diuretics, which can precipitate low intravascular volume and hypokalemia! 2. As a result, patients with HE and hypokalmiea require potassium repletion in addition to intravascular volume repletion LE  What is the underlying etiology of a patient with black stools and progressive confusion? He has a past medical history of hepatitis C and physical exam shows asterixis. Hepatic encephalopathy secondary to gastrointestinal bleed U SM HE is disorder of nervous system seen in patients with decompensated liver disease. Look for precipitating factors. • Hepatic Encephalopathy vs SBP Spontaneous Bacterial Peritonitis) Hepatic Encephalopathy: NO abdominal pain, usually no ascites, forgetful/confused SBP Triad: Abdominal Pain, Ascites, Altered Mental Status AMS HepatoPulmonary Syndrome Hepatopulmonary syndrome results from intrapulmonary vascular dilations AV Shunting) in the setting of chronic liver disease. Condition characterized by hypothermia, intrapulmonary vasodilation, and portal htn in the presence of cirrhosis Patients may have with platypnea (dyspnea worse when moving from lying to sitting) GIT 491 https://t.me/usmleinnercircle Version 2 Orthodeoxia (major clue): hypoxia upon sitting upright HepatoRenal Syndrome Risk factors Precipitating factors Diagnosis . . .• . • .. . Hepatorenal syndrome Advanced cirrhosis with portal hypertension & edema Reduced renal perfusion GI bleed, vomiting, sepsis, excessive diuretic use, SBP Reduced glomerular pressure & GFR 0 NSAID use (constricts afferent arterioles) Renal hypoperfusion Fe Na <1 % (or urine Na <10 mEq/L) Absence of tubular injury No RBC, protein, or granular casts in urine No improvement in renal function with fluids • Address precipitating factors (eg, hypovolemia, anemia, infection) • Splanchnic vasoconstrictors (midodrine, octreotide, norepinephrine) • Liver transplantation Treatment FeNa = fractional excretion of sodium; GFR = glomerular filtration rate; GI = gastrointestinal;RBC = red blood cells; SBP = spontaneousbacterial peritonitis. D/D Hypovlemic AKI; but it should improve with fluids unlike with Hepatorenal  Intravenous colloid solutions (eg, albumin) are used in the treatment of hepatorenal syndrome and spontaneous bacterial peritonitis. Budd Chiari Budd-Chiari syndrome • Hepatic venous outflow obstruction • Usually due to: Etiology 0 Myeloproliferative disorder (eg, PV) 0 Malignancy (eg, hepatocellular carcinoma) 0 Oral contraception use/pregnancy • Acute o Jaundice, hepatic encephalopathy, variceal bleeding 0 Manifestations • Prolonged INR/PTT; elevated transaminases Subacute/chronic o Vague, progressive abdominal pain Diagnosis 0 Hepatomegaly, splenomegaly, ascites 0 Mild/moderate elevation in bilirubin, transaminases • Abdominal Doppler ultrasound - t hepatic vein flow • Investigation for underlying disorders (eg, JAK2 testing for PV) INR = internationalnormalizedratio; PTT= partialthromboplastintime; PV = polycythemiavera. Liver Abscess GIT 492 https://t.me/usmleinnercircle Version 2 • Pyogenic liver abscesses typically present with fever, RUQ pain, hepatomegaly, leukocytosis, and elevated liver enzymes; typically with greater increases in ALP and bilirubin than in aminotransferases. an associated right-sided pleural effusion may occur. Diagnosis requires abdominal imaging(CT, USG, and management includes blood cultures, antibiotics, and drainage. rC irc le Liver abscess Bacterial Pyogenic) Liver Abscess ne  CT scan classically demonstrates a well-defined, hypoattenuating, rounded lesion, often surrounded by a peripherally enhancing abscess membrane. In T/t: Blood cultures, antibiotics, and percutaneous aspiration and drainage LE  Amoebic Liver abscess is treated with Metronidazole NOT drained); unless it fails to improve with medication or very large abscess. U SM Gall Bladder & Bile Duct GIT 493 https://t.me/usmleinnercircle Version 2 Evaluation of elevated alkaline phosphatase Elevated serum alkaline phosphatase Nonna! GGT Alkaline phosphatase likely of bone origin Elevated GGT Alkaline phosphate likely of brha,y ong,n AMApos~ive OR Abnormal hepatic parenchyma on uttrasound OleckRUQ IMtrasound & AMA Dilated bde ducts Both nonnal Consider liver biopsy, ERCP, observation Liver biopsy AMA= antinitodlondrial anlibody; ERCP = endoscopicretrogradedlolangiopancreatogram; GGT = gamma-gkJtamytransferase; RUQ=ri!lhtupperquadrant OUWond Alcohol raises GGT What are the differentials for air in the gallbladder? • Emphysematous cholecystitis (gb infected with gas-forming organisms like Clostridium) • Pneumobilia (air secondary to gallstone ileus) Management of gallstones Gallstones without symptoms Gallstones with typical biliary colic symptoms Complicated gallstone disease* . .. . No treatment required in most patients Elective laparoscopic cholecystectomy Possible ursodeoxycholic acid in poor surgical candidates Cholecystectomy within 72 hr •Acute cholecystitis, choledocholithiasis, gallstone pancrealitis.  Mild complication, do cholecystectomy within 3 days, if severe complication like Severe Pancreatitis: wait for resolution of inflammation. If the patient is a good surgical candidate, then the patient should have a cholecystectomy during their initial hospitalization as this decreases hospital readmission rates and mortality. Features that distinguish biliary colic from cholecystitis are pain resolution within 46 hours and absence of abdominal tenderness, fever, and leukocytosis. GIT 494 https://t.me/usmleinnercircle Version 2  Acute cholecystitis typically presents with persistent right upper quadrant pain, fever, nausea, vomiting, and leukocytosis. Diagnosis is confirmed when ultrasound reveals choleliths with gallbladder wall thickening or sonographic Murphy sign. If ultrasound is negative or inconclusive, a hepatobiliary iminodiacetic acid HIDA scan is the next diagnostic test of choice and is usually confirmatory. le Acalculous Cholecystitis Acalculous cholecystitis Prolonged fasting or TPN Critical illness (eg, sepsis, ICU) Fever, leukocytosis, t LFTs, RUQ pain Clinical presentation Jaundice & RUQ mass less common HIDA or CT scan if needed Enteric antibiotic coverage Treatment rC Abdominal ultrasound (preferred) Diagnosis irc Severe trauma or recent surgery Risk factors Cholecystostomy for initial drainage ne Cholecystectomy once clinically stable  Acalculous cholecystitis can present with unexplained fever and diffuse or right upper quadrant In (RUQ) abdominal pain Emphysematous Cholecystitis LE .. . .. .. . Emphysematous cholecystitis U SM Risk factors Clinical presentation Diagnosis Treatment GIT .. Diabetes mellitus Vascular compromise lmmunosuppression Fever, right upper quadrant pain, nausea/vomiting Crepitus in abdominal wall adjacent to gallbladder Air-fluid levels in gallbladder, gas in gallbladder wall Cultures with gas-fomning C/ostridium, Escherichia coli Unconjugated hyperbilirubinemia, mildly elevated aminotransferases Emergency cholecystectomy Broad-spectrum antibiotics with C/ostridium coverage (eg, piperacillin-tazobactam) 495 https://t.me/usmleinnercircle Version 2 Emphysematous cholecystitis Acute Cholangitis Acute cholangitis Etiology • Ascending infection due to biliary obstruction Clinical presentation • Fever, jaundice, RUQ pain (Charcot triad) • ± Hypotension, AMS (Reynolds pentad) • Cholestatic liver function abnormalities Diagnosis 0 j Direct bilirubin, alkaline phosphatase 0 Mildly j aminotransferases • Biliary dilation on abdominal ultrasound or CT scan Treatment • Antibiotic coverage of enteric bacteria • Biliary drainage by ERCP within 24-48 hr AMS = altered mental status; ERCP = endoscopic retrograde cholangiopancreatography;RUQ = right upper quadrant. An anion gap metabolic acidosis commonly occurs from lactic acidosis with severe sepsis. Very high levels of ALP (vs Acute Cholecystitis) GIT 496 https://t.me/usmleinnercircle irc le Version 2 rC Common bile duct dilation in Acute cholangitis  What surgical procedure is preferred for biliary drainage for patients with acute cholangitis? ERCP with sphincterotomy ne other options include percutaneous transhepatic cholangiography and open surgical decompression In Primary Sclerosing Cholangitis Primary sclerosing cholangitis • Asymptomatic • Fatigue & pruritus LE Clinical features • Associated with IBD, particularly UC (-90% of patients) Laboratory/imaging • Cholestatic liver injury (i alkaline phosphatase, j bilirubin) • Multifocal stricturing/dilation of intrahepatic &/or extrahepatic bile U SM ducts on cholangiography Liver biopsy Complications • Fibrous obliteration of small bile ducts, with concentric replacement by connective tissue in onion-skin pattern • Biliary stricture • Cholangitis or cholelithiasis • Cholangiocarcinoma, colon cancer, biliary cancer • Cholestasis (eg, t fat-soluble vitamins, osteoporosis) IBD = inflammatorybowel disease; UC = ulcerativecolitis. Not curative: Liver Transplant  What is the best diagnostic test for patients with primary sclerosing cholangitis? MRCP magnetic resonance cholangiopancreatography) or ERCP; MRCP is imaging technique using MRI to visualize the biliary and pancreatic ducts GIT 497 https://t.me/usmleinnercircle Version 2 Biopsy is not necessary for diagnosis but will show a "onion-skin" pattern. Biopsy will not be the answer Primary sclerosing cholangitis What is the likely diagnosis in a 42 y.o. male with abdominal pain, bloody stools, and fatigue? Labs show elevated alkaline phosphatase. IBD Ulcerative colitis) with primary sclerosing cholangitis Primary Biliary Cholangitis GIT 498 https://t.me/usmleinnercircle Version 2 Primary biliary cholangitis Pathogenesis • Autoimmune destruction of intra hepatic bile ducts • Affects middle-age women • Insidious onset of fatigue & pruritus Clinical features • Progressive jaundice, hepatomegaly, cirrhosis • Cutaneous xanthomas & xanthelasmas • Cholestatic pattern of liver injury ( f alkaline phosphatase, aminotransferases) • Antimitochondrial antibody • Severe hypercholesterolemia • Ursodeoxycholic acid (delays progression) Treatment irc • Liver transplantation for advanced disease le Laboratory findings • Malabsorption, fat-soluble vitamin deficiencies Complications • Metabolic bone disease (osteoporosis, osteomalacia) rC • Hepatocellular carcinoma Metabolic bone disease seen in patients with PBC may be due to the inhibition of osteoblast activity by a retained toxin rather than vitamin D malabsorption In ne PBC is commonly associated with severe hyperlipidemia (elevation of HDL out of proportion to LDL, hence the xanthelasmas  The diagnosis primary biliary cholangitis requires 2 out of 3 of the following: 1. Positive anti-mitochondrial antibodies LE 2. Elevated alk-phosphatase 3. Liver biopsy revealing destruction of intrahepatic bile ducts U SM Post-cholecystectomy syndrome Complications of cholecystectomy • Injury to bile ducts or the hepatic artery (especially with accidental incorrect clipping) • Gallbladder fistulae • Hemorrhage • Subhepatic abscess • Postcholecystectomysyndrome • Description: persistent abdominal pain or new symptoms following gallbladder removal • Frequency: 10-15% of patients • Etiology: both biliary (e.g, biliary injury, retained cystic duct, sphincter of Oddi dysfunction) and extrabiliary causes (e.g., irritable bowel syndrome, pancreatitis) have been identified c;;::i • Clinical features: abdominal pain and upper GI tract (e.g., dyspepsia) or lower GI tract (e.g., diarrhea) symptoms • Diagnosis: ultrasound or CT scan followed by ERCP(preferred test if intervention is planned) or MRCP • Treatment: depends on the identified cause, but may involve endoscopic sphincterotomy (e.g., in patients with sphincter of Oddi dysfunction) or transduodenal sphincteroplasty GIT 499 https://t.me/usmleinnercircle Version 2 Table 15.1 Postcholecystectomy syndrome (PCS) Etiology Features Residual stone in CBD Gallstone in cystic duct stump Dysfunction of the biliary tree Other This can lead to pancreatitis, cholangitis, or biliary obstruction Patients with anatomically longer cystic ducts are at risk for retained gallstones Increased pressure at the sphincter of Oddi can lead to impaired function of the biliary tree Gastritis, peptic ulcer disease  Patients who complain of persistent abdominal pain after cholecystectomy should be evaluated for post-cholecystectomy syndrome. Diagnosis involves ultrasound or CT followed by ERCP. evaluated via abdominal imaging (e.g. ultrasound) followed by direct visualization (e.g. ERCP, MRCP to find the causative factor • Sphincter of Oddi dysfunction (stenosis) produces recurrent, episodic pain in the right upper quadrant or epigastric region with elevations in AST/ALT. visualization of a dilated common bile duct in the absence of stones favors this diagnosis Dx: Sphincter of Oddi Manometry Gold Standard) Opioid analgesics (e.g. morphine): precipitate symptoms of sphincter of Oddi dysfunction Biliary leakage should be suspected in patients with persistent abdominal pain, fever, and right upper quadrant tenderness 210 days after laparoscopic cholecystectomy. Laboratory studies often demonstrate elevated liver function tests (especially bilirubin), with normal-appearing bile ducts (often with an intraabdominal fluid collection) on imaging. Pancreas  What is the first test or imaging study that should be ordered to diagnose pancreatitis in a patient with acute epigastric abdominal pain that radiates to the back? Serum amylase and lipase if amylase or lipase is  3x normal, may not need confirmatory imaging for diagnosis Order amylase and lipase to confirm diagnosis, then do imaging to identify the etiology (i.e. gallstones)  What imaging modality is recommended to evaluate patients with suspected gallstone pancreatitis? Ultrasound RUQ CT is not as sensitive as US for detecting gallstones  What imaging modality is preferred for the diagnosis of acalculous cholecystitis? Ultrasound GIT 500 https://t.me/usmleinnercircle Version 2 abdominal CT scan or HIDA scans are more sensitive and specific; use of US is unclear  What imaging modality is most useful for diagnosing acute pancreatitis if laboratory testing is unclear? Contrast-enhanced CT scan nearly 30% of patients have an ileus with bowel gas that prevents ultrasound from fully visualizing the pancreas le Acute pancreatitis • Chronic alcohol use (-40%) • Gallstones (-40%)Both comprise 2/3 cases • Hypertriglyceridemia 15% • Drugs (eg, azathioprine, valproic acid, thiazides) • • Iatrogenic (post-ERCP, ischemic/atheroembolic) Infections (eg, CMV, Legionella, Aspergil/us) Diagnosis (requires 2 of the follow) rC • Acute epigastric pain radiating to the back irc Etiology • Amylase or lipase >3 limes normal limit Clinical presentation • Abnormalities on imaging consistent with pancreatilis Other findings ne • ALT level >150 U/L suggests gallstone pancreatitis • Severe disease: fever, tachypnea, hypoxemia, hypotension Idiopathic Gall stone Etoh Trauma Steroids Mumps and other viruses Autoimmune ds stiing bite Hyper trigliseridemia/ calcemia ERCP Drugs: furosemide, In ALT= alanine aminotransferase;CMV = cytomegalovirus;ERCP = endoscopicretrogradecholangiopancreatography . I Get Smashed  Patient with RUQ pain radiating to back, hypertension controlled with thiazides. Hypercalcemia causing pancreatitis LE • HyperCa+ leads to secretory block in pancreatic duct • Hypercalcemia can cause pancreatitis but pancreatitis can cause hypocalcemia d/t saponificaiton, thus predisposing hypercalcemia may be missed U SM  Boy was riding bike and fell, high amylase/lipase → traumatic pancreatitis • NPO w/pain control and IV fluids • Bad prognostic indications: increased BUN, low Ca, high WBC, low Hct, high LDH Glasgows prognostic indicators CLASGOW Ca< 8 LDH> 600 Age> 55 S.albumin< 2.8 Glucose>180 PaO2<60 Wbc>16k urea>16 GIT 501 https://t.me/usmleinnercircle Version 2 Acute pancreatitis Drugs associated with drug-induced pancreatitis • Acetaminophen Analgesics • Nonsteroidal anti-inflammatory drugs • Mesalamine, sulfasalazine . • Opiates Antibiotics lsoniazid • Tetracyclines • Metronidazole • Trimethoprim-sulfamethoxazole Antiepileptics Antihypertensives Antivirals lmmunosuppressants Others • Valproic acid • Carbamazepine • Thiazides, furosemide • Enalapril, losartan • Lamivudine • Didanosine • Azathioprine, mercaptopurine • Corticosteroids • Asparaginase • Estrogens • Diuretics, including thiazides (eg, hydrochlorothiazide) and most loop diuretics (eg, furosemide), are among the most common offenders. These drugs may trigger pancreatitis via hypersensitivity to the sulfonamide molecule, pancreatic ischemia due to reduced blood volume, and/or increased viscosity of pancreatic secretions. Acute pancreatitis from uncorrectable causes (eg, ischemia, atheroembolism) can be conservatively managed with analgesics and intravenous fluids. • Infected pancreatic necrosis should be considered in patients with acute pancreatitis who have persistent abdominal pain or have initial improvement followed by clinical deterioration (eg, leukocytosis, fever). CT scan showing gas within pancreatic necrosis is diagnostic. Management includes intravenous antibiotics. Severe Acute Pancreatitis Initial management requires the use of intravenous antibiotics capable of penetrating pancreatic necrosis (eg, meropenem, fluoroquinolone + metronidazole  What is the cause of hypotension in severe acute pancreatitis? Increased vascular permeability release of activated pancreatic enzymes and inflammatory mediators leads to widespread vasodilation and vascular permeability Acute pancreatitis can produce multiple organ dysfunction syndrome MDOS/SIRS/shock GIT 502 https://t.me/usmleinnercircle Version 2 Predictors . . . . . Acute pancreatitis with organ failure (eg, res12iratoi:y, cardiovascular, renal} persisting >48 hr Patient factors: 0 Older age (eg, age >55) 0 Comorbidities, including obesity (BMI >30 kg/m2 ) Clinical findings: 0 Altered mental status 0 SIRS (eg, leukocytes >12,000/mm3 , temperature >38 C [100.4 Fl) Laboratory findings of intravascular volume depletion: 0 t BUN (>20 mg/dl) and/or j creatinine (>1.8 mg/dl) 0 j HCT(>44%) Radiologic findings: 0 CXR: pulmonary infiltrates, pleural effusions 0 Abdominal CT scan: severe pancreatic necrosis le Definition Severe acute pancreatitis Clinical features of severe pancreatitis rC • Fever, tachycardia, hypotension irc BUN = blood urea nitrogen; CXR = chest x-ray; HCT = hematocrit; SIRS = systemic inflammatory response syndrome. • Dyspnea, tachypnea &/or basilar crackles Clinical presentation • Abdominal tenderness &/or distension • Cullen sign: periumbilical bluish coloration indicating hemoperitoneum • Age >75 • Obesity • Alcoholism ne • Grey Turner sign: reddish-brown coloration around flanks indicating retroperitoneal bleed • C-reactive protein >150 mg/dl at 48 hr after presentation pancreatitis • Rising blood urea nitrogen & creatinine in the first 48 hr In Associated with j risk of severe • Chest x-ray with pulmonary infiltrates or pleural effusion • CT scan/magnetic resonance cholangiopancreatography with pancreatic necrosis & extra LE pancreatic inflammation • Pseudocyst • Peripancreatic fluid collection U SM Complications • Necrotizing pancreatitis • Acute respiratory distress syndrome • Acute renal failure • Gastrointestinal bleeding Pancreatic Pseudocyst GIT 503 https://t.me/usmleinnercircle Version 2 Pancreatic pseudocyst  Pseudocysts are mature walled-off pancreatic fluid collections (usually no necrosis or solid material) surrounded by a thick fibrous capsule and containing enzyme-rich fluid, tissue, and debris. They can leak amylase-rich fluid into the circulation and increase serum amylase. Complications include spontaneous infection, duodenal or biliary obstruction, pseudoaneurysm (due to digestion of adjacent vessels), pancreatic ascites, and pleural effusion. T/t: Asymptomatic: expectant management (eg, symptomatic therapy, NPO. Symptomatic: Endoscopic drainage Pancreatic Cyst  Most pancreatic cysts are benign and can be managed conservatively (eg, surveillance imaging)  Cysts with higher risk for malignancy: • Large size 3 cm) • Solid components or calcifications • Main pancreatic duct involvement (ie, ductal dilation) • Thickened or irregular cyst wall Require further evaluation with endoscopic ultrasound–guided biopsy and possibly surgical resection. Chronic Pancreatitis  What is the likely diagnosis in a patient with a history of alcohol use and chronic, intermittent epigastric abdominal pain that is relieved by leaning forward? GIT 504 https://t.me/usmleinnercircle Version 2 Chronic pancreatitis other common symptoms include steatorrhea, weight loss, and diabetes Overviewof chronic pancreatitis • Alcohol use • Cystic fibrosis (common in children) Etiology • Autoimmune Clinical presentation irc • Chronic epigastric pain with intermittent pain-free intervals le • Ductal obstruction (eg, malignancy, stones) • Malabsorption-steatorrhea, weight loss • Diabetes mellitus • Amylase/lipase can be normal & nondiagnostic • CT scan or MRCP can show calcifications, dilated ducts & enlarged pancreas rC Laboratory results/imaging • Pain management • Alcohol & smoking cessation Treatment ne • Frequent, small meals In • Pancreatic enzyme supplements Management of chronic pancreatitis .. . • Alcohol & tobacco abstinence Frequent, small meals Pancreatic enzyme supplements LE Pain Nonsteroidal anti-inflammatory drugs • Opioids .. . .. Low-fat diet Pancreatic enzyme supplements Fat-soluble vitamin supplements U SM Malabsorption Diabetes Metformin Insulin The presence of pancreatic calcifications on abdominal imaging helps establish a diagnosis of chronic pancreatitis. In patients with pancreatic insufficiency, pancreatic enzyme deficiency leads to the release of high volumes of CCK, resulting in a loop of pancreatic hyperstimulation. In association with pancreatic inflammation and ischemia, this results in the characteristic postprandial abdominal pain common in patients with chronic pancreatitis. GIT 505 https://t.me/usmleinnercircle Version 2 Pancreatic enzyme supplementation, which typically contains lipase, protease, and amylase, helps alleviate pain by reducing pancreatic hyperstimulation • Pancreatic ascites is a rare complication of chronic pancreatitis that results from damage to the pancreatic duct, leading to leakage of pancreatic juice into the peritoneal space serosanguinous or straw-colored ascitic fluid with analysis showing high amylase (often 1000 U/L • Pancreatogenic diabetes mellitus is caused by alpha- and beta-cell destruction; chronic pancreatitis is a risk factor. As in other types of diabetes mellitus, catabolic symptoms (eg, weight loss, frequent urination) and hyperglycemia suggest severe insulin deficiency and necessitate insulin. Medications targeting gastrointestinal peptides (eg, dipeptidyl peptidase-4 inhibitors, glucagonlike peptide-1 agonists) increase the risk for pancreatitis and should be AVOIDED. Spleen Clinical features of splenic abscess .. • Infection (eg, infective endocarditis) with hematogenous spread Risk factors Hemoglobinopathy (eg, sickle cell disease) lmmunosuppression (eg, HIV) • Intravenous drug use • Trauma • Classic triad of fever, leukocytosis & left upper-quadrant abdominal pain • Left-sided pleuritic chest pain with left pleural effusion commonly seen Clinical presentation • Possible splenomegaly Treatment . • . Most commonly due to Staphylococcus, Streptococcus & Salmonella Usually diagnosed by abdominal computed tomography scan Combination of broad-spectrum antibiotics & splenectomy • Possible percutaneous drainage in poor surgical candidates  What is the likely diagnosis in a patient with recently diagnosed infective endocarditis that now presents with fever and left-sided chest/abdominal pain? CT reveals a left-sided pleural effusion and splenomegaly with a splenic fluid collection. Splenic abscess (secondary to IE splenic abscess usually presents with a triad of fever, leukocytosis, and LUQ abdominal pain; treatment is with antibiotics plus splenectomy Small Intestine Malabsorption Syndrome GIT 506 https://t.me/usmleinnercircle Version 2 Chronic pancreatitis .. .. . Celiac disease .. Lactose intolerance Malabsorption syndromes Diarrhea after lactose-containing meals i Stool osmotic gap L Stool pH + Lactose hydrogen breath test Greasy stools • Abdominal pain radiating to back i Stool osmotic gap Microcytic anemia, iron deficiency .. Macrocytic anemia, B 12 deficiency + Lactulose breath test irc Small intestinal bacterial overgrowth le • Villous atrophy D-Xylose Test rC D-xylose is a monosaccharide that can be absorbed in the proximal small intestine without degradation by pancreatic or brush border enzymes.  A false-positive D-xylose test (ie, low urinary D-xylose level despite normal mucosal absorption) can be seen in the following: ne • Delayed gastric emptying • Impaired glomerular filtration In • Small intestinal bacterial overgrowth SIBO, characterized by alterations in small intestinal flora (due to abnormal intestinal anatomy or motility), leading to bacterial fermentation of the D-xylose IBS LE before it can be absorbed. U SM Clinical features of irritable bowel syndrome Rome IV diagnostic criteria Alarm features Recurrent abdominal pain/discomfort ;:,,1day/week for past 3 months & ;:,,2of the following: • Related to defecation (improves or worsens) • Change in stool frequency • Change in stool form • Older age of onset (;:o,50) • Gastrointestinal bleeding • Nocturnal diarrhea • Worsening pain • Unintended weight loss • Iron deficiency anemia • Elevated C-reactive protein • Positive fecal lactoferrin or calprotectin • Family history of early colon cancer or IBD IBD = inflammatory bowel disease. GIT 507 https://t.me/usmleinnercircle Version 2 • IBS with diarrhea: o Stool cultures o Celiac disease screening Evaluation o 24-hr stool collection o Colonoscopy or flexible sigmoidoscopy& biopsy • IBS with constipation: o Radiography o Flexible sigmoidoscopy& colonoscopy Evaluation of suspected irritable bowel syndrome Abdominal pain .e1days/week • ;,3 months and .e2of the following: • linked to defecation • Change in stool frequency • Change in stool form & Plus lack of alarm symptoms· Constipation predominant Diarrhea predominant ! •CBC • Stool culture • Celiac disease serologic testing • CRP or fecal calprotectin/lactoferrin Normal ] , Treat for IBS-C J , Colonoscopy ] Abnormal ! ! Treat for IBS-D ] • Treat underlying cause • Additional evaluation if needed (eg, colonoscopy) 'Alarm symptoms· •Age >50 • Rectal bleeding/melena • Fasting diarrhea (eg, nocturnal) • vvorsen1ng abdominalpain • Familyhistoryof inflammatory boweldiseaseor colorectalcancer CBC = completebloodcount:CRP = C-reactiveprotein,IBS-C = constipation-predominant Irritablebowelsyndrome, 18S-D= diarrhea-predominant irritablebowelsyndrome Abdominal pain Diarrhea Constipation . . .. .. C)UWorld Pharmacologic management of IBS First line: antispasmodic therapy (eg, hyoscyamine) as needed Second line: tricyclic antidepressants (eg, amitriptyline)* First line: soluble fiber (eg, psyllium), loperamide Second line: bile acid sequestrants (eg, cholestyramine), serotonin-3 receptor antagonists (eg, alosetron) First line: soluble fiber (eg, psyllium), osmotic laxatives (eg, PEG) Second line: lubiprostone, guanylate cyclase agonists (eg, linaclotide), sodium/hydrogen exchanger 3 inhibitor (eg, tenapanor) *Preferred for IBS with diarrhea; avoid with constipation. IBS = irritable bowel syndrome; PEG = polyethylene glycol. GIT 508 https://t.me/usmleinnercircle Version 2 Due to Smooth Muscle Hypersensitivity to Cholecystokinin  For IBSC, fiber supplementation improves constipation and may reduce abdominal pain. IBSD is usually treated with antidiarrheal agents such as loperamide. Patients with IBSD should be screened for celiac disease because both disorders may le present with abdominal pain, diarrhea, and bloating; screening is not indicated in IBSC. irc • Colonoscopy is recommended in patients with alarm features to evaluate for malignancy or inflammatory bowel disease rC IBD Classification & management of mild ulcerative colitis • <4 watery bowel movements per day • Hematochezia is rare or intermittent No anemia Laboratory findings • • Normal ESR & CRP • 5-Aminosalicylic acid agents (eg, mesalamine, Treatment ne Clinical features sulfasalazine) In CRP = C-reactive protein; ESR = erythrocyte sedimentation rate. LE Smoking is a major risk factor that likely contributes to disease flare-ups in Crohns. U SM Might be protective in UC. Use Glucocorticoids for immediate improvement of moderate to severe Crohnʼs. Biological agents weeks to works. • Pregnancy is a high-risk period for patients with UC due in part to placental cytokines that worsen colonic inflammation. Maternal complications include worsening UC disease activity, which can lead to severe hematochezia, anemia, and, in rare cases, toxic megacolon. Fetal risks include preterm delivery and small for gestational age. In addition, fertility rates are lower in those with active UC.  Most medications used in the management of UC (eg, mesalamine, sulfasalazine, TNF a inhibitors) are considered safe for continuation throughout pregnancy GIT 509 https://t.me/usmleinnercircle Version 2 can also be continued during breastfeeding (except salfasalazine) Celiac Disease Features of malabsorption in celiac disease Nutrients Symptoms General Bulky, foul-smelling, floating stools Loss of muscle mass, loss of subcutaneous fat, fatigue Fat & protein Iron Pallor (anemia), fatigue Calcium & vitamin D Bone pain (osteomalacia), fracture (osteoporosis) Vitamin K Easy bruising Vitamin A Hyperkeratosis Clinical manifestations of celiac disease • Diarrhea, ± steatorrhea, weight loss Gastrointestinal Mucocutaneous Endocrine Bone disorders Hematologic Neuropsychiatric GIT • Abdominal pain • Flatulence/bloating • Late manifestations: ulcerative jejunitis, enteropathy-associated T-cell lymphoma • Dermatitis herpetiformis • Atrophic glossitis • Vitamin D deficiency • Secondary hyperparathyroidism • Osteomalacia/osteoporosis (adults) • Rickets (children) • Iron deficiency anemia • Peripheral neuropathy • Depression/anxiety 510 https://t.me/usmleinnercircle Version 2 Celiac disease • First-degree relative with celiac disease Risk factors • Down syndrome • Autoimmune disorders (eg, type 1 diabetes mellitus, autoimmune thyroiditis) Classic • ± Abdominal pain, distension, bloating, diarrhea symptoms • General: failure to thrive/weight loss, short stature,* delayed puberty/menarche* • Oral: enamel hypoplasia, atrophic glossitis Extraintestinal • Dermatologic: dermatitis herpetiformis manifestations • Hematologic: iron deficiency anemia (due to malabsorption) (may be sole le • Neuropsychiatric: peripheral neuropathy, mood disorders (eg, anxiety, presentation) depression) • j Tissue transglutaminase lgA antibody Diagnosis irc • Musculoskeletal: arthritis, osteomalacia/rickets* (due to vitamin D malabsorption), • Proximal intestinal biopsy (villous atrophy, crypt hyperplasia, intraepithelial lymphocytosis) • Gluten-free diet Treatment rC • Dapsone for dermatitis herpetiformis *Pediatric findings. . Nutritional deficiencies . .. . . Dietary counseling with a dietician Monitor lifelong adherence to gluten-free diet Access to an advocacy group Most common deficiencies are iron, calcium, vitamin D, folic acid & rarely thiamine Increased risk for osteopenia & osteoporosis Obtain DXA at diagnosis LE Prevention of bone loss .. Management of celiac disease In Gluten-free diet/education ne Patients with celiac disease commonly report fatigue and foul-smelling, greasy diarrhea. Vaccination . Pneumococcal vaccination Dapsone in addition to gluten-free diet U SM Dermatitis herpetiformis Repeat DXA 1 year later if osteoeenia is eresent Pneumococcal vaccine due to associated hyposplenism.  The presence of IgA anti-endomysial and anti-tissue transglutaminase antibodies are highly predictive of celiac disease. absence does not rule out celiac disease as many patients have concurrent selective IgA deficiency. In such cases use IgG antibodies for diagnosis.  In children, a high degree of suspicion must be maintained because the main presenting sign of celiac disease may be growth delay (eg, weight loss, poor linear velocity). Growth delay can occur in the absence of diarrhea, which is present in only two-thirds of patients.  In some patients, extraintestinal symptoms with no gastrointestinal features may be the sole presentation. GIT 511 https://t.me/usmleinnercircle Version 2  Dermatitis herpetiformis is another extraintestinal manifestation and presents as grouped pruritic papules or vesicles(eg, fluid-filled) typically on extensor surfaces (eg, elbows, knees). Symptoms typically respond to a gluten-free diet; dapsone should also be given for acute management of dermatitis herpetiformis SIBO Small intestinal bacterial overgrowth • Anatomic abnormalities (eg, strictures, surgery, small bowel diverticulosis) Risk factors Clinical manifestations Diagnosis Treatment • Motility disorders (eg, diabetes mellitus, scleroderma, opioid use) • Immunodeficiency (eg, lgA deficiency) • Chronic pancreatitis • Gastric hypochlorhydria, proton pump inhibitor use • Bloating, flatulence • Chronic watery diarrhea • Possible malabsorption • i Vitamin B12 (bacterial consumption), j folate (bacterial synthesis) • Carbohydrate breath testing (lactulose or glucose) • Endoscopy with jejunal aspirate/culture • Oral antibiotics (eg, rifaximin, ciprofloxacin, doxycycline) • Do NOT consider SIBO in patients with regular bowel movement • Carbohydrate breath test: a dose of fermentable carbohydrate (ie, lactulose, glucose) is taken orally, and breath samples for hydrogen and methane (produced by gut bacteria) are taken at regular intervals. Normally, colonic bacteria ferment any nonabsorbed substrate after 23 hours; in contrast, an earlier rise in breath hydrogen (at 1.5 hours) suggests fermentation in the small bowel, indicating SIBO. Appendicitis  What is the preferred imaging modality for diagnosis of acute appendicitis during pregnancy? Ultrasound (graded compression technique) if ultrasound is non-diagnostic, MRI can be performed for further assessment GIT 512 https://t.me/usmleinnercircle Version 2 Management of suspected appendicitis Suspected appendicitis (eg, modified Alvarado score• ~4) [ Perform diagnostic imaging ] I Child or pregnant female? I No ! Yes 1 Evaluate for other diagnoses Nonperforated Management depends on specific modified Alvarado score Antibiotics & appendectomy S12 hr •Modified Alvarado score appercitis Perforated appendicitis le 'T" Nonvisualized appendix (ie, nondiagnostic) ! Antibiotics & bowel rest; PCD (contained abscess) vs l&D with appendectomy (diffuse contamination) rC Normal US±MRI irc Abdominopelvic CT scan 1 point each: migratory RLQ pain, anorexia, nausea or vomiting, RLQ rebound tenderness, fever >37.5 C (99.5 F). 2 points each: RLQ tenderness, leukocytes >10,000/mm3. ne l&D = irrigation & drainage; PCD = percutaneous drainage; RLQ = right lower quadrant; US = ultrasound. CUWo<ld to-upper quadrant or right flank. LE may mimic Cholecystitis pain In  During pregnancy, the appendix undergoes cephalad displacement by the gravid uterus; therefore, with increasing gestational age, patients may have an atypical presentation of pain in the right mid-  What is the initial management for patients with an appendiceal abscess? IV hydration, antibiotics, and bowel rest U SM appendectomy should not be performed right away (may return in 68 weeks for elective appendectomy, though this is controversial) • Infections are the most common complication of appendectomy, and risk of intra-abdominal abscess (e.g. Sub-phrenic Abscess) is significantly greater with laparoscopic appendectomy than laparotomy. Manifestations typically include recurrent fever and abdominal symptoms (eg, pain, vomiting) several days after an abdominal operation GIT 513 https://t.me/usmleinnercircle Version 2 Examination signs of appendicitis Sign Findings . . . Peritoneal signs Rebound tenderness Involuntary guarding Abdominal rigidity Psoas sign Obturator sign Rovsing's sign Rectal tenderness .. . . . . . Significance Acute increase in pain after removing the hand from applying pressure Tensing of abdominal wall muscles during palpation of abdomen Persistent tension of abdominal wall muscles Peritoneal irritation (rupture or impending rupture) RLQ pain with extension of right thigh Abscess adjacent to psoas or retrocecal appendix RLQ pain with internal rotation of right thigh Pelvic appendix or abscess RLQ pain with LLQ palpation & retropulsion of colonic contents Acute appendicitis Right pelvic pain during rectal examination, especially with pressure on the right rectal wall Pelvic appendix or abscess Infectious ileocecitis (Pseudo-appendicitis) Campy/obacter gastroenteritis Epidemiology Clinical features Treatment Complications . .. .. .. Most commonly transmitted via undercooked poultry Fever, abdominal pain, diarrhea (mucoid ± blood) Pseudoappendicitis (RLQ pain due to acute ileocecitis) Supportive care (symptoms usually self-limited <7 days) Antibiotics only in severe or high-risk cases• Guillain-Barre syndrome Reactive arthritis *Duration >7 days; bloody stools; high fevers; patients who are pregnant, immunocompromised,or elderly. RLQ = right lower quadrant. T/t: Amoxicillin;l if severe D/D  Appendicitis Mild diarrhoea vs Profuse and Mucoid. Presence of other sick contact  Cecal diverticulitis: Mild diarrhoea can occur, profuse or mucoid diarrhoea is not seen. In addition, diverticulitis is exceedingly rare in children Obstruction GIT 514 https://t.me/usmleinnercircle Version 2 Small bowel obstruction irc le • Colicky abdominal pain, vomiting Inability to pass flatus or stool if complete (no obstipation if partial) Clinical presentation • • Hyperactive -----+absent bowel sounds • Distended & tympanitic abdomen • Dilated loops of bowel with air-fluid levels on plain film or CT scan Diagnosis • Partial: air in colon • Complete: transition point (abrupt cutoff), no air in colon • lschemia/necrosis (strangulation) Complications • Bowel perforation • Bowel rest, nasogastric tube suction, intravenous fluids Management • Surgical exploration for signs of complications rC Can be a complication of Crohns Disease  What is the initial step in the management of small-bowel obstruction that is complicated by fever, hypotension, and tachycardia? ne Urgent surgical exploration  What is the most common cause of small-bowel obstruction SBO in the U.S? Adhesions In typically result from abdominal operations or inflammatory processes Can be congenital Ladd Bands) in children No LE Is post-operative ileus characterized by a clear transition point on abdominal X-ray? U SM both small and large bowel dilation GIT 515 https://t.me/usmleinnercircle Version 2 post-operative ileus Small bowel obstruction vs ileus Small bowel obstruction Etiology Abdominal examination . .. Prior surgery (weeks to years) Distension Increased bowel sounds .. . .. lleus Recent surgery (hours to days) Metabolic (eg, hypokalemia) Medication induced Possible distension Reduced/absent bowel sounds Small bowel dilation Present Present Large bowel dilation Absent Present SBO vs ileus SBO • Nausea, vomiting Obstipation Clinical features • • Nausea ± vomiting • No flatus • Acute abdomen • Abdominal distension • Hyperactive or absent bowel sounds • Decreased or absent bowel sounds • Air fluid levels X-ray findings lleus . No transition point • Dilated proximal bowel, collapsed distal bowel • Dilated loops of bowel • Air in colon/rectum • Little/no air in colon/rectum SBC = small bowel obstruction. GIT 516 https://t.me/usmleinnercircle Version 2  What class of analgesics may contribute to prolonged post-operative ileus? Opiates • Paralytic ileus is most commonly a complication of abdominal surgery but can also be seen in other conditions such as retroperitoneal/abdominal hemorrhage, intraabdominal inflammation (eg, pancreatitis), intestinal ischemia, and electrolyte abnormalities. Evaluation of minimal bright red blood per rectum Age 4049 without red flags Age <40 without red flags Sigmoidoscopy or colonoscopy 11 No source _ identified No further evaluation necessary ©UWor1d ne Hemr~s Colonoscopy *Red flags = change in bowel habits, albdominal pain, weight loss, iron deficiency anemia, family history of colon cancer. In I Age 2'50 or red flags* rC I irc Minimal bright red blood per rectum le Large Intestine & Rectum LE What is the next step in management for an elderly patient with newly discovered iron deficiency anemia? colonoscopy and endoscopy U SM new IDA in an elderly patient is considered to be from GI blood loss until proven otherwise GIT 517 https://t.me/usmleinnercircle Version 2 Rectal prolapse External hemorrhoid Internal hemorrhoid Perianal abscess Anogenital wart Anorectal cancer Skin tags .. .. . .. .. . .. . .. . .. Anal & perianal masses Erythematous mass with concentric rings that occurs with Valsalva Mucus discharge, mild abdominal pain, mass sensation Dusky/purple lump or polyp Associated itching, bleeding Thrombosis: acute enlargement with pain Intermittent itching, painless bleeding, leakage of stool Detected with digital rectal exam or anoscopy (unless prolapsed) Fluctuant mass/swelling with erythema Fever Gradual onset Pink or flesh-colored pa pules, plaques, or cauliflower-shaped masses Chronic onset Mild itching, bleeding Squamous cell carcinoma most common Bleeding, pain Ulcerating, enlarging mass Small, flesh-colored papules May represent external terminus of anal fissure (sentinel tag) Hemorrhoids Pathophysiology Risk factors Presentation Management GIT .. .. .. . . . External hemorrhoids Hemorrhoidal venous plexus distension lntravascular inflammation, which leads to hemorrhoid thrombosis Constipation, low-fiber diet, prolonged sitting Pregnancy, advanced age Anal pruritus, bleeding, discharge of mucus, fecal incontinence, sensation of fullness in anorectum Thrombosis (severe anorectal pain) Purple or blue bulge on examination Conservative management: 0 High-fiber diet, stool softeners, sitz baths 0 Topical analgesics, anti-inflammatories & antispasmodics (eg, lidocaine, glucocorticoid suppositories, nitroglycerin cream) Refractory or thrombosed hemorrhoids: 0 Conservative measures when mild 0 Hemorrhoidectomy when severe 0 Hemorrhoid incision with thrombus removal for temporary relief 518 https://t.me/usmleinnercircle Version 2 Initial management of hemorrhoids .. .. .. Dietary factors . .. . Behavioral factors Increased fiber intake (foods, fiber supplements) Reduced fat intake Moderation of alcohol intake Limit time sitting on toilet (eg, 3 minutes) Limit defecation to once daily Avoid straining during defecation Analgesics (eg, benzocaine) Astringents (eg, witch hazel) Hydrocortisone le Topical agents Increased fluid intake irc • Infrared coagulation, rubber band ligation, and sclerotherapy are all used to treat symptomatic nonthrombosed internal hemorrhoids that fail conservative management. rC These techniques are avoided with external hemorrhoids because their location below the dentate is highly innervated and these procedures would cause severe pain. Diverticulitis & Diverticulosis Diagnosis Management • Abdominal pain • Nausea/vomiting, altered bowel movements • Fever, leukocytosis • Possible sterile pyuria • CT scan of the abdomen (oral & intravenous contrast) • Bowel rest • Antibiotics (eg, ciprofloxacin, metronidazole) In Clinical presentation ne Clinical features of acute diverticulitis LE • Colonoscopy 6-8 weeks after resolution Complications U SM • Other Signs: • Abscess, obstruction, fistula, perforation  A palpable, tender mass(from focal inflammation)  Dysuria, or urinary urgency or frequency due to bladder irritation from an adjacent inflamed sigmoid colon. GIT 519 https://t.me/usmleinnercircle Version 2 Acute diverticulitis • Acute (left) lower abdominal pain & tenderness • CT scan: focal bowel wall thickening, t visible diverticula No high-risk features or complications High risk features: •Age>70 • Comorbidities • lmmunosuppression • Sepsis/SIRS Outpatient management • Oral fluids • t Oral antibiotics • Close follow-up I Inpatient management • Bowel rest • IV fluids • IV antibiotics Complications i Abscess ] 1 Perforation, obstruction, fistula IV antibiotics t percutaneous drainage • IV antib1ot1cs • Surgery IV= intravenous;SIRS= syslemic lnflammalory response syndrome (le, significantfever, tachycardia, tachypnea, lelA<ocytosis) • CT Scan: Focal bowel wall thickening and inflammatory signs (eg, stranding) in pericolonic fat.  What is the next step in management of a patient with diverticulitis complicated by an abscess  4 cm? CT-guided percutaneous drainage surgical drainage can be attempted if percutaneous drainage fails  Small abscesses 4 cm) often respond to IV antibiotic therapy alone without drainage  What is the most common cause of gross lower GI bleeding in adults? Diverticulosis  What dietary habits increase the risk of diverticulosis complications (e.g. hemorrhage, diverticulitis)? Heavy meat consumption, low fiber NSAIDs, obesity, and possibly smoking are also correlated with increased complications Angiodysplasia  What is the likely diagnosis for an adult with episodic, painless dark maroon-colored stool? Angiodysplasia typically right-sided (versus diverticulosis, which is typically left-sided with passage of bright red blood(arterial); anemic patients may be treated with cautery GIT 520 https://t.me/usmleinnercircle Version 2 Patients with angiodysplasia are more-likely to bleed if they have what other coexisting pathologies? End stage renal disease, vWD, Aortic stenosis Pathogenesis (low-yield but informative? • Aortic stenosis/AV replacement ⟶ shear stress cleaves vWF multimers ⟶ ↓ platelet activation ⟶ bleeding (Heyde's Syndrome) le • vWF normally suppresses angiogenesis ⟶ ↓ vWF  ↑ angiogenesis = angiodysplasia Diagnosis . .. Crohn disease Malignancy (colon, bladder, pelvic organs) Pneumaturia (air in urine) Fecaluria (stool in urine) Recurrent urinary tract infections (mixed flora) CT scan of the abdomen with oral or rectal (not intravenous) contrast Colonoscopy to exclude colonic malignancy In Toxic Megacolon Diverticular disease (sigmoid most common) rC Clinical presentation .. . .. ne Etiology Clinical features of colovesical fistula irc Colovesical Fistula C/ostridioides difficile colitis LE • Recent antibiotic use or hospitalization • Advanced age (>65) Risk factors • Gastric acid suppression (eg, PPI, H2 blocker) • Underlying inflammatory bowel disease U SM • Chemotherapy Clinical presentation • Profuse watery diarrhea • Leukocytosis (-15,000/mm 3) • Fulminant colitis or toxic megacolon • Stool PCR for C difficile genes* Diagnosis • Stool EIA for C difficile toxin & glutamate dehydrogenase antigen Infection control • Hand hygiene with soap & water • Contact isolation • Seoricidal disinfectants (eg, bleach) *Genes specific to toxigenic strains are assessed. EIA = enzyme immunoassay;H2 = histamine-2receptor; PPI = proton pump inhibitor. Usually watery diarrhea but CAN be bloody too ! GIT 521 https://t.me/usmleinnercircle Version 2 Pathophysiology Clinical features Diagnosis Treatment .. . • . . .. Toxic megacolon Colonic smooth muscle inflammation & paralysis Complication of IBD or infectious colitis j Risk with use of antimotility agents (eg, loperamide) or opioids Systemic toxicity (eg, fever, tachycardia, hypotension) Abdominal pain & distension following diarrheal illness Colonic dilation (>6 cm) on imaging Bowel rest/decompression, antibiotics Corticosteroids if IBD-associated • Surgery for perforation, peritonitis, clinical deterioration IBD = inflammatorybowel disease. Dx for Clostridium: Antigen Stool Test. The presence of C. difficile itself does not indicate infection as many individuals 815% are asymptomatic carriers. Treatment involves antibiotics and consideration of surgical resection or repair in the setting of bowel perforation. Confirm via X-ray. Do not perform enema or colonoscopy due to risk of toxic perforation. • Toxic megacolon is a medical emergency that requires nasogastric decompression, antibiotics, IV fluids +/- steroids (if IBD associated). Megacolon ie, colonic diameter 6 cm on abdominal x-ray,  What IBD complication presents with colonic dilation on X-ray and fever, tachycardia, leukocytosis, and/or anemia? Toxic megacolon may also have severe bloody diarrhea; highest risk of developing toxic megacolon is early on in the disease GIT 522 https://t.me/usmleinnercircle Version 2 Management of Clostridioides difficile infection Fulminant CDI Severe CDI + either. • Hypotension or shock • lieus or megacolon Severe CDI Symptoms + either. • Leukocytes >15,000/mm' • Serum creatinine >1.5 mg/dl l l Oral therapy• • Fidaxomicin ·Vancomycin Oral vancomycin.. and IV metronidazole l le Nonsevere CDI Classic symptoms: • Profuse watery diarrhea • Abdominal pain irc Refractory CDI • Consider fecal microbiota transplantation • Consider surgical intervention COi = C/ostndioides difficile infection:IV= intravenous. "Oralmetronidazole canbe considered fornonsevere CDI if fidaxomicin andvancomycin are unavailable . .:Consideracldmgvancomyanenemasif ileusis presenl CUW<><ld Not IV  C. diff is one of the rare indications for ORAL vancomycin (in C. difficile infections, however, intravenous rC administration is ineffective, as vancomycin is hardly excreted into the colon (the main site of infection) Antibiotics & Clostridioides difficile infection • Fluoroquinolones • Clindamycin • 3rd-14th-generation cephalosporins In • Carbapenems Low COAD risk ne High COAD risk • Monobactams • TMP-SMX • Macrolides • Tetracyclines • Aminoglycosides CDAD = Clostridioides difficile-associated disease; LE TMP-SMX = trimethoprim-sulfamethoxazole.  The diagnosis of C difficile–associated diarrhea CDAD requires both of the following: • Characteristic symptoms Watery diarrhea 3 loose stools in 24 hr) is the hallmark symptom of C U SM difficile infection; other classic symptoms include lower abdominal pain, low-grade fever, and leukocytosis. • Positive stool testing for toxigenic C difficile When characteristic symptoms are present, a positive test generally represents active infection (vs carrier status). Treatment with 10 days of oral fidaxomicin or vancomycin is generally curative, but recurrence occurs within 30 days of antibiotic cessation in approximately 25% of patients. Recurrence is marked by the reemergence of characteristic symptoms with positive C difficile stool testing. • Recurrence of Clostridioides difficile infection is common, especially in patients on prolonged antibiotics for other conditions. Recurrence is typically due to germination of persistent spores from the initial infection. GIT 523 https://t.me/usmleinnercircle Version 2 To reduce the risk of recurrence, treatment for CDI is often extended throughout the course of other antibiotic therapies. Colonic Pseudo-obstruction (Ogilvie Syndrome) Acute colonic pseudoobstruction (Ogilvie syndrome) Etiologies • Major surgery, traumatic injury, severe infection • Electrolyte derangement (! K, ! Mg, ! Ca) • Medications (eg, opiates, anticholinergics) • Neurologic disorders (eg, dementia, stroke) • Abdominal distension, pain, obstipation, vomiting Clinical findings • Tympanic to percussion, ! bowel sounds • If perforation: guarding, rigidity, rebound tenderness Imaging • X-ray: colonic dilation, normal haustra, nondilated small bowel • CT scan: colonic dilation without anatomic obstruction Management • NPO, nasogastric/rectal tube decompression • Neostigmine if no improvement within 48 hr Volvulus . Risk factors Presentation Imaging Management GIT . . . .. . . Sigmoid volvulus Sigmoid colon redundancy (eg, dilation/elongation from chronic constipation) Colonic dysmotility (eg, underlying neurologic disorder) Slowly progressive abdominal discomfort/distension ± obstructive symptoms (eg, nausea, emesis, obstipation) Abdomen distended & tympanitic to percussion X-ray: dilated, inverted, U-shaped loop of colon (coffee bean sign) CT scan: dilated sigmoid colon, mesenteric twisting (whirl sign) Endoscopic detorsion (eg, flexible sigmoidoscopy) & elective sigmoid colectomy Emergency sigmoid colectomy if perforation/peritonitis present 524 https://t.me/usmleinnercircle Version 2 CUWofld LE In ne rC Sigmoid volvulus irc le Cecal volvulus U SM  Cecal volvulus forms a closed-loop obstruction. With continued gas formation by intraluminal bacteria, the lumen of the obstructed bowel loop gradually expands. This leads to slowly progressive (eg, over 2 days) abdominal distension. Sigmoid Volvulus Caecal Volvulus M/c in old Younger T/t: Endoscopic Detorsion 1st line T/t: Emergency Laprotomy 1st line Ischemic Colitis GIT 525 https://t.me/usmleinnercircle Version 2 Colonic ischemia • Usually nonocclusive, "watershed" ischemia Pathophysiology • Underlying atherosclerotic disease • State of low blood flow (eg, hypovolemia) • Moderate abdominal pain & tenderness Clinical features • Hematochezia, diarrhea • Leukocytosis, lactic acidosis Diagnosis • CT scan: Colonic wall thickening, fat stranding • Endoscopy: Edematous & friable mucosa • Intravenous fluids & bowel rest Management • Antibiotics with enteric coverage • Colonic resection if necrosis develops  CT findings indicative of ischemic colitis include colonic wall thickening (edema) and fat stranding. also may have air (pneumatosis) in the bowel wall Microscopic Colitis Microscopic colitis Clinical findings Triggers Diagnosis Management • • Abdominal pain, fatigue, weight loss, arthralgias • Smoking, medications (eg, NSAIDs, PPls, SSRls) • Colonoscopic biopsy with lymphocytic infiltration of lamina propria Watery, nonbloody diarrhea; fecal urgency & incontinence 0 Collagenous: thickened subepithelial collagen band 0 Lymphocytic: high levels of intraepithelial lymphocytes • Remove possible triggers • Antidiarrheal medications & budesonide II NSAIDs= nonsteroidalanti-inflammatorydrugs; PPls = proton pump inhibitors;SSRls = selectiveserotoninreuptake inhibitors. GIT 526 https://t.me/usmleinnercircle Version 2 Proctalgia Fugax Proctalgia fugax • Spastic contraction of the anal sphincter • Pudenda! nerve compression • Female sex Pathophysiology • Other functional pathologies (eg, irritable bowel syndrome) Risk factors Manifestations Evaluation • Normal physical examination (eg, rectal, pelvic, prostate) Management • Reassurance irc • No laboratory abnormalities le • Psychosocial stress, anxiety • Recurrent rectal pain unrelated to defecation • Episodes lasting seconds to minutes (S30 min) • No pain between episodes Proctitis rC • Nitroglycerin cream ± biofeedback therapy for refractory symptoms Radiation proctitis Manifestations Management Diarrhea, mucus discharge, tenesmus Minimal bleeding Severe erythema Edema, ulcerations LE Endoscopic appearance .. .. .. Direct mucosal damage ne . Pathogenesis S8 weeks In . Postradiation onset Acute radiation proctitis Antidiarrheals (eg, loperamide) Butyrate enemas . .. .. .. .. Chronic radiation proctitis >3 months to years Obliterative endarteritis & chronic mucosal ischemia Submucosal fibrosis Severe bleeding ± Strictures with constipation & rectal pain Multiple telangiectasias Mucosal pallor & friability Endoscopic thermal coagulation Sucralfate or glucocorticoid enemas U SM Rectal Prolapse GIT 527 https://t.me/usmleinnercircle Version 2 Rectal prolapse • Women age >40 with history of vaginal deliveries/multi parity • Prior pelvic surgery Risk factors • Chronic constipation, diarrhea, or straining • Stroke, dementia • Pelvic floor dysfunction or anatomic defects • Abdominal discomfort Clinical presentation • Straining or incomplete bowel evacuation, fecal incontinence • Digital maneuvers possibly required for defecation • Erythematous mass extending through anus with concentric rings (full-thickness prolapse) or radial invaginations (non-fullthickness prolapse) • Medical Management 0 Considered for non-full-thickness prolapse 0 Adequate fiber & fluid intake, pelvic floor muscle exercises 0 Possible biofeedback therapy for fecal incontinence • Surgical 0 Preferred for full-thickness or debilitating symptoms (eg, fecal incontinence, constipation, sensation of mass) Rectal prolapse Mucosal prolapse Full thickness prolapse Anal sphincter ·;;: cu E w Protruding mucosa with radial invaginations Erythematous mass with concentric rings Anal Fissure GIT 528 https://t.me/usmleinnercircle Version 2 Anal fissures • Local trauma (eg, constipation, prolonged diarrhea, anal sex) • Inflammatory bowel disease (eg, Crohn disease) • Malignancy • Pain with bowel movements Clinical • Brighi red blood on toilet paper or stool surface presentation • Most common at posterior anal midline • Chronic fissure may have skin tag at distal end • High-fiber diet & adequate fluid intake • Stool softeners Treatment • Sitz baths • Topical anesthetics & vasodilators (eg, nifedipine, nitroglycerin) Anorectal fistula (fistula in ano) • Crohn disease Causes rC • Perianal abscess irc Anorectal Fistula le Etiology • Malignancy, radiation proctitis • Infection (eg, lymphogranuloma venereum) • Perirectal pain, discharge • Inflammatory papule/pustule • Palpable fistula tract ne Clinical manifestations • Assess extent of fistula In o Gentle probe Management 0 Imaging (endosonography, fistulogram, MRI) LE • Surgery (eg, fistulotomy) An anal fistula may present with recurrent perianal abscesses and signs and symptoms of fever, pain, and a fluctuant or indurated perianal mass. U SM Direct visualization and treatment are best accomplished with Anoscopy and Fistulotomy. Differentiated from Pilonidal tract disease by the presence of a true connection to the intestinal lumen. Anorectal Abscess GIT 529 https://t.me/usmleinnercircle Version 2 Etiology Risk factors Clinical presentation Management . .. . .. . .. . Anorectal abscesses Obstruction of anal crypt gland ----+bacterial overgrowth Constipation, anoreceptive intercourse Inflammatory bowel disease Diabetes mellitus, immunosuppression (eg, corticosteroids, HIV/AIDS) Pruritus, pain with defecation ----+severe, constant pain ± fever Superficial: visible erythematous area with tenderness, induration, ± fluctuance Deep: palpable area of induration or fluctuance on DRE Imaging (eg, CT scan) if concern for deep infection Prompt incision & drainage Systemic antibiotic therapy* if cellulitis, fever, or j risk for severe infection (eg, diabetes mellitus, immunosuppression) present *Consider in all patients;may t risk for fistula formation. DRE= digital rectal examination. Anorectal abscesses arise when one or more of the anal crypt glands become blocked, allowing for bacterial overgrowth. They vary in complexity based on their anatomic location: • Simple perianal abscesses are located within the dermis and subcutaneous tissue surrounding the anal orifice. 0 Because they are usually superficial and obvious on inspection as circumscribed, erythematous and tender masses, perianal abscesses can often undergo incision and drainage at the bedside. • Perirectal abscesses, in contrast, are located within the intersphincteric, supralevator, and ischiorectal spaces. Because of their deeper location, they frequently have no associated perianal skin changes; however, signs of systemic infection (eg, fever, tachycardia) and tenderness, fluctuance, and induration on DRE suggest the diagnosis. 0 When a perirectal abscess is suspected, imaging (eg, CT scan of the pelvis with IV contrast) should be performed to confirm the diagnosis and define the location and extent of the infection. Treatment involves prompt surgical drainage in the operating room (due to the deeper nature of the infection) and intravenous IV antibiotics. Peritoneum & Mesentery GIT 530 https://t.me/usmleinnercircle Version 2 Retroperitoneal abdominal organs rC irc le Ascending colon• C,""""1<1 ne ·Secondarilyretroperitoneal (devek>pedintraperitoneal & migratedretroperitoneal). Retroperitoneal Hematoma new-onset anemia CT below)? Retroperitoneal hematoma In  What is the likely diagnosis in a patient on warfarin who complains of weakness and back pain with U SM LE  Diagnosis of a retroperitoneal hematoma is confirmed with non-contrast CT scan of abdomen/pelvis or abdominal ultrasound. GIT 531 https://t.me/usmleinnercircle Version 2 • Flank pain with suprainguinal tenderness in patient who had recent cardiac catheterization. Diagnosis? Retroperitoneal hemorrhage  CT Scan  What cardiac catheterization complication presents with sudden hemodynamic instability and ipsilateral flank/back pain? Retroperitoneal hematoma typically occurs within 12 hours of catheterization; treatment is supportive Mesenteric Ischemia Acute mesenteric ischemia Presentation • Rapid onset of periumbilical pain (often severe) • Pain out of proportion to examination findings • Hematochezia (late complication) Risk factors • Atherosclerosis (acute on chronic) • Embolic source (thrombus, vegetations) • Hypercoagulable disorders Laboratory findings • Leukocytosis • Elevated amylase & phosphate levels • Metabolic acidosis (elevated lactate) Diagnosis • CT (preferred) or MR angiography • Mesenteric angiography, if diagnosis is unclear Treatment: • Unstable or signs of peritonitis → no imaging → emergency lap • Stable = endovascular approach (angioplasty, stent)  AMI typically presents with sudden-onset, severe, poorly localized (visceral) midabdominal pain accompanied by nausea and vomiting. Early in the course of illness, physical examination is typically unremarkable (eg, minimal, diffuse tenderness) despite the presence of severe pain; this finding is classically referred to as "pain out of proportion to the examination findings" GIT 532 https://t.me/usmleinnercircle Version 2 Chronic mesenteric ischemia Etiology • Atherosclerosis (smoking, dyslipidemia) Clinical features • Crampy, postprandial, epigastric pain • Food aversion & weight loss Diagnosis • Signs of malnutrition, abdominal bruit • CT angiography (preferred), Doppler ultrasonography • Risk reduction (eg, tobacco reduction), nutritional support • Endovascular or open surgical revascularization le Management Spontaneous bacterial peritonitis Clinical presentation • Abdominal pain/tenderness rC • Temperature ::,_37.8C (100 F) irc Peritonitis • Empiric antibiotics - third-generation cephalosporins (eg, cefotaxime) • Fluoroquinolones for SBP prophylaxis In Treatment ne • Altered mental status (abnormal connect-the-numbers test) • Hypotension, hypothermia, paralytic ileus with severe infection • PMNs ::,_250/mm3 Positive culture, often gram-negative organisms (eg, Escherichia coli, Klebsiella) Diagnosis from ascitic fluid • • Protein <1 g/dl • SAAG ::,_1.1g/dl PMN = polymorphonuclear leukocytes; SAAG = serum-ascites albumin gradient; SBP = spontaneous bacterial peritonitis. LE Antibiotics AFTER paracentesis since AB therapy can cause negative ascites cultures Peritoneal dialysis-related peritonitis • Touch contamination of catheter lumen • Extension of catheter site skin infection • Abdominal pain ± fever & nausea Clinical presentation • Diffuse abdominal tenderness± rebound • Cloudy peritoneal fluid • Gram-positive bacteria (-65%) Microbiology • Gram-negative bacteria (-30%) • Fungal organism (-5%) • Clinical presentation (ie, abdominal pain, cloudy fluid) Diagnosis • Relative neutrophilia on fluid analysis • Positive fluid Gram stain or culture U SM Pathophysiology Treatment . lntraperitoneal antibiotics (eg, vancomycin + gentamicin)* •Empiric therapy should cover both gram-positive& gram-negativeorganisms. GIT 533 https://t.me/usmleinnercircle Version 2 Hypotension and weakness in the setting of peritoneal dialysis are concerning for peritonitis and sepsis. Hernia Umbilical Hernia Congenital umbilical hernia Pathophysiology Clinical features Management . .. . .. Incomplete closure of abdominal muscles Soft, nontender bulge at umbilicus Protrudes with increased abdominal pressure Typically reducible Observe for spontaneous closure Elective surgery around age 5 Children with large or persistent defects are less likely to experience spontaneous closure and may undergo progressive enlargement of defect over time. Incisional Hernia lncisional hernia Pathogenesis • Breakdown of prior fascial closure . • • . • Obesity Risk factors Clinical features Diagnosis Tobacco smoking Poor wound healing (eg, immunosuppression, malnutrition) Vertical or midline incision Surgical site infection • Abdominal mass that enlarges with Valsalva • Palpable fascial edges in nonobese patients • Possible delayed presentation (months-years) • Clinical • CT scan of abdomen Rectus Abdominis Diastasis GIT 534 https://t.me/usmleinnercircle Version 2 Rectus abdominis diastasis Linea alba Skin ----------:7 Subcutaneous tissue -- Peritoneum ---,--,,-"::allllP Omentum ---- le Intestines ---CUWo~d Risk factors rC  Chronic abdominal stretching (eg, pregnancy, multiparity), irc due to weakening of the linea alba  Surgical weakening (eg, prior cesarean deliveries), and ne  Increased intra abdominal pressure (eg, constipation)  Unlike a true hernia, rectus abdominis diastasis has no associated fascial defect managed conservatively with observation and reassurance In NOT palpable while supine (vs Incisional Hernia) LE GIT Tumors Microcytic (iron deficiency) anemia in an elderly patient is more likely due to colon or U SM gastric cancer than to pancreatic cancer, which does not typically bleed into the gastrointestinal tract. Esophagus GIT 535 https://t.me/usmleinnercircle Version 2 Screening & management of Barrett esophagus Multiple risk factors for BE? • Chronic GERO (eg, >5 yr) • Male sex • Vllhite race • Hiatal hernia • Central obesity • Tobacco use • First-degree relative with BE or EAC l l Screening upper GI endoscopy Changes in distal esophagus: • Columnar epithelium • Intestinal metaplasia (ie, goblet cells) No dysplasia ] Low-grade dysplasia , High-grade dysplasia ] l l l PPI repeat endoscopy PPI surveillance or endoscopic eradication PPI endoscopic eradication BE= Barrettesophagus:EAC = esophagusadenocarcinoma,GERO= gastroesophagealrefluxdisease: GI = gastromtes nal; PPI = proton pumpmhlbitor. CIUW..ld  Patient with a history of Barrett's Esophagus and the patient has NO dysplasia: Screening/Surveillance EGDs every 35 years  Patient with a history of Barrett's Esophagus and dysplasia present: Screening/Surveillance every 12 years (less than every 3 years)  Man over age 50 with chronic GERD and other bad RF (smoking, obesity, high BMI  EGD screening/surveillance recommended Stomach GIT 536 https://t.me/usmleinnercircle Version 2 Staging of gastric adenocarcinoma Initial endoscopy/biopsy positive for adenocarcinoma CT abdomen and pelvis Chemotherapy +/- Palliative surgery irc Surgical resection le PET/CT, endoscopic ultrasound Laparoscopy, CT chest +/- Paracentesis/peritoneal lavage IDUWor1d imaging helps with disease staging, which determines prognosis and treatment options ne rC Pancreas Major risk factors for pancreatic cancer • First-degree relative with pancreatic cancer • Hereditary pancreatitis Hereditary In • Germline mutations (eg, BRCA1, BRCA2, Peutz-Jeghers syndrome) LE Environmental • Cigarette smoking (most significant) • Obesity, low physical activity • Nonhereditary chronic pancreatitis U SM Alcohol can cause chronic pancreatitis, which is associated with increase risk of pancreatic cancer, but it is not as significant as association bt. smoking and pancreatic cancer GIT Pancreatic adenocarcinoma Risk factors • • • • Smoking Hereditary pancreatitis Nonhereditary chronic pancreatitis Obesity & lack of physical activity Clinical presentation • Systemic symptoms (eg, weight loss, anorexia) (>85%) • Abdominal pain/back pain (80%) • Jaundice (56%) • Recent-onset atypical diabetes mellitus • Unexplained migratory superficial thrombophlebitis • Hepatomegaly & ascites with metastasis Laboratory studies • Cholestasis ( 1alkaline phosphatase & direct bilirubin) • f Cancer-associated antigen 19--9(not as a screening test) • Abdominal ultrasound (if jaundiced) or CT scan (if no jaundice) 537 https://t.me/usmleinnercircle Version 2  What imaging modality is preferred for detecting pancreatic tumors of the body/tail (e.g. nonjaundiced patients)? Abdominal CT scan ultrasound is less sensitive for visualizing the pancreatic body/tail due to overlying bowel gas • Pancreatic cancer classically presents with constant and/or gnawing epigastric pain that is worse at night. other classic signs include jaundice, anorexia with weight loss, and migratory thrombophlebitis • Depression and associated anxiety may be prodromal features in more than one-third of patients with pancreatic cancer. Patients may describe nonspecific anxiety, a premonitory sensation ("something bad is going to happen"), and feeling "low" for no reason. Tumors in the body or tail of the pancreas may present with progressive, constant back pain that is worse at night when lying supine.  Pancreatic surgery C/I is tumor encasement in SMA artery. Tumor in head has better prognosis than in tail. Atypical DM (eg, DM presenting in a thin, older patient) should raise suspicion for pancreatic cancer  What is the next step in management for an older patient with a significant smoking history that presents with painful, pruritic red streaks on the arm, with a similar episode two weeks prior on the chest that self-resolved, and mild epigastric pain? Abdominal CT this patient has migratory superficial thrombophlebitis (Trousseau's syndrome), which is associated with occult visceral malignancy (pancreas, stomach, lung, prostate) Presents with unexplained superficial venous thrombosis at unusual sites Trousseau's Syndrome migratory thrombophlebitis (associated with pancreatic cancer) GIT 538 https://t.me/usmleinnercircle Version 2 Advanced pancreatic adenocarcinoma, can obstruct the common bile duct CBD and cause extrahepatic cholestasis. Patients typically develop jaundice and pruritus due to direct (conjugated) hyperbilirubinemia and are at increased risk for cholangitis. Given the poor overall prognosis in surgically unresectable pancreatic cancer, palliative care is usually preferred. le Endoscopic stent placement can effectively relieve CBD obstruction in most patients and is less invasive and less risky than surgery. irc Surgical bypass (eg, anastomosis between the gallbladder or CBD and jejunum) is sometimes considered as a second-line option in patients in whom stent placement would be technically challenging rC REMEMBER this, this is important ! ne Colon Colon cancer screening • Start at age 45: Patients at average risk o LE o Patients with FDR with CRC or high-risk adenomatous polyp* U SM Colonoscopy every 10 years In o Patients with ulcerative colitis gFOBT or FIT every year o FIT-DNA every 1-3 years o CT colonography every 5 years Flexible sigmoidoscopy every 5 years (or every 10 years with annual FIT) • Colonoscopy at age 40 (or 10 years prior to age of diagnosis in FDR, whichever comes first) • Repeat every 5 years (every 10 years if FDR diagnosed at age >60) • Start screening 8-10 years after diagnosis • Colonoscopy every 1-3 years •Adenomatous polyp ;,10 mm, high-grade dysplasia, villous elements {for example). CRC = colorectal cancer; FDR= first-degree relative; FIT= fecal immunochemical test; FIT-DNA= multitarget stool DNA test; gFOBT = guaiac-based fecal occult blood test. Tests of the stool for occult blood rely on heme pseudoperoxidase activity, which can be interfered with by vitamin C and renders the test susceptible to false negative results. Patients should abstain from vitamin C, citrus, and red meat for several days prior to FOBT. GIT 539 https://t.me/usmleinnercircle Version 2 Patients with adenomatous polyps found on screening sigmoidoscopy should undergo colonoscopy for visualization of the entire colon and detection of synchronous adenomas and advanced neoplasia. Colon cancer screening in high-risk patients Family history of adenomatous polyps or CRC • First-degree relative at age <60 Inflammatory bowel disease • Ulcerative colitis • Crohn colitis • Colonoscopy at age 40 or 10 years before the age of diagnosis in the relative (whichever comes first) • Repeat every 3-5 years • Begin 8 years post diagnosis (12-15 years if disease only in left colon) • Colonoscopy with biopsies every 1-2 years Familial adenomatous polyposis • Begin at age 10-12 • Colonoscopy every year Hereditary nonpolyposis CRC (Lynch syndrome) • Begin at age 20-25 • Colonoscopy every 1-2 years Risk factors for colon cancer Lifestyle factors Medical/family history Protective factors . .. .. . .. • Frequent consumption of red/processed meat Tobacco, alcohol use Personal/family history of adenomatous polyps or colon cancer Inherited colon cancer syndromes (eg, familial adenomatous polyposis, Lynch syndrome) Ulcerative colitis Diabetes/obesity Prior abdominopelvic radiation High-fiber diet Aspirin/NSAID use NSAID = nonsteroidalanti-inflammatorydrug.  For individuals with a history of abdominopelvic radiation treatment, colon cancer screening should commence at an earlier age (eg, age 3040 than that recommended for average-risk patients (ie, age 45. Colonoscopy is preferred, although acceptable alternatives include fecal occult blood testing and fecal DNA testing.  What characteristics (growth pattern, histology) of colonic adenomatous polyps suggest greater malignant potential? sessile growth and villous histology other signs of malignant potential include large size  1 cm) and high number  3 concurrent adenomas)  Due to their exceedingly high risk of CRC, patients with classic FAP (and sometimes attenuated FAP need an elective total proctocolectomy in their late teens or early twenties. However, in younger patients (such as 9-year-old boy), surgery is usually delayed in favor of close surveillance with GIT 540 https://t.me/usmleinnercircle Version 2 frequent colonoscopy until patients have completed puberty and matured both physically and emotionally. • Urgent total proctocolectomy is performed no matter the patient's age in those with CRC or adenomas with high-grade dysplasia; other indications for colectomy include severe symptoms related to colonic neoplasia (eg, hemorrhage) and a significant increase in polyp number during the screening interval irc le • Regular aspirin or nonsteroidal anti-inflammatory drug use has been associated with reduced incidence of CRC in patients at average risk but does not influence the chances of developing CRC in FAP.  Whenever possible, patients with adenocarcinoma of the colon should be offered surgical resection with curative intent. When metastatic spread is confined to the liver, surgical resection of both the rC hepatic mass and the primary tumor can be curative, and often provides increased long-term 5 yr) survival even when not curative. Combined chemotherapy and radiation are used for nonoperable rectal adenocarcinoma as well as anal squamous cell carcinoma; ne however, radiation therapy is typically avoided in tumors proximal to the rectum because the adverse effects (eg, radiation enteritis) can be severe. In Surveillance after colon cancer resection • Colonoscopy in 1 yr & then every 3-5 yr Stage I • Colonoscopy in 1 yr & then every 3-5 yr • Periodic CEA testing LE Stages II & Ill • Annual CT scan of the chest, abdomen(± pelvis) Stage IV • Individualize • Consider stage 11/111 strategy but more frequent CT scans U SM CEA = carcinoembryonic antigen. Staging evaluation for rectal adenocarcinoma Tumor markers Imaging Endoscopy/direct visualization Carcinoembryonic antigen CT scan: chest, abdomen, pelvis Colonoscopy PJ Syndrome GIT 541 https://t.me/usmleinnercircle Version 2 Peutz.Jeghers syndrome Etiology • Autosomal dominant disorder • Tumor suppressor gene mutation causes unregulated tissue growth • Pigmented macules (eg, lips, buccal mucosa, palms/soles) • ;?2gastrointestinal hamartomatous polyps o Abdominal pain due to obstruction or intussusception Clinical features o Anemia due to acute/chronic bleeding 0 Rectal prolapse • f Cancer risk (eg, gastrointestinal, breast, genital tract) • Positive family history Diagnosis Management • Genetic testing • Annual anemia screening • Cancer screening (eg, upper/lower endoscopy) Liver Solid liver masses Focal nodular hyperplasia Hepatic adenoma Regenerative nodules Hepatocellular carcinoma Manifestations Imaging Treatment Complications • Acute or chronic liver injury (eg, cirrhosis) • Systemic symptoms • Chronic hepatitis or cirrhosis • Elevated a fetoprotein • Single/multiple lesions • Known extrahepatic malignancy Liver metastasis Epidemiology • Associated with anomalous arteries • Arterial flow & central scar on imaging • Women on long-term oral contraceptives • Possible hemorrhage or malignant transformation .. .. .. .. .. Hepatic adenoma Benign epithelial liver tumor Primarily young women on oral contraception Often asymptomatic (incidentally found) Episodic right upper quadrant pain Solitary, solid lesion in right lobe of liver Multiple lesions occasionally occur Asymptomatic & <5 cm - stop oral contraception Symptomatic or >5 cm - surgical resection Malignant transformation (-10%) Rupture & hemorrhagic shock  Is needle biopsy recommended for a patient with a hepatic adenoma? GIT 542 https://t.me/usmleinnercircle Version 2 No (due to risk of bleeding) surgical excision is preferred Hepatic adenoma can be seen in OCP use or Pregnancy le AFP is elevated in 50% of HCC cases; therefore, it can serve as an important diagnostic clue (when elevated) but cannot be used to rule out the diagnosis irc MALToma  90% MALToma cases are ass with H. pylori.  All patients with MALT lymphomas should be tested for H pylori infection, and patients with a positive result who have early-stage MALT lymphoma should undergo H pylori eradication therapy (eg, rC quadruple therapy). The majority of patients achieve complete remission with antibiotic treatment. ne  Patients with more advanced malignancies or with H pylori-negative tumors should be considered for radiation therapy, immunotherapy (eg, rituximab), or single-agent chemotherapy. Pernicious anemia is related to an increased risk of gastric adenocarcinoma and gastric LE Miscellaneous In carcinoid tumors NOT MALToma Areas of referred pain to the abdomen U SM Cholecystitis CUWor1d GIT 543 https://t.me/usmleinnercircle Version 2 Unintentional weight loss in elderly patients Definition • >5% involuntary weight loss over 6-12 months • Malignancy, depression • Chronic end-organ disease (eg, CKD, COPD) Causes • Neurologic disease (eg, oropharyngeal dysphagia) • Social factors (eg, poverty, isolation) • Diminished smell & taste, poor dentition CKD = chronic kidney disease; COPD = chronic obstructive pulmonary disease.  Advanced end-organ disease contributes to weight loss via components of both anorexia (ie, loss of appetite) and cachexia (ie, inflammatory loss of muscle mass). In such patients without findings to suggest malignancy (eg, blood in the stool, hypercalcemia, unexplained anemia), watchful waiting is typically most appropriate. This is especially true for elderly patients with limited life expectancy, and other explanations for weight loss (eg, CKD because watchful waiting avoids diagnostic testing that is unlikely to improve quality of life or life expectancy. Furthermore, unnecessary diagnostic testing may increase patient anxiety, physical discomfort, and financial stress. In addition, such patients with evidence of malnutrition (eg, BMI 18.5 kg/m2 often benefit from nutritional supplementation (eg, high-calorie supplement drinks), which can help reverse cachexia progression (protein-energy wasting). Abdominal Compartment Syndrome Abdominal compartment syndrome Etiology .. .. Clinical manifestations . .. . Management . t lntraabdominal pressure causing organ dysfunction Risk factors: massive fluid resuscitation, major intraabdominal surgery or pathology Tense, distended abdomen t Ventilatory requirements (diaphragmatic elevation, t intrathoracic pressure) t CVP (venous compression but L venous return & cardiac preload) Hypotension, tachycardia (L venous return & cardiac output) L Urine output (L intraabdominal organ perfusion) Temporizing measures: 0 Avoid over resuscitation with fluids 0 L lntraabdominal volume (eg, NG tube) 0 t Abdominal wall compliance (eg, sedation) Definitive management: surgical decompression CVP = central venous pressure;NG = nasogastric.  Although reduced cardiac preload is typically reflected in a low CVP, the two are uncoupled in ACS, where CVP is increased (despite reduced preload) due to external compression rather than increased intravenous volume. GIT 544 https://t.me/usmleinnercircle Version 2 • Bladder pressure measurement can estimate intraabdominal pressure when abdominal compartment syndrome ACS is suspected.  A patient develops hypotension, JVD, and distended abdomen 18 hours after abdominal surgery. He was transfused with 6 units of PBRC during surgery. Urine output is decreased. Labs show increased BUNCr. What is the treatment? Abdominal decompression by laparotomy with temporary closure (reopen abdomen and cover with plastic) le Abdominal compartment syndrome leads to decreased preload and thus hypotension and hypoperfusion  AKI, tachycardia irc  Patients receiving massive fluid resuscitation (eg, patients with severe burns) are at increased risk for ACS, especially when coupled with conditions (eg, trauma, burns) that cause a systemic rC inflammatory response, increased capillary permeability, and rapid third spacing of fluids (eg, into the abdominal cavity). The resulting increase in IAP may decrease perfusion to intraabdominal organs (ie, abdominal perfusion pressure = mean arterial pressure  IAP and result in the following organ dysfunction: • Renal impairment: Renal vein compression impairs renal venous drainage, leading to renal dysfunction (eg, decreased urine output, increased creatinine). ne • Pulmonary dysfunction Diaphragmatic elevation causes extrinsic lung compression, which increases intrathoracic pressure and leads to high ventilation pressures (eg, peak inspiratory pressure) and jugular venous distension JVD. In • Cardiovascular compromise Obstruction of venous return leads to decreased cardiac output (eg, TPN LE hypotension) and increased venous hydrostatic pressure in the lower extremities (eg, 3 pitting edema). Cardiac function is also diminished due to direct cardiac compression from an elevated hemidiaphragm. U SM • Enteral feeding is preferred to parenteral feedings for patients who have a functioning gastrointestinal system. The standard composition of 30 kcal/kg/day and 1 g/kg/day of protein is satisfactory for most patients with adequate baseline nutrition. Paeds GIT Tracheoesophageal Fistula GIT 545 https://t.me/usmleinnercircle Version 2 Pathogenesis Clinical features Diagnosis Management .. .. . .. .. Tracheoesophageal fistula with esophageal atresia Defective division of foregut into esophagus & trachea Most commonly results in proximal esophageal pouch & fistula between distal trachea & esophagus Coughing, choking, vomiting with feeding Excessive oral secretions Commonly part of VACTERL association Inability to pass enteric tube into stomach X-ray: enteric tube coiled in proximal esophagus Surgical correction VACTERL screening: echocardiography, renal ultrasound VACTERL= Vertebral,Anal, Cardiac,TracheoEsophageal,Renal, Limb defects. Foreign Body Ingestion  What is the next step in management for a symptomatic child with evidence of a swallowed coin in the esophagus on X-ray? Flexible endoscopy endoscopy is both diagnostic and therapeutic; asymptomatic children may be observed for 24 hours after ingestion (coins are not sharp and the metals used are not toxic) (vs Battery; necessary to prevent further mucosal damage and esophageal ulceration; removal is necessary for both symptomatic and asymptomatic patients) Trachea: Bronchoscopy; Esophagus: Endoscopy  What is the next step in management for a child that swallowed a battery that is located in the stomach on X-ray? Observation for 24  48 hours batteries located distal to the esophagus typically pass uneventfully GIT 546 https://t.me/usmleinnercircle Version 2 Suspected foreign body ingestion ! PA & lateral x-rays (CT scan if object not visible on x-ray) ! ! Endoscopic removal le No high-risk features A No transit: Endoscopic removal Object moving distally: No intervention IDUWO!ld ne PA = posteroanterior, 'Patient has resp,ratO()'or obstructJvesymptoms; object is a button battery,magne or sharp ,tem, rC Serial x-rays irc A High-risk features* • Sharp object (eg, needle, safety pin) in the esophagus, stomach, or proximal duodenum In If a sharp foreign body is distal to the proximal duodenum in an asymptomatic patient, observation with repeat x-ray in 1224 hours may be appropriate because these objects are usually excreted uneventfully LE • Symptoms of esophageal obstruction (eg, drooling, inability to swallow secretions) • Symptoms of respiratory compromise • Button battery in the esophagus (due to the risk of electrical and chemical injury) U SM • Magnets in the esophagus or stomach (due to the potential for bowel entrapment as a result of magnetic attraction across intestinal segments)  In cases of high-risk esophageal foreign bodies, such as magnets, button batteries, and sharp objects (fish bone), immediate endoscopic removal is required to prevent complications.  Ingestion of 2 magnets, especially if swallowed separately, can entrap bowel via attraction across varying intestinal segments and lead to necrosis, fistulae, or perforation. Therefore, magnets visualized in the esophagus or stomach on x-ray require immediate endoscopic removal even if patients are asymptomatic. In case of single magnet ingestion; observation can be considered. GIT 547 https://t.me/usmleinnercircle Version 2 Crying Infant Crying in young infants Diagnosis Normal . . . Key features Intermittent, consolable, <3 hr/day • 2:3 hr/day (usually evening), 2:3days/week Colic Gastroesophageal reflux disease Healthy infant age <3 months Frequent spit-up • Back-arching after feeding • Acute otitis media: bulging tympanic membrane, ± fever • Meningitis: fever, lethargy, bulging fontanel . . Infection • Septic arthritis: fever, limited extremity movement lntussusception UTI: fever, vomiting, poor feeding Episodic irritability with legs drawn to abdomen • ± Bilious emesis, bloody stools Torsion • Testicular swelling or abdominal distension (ovarian) • Hair tourniquet: hair accidentally wrapped around digit Trauma • Corneal abrasion: tearing, photophobia; + fluorescein test • Abuse/fracture: bruising, laceration, asymmetric movements UTI = urinary tract infection. Infantile Colic: benign; parents should be reassured and taught soothing/feeding techniques Constipation Pediatric constipation Risk factors History Examination .. .. .. . . Pathologic Functional Delayed meconium passage • Solid food introduction Down syndrome (associated with HD • Toilet training & intestinal atresia/stenosis) • School entry Poor weight gain or linear growth Narrow (ribbon) stools Blood mixed in stool Bilious vomiting or fever Displaced anus, t rectal tone, or sacral anomalies (eg, hair tuft) Abnormal lower extremity neurologic findings (eg, weakness) • Absence of pathologic . . . o Common (eg, celiac disease, Management hypothyroidism) 0 Urgent (eg, HD, CF, spinal dysraphism) Painful, infrequent, largecaliber or pellet-like stools* Fecal soiling (encopresis) • Absence of pathologic features Mild abdominal distension • Anal fissure (± rectal • Severe abdominal distension • Work up for organic cause features bleeding) • Age-appropriate toileting . . plan Dietary changes (t fiber/water) Laxatives (eg, polyethylene glycol) •Benign features refractory to functional constipation management may also be pathologic. CF = cystic fibrosis; HD= Hirschsprung disease. GIT 548 https://t.me/usmleinnercircle Version 2 Pediatric functional constipation • Initiation of solid food & cow's milk Risk factors • Toilet training • School entry • Painful/hard bowel movements Clinical features • Stool withholding • Encopresis (fecal incontinence) • Delayed passage of meconium • Fever or vomiting • "Ribbon" stools Alarm signs • Severe abdominal distension • Abnormal examination findings (eg, displaced anus, tuft at gluteal cleft) irc • Anal fissure Complications • Hemorrhoids • Enuresis/urinary tract infections Treatment • Laxatives (eg, polyethylene glycol) • Age-appropriate toileting guidance • ± Enemas/suppositories rC • j Dietary fiber & water intake • Limit cow's milk (<24 oz/day) le • Poor growth ne Recurrent cystitis (UTI's) (due to mass effect on the bladder, preventing voiding and leading to urinary stasis)  Initial management is dietary, including the addition of apple, prune, or pear juice/puree. In These nondigestible, osmotically active carbohydrates (eg, sorbitol) pull water into the gastrointestinal tract, thereby softening stool. LE Approach to the straining infant Straining infant Well-appearing U SM Ill-appearing or red flags* present i r Serious organic cause • Hirschsprung disease • Cystic fibrosis • Spinal dysraphism • Hypothyroidism Loose stools ± mucus & blood i Normal stool consistency l Hard or pellet-like stools ! ! ! Food induced protein enterocolitis Normal infant dyschezia Functional constipation (± anal fissure if blood present) *Severe abdominal distention, abnormal rectal tone or sacral findings, delayed passage of meconium, failure to thrive C)UWorld  Pathogenesis of infant dyschezia involves the following: • Failure to coordinate increased intraabdominal pressure with relaxation of the pelvic floor muscles GIT 549 https://t.me/usmleinnercircle Version 2 • Inadequate abdominal muscle tone to produce an effective Valsalva maneuver These mechanisms result in characteristic findings of crying, turning red in the face, and straining for greater than 10 minutes, followed by passage of a soft, nonbloody stool. T/t: Reassurance only Vomiting Differential diagnosis of regurgitation & vomiting in infants Diagnosis Clinical features • Physiologic Gastroesophageal reflux Milk protein allergy • Reassurance 0 Asymptomatic 0 "Happy spitter" • Positioning therapy • Pathologic (GERO) • Thickened feeds 0 Failure to thrive • Antacid therapy 0 Significant irritability • If severe, esophageal pH probe monitoring 0 Sandifer slndrome • Regurgitation/vomiting • Eczema • Bloody stools • Projectile nonbilious vomiting Pyloric stenosis Management • Olive-shaped abdominal mass • Dehydration, weight loss & upper endoscopy • Elimination of dairy & soy protein from diet • Abdominal ultrasonography • Pyloromyotomy GERD = gastroesophagealreflux disease. Positional therapy (e.g. hold infant upright for 2030 minutes after frequent, small-volume feeds)  What is the next step in management for an infant with failure to thrive that spits up formula after each feed? proton pump inhibitors and/or thickened feeds (e.g. adding oatmeal) failure to thrive indicates that this is pathologic GERD (vs Adequate wt gain in physiologic) be careful with PPIs in infants— loss of acidity predisposes to increased risk of infections GIT 550 https://t.me/usmleinnercircle Version 2 Evaluation of bilious emesis In the neonate Bilious emesis j Unstable Stable ' Abdominal x-ray ] Emergency laparotomy Increased rectal tone and/or delayed passage o~meconium ' - No .... ____ Upper GI series ___ 1 Yes ' Right-sided ligament of Treitz + + Microcolon Rectosigmoid transition zone t 't Hirschsprung disease ne CVWol1d Malrotation l Duodenal atresia rC Contrast enema Meconium ileus Double bubble sign J Normal le Dilated loops of bowel irc Free air Cyclical Vomiting Syndrome In Cyclic vomiting syndrome • Personal or family history of migraines • Episodes often have identifiable trigger (eg, infection, stress) History Stereotypical vomiting episodes LE . U SM Symptoms o Acute onset of nausea, abdominal pain, headache, vomiting 0 Self-limited, lasting 1-2 days • Between episodes 0 Usually asymptomatic 0 Often regular intervals (eg, 2-4 weeks) Diagnosticcriteria of cyclic vomiting syndrome • ~3 episodes in a 6-month period • Easily recognizable to family (stereotypical) • Lasts 1-10 days • Vomiting ~4 times/hr at peak • No symptoms in between vomiting episodes • No underlying condition can be identified  What is the likely diagnosis in a child that presents with selflimiting, recurrent nausea and vomiting at the same time each month with no symptoms in between episodes? Cyclic vomiting syndrome GIT 551 https://t.me/usmleinnercircle Version 2 Treatment: Abortive: Triptans Supportive: Anti-emetics Food Allergen Proctocolitis Milk- or soy-protein-induced colitis Risk factors • Family history of allergies, eczema, or asthma Clinical features • • • • Presents at age 2-8 weeks Regurgitation or vomiting +/-Painless bloody stools +/-Eczema • Elimination of milk & soy from maternal diet of exclusively breastfed infants Treatment • Initiation of hydrolyzed formula in fonnula-fed infants Prognosis • Spontaneous resolution by age 1 year Management of food protein-induced allergic proctocolitis (FPIAP) Infant with presumed FPIAP• Formula-feeding ) Breastfeeding ) i i Eliminate common triggers from maternal diet (eg, dairy, soy) Switch to hypoallergenic (eg, hydrolyzed) formula l Symptom resolution? ] FPIAP confirmed: Reintroduce offending protein around age 1 - Yes 1 No---+ Consider evaluation for alternate diagnosis (eg, flexible sigmoidoscopy) •Well-appearingInfant age <6 months with blood-streaked stools and nonlocal examination CUWond D/D MECKEL but very rare in less than 6 months age Symptoms suggestive of a different diagnosis include failure to thrive, profuse diarrhea, and forceful vomiting GIT 552 https://t.me/usmleinnercircle Version 2 lgE- & non-lgE-mediated food allergies lgE mediated Example Age Anaphylaxis Any Immediate (<1 hr) Food proteininduced allergic Clinical Symptom onset <6 months Insidious <12 months Within hours proctocolitis features • Urticaria • Vomiting, wheezing • Angioedema, hypotension • Painless, bloody stools • Well appearing Non-lgE Food proteinenterocolitis Breastfeeding Benefits Contraindications • Active untreated tuberculosis (mothers may breastfeed after 2 weeks of anti-tuberculin therapy) ne • More rapid uterine involution & decreased postpartum bleeding rC Breastfeeding benefits & contraindications • Faster return to prepartum weight • Improved child spacing In Maternal blood), dehydration, lethargy • Ill appearing syndrome le • Profuse vomiting, diarrhea (± induced irc mediated • Improved maternal-infant bonding LE • Reduced risk of breast & ovarian cancer • Maternal HIV infection (in developed countries where fonnula is readily available) • Herpetic breast lesions • Varicella infection <5 days prior lo or within 2 days of delivery • Specific maternal medications • Chemotherapy or ongoing radiation therapy • Active abuse of street drugs or alcohol • Improved immunity U SM • Improved gastrointestinal function Infant • Prevention of infectious diseases: o Otitis media o Gastroenteritis o Respiratory illnesses o Urinary tract infections • Galactosemia • Decreased risk of childhood cancer, type I diabetes mellitus & necrolizing enlerocolitis  Casual, moderate alcohol use is considered safe and not a contraindication to breastfeeding.  Mothers with Hep B and Hep C can breast feed given their nipples are not cracked or bleeding. GIT 553 https://t.me/usmleinnercircle Version 2 Pregnant patients on medication-assisted treatment (eg, methadone) for opioid use disorder can be encouraged to breastfeed if they are enrolled in treatment programs during pregnancy and postpartum and demonstrate adherence to therapy without active recreational drug use.  Neonates should breastfeed every 2  3 hours for > 10  20 minutes per breast during the first month of life or approximately 8  12 times per day  Many breastfed infants have a decrease in the frequency of infant bowel movements after the first month of life. Parents should be reassured that this is normal and requires no treatment. Exclusive breastfeeding is recommended for the first 6 months of life. All exclusively breastfed infants should also be started on vitamin D supplementation, Iron supplementation for Premature babies 6 months and Vitamin B12 for babies with strict vegetarian mothers. Timeline of infant nutrition 6 months 1 year Introductionof pureedfoods Introduction of cow'smilk Birth Exclusivebreastfeeding  By age 46 months, term infants can typically sleep through the night without requiring overnight feeds to meet their metabolic demands. Offering feedings during nocturnal awakenings trains infants to wake up to feed. To break this cycle, overnight feeds should be avoided. Dehydration Mild dehydration (3-5% volume loss) presents with a history of decreased intake or increased fluid loss with minimal or no clinical symptoms. Moderate dehydration (6-9% volume loss) presents with decreased skin turgor, dry mucus membranes, tachycardia, irritability, a delayed capillary refill (2-3 seconds), and decreased urine output. Severe dehydration (10-15% volume loss) presents with cool, clammy skin, a delayed capillary refill (>3 seconds}, cracked lips, dry mucous membranes, sunken eyes, sunken fontanelle (if still present), tachycardia, lethargy, and minimal or no urine output. Patients can present with hypotension and signs of shock when severely dehydrated. Mild-moderate: Oral Rehydration GIT 554 https://t.me/usmleinnercircle Version 2 Moderate-severe: IV bolus of isotonic solutions 20Ml/ Kg (e.g. normal saline, lactated Ringer's) Evaluation of neonatal hydration <7% • Continue exclusive breastfeeding • Follow-up at age 10-14 days to check that infant has regained birth weight 2':7% • • • • Assess for oromotor dysfunction Assess for lactation failure Daily weights Consider formula supplementation rC Management of weight loss irc Signs of dehydration Decreased wet diapers Absence of tears Sunken fontanelle Dry mucous membranes Decreased skin turgor Delayed capillary refill le • • • • • • Neonatal weight trends course • Weight loss in first 5 days of life (eg, excretion of excess fluid, delayed lactogenesis) • Weight regained (ie, back to birth weight) by age 2 weeks ne Natural • S10% of birth weight lost 0 output, sunken fontanelle) o Assess for breastfeeding complications (eg, tongue-tie) In • 0 Measure weights daily 0 Consider formula supplementation 0 Consider serum electrolytes & glucose measurements LE Management Provide reassurance & continue to monitor >10% of birth weight lost &/or signs of dehydration (eg, poor urine  Healthy infants may lose up to 7% of their birth weight in the first 5 days of life. U SM due to excretion of excess fluid acquired in utero and during labor; no treatment is required  Approximately how many wet diapers should a 5-day-old newborn make per day?  5 wet diapers/per day as a general rule, the number of wet diapers should equal age in days for the first week of life; after the first week, infants should have  6 wet diapers per day Midgut volvulus GIT 555 https://t.me/usmleinnercircle Version 2 Surgical emergency that classically presents in a neonate with congenital malrotation, manifesting as bilious vomiting and abdominal distention. diagnosed using an upper GI series, which may show a "corkscrew" pattern; untreated volvulus can progress to bowel ischemia and perforation Malrotation with midgut volvulus Pathogenesis • Failure of normal embryonic gut rotation • Narrow mesenteric base allows for f small bowel mobility • Twisting of small bowel around superior mesenteric artery --+ gut ischemia & necrosis • Most common in infancy (usually age <1 month) Clinical • Acute: bilious emesis, abdominal distension features • Chronic: intermittent abdominal pain & vomiting, failure to thrive • Untreated: hematochezia, peritonitis & shock • Abdominal x-ray: ± dilated bowel, air-fluid levels, pneumoperitoneum Diagnosis • Upper gastrointestinal series (gold standard): ligament of Treitz on right; corkscrew, or bird's-beak, duodenum Treatment • Emergency laparotomy (to relieve volvulus) • Ladd procedure (to reposition malrotated bowel) Acute Symptoms in Infants. Chronic in Older Children and adults.  For neonates with suspected volvulus (eg, bilious emesis, x-ray with dilated loops of bowel, normal rectal examination) who are hemodynamically stable, upper GI series (consisting of x-rays with contrast material for improved visualization) is the diagnostic gold standard. In contrast, in a neonate with bilious emesis who is hemodynamically unstable (eg, hypotension, tachycardia) or has peritoneal signs (eg, firm, distended, tender abdomen), the next step in management is exploratory laparotomy. Surgical exploration should not be delayed for diagnostic or repeat imaging due to increased risk of mortality GIT 556 https://t.me/usmleinnercircle Version 2 Volvulus \ ' r-.. Pyloric Stenosis rC irc --- le .... Infantile hypertrophic pyloric stenosis ne • First-born boy Risk factors • Erythromycin • Bottle feeding • Projectile nonbilious emesis • Poor weight gain In Presentation • Dehydration • Olive-shaped abdominal mass LE Diagnostic studies Treatment • Hypochloremic metabolic alkalosis • Thickened pylorus on abdominal ultrasound • Intravenous rehydration • Pyloromyotomy U SM U/S can also detect pyloric muscle diameter and thickness • Erythromycin : either through direct administraton or through breast milk; pyloric stenosis is also associated with formula feeds  How soon after birth does pyloric stenosis typically present?  3  5 weeks takes time for hypertrophy to develop; helps distinguish from intestinal atresia (bilious vomit within 1  2 days)  What acid-base disturbance is associated with pyloric stenosis? Hypokalemic, hypochloremic metabolic alkalosis secondary to vomiting of gastric acid and subsequent volume contraction; should be corrected prior to pyloromyotomy to reduce risk of post-operative apnea GIT 557 https://t.me/usmleinnercircle Version 2 Neonatal Jaundice Management of neonatal unconjugated hyperbilirubinemia Total bilirubin elevated for age J Unconjugated bilirubin >25 mg/dl ] !~----No-----~-----Yes-----~ Unconjugated bilirubin above phototherapy threshold for age Increase feed volume/frequency & monitor Initiate phototherapy - Unresponsiveto therapy (eg, rapid rate of rise) - • Initiate exchange transfusion • Evaluate for ~athol ic causes· •eg, RhD lncompahblhty. glucose-6-phospt,atase deficiency. CUWorld Approach to neonatal cholestasis Neonatal cholestasis• ] I Abdominal ultrasound ] Abnormal or absent gallbladder Cystic dilation of biliary tree j Biliary cyst Biliary atresia Isolated hepatomegaly or normal j Infectious evaluation l CMV, toxoplasmosis, HSV, UTl/sepsis j Genetic/metabolic evaluation AAT deficiency Dubin-Johnson syndrome Galactosemia Cystic fibrosis "Dark unne, aciholicstools, conjugated hyperblllrublnemla. AAT = atpha-1 antitryps,n; CMV = cytomegalovirus; HSV = herpes simplex virus; Ult= urinary tract infection. IC>UW<><ld Most neonates with symptomatic congenital infections typically have other manifestations, such as growth restriction (eg, microcephaly) or hepatosplenomegaly. GIT 558 https://t.me/usmleinnercircle Version 2 Neonatal indirect hyperbilirubinemia t Bilirubin clearance t Enterohepatic circulation . .. .. .. Immune-mediated hemolysis (Coombs positive) 0 Rh isoimmunization 0 ABO incompatibility Nonimmune-mediated hemolysis (Coombs negative) 0 RBC membrane defects (eg, spherocytosis) 0 RBC enzyme defects (eg, G6PD deficiency) Cephalohematoma Polycythemia Gilbert syndrome Crigler-Naiiar syndrome le t Bilirubin production . Examples Lactation failure jaundice Breastmilk jaundice G6PD = glucose-6-phophatasedehydrogenase;RBC = red blood cell. irc Cause Diagnosis Timing Lactation failure jaundice Age <1 week rC Lactation failure jaundice vs breast milk jaundice Pathophysiology Insufficient intake of breast milk: ne Breast milk jaundice • l Bilirubin elimination • j Enterohepatic circulation j 13-glucuronidase in breast milk: Age >1 week • j Deconjugation of intestinal bilirubin • j Enterohepatic circulation (peaks at 2 weeks) Clinical features • Suboptimal breastfeeding • Signs of dehydration • Adequate breastfeeding • Well-hydrated In Signs of dehydration include decreased urine/stool output and "brick-red" urate crystals in the diaper LE  Treatment of breast milk jaundice includes frequent follow-up and monitoring of the infant's hyperbilirubinemia. Exclusive breastfeeding should be continued and encouraged as the jaundice should resolve spontaneously by age 3 months U SM  What is the recommended management for infants with breastfeeding failure jaundice? Increase the frequency and duration of feeds if the bilirubin continues to rise (e.g. mother's milk supply is inadequate), formula supplementation may be necessary  What is the initial treatment for rapidly rising hyperbilirubinemia in a newborn? non-UV phototherapy (for kernicterus prevention) exchange transfusion is indicated for total bilirubin levels  25 mg/dL Bilirubin induced Neurologic Dysfunction GIT 559 https://t.me/usmleinnercircle Version 2 BIND Pathophysiology Risk factors Bilirubin-induced neurologic dysfunction • Excess levels of free, unconjugated bilirubin cross the blood-brain barrier • Deposition of bilirubin in basal ganglia & brainstem nuclei • Neuronal damage, necrosis & atrophy • Prematurity • Hemolysis (eg, G6PD deficiency) • Birth trauma (eg, cephalohematoma) • Exclusive breastfeeding with excessive weight loss • ± Reversible (treatment: phototherapy, exchange transfusion) Acute encephalopathy • Clinical findings over 1st days of life 0 Lethargy or inconsolability 0 Hypotonia (early) or hypertonia (late) o Apnea/respiratory failure, feeding difficulties, seizures • Irreversible over 1st years of life • Clinical findings Chronic 0 Developmental delay encephalopathy 0 Sensorineuralhearingloss 0 Choreoathetoid movements 0 Upward gaze palsy Biliary Atresia Biliary atresia Pathogenesis • Extra hepatic bile duct fibrosis • Asymptomatic at birth Clinical findings • Infants age 2-8 weeks: o Jaundice, acholic stools, dark urine 0 Hepatomegaly • Direct hyperbilirubinemia • Ultrasound: o Diagnostic evaluation Absent/abnormal gallbladder &/or CBD • Liver biopsy: 0 lntrahepatic bile duct proliferation 0 Portal tract inflammation & edema 0 Fibrosis • lntraoperalive cholangiography (gold standard): 0 Treatment Biliary obstruction • Surgical hepatoportoenterostomy (Kasai procedure) • Liver transplant CBD = commonbile duct.  What is the recommended management of patients with biliary atresia prior to a liver transplant? Hepatoportoenterostomy Kasai procedure) allows time for growth and reduces morbidity/mortality of liver transplant Biliary cyst GIT 560 https://t.me/usmleinnercircle Version 2 BIiiary cysts Cystic dilation of biliary tree Most common: single, extrahepatic dilation of common bile duct (type I) May be incidental finding on imaging Diagnosis Complications Treatment .. . . • • . • . Type I Type II Abdominal pain 0 RUQ mass 0 Jaundice Neonates: jaundice, acholic stools, dark urine, hepatomegaly ± Nausea, vomiting Ultrasound ± CT scan or MRCP Type Ill le 0 Presentation Normal Classic triad• in children/adults Type IV TypeV irc .. .. Cholangiocarcinoma m/c Acute cholangitis Pancreatitis Stone formation Cyst resection (to L risk for malignancy) ± Roux-en-Y hepaticojejunostomy (to allow for biliary drainage) •often, only two-thirdsof findings are present. ne MRCP = MR cholangiopancreatography;RUQ = right upper quadrant. rC Pathogenesis Biliary cyst of cholangiocarcinoma. In • Biliary cysts require surgical resection to relieve obstruction and prevent the development • Acute complications: Pancreatitis, cholangitis, and stone formation LE  There is still a small risk for developing cancer in the remaining bile ducts after surgery, and serial laboratory testing with or without imaging is usually recommended postoperatively. U SM Intestinal Atresia Duodenal atresia Jejunal atresia GIT 561 https://t.me/usmleinnercircle Version 2  Atresia of the jejunum or ileum is thought to occur due to a vascular accident in utero that causes necrosis and resorption of the fetal intestine, leaving behind blind proximal and distal ends of intestine. Risk factors include poor fetal gut perfusion from maternal use of vasoconstrictive medications or substances such as cocaine and tobacco. In contrast to duodenal atresia, jejunal and ileal atresia are not associated with chromosomal abnormalities. Hirschsprung Delayed passage of meconium Hirschsprung disease Meconium ileus • Failure of neural crest cell migration • Obstruction by inspissated stool Level of obstruction • Rectosigmoid • Ileum • Increased tone • Normal tone Rectal examination Positive squirt sign• • • Negative squirt sign• Meconium consistency • Normal • lnspissated • Dilated proximal colon ± small bowel • Dilated small bowel Imaging • Narrow rectosigmoid • Microcolon Associated disorder • Down syndrome • Cystic fibrosis Pathophysiology *Expulsionof gas/stool on rectal examination. 99% of full-term infants stool within 48 hours of birth. Meconium ileus T/t: Hyperosmolar Enema/ Surgical Management GIT 562 https://t.me/usmleinnercircle Version 2 irc le Hirschsprung disease ·Betweennam>W, aganglionic colon& dilatedPfOXimal colon. Cuw,,• rC Patients with a long segment of affected colon in Hirschsprung classically present in the newborn period with delayed passage of meconium. However, in patients with a short aganglionic colonic segment, symptoms often develop in infancy or early childhood and include chronic, refractory constipation and poor growth or failure to thrive. ne Patients do not respond to standard treatment of constipation. In Intussusception lntussusception . • Telescoping of one bowel segment into adjacent segment (most often ileocecal) LE Pathogenesis & epidemiology U SM Risk factors Clinical presentation Diagnosis & management Bowel edema -> ischemia & necrosis • Age 6 months to 3 years most common .. .. .. . . Hypertrophy of intestinal Peyer patches (eg, recent viral illness) Pathologic lead point (eg, Meckel diverticulum, t:!2e,intestinal tumor) Sudden, intermittent abdominal pain & vomiting Sausage-shaped mass in right abdomen Currant jelly stools Lethargy or altered mental status Ultrasonography: target sign • Air (pneumatic) or saline enema Surgical intervention for failed enema reduction or signs of (leritonitis HSP = Henoch-Sch6nlein purpura. Barium contrast enema is generally avoided in patients with suspected intussusception due to the risk of peritonitis if perforation occurs. GIT 563 https://t.me/usmleinnercircle Version 2 • Laparotomy is indicated if enema reduction is ineffective, if a pathological lead point is identified, or if the patient has signs of perforation (eg, free air on x-ray, rigid abdomen).  Most common age group: 636 months  A pathologic lead point should be suspected in patients with intussusception and the following features: • Recurrent episodes • Atypical location (eg, small bowel into small bowel) • Atypical age • Persistent rectal bleeding despite reduction The most common lead point is a Meckel diverticulum Dx with Nuclear Scintigraphy lleocolic intussusception GIT 564 https://t.me/usmleinnercircle Version 2 rC irc le lntussusception ne Meckel's Diverticulum Meckel's diverticulum In Rule of 2s • • • • 2% prevalence 2:1 male-to-female ratio 2% are symptomatic at age 2 Located within 2 feet of the ileocecal valve Clinical presentation • • • • • Asymptomatic incidental finding Painless hematochezia lntussusception Intestinal obstruction Volvulus Diagnosis Technetium-99m pertechnetate scan Treatment Surgery for symptomatic patients U SM LE Epidemiology NEC GIT 565 https://t.me/usmleinnercircle Version 2 Necrotizing enterocolitis • Gut mucosa! wall invasion by gas-producing bacteria Pathogenesis • Intestinal inflammation, necrosis • Prematurity Risk • Very low birth weight (<1.5 kg [3.3 lb]) factors • Enteral feeding • Nonspecific: apnea, lethargy, vital sign instability • Gastrointestinal Clinical findings X-ray o Abdominal distension o Feeding intolerance, bilious emesis o Bloody stools • Pneumatosis intestinalis (air in bowel wall) findings • Pneumoperitoneum (free air under diaphragm) Complications • Sepsis, disseminated intravascular coagulation • Late: strictures, short-bowel syndrome  Term infants with reduced mesenteric oxygen delivery from cyanotic congenital heart disease and/or hypotension are also at risk for intestinal ischemia and infarction.  Given the risk of lethal septic shock, empiric broad-spectrum antibiotics should be started immediately. Management of necrotizing enterocolitis • Discontinuation of enteral feeds Immediate • Nasogastric decompression interventions • Blood cultures & empiric antibiotics • Intravenous fluid repletion Monitoring • Serial complete blood count & electrolytes • Serial abdominal examinations & imaging Indications • Bowel perforation (pneumoperitoneum) for surgery • Clinical deterioration despite medical management (suggestive of bowel necrosis) Culture before antibiotics Necrotizing enterocolitis GIT 566 https://t.me/usmleinnercircle Version 2 Reye Syndrome Reye syndrome • Aspirin use in children during viral infection (eg, influenza, varicella) Pathophysiology . • Microvesicular fat deposits in the liver Cerebral edema • Acute liver failure Clinical features 0 Hepatomegaly without jaundice • Rapidly progressive encephalopathy 0 . • • . Vomiting, lethargy, seizure, coma Treatment i PT, INR, PTT ! Glucose Metabolic acidosis irc Laboratory findings le • i AST, ALT, ammonia Normal bilirubin • Supportive rC ALT= alanine aminotransferase(SGPT);AST= aspartate aminotransferase(SGOT). ne • Inc. ICP (secondary to hyperammonemia causing astrocyte edema (inc. opening pressure on LP major cause of death in Reye In Malnutrition LE Severe malnutrition U SM Types Management Complications of management .. . . .. . . . Marasmus (wasting) Kwashiorkor (edematous malnutrition) Combination of above Rewarming for hypothermia Antibiotics for presumed systemic infection Rehydration 0 Oral rehydration solution preferred 0 Intravenous fluids if in shock Refeed cautiously Heart failure Refeeding syndrome Functional Abdominal Pain GIT 567 https://t.me/usmleinnercircle Version 2 Functional abdominal pain Clinical features Management .. .. . .. Chronic Poorlylocalizedor periumbilical No vomiting, diarrhea, weight loss Normal examination Negative stool guaiac Reassurance Symptom diary GIT Surgery • Laparoscopic intraabdominal surgery (eg, cholecystectomy) requires insufflation of CO2 into the abdomen to create space for surgical maneuvering and visibility. The increased intraabdominal pressure stimulates stretch receptors on the peritoneum that respond by triggering an increase in vagal tone. Consequently, patients can develop severe bradycardia, atrioventricular block, and sometimes asystole. Bariatric Surgery Preparation for bariatric surgery • BMI ~40 kg/m2 • BMI ~35 kg/m 2 with serious comorbidity (eg, T2DM, hypertension, OSA) • BMI ~30 kg/m 2 with resistant T2DM or metabolic syndrome • Review previous attempts at weight loss, diet, exercise habits Intake Review psychiatric history, coping skills, readiness to change assessment • • Review risk for cardiac (eg, CAD) and pulmonary (eg, OSA) disease Indications CAD= coronary artery disease; OSA = obstructive sleep apnea; T2DM = type 2 diabetes mellitus. • Weight-loss medication is indicated for patients with a BMI 30 kg/m2 (obese) and those with a BMI of 2529.9 kg/m2(overweight) with weight-related complications  Due to reductions in dietary intake and malabsorption associated with bariatric surgery, patients can have micronutrient deficiencies in folate, iron, calcium, and vitamins D and B12 that can adversely affect fetal outcomes (eg, preterm delivery, stillbirth). Therefore, reproductive-aged women are recommended to delay pregnancy for at least a year after bariatric surgery to optimize weight loss and stabilize nutritional status. During this period, a nonoral form of contraception (eg, intrauterine device, implant) is recommended due to decreased absorption rates with oral contraceptive pills. Most common deficiency: Vitamin B1 GIT 568 https://t.me/usmleinnercircle Version 2 Gastric Bypass Biliopancreatic limb Jejunojejunal anastomosis ne Common limb rC Alimentary limb (Roux limb) Pancreatic enzymes & bile acids irc Distal stomach remnant le Roux-en-Y gastric bypass Stomal (anastomotic) stenosis, is caused by progressive narrowing of the GJ anastomosis that leads to obstruction of gastric pouch outflow. This complication usually occurs within the first year after In surgery, likely due to local tissue ischemia and ulceration. Patients typically have progressive symptoms including nausea, postprandial vomiting, gastroesophageal reflux, and dysphagia, to the point of not tolerating liquids. LE Diagnosis requires visualization of the GJ anastomosis via esophagogastroduodenoscopy EGD, during which balloon dilation can be performed to open the narrowing. Patients sometimes require surgical revision if balloon dilation fails. U SM  Right upper quadrant ultrasound is useful in diagnosing cholelithiasis, which is a common complication after Roux-en-Y, especially in the setting of rapid weight loss. Complications of Roux-en-Y gastric bypass surgery Early • Anastomotic leak (sepsis) • Bowel ischemia (diffuse abdominal pain) • Anastomotic stricture (dysphagia, bowel obstruction) Late • Marginal ulcer (abdominal pain, bleeding, peri-anastomotic perforation) • Cholecystitis (right upper quadrant pain) • Dumping syndrome (diarrhea, crampy abdominal pain, vasomotor) GIT 569 https://t.me/usmleinnercircle Version 2 Pathogenesis Etiology Symptoms Timing Management .. .. .. • . .. . Dumping syndrome Destruction or bl£1:1ass of the E!l£loricsi:1hincter Rapid emptying of hypertonic gastric contents Esophageal/gastric resection or reconstruction Vagal nerve injury (eg, Nissen fundoplication) Abdominal pain, diarrhea, nausea Hypotension/tachycardia Dizziness/confusion, fatigue, diaphoresis 15-30 min after meals Clinical diagnosis Small, frequent meals Replacement of simple sugars with complex carbohydrates • Incorporation of high-fiber & protein-rich foods  Anastomotic leak is a serious postoperative complication that can present with fever, abdominal pain, tachypnea, and tachycardia, usually within the first week after bariatric surgery. The diagnosis is best confirmed by oral contrast–enhanced imaging (either abdominal CT scan or upper gastrointestinal series), and treatment requires urgent surgical repair. Heart rate 120/min has been shown to be the most sensitive predictor of postoperative anastomotic leak. Billroth 2 Billroth II Pancreas & ducts , Du ' ,' Jejunum stomy Partial gastrectomy with gastrojejunostomy is most often performed to treat complicated peptic ulcer disease (eg, perforation, malignancy, gastric outlet obstruction) or ulcers refractory to medical management. In a Billroth II gastrojejunostomy, the gastric antrum is removed to decrease gastrin GIT 570 https://t.me/usmleinnercircle Version 2 production and for histopathologic evaluation. A side-to-side anastomosis is then made between the jejunum and the gastric body, bypassing the duodenum and proximal jejunum. Iron absorption occurs predominantly in the duodenum and proximal jejunum, and bypass of this segment of small bowel results in iron deficiency anemia. The post-surgical decrease in gastric acidity also diminishes iron absorption and may contribute to iron deficiency in these patients. Treatment is accomplished with pharmacologic iron supplementation, which allows for adequate iron absorption at secondary absorption sites in the distal small bowel. le Deficiency involving thiamine, folate, vitamin B12, fat-soluble vitamins (especially vitamin D, and calcium is also common following gastrojejunostomy. irc Nissenʼs Fundoplication Postoperative complications of Nissen fundoplication syndrome Gastroparesis Possibly caused by disruption of peristalsis due to tightening of the LES Symptoms develop within 12 weeks of surgery rC Gas-bloat .. . .. . .. .. Diagnosed clinically & usually self-resolves Likely caused by gastric air trapping due to tightening of the LES Symptoms include bloating & inability to belch Diagnosed clinically & usually resolves with conservative management* Caused by inadvertent vagal nerve injury ne Dysphagia Symptoms: bloating, early satiety, postprandial emesis, food aversion, weight loss Diagnosed via gastric scintigraphy + EGO to rule out obstruction Managed with small, low-fat, low-fiber meals± promotility agents** In *Including simethicone & avoidance of carbonated beverages. **Metoclopramidetypically first-line. U SM LE EGD = esophagogastroduodenoscopy;LES = lower esophageal sphincter. GIT 571 https://t.me/usmleinnercircle Version 2 Nissen fundoplication for gastroesophageal reflux disease (GERO) GERD Gastroesophageal junction (GEJ) Lower esophagealsphincter (LES) relaxation allows reflux ·Branchesof the Vagus nerve may be injuredduringthe procedure. C:,UW0<1d Pancreaticoduodenectomy  What are the complications of pancreaticoduodenectomy (Whipple procedure? • Anastomotic leak: hyperchloremic acidosis and high levels of amylase in abdominal secretions • Leakage of pancreatic secretions into abdominal cavity 0 Hyperchloremic acidosis → loss of HCO3 secretions in to the cavity; normal anion gap metabolic acidosis 0 Pancreatic fistula Prevention? Octreotide before and after pancreatic surgery to minimize pancreatic secretions Abdominal Trauma GIT 572 https://t.me/usmleinnercircle Version 2 Blunt abdominal trauma Hemodynamlcally unstable Hemodynamlcally stable Peritonitis? Peritonitis? Free fluid on FAST? Laparotomy + - 01' Inconclusive Consider CTAP (after resuscitation) or DPL. Evaluate for other sources of hemorrhage. Laparotomy - or inconduslve CTAP ConsiderCTAP en route to OR le + Free fluid on FAST? + Consider CTAP or serial abdominal examinations based on suspicion for intraabdominal injury. irc + rC CTAP= CT scan of the abdomen& pelvis, DPL = diagnosUcperitoneallavage, FAST = FocusedAssessmentwith SonographyIOI'Trauma; OR = operatingroom. ~UWond DPL is only used for blunt, NOT penetrating abdominal trauma evaluation (penetrating ne trauma warrants immediate surgical laparotomy)  Peritonitis from hollow viscus perforation should be suspected in patients experiencing suddenonset abdominal pain with significant tenderness and guarding. Patients with peritonitis tend to lie still In to minimize peritoneum irritation. An upright chest x-ray showing pneumoperitoneum can identify perforation. LE Do it before Lipase and Lactic acid as perforation requires immediate surgical intervention. Focused Assessment with Sonography for Trauma (FAST) Penetrating abdominal trauma U SM Indication for immediate laparotomy? Hemodynamic instability Pentonit,s Evisceration Impalement + Peritoneal penetration & significant organ injury (eg, based on imaging, local wound exploration)? + !- _j Close observation with serial abdominal examinations J Laparotomy IOUWorid GIT 573 https://t.me/usmleinnercircle Version 2 Rib fracture location & associated injuries Location Associated injuries Ribs 1-3 Subclavian vessels, brachia! plexus, mediastinal vessels (eg, aorta) Ribs 3-6 Cardiovascular Ribs 9-12 lntraabdominal: liver (right), spleen (left), kidney (posterior ribs 11 & 12) Any level Pulmonary  Any penetrating wound below the 4th intercostal space is considered to involve the abdomen. Therefore, suspicion of solid intraabdominal organ injury should prompt CT scan of the abdomen • Blunt abdominal trauma may result in damage to the mesenteric blood supply and bowel contusion, leading to subsequent necrosis and eventual GI perforation. can occur acutely or several days after the initial event (versus penetrating trauma which presents only acutely)  What is next step for blunt abdominal trauma with GI perforation identified on CT? Exploratory laparotomy  Which organs 2 are most commonly injured with blunt abdominal trauma? liver and spleen Liver laceration ClUWorld Trauma to the RUQ, right lower rib cage (eg, right eighth and ninth ribs), or right flank should raise suspicion for hepatic injury. Abdominal aortic injuries due to BAT are rare 1%; most people die before reaching hospital. GIT 574 https://t.me/usmleinnercircle Version 2 Pancreas is retroperitoneal; it can injure in BAT but will NOT cause intraperitoneal fluid collection Pancreatic Injury  The development of the following symptoms and signs in patients with recent BAT should raise suspicion for undiagnosed pancreatic injury: le • Persistent abdominal discomfort/tenderness • Persistent nausea/emesis • Peripancreatic fluid collection (due to pancreatic duct injury) irc • Increasing amylase over serial measurements Ductal injury may require cholangiopancreatography for diagnosis rC Splenic Rupture Atraumatic splenic rupture • Hematologic malignancy (eg, leukemia, lymphoma) ne • Infection (eg, CMV, EBV, malaria) • Inflammatory disease (eg, SLE, pancreatitis) Risk factors • Splenic congestion (eg, cirrhosis, pregnancy) In • Medications (eg, anticoagulation, G-CSF) • Diffuse or LUQ abdominal pain, peritonitis • Referred left shoulder pain (Kehr sign) Clinical presentation • Hemodynamic instability • Acute anemia LE Diagnosis Treatment • lntraperitoneal free fluid on imaging • Catheter-based angioembolization (stable patients) • Emergency splenectomy (unstable patients) CMV = cytomegalovirus;EBV = Epstein-Barrvirus; G-CSF = granulocyte-stimulating U SM colony factor; LUQ = left upper quadrant; SLE = systemic lupus erythematosus. • Infectious mononucleosis, caused by Epstein-Barr virus, is associated with splenomegaly in up to 50% of cases, and splenic rupture within a month of symptom onset is a rare but serious complication. Because splenic rupture can occur even if the spleen is not initially palpable, activities that increase the risk of rupture (eg, contact sports) should be avoided for 34 weeks from the time of initial diagnosis.  Patients with spontaneous splenic rupture should ideally be managed non-operatively (eg, observation with serial hemoglobin measurement, embolization) to preserve splenic function. However, patients who remain hemodynamically unstable despite adequate resuscitation (eg, due to severe ongoing hemorrhage) may require exploratory laparotomy and splenectomy Stabilize hypovolemia first. GIT 575 https://t.me/usmleinnercircle Version 2 Splenic laceration  If a hemodynamically stable and alert patient with suspected splenic injury has a normal FAST exam despite high-risk features (e.g. guarding), what is the next step? CT scan of abdomen Duodenum Perforation When viscus perforation occurs within the retroperitoneum (eg, duodenal tear), classic symptoms and signs (eg, fever, diffuse abdominal pain) may be delayed. Retroperitoneal free air may be present on abdominal imaging. Duodenal Hematoma • Duodenal hematomas classically develop in pediatric patients 24 - 36 hours after an initial injury, causing epigastric pain and vomiting. symptoms due to failure to pass gastric contents beyond the obstructing hematoma Dx: Abdominal CT  Management of a duodenal hematoma typically involves gastric decompression NG tube) and possibly parenteral nutrition. surgery or percutaneous drainage may be considered to evacuate hematoma if non-operative management fails if unstable and hypotensive • Angiography with embolization Often due to gastroduodenal artery) GIT 576 https://t.me/usmleinnercircle Version 2 rC irc le Duodenal hematoma Rectus Sheath Hematoma ne Rectus sheath hematoma • Abdominal trauma, forceful abdominal contractions (eg, coughing) Risk factors • Anticoagulation • Older age, female sex features • Acute-onset abdominal pain with palpable abdominal mass In Clinical • Blood loss anemia, leukocytosis • ± Nausea, vomiting, fever LE • Hemodynamically stable: serial monitoring of CBC, reverse anticoagulation and transfuse blood products when Management appropriate • Unstable: angiography with embolization, surgical ligation U SM CBC = complete blood count.  RSH typically occurs due to rupture of the inferior epigastric artery  Manifestations include acute abdominal pain, often associated with rebound or guarding, and a palpable abdominal wall mass. The mass does not cross the midline and does not change with movement of the lower extremities Fothergill sign). Wound Dehiscence GIT 577 https://t.me/usmleinnercircle Version 2 Evisceration Skin ----------c;;..-~, Subcutaneous tissu Peritoneum ----:---:---iil"":olllll, Omentum ---'-:---- CUWOfld  Postoperative abdominal wounds can be categorized based on fascial involvement: • Superficial wound dehiscences are separations of the skin and subcutaneous tissue with intact rectus fascia. These typically develop within the first postoperative week due to an abnormal subcutaneous fluid buildup (eg, seroma) and can often be managed conservatively with careful dressing changes. • Deep (fascial) wound dehiscences involve the rectus fascia (ie, nonintact) and result in exposure of the intraabdominal organs to the external environment. T/t: Emergency Surgery Pilonidal Sinus  What disease often manifests in young males as a painful, fluctuant mass above the anus in the intergluteal region with associated discharge? What is the treatment? Pilonidal disease; treat with incision and drainage due to an occluded, infected hair follicle; most frequently affects males age 1530, obese individuals, those with sedentary lifestyles, and those with deep gluteal clefts GIT 578 https://t.me/usmleinnercircle Version 2 GI Bleed • Packed red blood cell transfusions are recommended in acute gastrointestinal bleeding for patients with hemoglobin 7 g/dL. A higher threshold of hemoglobin 9 g/dL is considered for unstable patients with acute coronary syndrome or with active bleeding and hypovolemia. In addition, the hemoglobin level may drop significantly as blood volume is replaced by the infusion of crystalloid solutions and the mobilization of interstitial fluid. le • Platelet transfusions are generally reserved for patients with active bleeding and platelet count 50,000/mm3 or 10,000/mm3 without bleeding irc • Proton pump inhibitors reduce rebleeding and the need for transfusions, and help stabilize clots in patients with UGIB.  Patients with upper (but not lower) GI bleeding often have an elevated blood urea nitrogen (BUN) and rC elevated BUN/creatinine ratio. ne Possible causes include increased urea production from intestinal breakdown of hemoglobin and increased urea reabsorption in the proximal tubule due to associated hypovolemia. Age-based evaluation of occult gastrointestinal bleeding Occult gastrointestinal bl~ In l U SM LE Upper endoscopy & colonoscopy I Source not found + Small bowel bleed evaluation j Adult Child Capsule endoscopy Tc 99m pertechnetate scan i i .- Source not found - Angiodysplas,a. neoplasia, ulcers Meckel d1verticulum I Source not found i CT/MR enterography • Malignancy/mass ·sa11oon-ass1sted enteroscopymay alsobe consideredif sourceremainsunidentified Tc 99m = technetium Tc 99m. C)UWO<ld Variceal Bleeding GIT 579 https://t.me/usmleinnercircle Version 2 What procedure should be done prior to endoscopic treatment in patients with an upper GI bleed who have a depressed level of consciousness and continued hematemesis? Endotracheal intubation these patients are at high risk for aspiration and must have their airway protected before upper endoscopy Variceal hemorrhage bleed algorithm Suspected variceal hemorrhage Place 2 large-bore IV catheters • Volume resuscitation • IV octreotide •Antibiotics Urgent endoscopic therapy of esophageal varices No further bleeding Continued bleeding Early rebleeding Initiate secondary prophylaxis Balloon tamponade (temporary) Repeat endoscopic therapy Beta blocker + endoscopic band ligation 1-2 weeks later Recurrent hemorrhage I TIPS or shunt surgery J rv = intravenous:TIPS= transjugularintrahepalic portosvstemicshunt. OUWorld Octreotide causes splanchnic vasoconstriction and reduces portal blood flow by inhibiting release of glucagon Bacterial infections (like SBP) can develop in up to 50% of patients who are hospitalized for acute variceal bleeding; therefore, these patients should be treated prophylactically with antibiotics. The currently preferred regimen is intravenous ceftriaxone for 7 days, with a transition to trimethoprim-sulfamethoxazole or an oral fluoroquinolone (norfloxacin, ciprofloxacin) if patients are ready for discharge prior to completion of the intravenous antibiotic course. Hematochezia GIT 580 https://t.me/usmleinnercircle Version 2 Hematochezia Hemodynamically stable Hemodynamically unstable or UGIB suspected 1) Resuscitate 2) +/- Surgery/lR consult J)EGD Source not found Source not found Colonoscopy __ Source not found rC Obscure GI bleed evaluation (eg, capsule endoscopy, repeat EGD/colonoscopy) -11 Angiography I irc Source not found Hemodynamically unstable Source not found, Hemodynamically le Hemodynamically stable EGD = esophagogastroduodenoscopy; GI = gastrointestinal; IR = interventional radiology; UGIB = upper gastrointestinal bleed. ne Hemobilia ©UWor1d In  Hemobilia is a rare cause of upper gastrointestinal bleeding UGIB that usually occurs as a complication of hepatic or biliopancreatic interventions (eg, liver biopsy, cholecystectomy, endoscopic cholangiopancreatography); other etiologies include hepatobiliary tumors and blunt trauma. LE Patients typically present with RUQ pain, jaundice, and UGIB  Massive hemobilia results in hemodynamic instability and UGIB that occurs immediately after the procedure; U SM intraductal hematoma formation more commonly results in delayed UGIB until after dissolution of the clot (usually 5 days post-procedure). T/t: Usually self limited, managed conservatively GIT 581 https://t.me/usmleinnercircle Version 2 Blood & Oncology RBC Microcytic Anemia Macrocytic Anemia Hemolytic anemia G6PD PNH Sickle Cell Anemia Hereditary Spherocytosis Macroangiopathic Hemolytic Anemia Acute Intermittent Porphyria WBC Acute Leukemia Chronic Leukemia Hodgkin Lymphoma Plasma Cell Dyscarias Multiple Myeloma Platelets Hemostasis Vitamin K Deficiency ITP TTP HUS DIC Hemophilia Hereditory Thrombophilias Pseudothrombocytopenia Drugs Heparin EPO Tumour Blood Oncology Therapies Radiotherapy PET Scan Cancer Pain Miscellaneous Lymphangitis Idiopathic Anemia of Ageing Blood Transfusion Transfusion Overload Catheter Induced Septic Thrombophlebitis Blood & Oncology 582 https://t.me/usmleinnercircle Version 2 Paeds Blood Polycythemia Anemia of Prematurity Physiologic Anemia of Newborn IDA Pancytopenia Anemia of chronic disease Malignancy • Chronic infection Rheumatic disease Common Etiologies • • Obesity . irc . Diabetes mellitus rC Pathogenesis • j inflammatory cytokines (eg, hepcidin) • Inhibition of ferroportin on enterocytes and macrophages • t iron absorption and j iron sequestration • Reduced circulating iron _, impaired erythropoiesis le RBC • • Normocytic/slightly microcytic anemia t serum iron, iron-binding capacity, reticulocyte count Laboratory findings • • j bone marrow iron • Lower than expected erythropoietin for degree of anemia Microcytic Anemia ne • Treat underlying condition causing inflammation In Treatment Congestive heart failure of thalassemia? CBC LE  What is the most appropriate screening test in a preconception patient with a family history U SM If there is anemia with a reduced MCV, then further testing is required (Hb electrophoresis)  What is the likely underlying cause of microcytic anemia in a patient with chronic kidney disease that recently began erythropoietin therapy? Iron deficiency the erythropoietin-induced surge in RBC production can precipitate iron deficiency Remember, patients with CKD can develop anemia due to loss of EPO. When EPO is given, an iron deficiency anemia can develop due to depletion of iron stores during hematopoiesis after administration, especially if iron stores are already low. The treatment of choice for iron deficiency in patients on dialysis is intravenous iron. Blood & Oncology 583 https://t.me/usmleinnercircle Version 2 Iron deficiency Parameter anemia a-thalassemia minor 13-thalassemia minor MCV t t t ROW i Normal Normal RBCs t Normal Normal Target cells Target cells t Iron & ferritin i TIBC Normal/j iron & ferritin (RBC Normal/j iron & ferritin (RBC turnover) turnover) i Hemoglobin No improvement No improvement Normal Normal i Hemoglobin A2 Microcytosis, Peripheral smear Serum iron studies hypochromia Response to iron supplementation Hemoglobin electrophoresis MCV = mean corpuscular volume; RBCs = red blood cells; ROW= red cell distribution width; TIBC = total iron-binding capacity. RDW 20% in IDA  What is a useful way to tell Iron Deficiency Anemia from minor thalassemia on blood count from MCV and RBC count? Mentzer index: MCV/RBC 13 is minor thalassaemia 13 is IDA This is useful if they are really vague with their question with limited information available IDA may be associated with reactive thrombocytosis (platelets 400,000/mm3 in response to low red blood cell count. This change is due to megakaryocytes and erythrocytes sharing a common progenitor cell. Macrocytic Anemia Common causes of macrocytosis • Hemolytic anemia • Bone marrow recovery Increased reticulocytes Impaired RBC maturation • Vitamin Bdfolate deficiency • Medications (eg, hydroxyurea, methotrexate) • Myelodysplastic syndrome Bone marrow disorders Lipid abnormalities • Sideroblastic anemia • Multiple myeloma • Liver disease • Hypothyroidism RBC= red bloodcell. Blood & Oncology 584 https://t.me/usmleinnercircle Version 2  What is the most common cause of vitamin B12 deficiency? Pernicious anemia  What is the likely underlying cause of anemia in a patient with a history of gastrectomy that presents with a shiny tongue, pale palmar creases, and laboratory evidence of hemolysis? Labs show indirect hyperbilirubinemia and elevated LDH. Vitamin B12 deficiency (impaired DNA synthesis) deficiency due to loss of intrinsic factor; high numbers of immature megaloblasts in the bone irc le marrow results in increased intramedullary hemolysis, thus causing indirect hyperbilirubinemia and elevated LDH Patients with moderate to severe megaloblastic anemia can have severe hypokalemia during the first 48 hours of treatment with vitamin B12. U SM LE In ne rC Close monitoring and supplementation of potassium is required during this period. Triphalangeal thumbs in Diamond Blackfan Hemolytic anemia G6PD  What is the likely diagnosis in an African-American patient that presents with dark urine that stains positive with Prussian blue after being treated for a urinary tract infection? G6PD deficiency positive stain indicates presence of hemosiderin in the urine due to hemolysis; hemolytic episodes are often precipitated by infection or medications (increased oxidative stress)  What is the likely diagnosis in an African-American male that develops dark urine, fatigue, and jaundice after being treated with TMPSMX? Peripheral smear reveals bite cells, but a G6PD activity assay is normal. Blood & Oncology 585 https://t.me/usmleinnercircle Version 2 G6PD deficiency most erythrocytes with severe G6PD deficiency are hemolyzed early during the acute episodes, thus the G6PD assay may initially be abnormally normal; it is best to wait 3 months after the episode before re-testing Hemoglobinuria – heme is excreted in the kidneys, which causes dark-colored urine and a false-positive urine dipstick for blood the heme in hemoglobin causes a positive urine dipstick test, but no erythrocytes are seen on urine microscopy Medications that often trigger hemolysis in G6PD deficiency* Diaminodiphenyl sulfone (dapsone) lsobutyl nitrite Avoid Nitrofurantoin Rasburicase Primaquine Acetaminophen Acetylsalicylic acid (aspirin) Chloramphenicol Chloroquine Colchicine Diphenhydramine (Benadryl) Use with caution Glyburide lsoniazid L-Dopa Quinine Sulfamethoxazole Trimethoprim Vitamin K 'This list is not exhaustive; it includes medications commonly used in the clinical setting. G6PD = glucose-6-phosphate dehydrogenase. PNH Blood & Oncology 586 https://t.me/usmleinnercircle Version 2 fatigue • Hemolysis • Cytopenias (impaired hematopoiesis) • Venous thrombosis (intraabdominal, cerebral veins) Workup • Complete blood count (hypoplastic/aplastic anemia, thrombocytopenia, leukopenia) • Elevated lactate dehydrogenase & low haptoglobin (hemolysis) • Indirect hyperbilirubinemia • Urinalysis (hemoglobinuria) • Flow cytometry (absence of CD55 & CD59) Treatment • Iron & folate supplementation • Eculizumab (monoclonal antibody that inhibits complement activation) irc Clinical manifestations le Clinical features of paroxysmal nocturnal hemoglobinuria • Reason for increase thrombosis Venous thrombosis is caused by vasoconstriction and platelet rC aggregation as a result of free hemoglobin (due to hemolysis) scavenging serum nitric oxide (a vasodilator) and, according to the prevalent hypothesis, activating the endothelial lining of blood vessels. ne Sickle Cell Anemia Cause Reticulocytes Aplastic crisis In Acute severe anemia in sickle cell disease Key features • Transient arrest of erythropoiesis L Splenic sequestration crisis LE i • Secondary to infection (eg, parvovirus B19) • Splenic vaso-occlusion _, rapidly enlarging spleen • Occurs in children prior to autosplenectomy U SM Parvovirus B19 infection can result in an aplastic crisis with no preceding viral symptoms. Blood transfusions are the mainstay of treatment. Management of sickle cell anemia • Vaccination Maintenance • Penicillin (until age 5) • Folic acid supplementation • Hydroxyurea (for patients with recurrent vaso-occlusive crises) • Hydration Acute pain crises • Analgesia • +/- Transfusion Pneumococcal Vaccine, Twice daily penicillin Blood & Oncology 587 https://t.me/usmleinnercircle Version 2 Vaso-occlusive pain episode Clinical features Management . . . . .. . Infections in sickle cell disease Infection Acute, severe pain Pain at ~1 site (eg, dactylitis) Pneumonia ± Low-grade fever May be triggered (eg, by dehydration) Osteomyelitis/septic arthritis Analgesics (NSAIDs, opiates) Hydration Bacteremia/sepsis ± RBC transfusion NSAID = nonsteroidal anti-inflammatory drug; RBC = red blood cell. Meningitis . Etiology Streptococcus pneumoniae • Staphylococcus aureus • Salmonella species . • Streptococcus pneumoniae Haemophilus influenzae type B • Streptococcus pneumoniae IV Opiates (e.g Morphine) are given for adequate Analgesia in emergency situation.  Transfusion should be administered cautiously (eg, half of normal volume) because RBCs in the spleen may return to circulation, resulting in a rapid increase in hemoglobin and complications such as hyperviscosity syndrome. Acute splenic sequestration Pathophysiology • Complication of sickle cell disease seen in early childhood • Vasoocclusion within spleen, causing trapping of red blood cells & platelets • Abdominal pain Clinical features Laboratory findings • Palpable splenomegaly • Signs of anemia (tachycardia, pallor, fatigue) • Hypotensive shock • Acute drop in hemoglobin • Reticulocytosis • Thrombocytopenia • Isotonic fluid resuscitation Treatment • Red blood cell transfusion • ± Splenectomy Thrombocytopenia (as platelets are also trapped in the spleen) • Hyposthenuria is an impairment in the kidney's ability to concentrate urine producing nocturia in patients with sickle cell anemia and trait. Hyperosmolar conditions of the renal medulla, red blood cells sickle in the vasa recta, impairing free water reabsorption and countercurrent exchange Urine osmolality is low; however, normal serum sodium is maintained due to intact ADH In patients with sickle cell disease, chronic damage and autoinfarction typically causes functional asplenia by age 5. Blood & Oncology 588 https://t.me/usmleinnercircle Version 2 How long is risk of sepsis in patients with splenectomy ? 30 years  What is the most common cause of sepsis in patients with sickle cell disease? Streptococcus pneumoniae le Broad-spectrum empiric antibiotics (ceftriaxone) should be given to patients with SCD presenting with sepsis irc  What is the likely diagnosis in a child with a history of sickle cell disease that presents with hypovolemic shock in the setting of LUQ tenderness and splenomegaly? Splenic sequestration rC hypovolemic shock occurs due to pooling of RBCs within the spleen  What is the most common complication of sickle cell trait? Painless hematuria (microscopic or gross) due to sickling in the renal medulla; isosthenuria (inability to concentrate urine) is common as ne well, and manifests as nocturia and polyuria Electrophoresis patterns in sickle cell syndromes HbA2 HbF HbS HbC ++++ + + None None Sickle cell trait +++ + + +++ None Sickle cell anemia (SCA) None + + ++++ None SCA on hydroxyurea None + ++ +++ None Hemoglobin SC disease None + + +++ +++ U SM LE Normal In HbA Blood & Oncology 589 https://t.me/usmleinnercircle Version 2 Stroke in sickle cell disease • lschemic stroke 0 Intimal hyperplasia & stenosis (vasculopathy) o Adhesion of sickled red blood cells to vasculature Pathogenesis • Hemorrhagic stroke 0 Weakened cerebral vessels 0 Cerebral aneurysm rupture • Focal neurologic symptoms (eg, hemiparesis) • Seizure Presentation • Altered mental status • MRI of the head: ischemic stroke (large vessel region or watershed area) • CT scan of the head: hemorrhagic stroke • Exchange transfusion Imaging Treatment • Simple transfusion if exchange transfusion is unavailable • Transcranial Doppler screening during childhood • Hydroxyurea Primary prevention • ± Chronic transfusions Differential diagnosis of bone pain in sickle cell disease Cause Vaso-occlusive crisis .. .. Osteomyelitis • Acute, severe pain Clinical features . .. . Avascular necrosis • Acute or subacute pain Pain ~1 site (eg, dactylitis) +/. Low-grade fever Erythema & warmth May be preceded by trigger (eg, dehydration) Focal pain at 1 site (eg, long bone) . Chronic, worsening pain (eg, femoral head) Prolonged fever • Absence of fever Erythema & warmth • Absence of warmth or erythema Positive blood culture • Dactylitis presents at age 6 months to 4 years with an acute onset of pain and symmetric swelling of the hands and feet. Chronic dyspnea in sickle cell disease Cause Asthma Symptoms • Intermittent/chronic wheezing • May be worse at night or with exercise or upper . reseirato!}'. infection Pulmonary hypertension Pulmonary fibrosis Blood & Oncology . . • Exertional dyspnea Exertional dyspnea • Progressive . • . Pulmonary function testing showing reversible airway obstruction • Tricuspid regurgitation on echocardiography Signs of right-sided heart failure (eg, jugular venous distension, edema) Diagnostic findings i Pulmonary arterial pressure on right-sided heart catheterization Honeycomb pattern on chest CT scan Pulmonary function testing showing restrictive pattern 590 https://t.me/usmleinnercircle Version 2 Sickle cell disease in pregnancy • Baseline 24-hr urine collection/testing for total protein • Baseline chemistry panel • Serial urine cultures Prenatal care • Pneumococcal vaccination • Felic acid supplement • Aspirin • Serial fetal growth ultrasound examinations Hypoplastic Thumb in Fanconi Anemia • Spontaneous abortion le Obstetric complications • Preeclampsia, eclampsia • Abruptio placentae irc • Antepartum bleeding • Fetal growth restriction Fetal complications • Oligohydramnios rC • Preterm birth  What are some complications of chronic hemolytic anemia in Sickle Cell patients? • Baseline bilirubin increase ne • Anemia • Increase reticulocyte count (except aplastic crisis) In • Folate deficiency • Iron deficiency anemia .. LE Pathogenesis U SM . Diagnostic criteria Initial treatment Blood & Oncology .. . Acute chest syndrome Vasoocclusion of pulmonary vasculature Most commonly triggered by fat embolus (usually adults) or infection (usually children) New pulmonary infiltrate on chest x-ray PLUS .:1 of the following: 0 Increased work of breathing, cough, tachypnea, or wheezing o Temperature >38.5 C (101.3 F) 0 Hypoxemia 0 Chest pain Ceftriaxone & azithromycin Intravenous fluids Pain control 591 https://t.me/usmleinnercircle Version 2 Pathophysiology of acute chest syndrome Acute vasoocclusive pain Atelectasis (from splinting/pain) Fat embolus (from bone marrow necrosis) l Pulmonary infarction/inflammation Pulmonary infection, asthma exacerbation l j Regional alveolar hypoxia L ,,L""''"'. ~.-,.,,l, in pulmonary vasculature ! Acute chest syndrome" RBC = red blood cell. ·New pulmonarydensityon chestimagingwithfever,chestpain,&/or respiratorysymptoms. QIJV!,lorld Hereditary Spherocytosis  What is the likely diagnosis in a child with a family history of anemia that presents with Coombsnegative hemolytic anemia, jaundice, and splenomegaly? Hereditary Spherocytosis Macroangiopathic Hemolytic Anemia  Mechanical aortic valves often cause mild, asymptomatic hemolysis. However, valve deterioration can trigger progressive symptomatic anemia (eg, fatigue, dyspnea with exertion) with signs of erythrocyte lysis (eg, jaundice, dark urine) and reticulocytosis. Thrombocytopenia can also occur because platelets may be damaged as they pass through the rigid valve opening. • Hepatosplenomegaly can develop as the fragmented erythrocytes are cleared in the macrophages and monocytes within the liver and spleen. Peripheral blood smear generally shows schistocytes. A transthoracic echocardiogram should be performed to visualize valve function and determine valvular pressure gradients. Acute Intermittent Porphyria Blood & Oncology 592 https://t.me/usmleinnercircle Version 2 Acute intermittent porphyria Etiology • Autosomal dominant disorder with low penetrance • Reduced activity of porphobilinogen deaminase • Medications (eg, cytochrome P-450 inducers, progesterone) Exacerbating factors • Physiologic stress (eg, fasting, surgery, illness) • Alcohol, tobacco • Abdominal pain • Peripheral neuropathy most pronounced in the proximal upper extremities Manifestations • Autonomic dysfunction (eg, tachycardia, diaphoresis, hypertension) Management irc Laboratory findings • Red-tinged urine that oxidizes with light/air exposure • Elevated serum & urinary PBG, ALA, porphyrins • ± Hyponatremia, elevated transaminases • Glucose & hemin (heme analogue) le • Neuropsychiatric (eg, hallucinations, anxiety, psychosis) ALA= aminolevulinicacid; PBG = porphobilinogen. rC Abdominal pain: Because the pain is neuropathic, it can be diffuse, although tenderness, rebound, and guarding are absent. Nausea/vomiting, hypoactive bowel sounds, and constipation may occur. WBC U SM LE Acute Leukemia In ne Urobilinogen levels are typically elevated, and elevated urinary porphyrin levels confirm the diagnosis. Blood & Oncology 593 https://t.me/usmleinnercircle Version 2 Acute lymphoblastic leukemia Epidemiology • Most common childhood cancer • Peak age: 2-5 years • Nonspecific systemic symptoms (eg, fever, weight loss) • Leukemic cells overcrowd bone marrow o Pallor/fatigue (anemia) o Easy bruising/petechiae (thrombocytopenia) o Bone pain • Extramedullary spread Clinical features o Lymphadenopathy o Hepatosplenomegaly o Testicular enlargement • Mediastinal mass (T cell lineage): airway compression &/or superior vena cava syndrome • Leptomeningeal spread: neurologic symptoms (eg, cranial nerve deficits, meningismus) • Complete blood count* Evaluation & diagnosis • Bone marrow biopsy (>20% blasts is diagnostic) with flow cytometry • Lumbar puncture to evaluate for CNS involvement •;,2 cytopenias (leukocytes may be L or j), ± blasts on peripheral smear. - Chronic Leukemia Leukemoid reaction Chronic myeloid leukemia Leukocyte count >50,000/mm3 Elevated (often >100,000/mm3 ) Cause Severe infection BCR-ABL fusion LAP score High Low Neutrophil precursors More mature (metamyelocytes > myelocytes) Less mature (metamyelocytes < myelocytes) Absolute basophilia Not present Present LAP = leukocytealkalinephosphatase. Blood & Oncology 594 https://t.me/usmleinnercircle Version 2 Chronic lymphocytic leukemia Lymphadenopathy (cervical, supraclavicular, axillary) Hepatosplenomegaly Mild thrombocytopenia & anemia Often asymptomatic Diagnostic • Severe lymphocytosis & smudge cells • Flow cytometry • Lymph node & bone marrow biopsy not generally needed Prognostic • Median survival 10 years • Worse prognosis with: o Multiple cfiain lymphadenopathy o Hepatosplenomegaly o Anemia & thrombocytopenia • Infection • Autoimmune hemolytic anemia • Secondary malignancies (eg, Richter transformation) rC irc Complications Hairy cell leukemia Manifestations Diagnosis . . BRAF mutation Pancytopenia due to bone marrow fibrosis o Granulocytopenia (infections) o Anemia (fatigue, weakness) o Thrombocytopenia (bleeding, bruising} Splenomegaly (early satiety} Hepatomegaly/lymphadenopathy rare Peripheral smear - "hairy" leukocyte cells Bone marrow biopsy with flow cytometry Hairy Cell Chemotherapy (for moderate/severe} Life expectancy is often near-normal U SM Treatment . . . . Middle age/older adults ne . Clonal B-cell neoplasm In . . . LE Features le • • • • Clinical Hodgkin Lymphoma  What is the likely diagnosis in a patient with PMHx of Hodgkin lymphoma statuspost chemotherapy/radiation who presents with cough, dyspnea, and chest pain? Secondary malignancy there is an 18.5-fold increased risk for developing a second cancer in HL patients compared to the general population (likely related to chemo- and/or radiation therapy at a young age); most common sites are lung, breast, thyroid, bone, and GI  Patients with Hodgkin lymphoma HL are generally treated with combination chemotherapy and radiation therapy, which cures 75% of cases. However, these treatments are associated with serious long-term complications, including: Blood & Oncology 595 https://t.me/usmleinnercircle Version 2 • HL relapse (or recurrence): The leading cause of death for the first 810 years after treatment is HL relapse. Relapses primarily occur in the first 2 years, but risk remains mildly elevated for life. • Secondary malignancy Radiation exposure increases the risk of solid organ (eg, breast, lung) malignancy, and chemotherapy increases the risk of hematologic malignancy. Most cases arise 5 years after treatment. Secondary malignancies are the leading cause of death in those who have been cured of HL. • Cardiovascular disease Risk of coronary artery disease, valve damage, peripheral vascular disease, and cardiomyopathy are increased in those who have been treated for HL. Cardiovascular disease is the leading nonmalignant cause of death in long-term HL survivors. Other common treatment-related complications include pulmonary disease (eg, fibrosis, bronchiectasis) and hypothyroidism from chest radiation and neuropathy from chemotherapy. .,. .,..... .,..... .,..... Second malignancy / / Recurrence / .,..... 15 Years after treatment  Patients with HL occasionally have severe pain in areas of lymphadenopathy (eg, chest) following exposure to small quantities of alcohol (eg, drinking a beer). The etiology is unclear, but the pain may be due to alcohol-induced vasodilation within the lymph nodes, causing capsular distension. Plasma Cell Dyscarias Multiple Myeloma Blood & Oncology 596 https://t.me/usmleinnercircle Version 2 irc le Multiple myeloma (MM) Waldenstrom macroglobulinemia Peripheral smear • • • • Osteolytic lesions/fractures Anemia Hypercalcemia Renal insufficiency lgM lgG, lgA, light chains Rouleaux Rouleaux >10% clonal B cells >10% clonal plasma cells LE Bone marrow biopsy Hyperviscosity syndrome Neuropathy Bleeding Hepatosplenomegaly Lymphadenopathy ne Monoclonal antibody • • • • • Multiple myeloma In Major manifestations OI..JWofk: rC "Moth-eaten" appearancedue to aggressive,irregUarbonemarrowdestruction(nonspecific for MM) U SM  What screening tests would be useful for evaluating an elderly patient with chronic arm pain, anemia, and back pain? Serum/urine protein electrophoresis (best initial test) or free light chain analysis  A patient is diagnosed with multiple myeloma. What imaging should be performed? Full body XRay skeletal survey looking for lytic bone lesions/pathological fractures Bone scans are not useful in the diagnosis of MM as they detect only osteoblastic activity Platelets Blood & Oncology 597 https://t.me/usmleinnercircle Version 2 Common causes of thrombocytopenia Viral infections (eg, Epstein-Barr virus, hepatitis C, HIV) Decreased platelet production Chemotherapy Myelodysplasia (especially for age >60) Alcohol use Congenital (eg, Fanconi syndrome) Vitamin Bl 2 or folate deficiency Systemic lupus erythematosus Increased platelet destruction • Medications (eg, heparin) Idiopathic thrombocytopenic purpura, disseminated intravascular coagulation, thrombotic thrombocytopenic purpura-hemolytic uremic syndrome Antiphospholipid syndrome Other Dilutional due to massive red blood cell transfusion Splenic sequestration Hemostasis Bleeding disorders Type Symptoms • Hemarthrosis Clotting defect Platelet aggregation defect • Deep tissue hematomas • Easy or prolonged mucosa! bleeding • Ecchymoses Thrombocytopenia • Petechiae Examples • Hemophilia A • Hemophilia B • von Willebrand disease • Bernard-Soulier syndrome • Idiopathic thrombocytopenic purpura Laboratory results 1'Activated partial thromboplastin time Abnormal platelet function testing -l-Platelet count • Leukemia Blood & Oncology 598 https://t.me/usmleinnercircle Version 2 Elevated PT &/or PTT Corrects, normal PT &/or PTT Does not correct, abnormal PT&/or PTT Factor deficiency, evaluate for individual factor assays Inhibitor likely present, test for coagulation factor inhibitors le 1:1 inhibitor mixing study irc ©UWo~d  Normal plasma provides coagulation factors; therefore, it corrects prolonged PTT in the setting of rC coagulation factor deficiency but fails to correct prolonged PTT in the setting of a coagulation inhibitor. One of the most common coagulation inhibitors is lupus anticoagulant LA, a type of an antiphospholipid antibody. LA binds to the phospholipids used in most PTT tests and prevents them from inducing coagulation (thereby prolonging PTT. ne This laboratory artifact is not improved with the addition of plasma (mixing test) but is resolved with the addition of phospholipids (which eventually overwhelm all the antibody binding sites). In Vitamin K Deficiency .. .. .. .. . Infantile vitamin K-deficient bleeding Low vitamin K stores (poor placental transfer, sterile gut, low content in breast milk) Inefficient vitamin K use by immature liver LE Pathophysiology U SM Clinical features Laboratory findings Prevention . Classically presents on day 2-7 of life* Easy bruising Umbilical, mucosal & gastrointestinal bleeding lntracranial hemorrhage j PT j PTT (if severe) Normal platelet count Intramuscular vitamin Kat birth •can occur up to age 6 months. Blood & Oncology 599 https://t.me/usmleinnercircle Version 2 Vitamin K deficiency • Inadequate dietary intake (eg, malnutrition) • Disorders of fat malabsorption Risk factors . • Clinical features Laboratory findings . • • . 0 Cystic fibrosis 0 Biliary atresia Disorders of intestinal inflammation 0 Celiac disease 0 Inflammatory bowel disease Decreased production by bacterial flora (eg, frequent antibiotic use) Easy bruising Mucosal bleeding Gastrointestinal bleeding t PT & INR • If severely deficient, t PTT  What is the likely diagnosis in an alcoholic patient that begins bleeding from an IV site on postoperative day 7 after having an emergency surgery? Laboratory exam reveals anemia with elevated PT/PTT and normal platelet counts. Vitamin K deficiency acutely ill patients with underlying liver disease can become vitamin K deficient in 7  10 days, especially if they receive broad-spectrum antibiotics! PTT can be normal or elevated ITP  What two viruses should be tested for in an asymptomatic patient with idiopathic thrombocytopenic purpura? HIV and hepatitis C Secondary ITP may be the initial presentation of HIV infection in 510% of patients Immune thrombocytopenia Etiology Clinical findings Laboratory findings • Platelet autoantibodies • Preceding viral infection • Petechiae, ecchymosis • Mucosal bleeding (eg, epistaxis, hematuria) • Isolated thrombocytopenia <100,000/mm 3 • Few platelets (size normal to large) on peripheral smear • Children o Observe if cutaneous symptoms only o Glucocorticoids, IVIG, or anti-D if bleeding Treatment • Adults o Observation if cutaneous symptoms AND platelets .:30,000/mm 3 o Glucocorticoids, IVIG, or anti-D if bleeding or platelets <30,000/mm 3 IVIG = intravenousimmunoglobulin. Anti-D immune globulin (if Rh-positive and Coombs-negative) Blood & Oncology 600 https://t.me/usmleinnercircle Version 2 • Second-line therapies :Rituximab, thrombopoietin receptor agonists, and splenectomy • Platelet transfusions are reserved for life-threatening hemorrhage in patients with ITP TTP Thrombotic thrombocytopenic purpura Pathophysiology • t ADAMTS13 level -+ uncleaved vWF multimers -+ platelet trapping & activation • Acquired (autoantibody) or hereditary • Hemolytic anemia (i LDH, t haptoglobin) with schistocytes LDH = lactate dehydrogenase;vWF = von Willebrandfactor. irc Management • Renal failure • Neurologic manifestations • Fever • Plasma exchange • Glucocorticoids • Rituximab rC Clinical features le • Thrombocytopenia (i bleeding time, normal PT/PTT) Sometimes with: UPDATE Caplasizumab added in management Text ne  TTP is life threatening and must be treated urgently with plasma exchange PEX. PEX removes the patient's plasma and replaces it with donor plasma. This replenishes ADAMTS13 and removes the autoantibodies. In Without emergent PEX, the mortality rate is approximately 90%. LE HUS Hemolytic uremic syndrome U SM Pathogenesis Clinical features Laboratory findings Treatment • Escherichia coli serotype 0157:H? or Shigella dysenteriae • Vascular damage & microthrombi formation • Preceding bloody diarrhea • Fatigue, pallor • Bruising, petechiae • Oliguria, edema • Hemolytic anemia (schistocytes, j bilirubin) • Thrombocytopenia • Acute kidney injury (i BUN, j creatinine) • Fluid & electrolyte management • Blood transfusions • Dialysis BUN = blood urea nitrogen.  Treatment of the prodromal diarrhea with antibiotics or antimotility agents (eg, loperamide), as in this case, can increase the risk of developing HUS and is NOT recommended. Blood & Oncology 601 https://t.me/usmleinnercircle Version 2 DIC Acute vs chronic disseminated intravascular coagulation (DIC) Acute DIC Chronic DIC Sepsis Common etiologies Severe trauma Malignancy (eg, pancreatic) Obstetric complications Coagulation studies Prolonged Often normal Platelets Low Often normal Fibrinogen Low Often normal D-dimer High High Very high Mildly increased Bleeding risk Thromboembolism risk Mildly increased Very high Hemophilia  What is the likely diagnosis in an adolescent with a history of hemophilia A that presents with gradually worsening pain and limited motion of his knee? Hemophilic arthropathy due to hemosiderin (iron) deposition leading to synovitis and fibrosis within the joint; risk is significantly reduced with prophylactic factor concentrates Normal knee Hemophilic arthropathy • Inhibitor development occurs in approximately 25% of patients with severe hemophilia A (ie, frequent, spontaneous bleeds) as a complication of treatment. The immune system recognizes the infused factor as foreign, forming antibodies that interfere with factor function. Blood & Oncology 602 https://t.me/usmleinnercircle Version 2 Therefore, an inhibitor should be suspected when a patient with hemophilia A on factor replacement therapy develops increased bleeding frequency or has hemorrhage refractory to treatment. Treatment of an acute bleed in a patient with inhibitor development often involves bypassing products (eg, recombinant activated factor VII, activated prothrombin complex concentrates); such agents work downstream in the coagulation cascade to promote clotting without the need for factor VIII. le Hereditory Thrombophilias irc Hereditary thrombophilias* • Most common in those of white ethnicity • Activated protein C resistance Factor V Leiden . • 2nd most common in those of white ethnicity t Prothrombin levels Antithrombin deficiency • Inherited form is rare • Acquired: DIC, cirrhosis, nephrotic syndrome . JInactivation of factors Va & VIiia • Warfarin-induced skin necrosis (protein C only) ne Protein C or S deficiency rC Prothrombin mutation U SM LE In Pseudothrombocytopenia ©UWond !Clumps of platelets!  Pseudothrombocytopenia is a laboratory error caused by platelet aggregation in vitro. Most cases are due to incompletely mixed blood samples or the presence of serum antibodies to ethylenediaminetetraacetic acid EDTA), an anticoagulant used in hematologic testing. The error is generally identified when a patient with mild thrombocytopenia has peripheral blood smear evidence of large clumps of platelets Blood & Oncology 603 https://t.me/usmleinnercircle Version 2 Bleeding Reversal Reversal for life-threatening bleeding Drug Mechanism of action Tissue plasminogen . Activation of plasminogen {to plasmin) activators .! Xa Direct inhibition of clot- .. factors {eg, rivaroxaban, . apixaban) aminocaproic acid) .. .. Production of vitamin K-dependent clotting Direct inhibition of factor Dabigatran . Heparin .t bound & free thrombin Ill Cryoprecipitate Antifibrinolytic agents {eg, tranexamic acid, t fibrin degradation Warfarin Factor Xa inhibitors .. Reversal PCC Vitamin K PCC Recombinant modified factor Xa {eg, andexanet) PCC ldarucizumab {mAb fragment that binds dabigatran) Activity of antithrombin . Protamine sulfate mAb = monoclonalantibody; PCC = prothrombincomplex concentrate(containsvitamin K- dependent clottingfactors). Management of supratherapeutic INR INR Bleeding Treatment <4.5 None or minimal 4,5-10 None or minimal >10 None or minimal Hold warfarin & administer high-dose (2.5-5 mg) oral vitamin K Any Serious or life threatening Hold warfarin & administer intravenous vitamin K 10 mg as well as PCC Hold warfarin for 1 or 2 da:t'.sor decrease dose Hold warfarin & resume when INR is therapeutic; administer low-dose (1-2.5 mg) oral vitamin K if there is increased risk of bleeding PCC = prothrombin complex concentrate. Blood & Oncology 604 https://t.me/usmleinnercircle Version 2 Drugs/supplements that affect warfarin metabolism (selected) • Acetaminophen, NSAIDs • Antibiotics/antifungals (eg, metronidazole) • Amiodarone • Cimetidine • Cranberry juice, Ginkgo biloba, vitamin E • Omeprazole • Thyroid hormone • SSRls (eg, fluoxetine) t Warfarin effect ( t bleeding risk) CYP450 • • • • • Inducers I Warfarin effect U in efficacy) Carbamazepine, phenytoin Ginseng, St. John's wort Oral contraceptives Phenobarbital Rifampin le Inhibitors irc CYP450 CYP450 = cytochrome P-450; NSAIOs = nonsteroidal anti-inflammatory drugs; SSRls = selective serotonin reuptake inhibitors. ©UWorld weight heparin Optimal timing Unfractionated heparin SubQ Warfarin Oral or IV . .. . Throughout SubQ pregnancy Preconception & None Used in renal insufficiency None Best for high bleeding risk (term pregnancy, surgery) Sometimes used in 2nd & 3rd trimester for patients with mechanical heart valve • Teratogenic (bone . & cartilage) i bleeding LE IV= intravenous;subQ = subcutaneous. Ease of use, predictable effect • Avoid in severe renal insufficiency Before delivery postpartum Fetal effects Maternal considerations ne Low-molecular- Route pregnancy In Anticoagulant during rC Anticoagulation  Unfractionated heparin is the anticoagulant of choice preceding delivery due to its rapid reversibility. U SM However, all anticoagulation is discontinued at the onset of labor due to the risk of hemorrhage and before epidural anesthesia due to the risk of epidural hematoma. Heparin  What is the recommended anticoagulation for pulmonary embolism in a patient with severe renal insufficiency GFR  30 mL/min/1.73m2? Unfractionated heparin followed by warfarin Warfarin is preferred long-term oral anticoagulant in ESRD patients, but needs to be bridged first LMWH and factor Xa inhibitors are not recommended in renal insufficiency because they are metabolized by the kidney Blood & Oncology 605 https://t.me/usmleinnercircle Version 2 When you increase the warfarin dose in subtherapeutic patients who have a high risk of hypercoagulability like APLA, bridge with heparin while increasing the warfarin dose to avoid transient hypercoagulability due to warfarin. Clinical features of type 2 heparin-induced thrombocytopenia Suspected with heparin exposure >5 days & any of the .. .. .. .. following: Clinical signs Diagnostic evaluation Therapy Platelet count reduction >50% from baseline Arterial or venous thrombosis Necrotic skin lesions at heparin injection sites Acute systemic (anaphylactoid) reactions after heparin Serotonin release assay: gold standard confirmatory test Start treatment in suspected cases prior to confirmatory tests Stop all heparin products Start a direct thrombin inhibitor (eg, argatroban) or fondaparinux (synthetic pentasaccharide) Pretest probability of heparin-induced thrombocytopenia 2 points Thrombocytopenia Timing of drop . . . . . . Onset 5-10 days or s1 day if prior heparin Confirmed new thrombosis, skin necrosis, .. . Platelet <30% or nadir 3 <1 O,OOO/mm Consistent drop at 5-10 days but unclear (eg, missing platelet counts) . recent s1 day, with prior heparin exposure within 30- exposure 100 days Progressive or recurrent thrombosis, nonnecrotizing skin lesions, or unproven suspected thrombosis Possible S4 days without Onset after 10 days IV heparin bolus . 0 points count drop Platelet count drop 30%-50% or nadir 10,000- or acute systemic reaction after None apparent . 19,000/mm 3 exposure within 30 days Thrombosis Other causes for Platelet count drop >50% & nadir 2:20,000/mm3 in platelet count thrombocytopenia 1 point . . None Definite Score 0-3: Low probability. 4-5: Intermediate probability. 6-8: High probability. IV = intravenous. • Warfarin is usually started after treatment with a non-heparin anticoagulant and platelet count recovery to 150,000/mm3. Blood & Oncology 606 https://t.me/usmleinnercircle Version 2 le Lesion at Heparin injection site irc Patients diagnosed with heparin-induced thrombocytopenia are advised to avoid all forms of heparin (including low-molecular-weight heparin) for life to limit the risk of recurrence. rC Unfractionated heparin, low-molecular-weight heparin, heparin flushes for arterial lines, and heparin-coated catheters all require avoidance, and a "heparin allergy" should be listed in the medical record. ne EPO In  Up to 30% of patients receiving an ESA for CKD-induced anemia develop new or worsening hypertension, which typically occurs 28 weeks after treatment initiation. Hypertension is generally mild but can be severe. The mechanism by which ESAs cause hypertension appears to be independent of increased blood volume and likely involves systemic vasoconstriction caused by: • Activation of erythropoietin receptors on vascular smooth muscle cells LE • Heightened sensitivity of alpha receptors • Depletion of endothelium-derived nitric oxide U SM The risk of ESA-induced hypertension is increased by large doses and a rapidly rising hemoglobin soon after ESA administration. Following acute treatment of hypertension, subsequent ESA doses should be decreased with a goal of slowly increasing hemoglobin. Tumour Blood  The combination of a vascular tumor (e.g. hemangioma) and consumptive thrombocytopenia is indicative of Kasabach-Merritt syndrome. large or rapidly-growing tumors may trap platelets causing severe thrombocytopenia Oncology  Patients with suspected metastatic cancer should have an initial biopsy of a metastatic site (if possible). Blood & Oncology 607 https://t.me/usmleinnercircle Version 2 Enlarged scalene or supraclavicular lymph nodes are generally preferred due to their superficial location. Pancreas Gastric .. .• .• . • . Specificcancer risk factors Tobacco smoke Obesity Renal Dietary nitrates Alcohol & tobacco use Bladder • Helicobacter pylori Tobacco smoke Obesity Hypertension Tobacco smoke Occupational exposures (rubber, plastics, aromatic amine-containing dyes, textiles, leather) .• . .. .. • Hepatitis B & C Liver Colorectal Liver cirrhosis (any cause) Hemochromatosis Breast Aflatoxin Hereditary CRCsyndromes Inflammatory bowel disease Obesity .• . .• • Early menarche Prostate Late menopause Nulliparity BRCAmutations Increasing age African American Charred or fried foods CRC= colorectalcancer.  What is the recommended pharmacologic treatment for patients with cancer-related anorexia/cachexia syndrome CACS? Progesterone analogs (preferred) or corticosteroids e.g. megestrol acetate; Synthetic cannibinoids are useful for HIV cachexia, but not as useful for CACS Chemotherapy-induced cardiotoxicity Type I • Associated with anthracyclines Myocyte necrosis & destruction (fibrosis) Progression to overt clinical heart failure Less likely to be reversible • • • • Associated with trastuzumab Myocardial stunning/hibernation without myocyte destruction • Asymptomatic left ventricular systolic dysfunction More likely to be reversible • Type II •  Baseline cardiac function should be assessed prior to initiating trastuzumab in patients with HER2 positive breast cancer. Also routine echo is done during the therapy Therapies • Salvage therapy is defined as treatment for a disease when standard therapy fails. Blood & Oncology 608 https://t.me/usmleinnercircle Version 2 i.e. radiation therapy for prostate-specific antigen recurrence after radical prostatectomy for prostate cancer • Adjuvant therapy is defined as treatment given in addition to standard therapy • Induction therapy is an initial dose of treatment to rapidly kill tumor cells and send the patient into remission 5% tumor burden). A typical example is induction chemotherapy for acute leukemia. • Consolidation therapy is typically given after induction therapy with multidrug regimens to further reduce tumor burden. An example is multidrug therapy after induction therapy for acute leukemia. le • Maintenance therapy is usually given after induction and consolidation therapies (or initial irc standard therapy) to kill any residual tumor cells and keep the patient in remission. An example is daily antiandrogen therapy for prostate cance • Neoadjuvant therapy is defined as treatment given before the standard therapy for a particular disease. rC Radiotherapy Radiotherapy has applications as primary, adjuvant, or more commonly palliative therapy for many types of cancer. Tumor sensitivity to radiation depends on the rate of cell turnover, with rapidly growing tumors being more sensitive. In the same way, rapidly dividing normal body tissues such as ne blood cell precursors, epithelial surfaces in the skin, GI tract, and urinary tract, and the gonads (gametes) are also at risk for damage. Fibrosis and strictures due to diffuse scarring of the damaged tissues often occurs as a late U SM LE In complication of radiotherapy for prostate cancer, and may lead to obstructive uropathy. INCREASINGRADIATION DOSE Therapeutic ionizing radiation (eg, gamma rays, x-rays), commonly used to treat or palliate several types of cancer, can cause cell death through 2 major mechanisms: • DNA double-strand breakage Breakage of both strands is generally required, as single strand breaks are readily repaired by polymerases. • Free radical formation: Reactive oxygen species are formed by ionization of water; oxygen free radicals are then able to cause cellular and DNA damage. The effect of radiation is most pronounced in malignant cells as they are rapidly dividing and consequently less able to repair DNA damage. Epithelial surfaces (eg, bowel mucosa, skin) are also severely affected because they are rapidly dividing. Blood & Oncology 609 https://t.me/usmleinnercircle Version 2 A characteristic cell death curve of exposure to radiation shows a nearly flat line on initial exposure, followed by a steep increase in cell death as the radiation dose increases. The steep portion is due to a sharp increase in double-stranded DNA strand fractures and oxygen free radicals. -------,,,~- Linear Sigmoid Q) <I) C 0 a. a: Dose Most drugs exhibit a sigmoid-shaped dose response curve that shows no effect until a threshold concentration is exceeded. After this occurs, the effects increase with increasing drug concentration until a saturation threshold (ie, maximum effect) is reached. In contrast to this sigmoidal response, the risk of cancer following exposure to ionizing radiation shows a linear dose–response relationship (ie, risk increases proportionately with increased exposure). PET Scan Positron emission tomography PET scan with 18 fluorodeoxyglucose FDG, an analog of glucose, is often the imaging test of choice for Lymphoma. Neoplastic cells have a high metabolic rate and readily take up radiotracer on PET scan. However, radiotracer also pools in healthy organs with high glucose requirements such as the brain, kidneys, and liver; excretion of radiotracer also leads to enhancement throughout the urinary collecting system. Cancer Pain Blood & Oncology 610 https://t.me/usmleinnercircle Version 2 Cancer pain management Chronic cancer-related pain J Mild (eg, ~1-3/10 pain) Moderate/severe (eg, ~4-10/10 pain) ! ! Short-acting opioid • Oxycodone, morphine • ± nonopioid ± adjuvant Inadequaterelief irc Multipledaily doses or sleep interruption le Nonopioid • Acetaminophen or NSAID • ± adjuvant (eg, glucocorticoid, antidepressant, anticonvulsant) + rC Long-acting opioid • ER morphine, fentanyl patch • ± nonopioid ± adjuvant PLUS short-acting opioid for breakthrough pain• Cl.lWo<ld ne 'Short-actingopioidsfor breakthroughpainshouldalWaysbe available. ER= extended-release; NSAID = nonsteroidal anti-inflammatory drug  Can a patient with severe, acute pain refractory to NSAIDs and a history of IV drug abuse be given opioids for acute pain management? In Yes management of acute pain is similar for all patients, regardless of substance abuse history LE Acute pain management in patients with opioid use disorder Environment Withdrawal U SM prevention Analgesia • Supportive, nonjudgmental, shared decision-making • Do not decrease or stop maintenance opioid (eg, methadone, buprenorphine) • Maximize nonopioid medications (eg, acetaminophen, NSAIDs) • Use regional anesthesia when possible • Add opioids only as needed (shared decision-making required): 0 Patients with sustained remission are treated as opioid na"ive 0 Patients on maintenance may require higher doses 0 Convert to oral as soon as possible with rapid taper to baseline 0 Use for shortest period (3-5 days) if possible NSAIDs = nonsteroidal anti-inflammatory drugs. Blood & Oncology 611 https://t.me/usmleinnercircle Version 2  Inadequately treated pain in the postoperative patient can lead to hypertension, delayed recovery, and patient dissatisfaction. Patient-controlled analgesia is an option for treating moderate to severe pain, especially in previously opioid-tolerant patients or in patients unable to take oral pain medications. It provides small boluses of parenteral opioids with lockout intervals and maximum doses to prevent overdose. It can also allow the practitioner to calculate a total opioid requirement that can be utilized tor transitioning the patient to an adequate oral regimen Tumor Lysis Syndrome Tumor lysis syndrome Risk Manifestations Prophylaxis Treatment .. . .. . .. .. . Tumors with high cell burden or rapid turnover Combination chemotherapy/immunotherapy Severe electrolyte abnormalities 0 j Phosphorus, potassium, uric acid 0 L Calcium Acute kidney injury Cardiac arrhythmias Seizures Intravenous fluids Xanthine oxidase inhibitor• or rasburicase Intravenous fluids + rasburicase [I\"'-"-) Continuous telemetry Aggressive electrolyte monitoring & treatment *Allopurinol or febuxostat. Xanthine oxidase inhibitors (eg, allopurinol, febuxostat) simply prevent the production of additional uric acid (leaving the existing elevated uric acid levels unaffected), which is less preferable in the acute setting Blood & Oncology 612 https://t.me/usmleinnercircle Version 2 Tumor lysis syndrome Xanthine oxidase Hyperuricemia Urinary phosphate l Urinary uric acid Calcium phosphate stones Uric acid stones Hyperkalemia le Hyperphosphatemia rC l irc l Acutekidneyinjury Cardiacarrtlythmia ne Hypocalcemia In  Hyperphosphatemia can lead to calcium phosphate stone formation in the renal tubules, resulting in acute kidney injury due to renal tubular obstruction and direct cellular injury. U SM Lymphangitis LE Miscellaneous Epidemiology Manifestations Treatment .. .. . . Lymphangitis Cutaneous injury----+pathogen invasion of lymphatics in deep dermis Streptococcus pyogenes & MSSA Tender, erythematous streaks proximal to wound Regional tender lymphadenopathy (lymphadenitis) Systemic symptoms (eg, fever, tachycardia) Cephalexin MSSA = methicillin-sensitive Staphylococcus aureus. D/D Sprotrichosis which takes weeks (vs days here) Idiopathic Anemia of Ageing Elderly patients may present additional challenges in evaluating acute anemia. Besides other potential comorbidities that can cause chronic anemia at baseline (eg, renal insufficiency, myelodysplastic Blood & Oncology 613 https://t.me/usmleinnercircle Version 2 syndromes, occult malignancy, nutritional deficiencies), a significant number of elderly patients will have a baseline anemia for which no etiology is apparent, the so-called "idiopathic anemia of ageing." They are also more likely to have additional comorbidities such as congestive heart failure or chronic lung disease, which make them poorly tolerant of even mild anemia. Blood Transfusion Indications for specialized RBC treatments • Bone marrow transplant (BMT) recipients Irradiated • Acquired or congenital cellular immunodeficiency. • Blood components donated by first or second degree relatives • Chronically transfused patients Leukoreduced • CMV seronegative at-risk patients (e.g., AIDS, transplant patients) • Potential transplant recipients • Previous febrile nonhemolytic transfusion reaction • lgA deficiency • Complement-dependent autoimmune hemolytic anemia Washed • Continued allergic reactions (e.g., hives) with red cell transfusion despite antihistamine treatment Transfusion reactions timeline IDelayedhemolytic I , Febrilenonhemolytic • TRALI IAcutehemolytic I Anaphylaxis 1 hour 6 hours 2 days lOdays Time {not to scale) ©UWorld Blood & Oncology 614 https://t.me/usmleinnercircle Version 2 Blood transfusiion reactions associated with hypotension Clinical features & etiology Reaction • Rapid onset of shock, angioedema/urticaria & respiratory distress Anaphylactic • Within a few seconds to minutes of transfusion • Caused by recipient anti-lgA antibodies • Respiratory distress & signs of noncardiogenic pulmonary edema Transfusionrelated acute lung injury • Within 6 hours of transfusion • Transient hypotension often in patients taking angiotensin- converting enzyme inhibitors Primary hypotension reaction • Within minutes of transfusion . Bacterial sepsis Fever, chills, septic shock & DIC rC • Within minutes to hours of transfusion irc • Caused by bradyikinin 1inbl:ood products (normally degraded by angiotensin-converting enzyme) le • Caused by donor anti-leukocyte antibodies • Acute hemolytic transfusion reaction is distinguished from febrile non-hemolytic reaction by the presence of plasma free hemoglobin  25 mg/dL. ne may result in a "pink" appearing plasma; plasma free hemoglobin is  25 mg/dL in febrile nonhemolytic transfusion reactions In T/t: Cessation of Blood product, IV Normal Saline for hydration Immunologic blood transfusion reactions Onset* LE Transfusion reaction Anaphylactic Within seconds to minutes Cause Recipient anti-lgA antibodies directed against donor blood lgA Within ABO incompatibility (often 1 hr clerical error) Febrile nonhemolytic (most Within Cytokine accumulation during common reaction) 1-6 hr blood storage Within Recipient lgE against blood 2-3 hr product component Transfusion-related acute Within Donor anti-leukocyte lung injury 6 hr antibodies U SM Acute hemolytic Urticaria! Delayed hemolytic Graft versus host Within days to weeks Within weeks Anamnestic antibody response Donor T lymphocytes Key features • Angioedema, hypotension, respiratory . .. . . . .. .. . . distress/wheezing, shock lgA-deficient recipient Fever, flank pain, hemoglobinuria Disseminated intravascular coagulation Positive Coombs test Fever & chills Urticaria Respiratory distress Noncardiogenic pulmonary edema with bilateral pulmonary infiltrates Often asymptomatic Laboratory evidence of hemolytic anemia Positive Coombs test, positive new antibody screen Rash, fever, gastrointestinal symptoms, pancytopenia *Time after transfusion initiation. Blood & Oncology 615 https://t.me/usmleinnercircle Version 2 Bacterial sepsis Minutes to hours Bacterial contaminationof donor product • Fever,chills, septic shock & DIC Delayed hemolytic transfusion reaction Pathogenesis Clinical findings • Anamnestic antibody response: antibody production against minor RBC antigen to which patient was previously* exposed • Onset >24 hours to a month after transfusion • Often asymptomatic • Fatigue, dyspnea, tachycardia • Jaundice • Low-grade fever • Hemolytic anemia Laboratory 0 t indirect bilirubin, LDH, reticulocyte count findings 0 J hemoglobin, haptoglobin Management Prevention • New positive direct antiglobulin (Coombs) test • Supportive (eg, fluids) • Review transfusion history & prior antibody screens • Transfuse when necessary with extended-antigen cross-matched blood *Prior transfusion (eg, sickle cell disease patients), pregnancy, transplant. LDH = lactate dehydrogenase; RBC = red blood cell. Transfusion reactions Anaphylactic Mechanism Onset Findings Treatment • Anti-lgA antibodies (lgG or lgE) in lgA-deficient patient against donor blood lgA . Urticaria I Preformed recipient lgE antibodies against soluble allergen in donated plasma • Seconds to minutes • Respiratory distress/wheeze • Hours • Hives • Itching • Angioedema • Hypotension • Hives • Immediate cessation of transfusion • Epinephrine • Immediate cessation of transfusion • Antihistamines • Antihistamines, oxygen, fluids & vasopressors • Resume transfusion if patient is otherwise asymptomatic  After blood is ordered for transfusion, the following compatibility testing is usually performed. • First, the patient's ABO and Rh types are determined. • After this, the patient's serum is screened for unexpected antibodies, a procedure called pretransfusion antibody screening. Pretransfusion antibody screening is intended to detect any of all clinically significant RBC antibodies. 0 0 If negative, the patient can be safely transfused. If positive, further investigation is usually warranted to evaluate the identity of the antibody. The major problem that leads to difficulties finding cross-matched blood in patients with a history of multiple transfusions is alloantibodies (e.g., in patients with sickle cell anemia or myelodysplasia). Blood & Oncology 616 https://t.me/usmleinnercircle Version 2 0 0 The most commonly implicated RBC antigens in that case are E, L and K. Moreover, these patients tend to develop multiple alloantibodies that make finding compatible blood even more difficult. Transfusion Overload Transfusion-associated circulatory overload • Age <3 and >60 rC irc • Large transfusion volume or fast infusion rate • Respiratory distress Clinical features • t Heart rate ( <6 hr following t Blood pressure transfusion initiation) • • Pulmonary edema (eg, rales) • Respiratory support (eg, oxygen) Management • Diuresis (eg, furosemide) le • Underlying cardiac or renal condition Risk factors • inc BP (vs TRALI which has hypotension) ne Catheter Induced Septic Thrombophlebitis  Catheter-related bloodstream Infections are a common cause of fever in hospitalised patients and In can be complicated b catheter-associated septic thrombophlebitis. The infected thrombus serve as a nidus for microbial proliferation and presents with fever and signs and symptoms of bacteremia. The Involved vein may display erythema and induration and there may be purulent drainage from the catheter site. LE The diagnosis may be aided by duplex ultrasonography or with contrasted CT scan. Treatment Infectious source control with removal of the catheter and surgical excIsion of the affected vein, Intravenous antibiotics U SM Paeds Blood Polycythemia Blood & Oncology 617 https://t.me/usmleinnercircle Version 2 Neonatal polycythemia Definition Causes • Hematocrit >65% in term infants • Increased erythropoiesis from intrauterine hypoxia: maternal diabetes, hypertension, or smoking; intrauterine growth restriction • Erythrocyte transfusion: delayed cord clamping, twin-twin transfusion • Genetic/metabolic disease: hypothyroidism/hyperthyroidism, genetic trisomy (13, 18, 21) • Asymptomatic (most common) • Ruddy skin Clinical presentation • Hypoglycemia, hyperbilirubinemia • Respiratory distress, cyanosis, apnea • Irritability, jitteriness • Abdominal distension • Intravenous fluids Treatment • Glucose • Partial exchange transfusion  There may be significant variability in measurements with capillary samples (eg, heel prick). Therefore, if the hematocrit is 65% on a heel prick, it should be confirmed by rechecking a sample from peripheral venous blood, which is more reliable, unaffected by changes in temperature and blood flow, and often up to 15% lower than a hematocrit taken from a capillary sample  What is the recommended treatment for symptomatic neonates with polycythemia (e.g. respiratory distress, hypoglycemia)? Partial exchange transfusion blood is removed in exchange for normal saline to normalize the hematocrit; asymptomatic infants may be managed with rehydration (e.g. feeding, parenteral fluids) Anemia of Prematurity Anemia of prematurity • j Oxygenation at birth -+ ! EPO production 0 Pathogenesis Clinical features Laboratory findings Management Impaired transition from hepatic to renal EPO* • Exacerbating factors in premature infants: 0 ! RBC life span 0 Frequent blood draws 0 Iron depletion • Often asymptomatic • Tachycardia, poor weight gain, apnea, hypoxia • Normocytic, normochromic anemia • Inadequate reticulocyte response • RBC transfusion if severe or symptomatic • Minimize exacerbating factors (eg, iron supplementation, limit blood draws) *Typicallyoccurs in the 3rd trimester. EPO = erythropoietin;RBC = red blood cell. Blood & Oncology 618 https://t.me/usmleinnercircle Version 2  What hematologic pathology is characterized by normocytic anemia with a low reticulocyte count in preterm infants? Anemia of prematurity Suspect in premature infants with low RC and low Hb/Hct due to impaired ability to produce adequate EPO, short RBC life span, and frequent phlebotomy in the NICU; diagnosis of exclusion le Normocytic anemia — use reticulocyte counts to differentiate causes • Low: ineffective or decreased RBC production (renal failure, aplastic anemia, anemia of Physiologic Anemia of Newborn rC • High: increased RBC destruction (hemolysis), blood loss irc prematurity) Physiologic anemia of infancy • i Tissue oxygenation at birth --+ down-regulation of erythropoietin ne Pathogenesis • Asymptomatic term infant• age 2-3 months Clinical features • Normocytic anemia (hemoglobin 9-11 g/dl) • Low to normal reticulocyte count • Reassurance • Anemia resolves with i erythropoietin drive after age 3 months In Treatment/prognosis *Preterm infants have a lower nadir at a younger age (ie, anemia of prematurity) . • LE Findings suggestive of a pathologic cause of anemia, Hemoglobin 9 g/dL, • Signs of hemolysis (eg, elevated reticulocyte count), or U SM • Microcytosis IDA Blood & Oncology 619 https://t.me/usmleinnercircle Version 2 Iron deficiency anemia in young children .. . Prematurity Lead exposure Age <1 0 Risk factors Diagnosis Treatment . .. . Delayed introduction of solids (ie, exclusive breastfeeding after 6 months) 0 Cow's, soy, or goat's milk Age >1 0 >24 oz/day cow's milk 0 <3 servings/day iron-rich foods Screening hemoglobin at age 1 Hemoglobin <11 g/dl, ! MCV, i RDW Empiric trial of iron supplementation MCV = mean corpuscularvolume;ROW= red bloodcell distributionwidth.  What are causes of iron deficiency anemia in young children? • Excessive intake of cow's milk (low in iron) 0 24 oz/day • Delayed introduction of solids • Cereal-rich diet (low in iron but usually fortified) Treatment? Iron supplementation First do a trial of Iron, if it doesnt improve investigate other causes with Hb electropheresis etc.  What hematologic pathology is more common in preterm infants within the first 4  6 months of life? Iron deficiency anemia other risk factors for iron deficiency include maternal iron deficiency and introduction of cows milk before age 12 months full-term infants are born with adequate iron stores that prevent anemia in the first 4  6 months of life Pancytopenia Blood & Oncology 620 https://t.me/usmleinnercircle Version 2 Common pediatric causes of pancytopenia Pancytopenia ] Impairedcell production ] Bone marrow aplasia • Fanconianemia Acquired aplastic anemia • Idiopathic • Drugs & toxins • Infection (HIV. EBV) • Radiation • Immunologic (SLE) • Malnutrition OUWald U SM LE In ne EBV = EpsteinBarr-virus.SLE = systemiclupuserythematosus. l • Malignancy • Storage disease (Gaucher disease) irc lnhented aplastic anemia rC • Hypersplenism • Immunologic (SLE) Bone marrow infiltration le Enhanced cell destructJon ] Blood & Oncology 621 https://t.me/usmleinnercircle Version 2 Musculoskeletal Arthritis Osteoarthritis Rheumatoid Arthritis Felty Syndrome Juvenile Idiopathic Arthritis Septic Arthritis Disseminated Gonoccocal Infection Seronegative Spondyloarthritis Gout SLE APLA Mixed Connective Tissue Disorder Sjogren Syndrome Scleroderma Temporomandibular Joint Upper Limb Carpal Tunnel Shoulder Adhesive Capsulitis Rotator Cuff Tear Dislocation Suprascapular Nerve Entrapment De Quervain Tendinopathy Elbow Dupuytren Contractures Lower Limb Trochanteric Bursitis Knee Patellar tendon rupture Patellar Dislocation ACL Injury PCL Injury Patellofemoral Pain Syndrome Popliteal Cyst Meniscal Tear Ankle & Foot Planter Fasciitis Achilles Tendinopathy Calcaneal Apophysitis Spine Spinal Spondylosis Musculoskeletal 622 https://t.me/usmleinnercircle Version 2 Cervical Radiculopathy Cervical Spondylotic Myelopathy Vertebral Compression Fracture Stenosis Spondylolisthesis Back Pain Lumber Radiculopathy Spine Trauma Bone Pathology Avascular Necrosis le Osteomalacia Pagetʼs Disease Osteoporosis irc Osteomyelitis Fracture Femur Scaphoid rC Clavicle Humerus Radius Stress Fracture ne Buckle & Greenstick Fracture Pelvic Fracture Bursitis Olecranon Bursitis In Pes Anserine Bursitis Vasculitis Large Vessel Takayasu Medium Vessel Kawasaki LE GCA Polyarteritis Nodosa U SM Buerger Disease Small Vessel Behcet Mixed Cryoglobulinemia Granulomatosis with polyangitis Churg Strauss Syndrome Henoch-Schonlein Purpura Myopathy Myasthenia Gravis Raynaudʼs Phenomenon Drugs Bisphosphonate Osteonecrosis Tumors Osteoid Osteoma Osteosarcoma Musculoskeletal 623 https://t.me/usmleinnercircle Version 2 Ewingʼs Sarcoma Miscellaneous Prosthetic Joint Compartment Syndrome Charcot Joint Complex Regional Pain Syndrome Amputation Langerhans Histiocytosis Myositis Ossificans Ganglion Cyst Peads Musculocutaneous Genu Varum Toddlerʼs Fracture DDH Legg-Calve-Perthes Disease SCFE Clubfoot Growing Pains Congenital Muscular Torticolis Transient Synovitis Adolescent Idiopathic Scoliosis Strength Training Rheumatologic diseases & commonly associated autoantibodies Sensitivity(%) Specificity (%) Rheumatoid arthritis RF: 70-80 Anti-CCP: 95 Systemic lupus erythematosus ANA: 95 Anti-dsDNNanti-Sm: 96 Drug-induced lupus ANA: 95 Antihistone: 95 Diffuse systemic sclerosis ANA: 95 Anti-Scl-70: 99 Limited systemic sclerosis ANA: 95 Anticentromere: 97 Polymyosltis/dermatomyosltis ANA: 75 Anli-Jo-1: 99 ANA = antinuclear antibodies; anti-CCP = anti-cyclic citrullinated peptide; anti-dsDNA = anti- double-stranded DNA; anti-Scl•70 = anti-topoisomerase I; anti-Sm = anti-Smith; RF = rheumatoid factor. Arthritis Musculoskeletal 624 https://t.me/usmleinnercircle Version 2 Joint fluid characteristics Nonnal Noninflammatory Inflammatory (eg,OA) (eg, crystals, RA) Septic joint Translucent or Appearance Clear Clear Opaque WBCs(mm 3) <200 200-2,000 2,000-100,000 50,000-150,000 PMNs <25% 25% Often >50% >80%-90% opaque Differential diagnosis for nontraumatic joint swelling Acute Subacute/chronic Constant Worse in morning Timing of pain Able to bear weight? No Multiple joints? Laboratory findings Subacute/chronic Worse in evening/night Yes Uncommon Common Variable Variable f WBCs/platelets f WBCs/platelets, ! RBCs f Inflammatory markers f Inflammatory markers ! WBCs/platelets ne RBC = red blood cell; WBC = white blood cell. Neoplastic rC Onset lnflammatory/rheumatologic irc Infectious le OA = osteoarthritis; PMNs = polymorphonuclear leukocytes; RA= rheumatoid arthritis; WBCs = white blood cells. Evaluation of oligoarthritis LE In Joint pain/swelling .s_4joints Bone marrow elements U SM • lntraarticular fracture T • Gout • Pseudogout r&,uwortd.com Positive culture I• Infectious l"T' I • Rheumatoid arthritis • Viral/Lyme disease • Systemic lupus erythematosus • Sarcoidosis • Spondyloarthropathy Osteoarthritis Musculoskeletal 625 https://t.me/usmleinnercircle Version 2 Risk factors for osteoarthritis .. .. Modifiable Sedentary lifestyle Obesity Occupational joint loading Diabetes mellitus Nonmodifiable .. . • Advanced age Female sex Family history • Abnormal joint alignment Prior joint trauma Hand osteoarthritis Osteophyte Hereditary hemochromatosis predominantly affects the second and third metacarpophalangeal joints Musculoskeletal 626 https://t.me/usmleinnercircle Version 2 Osteoarthritis Morning stiffness Systemic symptoms Examination l Symptoms persist • Topical or oral NSAIDs as needed • Consider duloxetine, topical capsaicin • None/brief (<30 min) l • Absent • Hard, bony enlargement of joints • Reduced range of motion with crepitus Symptoms persisV significant impairment • Surgery (if possible) • Chronic pain management (nonsurgical candidates) NSAIDs = nonsterOldalanli-lnftammato,ydrugs. @UWorld rC findings Nonpharmacologic treatment (eg, exercise, weight loss) le Joint involvement • >40; prevalence increases with age • Knees • Hips • Distal interphalangeal joints • 1st carpometacarpal joint irc Age of onset Management of osteoarthritis ne OA develops within 10 years in most patients with anterior cruciate ligament injury  Examination findings suggesting OA in the knee include: In • Periarticular bony hypertrophy and tenderness • Limited range of motion with crepitus and pain LE • Small joint effusion without erythema or warmth • Varus or valgus angulation • Popliteal Baker) cyst behind U SM • Arthrocentesis shows clear fluid with few inflammatory cells and can be helpful in evaluating patients with acute symptoms the joint • ESR, CRP and serologic (eg, rheumatoid factor) markers are normal. Rheumatoid Arthritis  What musculoskeletal pathology is significantly more prevalent in patients with rheumatoid arthritis? osteopenia/osteoporosis due, in part, to increased levels of pro-inflammatory cytokines, corticosteroid therapy, and lack of physical activity Musculoskeletal 627 https://t.me/usmleinnercircle Version 2 .. . • .. . . . • . .. • .. Extraarticular manifestations of rheumatoid arthritis Systemic Pulmonary Cardiovascular Musculoskeletal Dermatologic Hematologic Neuropsychiatric Other Disease-modifying antirheumatic drugs Fever Agent Weight loss Mechanism Fatigue Fibrotic lung disease Adverse effects Methotrexate Purine antimetabolite • Hepatotoxicity • Stomatitis • Cytopenias Leflunomide Pyrimidine synthesis inhibitor • Hepatotoxicity • Cytopenias Hydroxychloroquine TNF & IL-1 suppressor • Retinopathy Sulfasalazine TNF & IL-1 suppressor • Hepatotoxicity • Stomatitis Pleural effusions Lung nodules Pulmonary hypertension • Atherosclerosis Vasculitis Osteopenia/osteoporosis • Hemolytic anemia Rheumatoid nodules Anemia TNF inhibitors Depression • Adalimumab • Certolizumab • Etanercept Neuroeath~ • Infection • Demyelination • Congestive heart failure • Malignancy • Golimumab • lnfliximab Sjogren s~ndrome Raynaud phenomenon Methotrexate side effect (macrocytic anemia) Scleritis, episcleritis  Much of the Methotrexate toxicity, including hepatotoxicity and stomatitis, can be mitigated by concurrent administration of folic (or folinic) acid, which does not reduce the effectiveness of the drug. RA has prolonged morning stiffness 30 minutes  Before starting Methotrexate, Patients should be tested for hepatitis B and C. Methotrexate should not be used in patients who are pregnant or are planning to become pregnant in the near future and those with severe renal insufficiency, liver disease, or excessive alcohol intake. Pathogenesis & risk factors Clinical features .. .. . . Methotrexate-induced lung injury Idiosyncratic (ie, not dose dependent) hypersensitivity pneumonitis Risk factors: rheumatoid arthritis, parenchymal lung disease Onset 1-12 months: pneumonitis--+ fibrosis (restrictive PFTs) CT scan with variable mix of patterns: inflammation (eg, GGO, consolidation) & fibrosis (eg, reticulation) BAL: lymphocytosis; peripheral blood: eosinophilia Trial of MTX cessation is diagnostic & therapeutic BAL = bronchoalveolar lavage; GGO = ground-glass opacities; MTX = methotrexate; PFTs = pulmonary function tests.  Patients treated with hydroxychloroquine should have a baseline ophthalmologic evaluation, with annual reassessment beginning after 5 years. Musculoskeletal 628 https://t.me/usmleinnercircle Version 2 Rheumatoid arthritis of the hand Rheumatoidarthritis Methotrexate Persistentsymptomsfor >6 months Parallel therapy Add another nonbiologicagent (eg, sulfasalazlne,hydroxychloroqulne) le Inadequateresponse irc Swttchto alternate TNF inhibttor & continue methotrexate Neck pain radiating to occipital region Slowly progressive spastic quadriparesis Painless sensory deficits in hands or feet Respiratory dysfunction (eg, from vertebral artery compression) Protruding anterior arch of atlas Scoliosis with loss of cervical lordosis LE Signs Clinical features of RA cervical myelopathy In Symptoms .. .. .. .. ne rC TNf= tumornecrosisbctor-a. Upper motor neuron signs (eg, spastic paresis, hyperreflexia, Babinski sign) Hoffman sign U SM RA = rheumatoid arthritis. • Hoffman sign (flexion and adduction of the thumb when flicking the nail of the middle finger) suggests a corticospinal tract lesion and supports the diagnosis of cervical myelopathy; however, it is nonspecific and may be seen in normal patients. Felty Syndrome Musculoskeletal 629 https://t.me/usmleinnercircle Version 2 Felty syndrome • Rheumatoid arthritis 0 Clinical features Diagnosis Severe erosive joint disease & deformity 0 Rheumatoid nodules 0 Vasculitis (mononeuritis multiplex, necrotizing skin lesions) • Neutropenia (ANC <2000/µL) • Splenomegaly • Anti-CCP & RF are positive in >90% of patients • Markedly elevated ESR, often >85 mm/hr • Peripheral smear & bone marrow biopsy to rule out other causes of neutropenia ANC = absolute neutrophil count; anti-CCP = anticyclic citrullinated peptide; ESR = erythrocyte sedimentation rate; RF= rheumatoid factor. Do not diagnose Felty in the absence of neutropenia! Juvenile Idiopathic Arthritis Juvenile idiopathic arthritis • Symmetric arthritis for at least 6 weeks Clinical features o Polyarticular: "'.5joints involved o Oligoarticular: <5 joints involved • Elevated inflammatory markers (erythrocyte sedimentation rate, C-reactive protein) . . . • Hyperferritinemia Laboratory findings Hypergammaglobulinemia Thrombocytosis Anemia Juvenile idiopathic arthritis Subtype Frequency Age of onset Clinical features Sex ratio • Arthritis in 2:1joint for 2:6weeks • Quotidian fever Systemic 10% Age <18 . for 2:2weeks F= M Evanescent rash • Hepatosplenomegaly • Lymphadenopathy Polyarticular 40% Age 2-5 or 10-14 . • Arthritis in 2:5joints May be complicated F>M by uveitis • Arthritis in <5 joints Oligoarticular 50% Age 2-4 • May be complicated F>M by uveitis Joint swelling is present, but pain may be minimal D/D ARF but it has Migratory Polyarthritis and fever  6 weeks (vs Systemic JIA Musculoskeletal 630 https://t.me/usmleinnercircle Version 2 ::~~!r:r;~~ in JJOltMtKubrontoet d•~ lno.d\'1'ffl('fllUSU1llyJ)rnmc"tlK, 1,,..,,ivcmcnt cl left Mitt "1th \,1lg~ irc le dcform11ycl lot,,;t"I' leg.ind ~ion contr.Klul't'ollntt'  What fever pattern is seen in patients with systemic-onset juvenile idiopathic arthritis? rC Daily fever spikes Quotidian Fever) ne Ophthalmologic screening examinations are performed regularly because untreated uveitis is often asymptomatic and can lead to irreversible vision loss. Septic Arthritis • Abnormal joint: OA, RA, prosthetic joint, gout • Age >80 • Diabetes • IV drug abuse, alcoholism • Intra-articular glucocorticoid injections U SM LE Risk factors In Septic arthritis Clinical features • Acute monoarthritis: hot, swollen, decreased ROM • Fever • Elevated ESR & CRP Diagnosis • Blood cultures • Synovial fluid analysis: leukocytosis (>50,000/mm3>,Gram stain, culture Initial treatment • Gram-positive cocci: vancomycin • Gram-negative rod: third-generation cephalosporin • Negative microscopy: vancomycin (+ third-generation cephalosporin if immunocompromised) CRP = C-reactive protein; ESR = erythrocyte sedimentation rate; IV= intravenous; OA = osteoarthritis; RA = rheumatoid arthritis; ROM = range of motion. Musculoskeletal 631 https://t.me/usmleinnercircle Version 2 Pathogenesis Clinical features Diagnosis Treatment .. .• . • .• . .. Pediatric septic arthritis Age <3 months: Staphylococcus aureus, group B Streptococcus, gram-negative bacilli Age ;,3 months: Staphylococcus aureus, group A Streptococcus Acute-onset joint pain, swelling, limited motion Refusal to bear weight Fever 2'38.5 C (101.3 F) i WBC, ESR, CRP Blood culture Joint aspiration (synovial WBC count of >50,000/mm3 ) Effusion on ultrasound/MRI Joint drainage & debridement IV antibiotics CRP ; C-reactiveprotein;ESR; erythrocytesedimentationrate; IV ; intravenous;WBC; white bloodcell.  What is the next step in management for a patient with a 3-day history of fever and increasing unilateral knee erythema, pain, and swelling? Synovial fluid analysis Arthrocentesis) both diagnostic and therapeutic any patient with possible septic arthritis should have urgent synovial fluid analysis; septic arthritis progressively worsens over a few days (versus gout which worsens over 12  24 hours). • Underlying joint disorders (eg, gout, pseudogout, osteoarthritis) increase the risk for secondary joint infection. In patients with crystal-induced arthritis (eg, gout), the presence of crystals alone does not rule out septic arthritis as these can be present in synovial fluid between attacks. • Lyme arthritis presents as an inflammatory monoarticular or asymmetric oligoarticular arthritis, most commonly in the knee. synovial fluid analysis typically reveals 25,000 WBCs/mm3 with negative gram stain/culture; arthritis is the most common complication of late lyme disease. In addition, fever is typically absent. Clenched-fist bite injury ("fight bite") Mechanism Pathogens Clinical features Management • Puncture through thin soft tissue overlying MCP joint • Enclosed bacterial proliferation under extensor tendon & within joint capsule • Oral flora (eg, Eikenella corrodens, beta-lactamase-producing anaerobes) • Skin flora (eg, streptococci, Staphylococcus aureus) • Small puncture wound appears minor initially; delay in care common • Septic arthritis: joint pain, swelling, erythema/warmth, restricted/painful ROM • Urgent surgical irrigation & debridement • Antibiotics (eg, ampicillin-sulbactam [IV], amoxicillin-clavulanate (PO]) IV; intravenous: MCP ; metacarpophalangeal; PO; orally; ROM ; range of motion. Musculoskeletal 632 https://t.me/usmleinnercircle Version 2 In general, these wounds should be irrigated and left open due to the high risk of infection. Disseminated Gonoccocal Infection Disseminated gonococcal infection Purulent monoarthritis &/OR Clinical presentation Triad of tenosynovitis, dermatitis (erythematous papules & pustules), asymmetric migratory le polyarthralgias Blood cultures (may be negative) orNAAT Culture or NAAT of urethra, cervix, pharynx, rectum irc Diagnosis Synovial fluid analysis: Inflammatory effusion with neutrophil predominance; Gram stain & culture Intravenous ceftriaxone, switch to oral (cefixime) when clinically improved Treatment Empiric azithromycin OR doxycycline for concomitant chlamydia! infection rC Joint drainage for purulent arthritis In ne Skin lesions in disseminated gonococcal infection LE This woman developed a fever, several painful joints, and exquisitely tender necrotic acral pustules. A culture from her cervix grew Neisseria gonorrhoeae. Seronegative Spondyloarthritis U SM  What is the most likely diagnosis in a young male with a history of back pain that is relieved with activity? He endorses a history of diarrhea that is worse with NSAID use. Inflammatory bowel disease (complicated by spondylarthritis) Further investigations would reveal sacroiliac joint inflammation (ankylosing spondylitis). NSAIDs are commonly used to treat the back symptoms, but will end up exacerbating the IBD. Musculoskeletal 633 https://t.me/usmleinnercircle Version 2 .• .• • .. . • .. • • . .• Ankylosing spondylitis Inflammatory back pain Examination findings Complications Laboratory Imaging Ankylosing spondylitis Insidious onset at age <40 Symptoms >3 months Relieved with exercise but not rest Nocturnal pain Arthritis (sacroiliilis) Reduced chest expansion & spinal mobility Enthesitis (tenderness at tendon insertion sites) Dactylitis (swelling of fingers & toes) Uveitis Osteoporosis/vertebral fractures Aortic regurgitation Cauda equina syndrome Elevated ESR & CRP HLA-B27 association X-ray of sacroiliac joints MRI of sacroiliac joints CRP ; C-reactiveprotein; ESR; erythrocytesedimentationrate. Chronic Back Pain Treatment of ankylosing spondylitis Nonpharmacologic measures Initial treatment Treatment failure/ disease progression . . .. .. Exercise (postural exercises, ROM/stretching exercises) Physical therapy NSAIDs (eg, ibuprofen, naproxen) COX-2 inhibitors (eg, celecoxib) TNF-a inhibitors (eg, etanercept, infliximab) Anti-lL-17 antibodies (eg, secukinumab) COX-2 ; cyclooxygenase-2; NSAIDs ; nonsteroidal anti-inflammatory drugs; ROM ; range of motion; TNF-a; tumor necrosis factor-alpha. Most patients with ankylosing spondylitis do well and have no functional or employment disabilities. There is no increased overall mortality or reduced life expectancy.  What is the likely diagnosis in a young male with dyspnea on exertion accompanied by chronic lower back pain that is worse at night and improves with exercise? Ankylosing spondylitis  Patients with longstanding AS can develop osteopenia/osteoporosis due to increased osteoclast activity in the setting of chronic inflammation (mediated by TNFα and interleukin-6). In addition, spinal rigidity in these patients can increase the risk of vertebral fracture, which often results from minimal trauma. Musculoskeletal 634 https://t.me/usmleinnercircle Version 2 Reactive arthritis • Genitourinary infection: Chlamydia trachomatis • Enteritis: Salmonella, Shigel/a, Yersinia, Campylobacter, C/ostridioides (formerly Preceding infection C/ostridium) difficile • Asymmetric oligoarthritis • Enthesitis Musculoskeletal • Dactylitis • Ocular: conjunctivitis, anterior uveitis • Genital: urethritis, cervicitis, prostatitis Extraarticular symptoms • Oral ulcers irc afebrile and low leukocyte count (vs Septic Arthritis) le • Dermal: keratoderma blennorrhagicum, circinate balanitis Ankylosing spondylitis Affected spine U SM LE Psoriatic arthritis In ne rC Healthy spine Gout Musculoskeletal 635 https://t.me/usmleinnercircle Version 2 Risk factors for gout Prevention of future gout attacks • Medications (eg, diuretics, low-<Joseaspirin) • Surgery, trauma, recent hospitalization • Decreased seafood & red meat intake • Volume depletion Increased risk Decreased risk • Weight loss to achieve BMI <25 kg/m2 • Low-fat diet • Diet: High-protein (meat, seafood), high-fat, fructose or sweetened beverages • Protein intake preferably from vegetable & low-fat dairy products • Avoidance of organ-rich foods (eg, liver & sweetbreads) • Heavy alcohol consumption • Avoidance of beer & distilled spirits • Underlying medical conditions (eg, hypertension, obesity, chronic kidney disease, organ transplant) • Avoidance of diuretics when possible • Dairy product intake • Vitamin C (~1500 mg/day) • Coffee intake (~6 cups/day) .. . Medications associated with gout Reduced uric acid excretion Increased uric acid production Rapid decline in uric acid levels . . .. Tophaceousgout Diuretics (eg, hydrochlorothiazide, furosemide) Salicylates (eg, low-dose aspirin) ACE inhibitors (eg, lisinopril) Cyclosporine Cytotoxic chemotherapy agents (tumor lysis syndrome) Xanthine oxidase inhibitors (eg, allopurinol) Uricosuric drugs (eg, probenecid) Losartan and CCB lower uric acid levels and likely reduce the risk of an attack. In patients with gout, synovial crystals may be seen between flares, so the presence of these crystals alone does not rule out septic arthritis. Septic arthritis can cause acute monoarticular arthritis, but it is typically associated with fever, systemic manifestations (eg, chills), and progressively worsening symptoms over a few days, rather than the abrupt onset seen in gout. • NSAIDʼs and colchicine are the first line treatments for acute gout flares but are contraindicated in patients with renal failure. Instead, corticosteroids should be used in these patients and can be injected locally in patients with a mono-articular arthritis or given orally in patients with multiple joint involvement Musculoskeletal 636 https://t.me/usmleinnercircle Version 2 Tophaceous gout rC irc le Gout C)UWo,ld "EroSIOf'lsare commonly near (but not at) the artiWar surface. In ne Characteristically show punched-out erosions with a rim of cortical bone U SM LE SLE Musculoskeletal 637 https://t.me/usmleinnercircle Version 2 Systemic lupus erythematosus Lurus Path: Autoimmune, Complex Formation Pt: Women > Men Blacks> Whites Pt: Malar Rash Discoid Rash Serositis Oral Ulcers Arthritis Photosensitivity Dx: l5lANA Then: ds-DNA - Anti-smith - Anti-Histone (drug induce) Lupus Nephritis - U/ A-+ Bx Kidney Flare - Compliment Levels ! in flare - Compliment levels j in infection Tx: Reduce flares: Hydroxychloroquine Control symptoms: NSAIDs Flare: Prednisone Severe: Cyclophosphamide Nephritis: Cyclophosphamide Blood Renal Failure ANA Immunologic Neurology Seizures Psychosis Cognitive dysfunction Pleuritis/ Pericarditis/ pericardia!effusion ©UWorld Pancytopenia: Immune mediated destruction Joint involvement in SLE tends to be symmetric, migratory, non-erosive, and associated with brief morning stiffness (versus RA) • Hydroxychloroquine is an anti-malarial agent that is particularly effective at improving arthralgias, serositis, and cutaneous symptoms in SLE. Low dose, short-term prednisone may be used in patients with acute mild manifestations of SLE, whereas higher dose steroids or other immunosuppressants are typically reserved for patients with more severe, solid-organ manifestations. • Lupus nephritis and subsequent renal failure is a major contributor to mortality in SLE. What test is imperative in investigating the severity of lupus nephropathy? Biopsy Performing screening UA's are imperative. If things are off, perform biopsy. Only a biopsy will be able to tell us the severity of lupus nephropathy (i.e. nephritis, or glomerulosclerosis, or glomerular scarring). It also guides treatment (e.g. scarring is not treatable). Anti-dsDNA antibodies can be used to follow disease activity in SLE patients and is associated with the development of lupus nephritis. Musculoskeletal 638 https://t.me/usmleinnercircle Version 2 Features of drug-induced lupus erythematosus Acute onset of: • Constitutional symptoms (eg, fever, malaise) Clinical features* • Arthralgias • Serositis (eg, pleuritis, pericarditis) • Rash (less common compared with SLE) Implicated drugs • Antihistone antibodies (>90%) • Antinuclear antibodies (>95%) le Laboratory findings • High drug dose, prolonged use (>3 months) • Slow acetylator status • Most common: procainamide, hydralazine, penicillamine • Others: minocycline, TNF-a inhibitors (eg, etanercept, infliximab), isoniazid *No minimum number of SLE criteria is needed for diagnosis. SLE = systemic lupus erythematosus; TNF-a = tumor necrosis factor-alpha. • Edema • Malar rash Clinical Presentation • Arthritis ne • Hematuria rC SLE nephritis in pregnancy irc Risk factors • Nephritic range proteinuria • Urinalysis with RBC & WBC casts Laboratory findings • Renal biopsy In Diagnosis • i Complement levels • t ANA titers • Preterm birth • Cesarean delivery • Preeclampsia LE Obstetric complications • Fetal growth restriction • Fetal demise U SM • Systemic lupus erythematosus flare during pregnancy is distinguished from preeclampsia by the presence of RBC casts on urinalysis and classic symptoms of SLE (e.g. joint pain, malar rash). other findings consistent with SLE flare include decreased complement levels and increased ANA titers APLA Musculoskeletal 639 https://t.me/usmleinnercircle Version 2 Diagnostic criteria for antiphospholipid antibody syndrome (1 clinical & 1 laboratory criterion must be met) Vascular thrombosis • Arterial/venous thrombosis Clinical Pregnancy morbidity • ~3 consecutive unexplained fetal losses before 10th week • ~1 unexplained fetal loss after 10th week • ~1 premature birth of normal neonate before 34th week due to preeclampsia, eclampsia, . Laboratory placental insufficiency Lupus anticoagulant • Anticardiolipin antibody (lgG/lgM - medium or high titer) • Anti-beta-2 glycoprotein 1 antibody (lgG/lgM - high titer) Mx: Anticoagulation  Heparin, Warfarin )  What is the recommended treatment for a pregnant woman with antiphospholipid syndrome? Aspirin and Heparin warfarin is contraindicated during pregnancy Heparin prophylaxis is also given in pregnant patients with Factor 5 Leiden mutation AD Mixed Connective Tissue Disorder Mixed connective tissue disease • Autoimmune disorder with variable features of: Definition Clinical features 0 Systemic lupus erythematosus 0 Systemic sclerosis 0 Polymyositis 0 Rheumatoid arthritis • Raynaud phenomenon* • Hand/finger swelling* • Arthritis/synovitis* • Inflammatory myopathy* • Pulmonary hypertension • Malar or discoid rash • Mild CNS &/or kidney disease Laboratory findings • Anti-U1 ribonucleoprotein • Antinuclear antibody • Rheumatoid factor, anti-cyclic citrullinated peptide • Elevated creatine kinase • Anemia/cytopenia •Diagnostic criteria. Sjogren Syndrome Musculoskeletal 640 https://t.me/usmleinnercircle Version 2 Sjogren syndrome Exocrine features Extraglandular features • Keratoconjunctivitis sicca • Dry mouth, salivary hypertrophy • Xerosis • Raynaud phenomenon • Cutaneous vasculitis • Arthralgia/arthritis • Interstitial lung disease • Non-Hodgkin lymphoma • Objective signs of decreased lacrimation (eg, Schirmer test) findings • Positive anti-Ro (SSA) &/or anti-La (SSB) • Salivary gland biopsy with focal lymphocytic sialoadenitis • Classification: primary if no associated CTD, secondary if comorbid CTD (eg, SLE, RA, scleroderma) le Diagnostic irc CTD = connective tissue disease; RA= rheumatoid arthritis; SLE = systemic lupus erythematosus; SSA/SSB = Sjogren syndrome (antibody) A/B. Age-related sicca syndrome due to age-related exocrine gland atrophy; rC  What is the likely diagnosis in an elderly patient that presents with dry eyes and dry mouth? ANA is negative. ne Sjogren syndrome typically presents in middle-aged patients with a positive ANA Exocrine output from lacrimal and salivary glands declines with age In Age-related dry eye syndrome vs Sjogren syndrome Age-related dry eye syndrome Age of onset Clinical • 50s-80s • Mild to moderate mucosa! dryness U SM features • Common LE Prevalence Diagnostic features . . Sjogren syndrome Uncommon to rare • 30s-60s .. . Mucosal dryness often severe Parotid enlargement Comorbid autoimmune disease (eg, scleroderma, rheumatoid arthritis) Normal ESR and autoimmune markers* • Secretory function objectively normal/nearnormal (eg, normal Schirmer test) . • Abnormal ESR and autoimmune markers* Secretory function objectively decreased (eg, abnormal Schirmer test) 'Examples: antinuclearantibody,anti-SSA(Ro}, anti-SSB (La}. E SR = erythrocytesedimentationrate. Sjogren's Syndrome diagnosis key points: • often a part of another autoimmune disease (esp SLE, but can be solo; diagnosis clinical but needs confirmation • Best initial blood test is SSARo and SSBLa testing • Best initial overall test is the Schirmer tear production test with a fliter paper on the eye Musculoskeletal 641 https://t.me/usmleinnercircle Version 2 • Most accurate test is a lip or parotid biopsy looking for the lymphocytic infiltration • Rose bengal staining reveals an abnormal corneal epithelium if performed Just a reminder that SS also cause: Dental caries secondary to lack of saliva Dyspareunia secondary to lack of vaginal secretions Scleroderma Systemic sclerosis can cause interstitial and perivascular fibrosis in the kidneys. The resultant arteriolar vasculitis and glomerulitis can cause scleroderma renal crisis, which manifests with: • Sudden-onset hypertension • Papilledema • Azotemia • Microangiopathic hemolytic anemia • Hematuria, proteinuria Treatment: ACE inhibitors reduce RAAS activity and improve renal function and blood pressure  Sclerosis of what organ is the most common cause of death in scleroderma? Lungs causes interstitial fibrosis and pulmonary hypertension; Second most common cause is kidney involvement (sclerodermal renal crisis) Temporomandibular Joint Musculoskeletal 642 https://t.me/usmleinnercircle Version 2 Temporomandibular joint disorder manifestations Diagnosis Management Psychiatric illness (eg, anxiety, history of abuse) Facial pain (worsens with jaw motion) Ear pain, tinnitus Headache (unilateral, worse on awakening) Jaw dysfunction Clinical, imaging not typically needed Tenderness of mastication muscles Tooth wear (evidence of bruxism) Crepitus or clicking with TMJ motion Education (eg, avoidance of triggers, soft diet) Dental splints (if bruxism suspected) NSAIDs (eg, naproxen) rC NSAIDs = nonsteroidal anti-inflammatory drugs; TMJ = temporomandibularjoint. Upper Limb le Clinical Joint trauma (eg, injury, bruxism) irc .. .. .. .. .. .. . Risk factors  What is the typical prognosis for newborns with Erb-Duchenne or Klumpke palsy secondary to shoulder dystocia? ne Spontaneous resolution within 3 months 80% rarely surgical intervention is considered for infants with no improvement by age 3  6 months LE In Klumpke and Erb-Duchennepalsy U SM lnterphalangeal Forearm joints flexed supinated Mechanism Physical findings Nonna! arm Wrist& fingers Normal arm Hexed Klumpke palsy Erb•Duchenne palsy ·c1awhand* -Waiter'stip"' Radial head subluxation (nursemaid's elbow) • Axial traction on forearm with elbow extended (child pulled, lifted, or swung by arm) • Arm held extended & pronated • No swelling, deformity, or focal tenderness • Hyperpronation of forearm Treatment OR • Supination of forearm & ftexion of elbow Exclusively under 5 Musculoskeletal 643 https://t.me/usmleinnercircle Version 2 Carpal Tunnel Risk factors Clinical presentation Confirmatory test .. .. .. .. . . . Carpal tunnel syndrome Obesity Pregnancy Diabetes mellitus Hypothyroidism Rheumatoid arthritis End-stage renal disease/hemodialysis Pain & paresthesia in median nerve distribution (first 3½ digits) Positive Phalen, Tinel, or Durkan (carpal compression) test Severe disease: weakness of thumb abduction & opposition, atrophy of thenar eminence Nerve conduction studies • Wrist splinting Treatment • Glucocorticoid injection Surgery for severe or refractory symptoms  When surgical intervention is being considered, electrodiagnostic testing is frequently performed to confirm the clinical diagnosis and evaluate the extent of nerve injury. Pathophysiology of carpal tunnel syndrome Cause Idiopathic/overuse Hypothyroidism Diabetes mellitus Rheumatoid arthritis Pregnancy End-stage renal disease Acromegaly Gout . . .. . . . .. Pathophysiologic features Swelling & fibrosis of tendons & soft tissue Soft tissue enlargement (mucopolysaccharides) Soft tissue enlargement Microvascular insufficiency & neovascularization Extrinsic compression from joint deformity Edema/fluid accumulation • Amyloid & calcium phosphate deposition . Access related (bleeding, venous hypertension during HD, vascular steal) Tendon enlargement Synovial edema Compression from tophi HD = hemodialysis. Provocative tests for carpal tunnel syndrome Flexion of wrist compresses nerve inside carpal tunnel Phalen test Tinel sign Limited sensitivity and specificity Musculoskeletal 644 https://t.me/usmleinnercircle Version 2 Shoulder .• . • .• .• .• . .• . Common causes of shoulder pain Adhesive capsulitis (frozen shoulder) Biceps tendinopathy or rupture Similar to rotator cuff tendinopathy Weakness with abduction & external rotation Age >40 Decreased passive & active range of motion Stiffness ± pain Anterior shoulder pain Pain with lifting, carrying, or overhead reaching Weakness (less common) Uncommon & usually caused by trauma Gradual onset of anterior or deep shoulder pain Decreased active & passive abduction & external rotation rC Glenohumeral osteoarthritis Normal range of motion with positive impingement tests (eg, Neer, Hawkins) le Rotator cuff tear Pain with abduction, external rotation Subacromial tenderness irc Rotator cuff impingement or tendinopathy • Pain over AC joint AC joint sprain • Passive shoulder adduction provokes pain ne  Shoulder trauma can also cause acute subacromial (subdeltoid) bursitis, but the pain is usually aggravated by abduction or flexion with the arm internally rotated. In • Neer test With the patient's shoulder internally rotated and forearm pronated, the examiner stabilizes the scapula and flexes the humerus. Reproduction of the pain is considered a positive test. LE  Adduction of the arm across the body (ie, cross-body adduction test), which compresses the AC joint, provokes pain in the AC joint in the superior shoulder in AC joint Sprain. U SM Rotator Cuff Tear causes weakness with pain (vs Tendinopathy which causes only pain) Musculoskeletal 645 https://t.me/usmleinnercircle Version 2 Popoye sign in Bicep tendon rupture Adhesive Capsulitis  What is the likely diagnosis in a patient with two months of progressive left shoulder stiffness? The patient has markedly decreased passive and active abduction, flexion, and rotation of the left shoulder. Adhesive capsulitis (frozen shoulder) • Characterized by stiffness out of proportion to pain and reduction in both active and passive ROM • Due to inflammation and fibrosis of the joint capsule DDX Rotator cuff tendonitis is pain with abduction and external rotation + subacromial tenderness Pathogenesis Clinical presentation Diagnosis Treatment Natural history Musculoskeletal .. . .. .. .. .. .. Adhesive capsulitis Glenohumeral capsule contracture Idiopathic or secondary to shoulder injury (eg, rotator cuff tear, surgery) Risk factors: diabetes mellitus, hypothyroidism Gradual onset, poorly localized shoulder pain & stiffness Decreased passive & active range of motion Clinical diagnosis X-ray, MRI, ultrasound to rule out other causes (eg, rotator cuff tendinitis, osteoarthritis) Range-of-motion exercises Nonsteroidal anti-inflammatory drugs Corticosteroid injection Arthroscopic distension of joint capsule or surgical release Muscle atrophy of shoulder in advanced disease Pain & stiffness may last years 646 https://t.me/usmleinnercircle Version 2 • Initial phase: acute (often severe) pain with I stiffness (-2-9 months) Clinical presentation • Secondary phase: stiffness but 1 pain (-4-12 months) • Recovery phase: gradual return of motion (months to years) Rotator Cuff Tear Diagnosis Management Age >40 Acute glenohumeral dislocation Lateral shoulder pain Decreased active range of motion (eg, abduction) Positive drop arm test* MRI Ultrasound Physical therapy Consider surgical repair le Clinical presentation .. .. . .. .. irc Risk factors Acute rotator cuff injury rC •The arm is held in 90-degree abduction & released; inability to hold the arm steady suggests a tear. Rotator cuff tear ne Droparmt.e.s.t (siu11raSJ1 ·natusmll5Cle) U SM LE In Patientjsaskedto lower the a.rmsl<Y,,fy from abduction. D/D Axillary Nerve injury can mimic RCI with pain and weakness of abduction; however, the associated sensory loss over the lateral shoulder area differentiates it from RCI. Dislocation Musculoskeletal 647 https://t.me/usmleinnercircle Version 2 Shoulder dislocation Nonnal shoulder Anterior glenohumeral dislocation Posterior shoulder dislocation Acute glenohumeral dislocation of injury • Blow to abducted/raised arm • Fall on outstretched hand Clinical • Anterior dislocation: arm held in abduction/external rotation, anterior prominence of humeral head Mechanism features Management • Violent muscle contraction (eg, seizure) • Posterior dislocation: arm held in adduction/internal rotation, loss of anterior contour, prominence of coracoid & acromion • Closed reduction (uncomplicated), surgical repair • Immobilization & progressive rehabilitation • Fracture (glenoid, proximal humerus, clavicle) Complications • Rotator cuff injury • Recurrent dislocation  What is the first choice treatment for anterior shoulder dislocation? Closed reduction Check for neurovascular (axillary) injuries BEFORE closed reduction is attempted Musculoskeletal 648 https://t.me/usmleinnercircle Version 2 Anterior Shoulder Dislocation is MOST common Suprascapular Nerve Entrapment Suprascapular nerve entrapment Suprascapular Superiortransverse scapularligament rC irc \ Compression of nerveat le nerve ne lnfraspinatus m.  Clinical features suggestive of SNE at the suprascapular notch include: In • shoulder pain • weakness of shoulder abduction (supraspinatus muscle) LE • weakness of external rotation (infraspinatus muscle) Initial management includes NSAIDs and activity modification U SM De Quervain Tendinopathy De Quervain tendinopathy Pathogenesis Symptoms • Myxoid degeneration of tendons/tendon sheaths • Involves abductor pollicis longus & extensor pollicis brevis . Lateral hand/wrist pain • Provoked by lifting objects, thumb or wrist movement Diagnosis • Based on clinical features (eg, positive provocative testing) • Tenderness at radial styloid • Pain with thumb flexion + ulnar wrist deviation Management • Thumb spica splint • Nonsteroidal anti-inflammatory drugs • Persistent/severe cases: corticosteroid injection Musculoskeletal 649 https://t.me/usmleinnercircle Version 2 Thumb spica splint De Quervain tendinopathy Extensor retinaculum 1--------== Abductor pollicis longus Thickened tendon sheaths Extensor pollicis brevis Elbow Count tforce elbow brace Lateral epicondylitis Pathophyslology Cllnlcal presentation Repetitive, forceful overuse of wrist & digit extensors • Angiofibroblastic tendmosis of extensor tendons at lateral epicondyle • Insidious pain: onset weeks to months • Pain 1 cm distal lo lateral ep1condyle • Pain elicited by resisted wnst extension & passive wnst Hexion • Diagnosis based on clinical presentation Management • Musculoskeletal ultrasound for doubtful cases • Initial treatment: activity modification, counterforce bracing/strap Some patients pre er a compression sleeve. Refractory symptoms: short-term NSAIDs. corticosteroid m1ect1on,surgery NSAJDs= nonstero,dalantJ-mflammatory drugs Dupuytren Contractures Musculoskeletal 650 https://t.me/usmleinnercircle Version 2 Dupuytren contracture Dupuytren contracture Risk factors • Male sex, age >50, family history • Diabetes mellitus • Tobacco & alcohol use • Thickening of palmar fascia at the 3rd, 4th & 5th digits rC irc Treatment • Discrete nodules along flexor tendons near distal palmar crease • Gradual decrease in extension of digits • Modification of hand tools (eg, cushion tape, padded gloves) • Needle aponeurotomy • lntralesional glucocorticoid injection • Surgery for contractures or advanced disease le Clinical presentation ne Lower Limb In Location of pain for common hip disorders LE Joint pathology (eg, osteoarthritis) U SM Greater trochanteric pain syndrome-----j (bursitis,tendonitis) Meralgiaparesthetica (lateralfemoralcutaneous nervecompression)  What should be suspected in a older patient with groin pain that is relieved with rest and worsens with activity? Examination shows pain on internal rotation of the leg. Osteoarthritis of the hip (coxarthrosis) Musculoskeletal 651 https://t.me/usmleinnercircle Version 2 Meralgia paresthetica Lateral femoral cutaneous nerve entrapment or injury due to: • Compression (eg, light clothing, obesity, pregnancy) Etiology • Iatrogenic injury (eg, total hip arthroplasty) • Paresthesia & t sensation at lateral thigh • Normal motor & reflex examination Clinical features • Avoidance of tight garments, weight loss Management • Nonopioid analgesics (eg, NSAIDs) • Anliconvulsants (eg, gabapenlin), nerve block, or surgical release for refractory cases NSAIDs = nonsteroidal anti-inflammatorydrugs. Trochanteric Bursitis Greater trochanteric pain syndrome (trochanteric bursitis) Risk factors Symptoms Diagnosis Treatment .. • . .. .. . .. . Age ~50 Women> men Obesity Low back & lower extremity disorders (eg, scoliosis, osteoarthritis, plantar fasciitis) Chronic lateral hip pain Pain worse with hip flexion or lying on affected side Focal tenderness over trochanter X-ray to rule out hip joint pathology Ultrasound: degeneration of tendons, tendinosis Exercise, physical therapy, activity modification Nonsteroidal anti-inflammatory drugs Corticosteroid injection Knee Musculoskeletal 652 https://t.me/usmleinnercircle Version 2 Special tests for knee examination Valgus stress test • Stabilize lateral thigh with 1 hand. Place the other along medial leg & app'ly outward pressure. MCL injury Laxity indicates MCL injury Anterior drawer test • Patient supine with knee flexed • Grip proximal tibia with both hands & pull anteriorly Lachman test ACL injury • Place knee at 30 degrees flexion le • Stabilize distal femur with 1 hand & pull proximal tibia anteriorly with the other Laxity of tibia indicates ACL injury Thessaly test irc • Patient stands on 1 leg with knee flexed 20 degrees • Patient then internally & externally rotates on flexed knee McMurray test Meniscal tear • Passive knee flexion & extension while holding the knee in internal or external rotation rC Pain, clicking, or catching indicates meniscal tear Clinical features Young female athletes • Subacute to chronic pain I with squatting, running, prolonged sitting, using stairs Patellar tendonitis Osgood-Schlatter disease Primarily athletes ("jumper's knee") • PreadolescenU adolescent athletes In Typical patient Patellofemoral syndrome Episodic pain & tenderness at inferior patella • Recent growth spurt . LE Diagnosis ne Differential diagnosis of anterior knee pain in the young patient I Pain with sports, relieved by rest • Tenderness & swelling at tibial tubercle • Patellofemoral compression test U SM Sa Patellofemoral associated with a sensation of instability or "buckling" at the knee. Musculoskeletal Osgood Schlatter 653 https://t.me/usmleinnercircle Version 2 Patellofemoral syndrome lliotibial band syndrome Pes anserine bursitis Patellar tendinopathy Pre patellar bursitis .. . .. . ... .. . • .. Common overuse injuries of the knee Poorly localized anterior pain Pain with squatting Common in runners, more common in women Poorly localized lateral pain Tenderness at lateral femoral epicondyle with flexion & extension Common in runners, cyclists Highly localized medial pain Point tenderness at pes anserine bursa Common with osteoarthritis, diabetes mellitus Localized pain in inferior patella Tenderness at tendon insertion at inferior patellar margin Common in jumping sports (eg, basketball, volleyball) Anterior knee bogginess & tenderness Propensity to secondary infection with Staphylococcus aureus Common in patients who work on their knees ("housemaid's knee") • Injury to the popliteal artery is the most feared complication of any knee dislocation because the resulting lower leg ischemia can cause irreversible injury, requiring above-the-knee amputation. Management begins with immediate reduction of the dislocated knee. Given the risk of vascular injury, this should be followed by a meticulous vascular examination that includes: • Palpation of the popliteal and distal pulses • Measurement of the ankle-brachial index ABI • Duplex ultrasonography (if available) Patellar tendon rupture Patellar tendon rupture Normal Patellar tendon rupture Superior displacement of patella Swelling Ruptured patellar tendon euwond Musculoskeletal 654 https://t.me/usmleinnercircle Version 2 Patellar tendon rupture rC irc le Normal knee Patella baja LE In ne Normal patella U SM  Risk factors include chronic kidney disease, hyperparathyroidism, fluoroquinolone antibiotics and anabolic steroid abuse.  Patients with patellar tendon rupture should undergo early surgical repair to optimize recovery of normal knee motion and prevent long-term disability.  In contrast, management of partial patellar tendon tears, in which the extensor mechanism remains intact, is typically nonoperative, with a period of knee immobilization (eg, 6 weeks) followed by physical therapy. Patellar Dislocation Musculoskeletal 655 https://t.me/usmleinnercircle Version 2 Risk factors Clinical presentation .. .. . .. . Patellar dislocation Joint laxity Misaligned lower extremity Tight iliotibial band Patellar subluxation Competitive sports, dance, military training Quick, twisting motion around a flexed knee Feeling of knee giving way, severe pain, popping noise Examination: lateral dislocation of patella, decreased extension Patellar dislocation Normal Laterally displaced patella (right knee) Dislocated patella Quadriceps tendon Lateral patellofemoral ligament  The quadriceps muscles normally exert a lateral force on the patella, especially during quadriceps contraction; therefore, in cases of patellar dislocation, lateral displacement with associated tear of the medial patellofemoral ligament (which normally provides an opposing medial force) is most common. Superior and medial dislocations are rare. T/t: Spontaneous Reduction or Closed Reduction ACL Injury Musculoskeletal 656 https://t.me/usmleinnercircle Version 2 Tests for anterior cruciate ligament tear Features of anterior cruciate ligament injury Injury mechanisms . Anterior drawer test Rapid deceleration or direction changes • Pivoting on lower extremity with foot planted . Popping sensation at the time of injury . Anterior laxity of the tibia relative to the femur (anterior • Pain: sudden onset, severe Examination • Significant swelling (effusion/hemarthrosis) • Joint instability Lachman test drawer test, Lachman test) findings le Symptoms • Plain x-rays usually normal, but fracture of tibial spine Treatment . . • or anterolateral tibial plateau suggests AGL tear MRI provides definitive diagnosis irc Imaging RICE (Rest, Ice, Compression, Elevation) measures ± Surgery depending on age & activity level rC ACL = anteriorcruciateligament. ne  What is the likely diagnosis in an athlete that felt a "popping sensation" while playing soccer, followed by rapid pain/swelling? Aspiration of the knee yields grossly bloody joint fluid. ACL injury In a "popping sensation" with rapid-onset pain/swelling and hemarthrosis are characteristic of ACL injuries (versus slowly progressive or absent effusion in meniscal tears) LE PCL Injury U SM Posterior cruciate ligament tear It can be disrupted when a strong posteriorly directed force strikes the anterior aspect of the proximal tibia while the knee is in a flexed position (ie, classic dashboard injury). Musculoskeletal 657 https://t.me/usmleinnercircle Version 2 Patellofemoral Pain Syndrome Patellofemoral pain syndrome (PFPS) Lateral view Anterior view • Atrophy or weakness of the quadriceps or hip abductors is common, and rotational or varus/valgus malalignment may be noted.  Dx: Provocation of pain during tonic contraction of the quadriceps with the knee flexed (eg, squatting, lunging); Imaging (eg, x-ray, MRI is usually normal and needed only to exclude other disorders. • Patellofemoral compression test : Extension of the knee while compressing the patella  What is the initial pharmacologic therapy for treatment of patellofemoral pain syndrome? NSAIDs additional management includes activity modification and strengthening exercises Popliteal Cyst Etiology Risk factors Clinical presentation Complications Musculoskeletal . .. .. .. . Popliteal (Baker) cyst Extrusion of ftuid from knee joint space into semimembranosus/gastrocnemius bursa Trauma (eg, meniscal tear) Underlying joint disease (eg, osteoarthritis, rheumatoid arthritis) Asymptomatic bulge behind knee that diminishes with ftexion Posterior knee pain, swelling, stiffness Venous compression (leg/ankle swelling) Dissection into calf (erythema, edema, positive Homans sign) Cyst rupture (acute calf eain, warmth, erythema, ecch:i'.mosis) 658 https://t.me/usmleinnercircle Version 2 le  What is the likely diagnosis in a patient that develops acute posterior calf pain/swelling while walking? Physical examination shows tenderness/induration at the medial head of the gastrocnemius irc and a crescent-shaped patch of ecchymosis at the medial malleolus. Ruptured popliteal cyst rC may resemble a DVT; before rupture, popliteal cysts present as a painless bulge in the popliteal space Meniscal Tear Examination Diagnosis ne Older patients: degeneration of meniscal cartilage Acute popping sensation Catching, locking, reduced range of motion Slow-onset joint effusion Joint line tenderness Pain or catching in provocative tests (Thessaly, McMurray) MRI Arthroscopy Patientrotatesbodysidetosidewithkneein20°flexion&footplanted Surgery (knee arthroscopy): persistent symptoms &/or impaired activity Conservative management: 0 Mild symptoms without impaired activity 0 Older patients with degenerative tears U SM Management Younger patients: rotational force on planted foot In Symptoms .. .. . .. .. .. Thessaly test for meniscal injury LE Etiology Meniscal tears Musculoskeletal 659 https://t.me/usmleinnercircle Version 2 McMurray test for meniscal injury Examiner maximally flexes & extends the knee while rotating the lower leg CUWorld External rotation of tibia t valgus force stressesmedialmeniscus Internal rotation of tibia t varus force stresseslateral meniscus Remember with L.I.M.E Ankle & Foot Differential diagnosis of heel pain . • . .. .• • Maximal pain on first stepping out of bed Plantar fasciitis Achilles tendinopathy Calcaneal stress fracture Tarsal tunnel syndrome Pain & tenderness at medial plantar heel, worse with toe dorsiflexion Posterior pain Swelling & tenderness 2-6 cm proximal to tendon insertion Pain that is worse with activity Pain reproduced by medial-lateral squeezing of the calcaneus Pain, paresthesia & numbness on the sole of the foot Percussion tenderness over the posterior tibial nerve in the tarsal tunnel Overview of running injuries of the foot & ankle Clinical features Injury • Insidious onset Stress fracture • Focal pain in navicular or metatarsals Plantar fasciitis • Plantar surface of the heel Achilles tendinopathy Morton neuroma Tarsal tunnel syndrome • Risk factors: abrupt increase in intensity of training, poor running mechanics, female with eating disorder • Worse when initiating running or first steps of the day • Burning pain or stiffness 2-6 cm above the posteriorcalcaneus • Numbness or pain between the 3rd & 4th toes I • Clicking sensation when palpating space between 3rd & 4th toes while squeezing the metatarsal joints • Compression of the tibial nerve at the ankle • Burning, numbness & aching of the distal plantar surface of the fooVtoes I  What is the likely diagnosis in a young female that presents with foot pain? The patient experiences pain and a clicking sensation when the 3rd/4th metatarsal heads are squeezed together. Musculoskeletal 660 https://t.me/usmleinnercircle Version 2 Morton neuroma mechanically-induced degenerative neuropathy; Treatment is conservative (e.g. metatarsal support, padded shoe inserts) Mulder sign: squeezing metatarsal joints will cause pain on the plantar surface of the foot along with crepitus between the third and fourth toes. Medial view Pain at the mal/eolar zone and Posterior marginor tip of medial malleolus • Tender at posterior margin/tip of medial malleolus OR • Tender at posterior margin/tip of lateral malleolus OR • Unable to bear weight 4 steps (2 on each foot) Malleolar zone Midfoot zone , Navicular Lateral view rC X-ray of the foot is required if: irc X-ray of the ankle is required if: le Ottawa ankle rules Pain at the midfoot zone and Posterior marginor \ tip of lateral malleolus • Tender at the navicular OR • Tender at the base of the 5th metatarsal OR ne • Unable to bear weight 4 steps (2 on each foot) In Use of a soft protective brace and early range of motion exercises through physical therapy is the next most appropriate step in management for this patient who most likely has an ankle sprain. U SM LE Twisting injuries of the ankle are common and more frequently result from inversion injuries to the lateral ankle. The most important step in the evaluation of ankle injuries is to determine if the patient requires imaging to rule out a clinically significant fracture. Planter Fasciitis Risk factors Symptoms Diagnosis Plantar fasciitis .. .. .. . • . • Pes planus Obesity Working or exercising on hard surfaces Pain at plantar aspect of heel & hindfoot Worse with weight bearing (especially after prolonged rest) Tenderness at insertion of plantar fascia Pain with dorsiflexion of toes Presence of heel spurs on x-ray has low sensitivity & specificity • Activity modification Treatment Musculoskeletal Stretching exercises Heel padslorthotics 661 https://t.me/usmleinnercircle Version 2 Plantar fasciitis Degeneration of plantar aponeurosis Calcaneus Heel pain with standing or walking C)UWo,1d Pain at Anteromedial Heel Achilles Tendinopathy Achilles tendinopathy Risk factors Clinical features Examination findings Diagnosis Management • Athletic activity, increase in activity • Systemic disorders: psoriasis, ankylosing spondylitis . Medications: glucocorticoids, fluoroquinolones • Swelling, warmth, pain at posterior heel • Tendon rupture: "popping" sensation & acute pain following rapid acceleration/direction change • Swelling, tenderness 2-6 cm proximal to tendon insertion • Rupture: positive Thompson test* . • • . Clinical findings Ultrasound: swelling, neovascularization MRI Acute: activity modification, ice, NSAIDs • Chronic: eccentric resistance exercises *With the patient prone and feet off the end of the table, squeeze the calf muscles; the absence of plantar flexion indicates tendon rupture. NSAIDs = nonsteroidal anti-inflammatorydrugs. Normal Achilles tendon Gastrocnemius muscle Soleus muscle Achilles tendon C>UW.ld Thompson Test: No Passive Planter Flexion on sqeezing Gastronemius Musculoskeletal 662 https://t.me/usmleinnercircle Version 2  Complete tendon rupture results in an impaired ability to walk on the tips of the toes because this movement requires the power of the gastrocnemius and soleus muscles Calcaneal Apophysitis Calcaneal apophysitis Calcaneal apophysitis (Sever disease) • Running/jumping sports Risk factors • Growth spurts . le • Athletic cleat use or footwear without heel padding • Heel pain (50% bilateral) Treatment Pain with calcaneal palpation or compression • Decreased gastrocnemius/soleus flexibility • Nonsteroidal anti-inflammatory drugs, ice irc Clinical features • Activity limitation Growth plate (apophysis) Calcaneus (heel bone) ne rC ,_ ____ Achillestendon  Diagnostic findings include tenderness with squeezing the heel (calcaneal compression test) and on palpation at the base of the heel over the apophysis. U SM LE In Spine Musculoskeletal 663 https://t.me/usmleinnercircle Version 2 Differential diagnosis of neck pain Condition .. Strain Facet osteoarthritis .. . .. . Radiculopathy Spondylitic myelopathy Spondyloarthropathy Spinal metastasis Vertebral osteomyelitis .. .. .. .. . .. . Clinical clues Antecedent history of neck injury Pain/stiffness with neck movement Older individuals Pain/stiffness worse with movement Relieved with rest Pain radiates to shoulder/arm Dermatomal sensory/motor/reflex findings Positive Spurling test LE weakness, gaiUbowel/bladder dysfunction Lhermitte sign Young men HLA-B27 Relieved with exercise Prolonged morning stiffness Constant pain Worse at night Not responsive to position changes Focal tenderness Fevers & night sweats IVDU, immune compromise, or recent infection IVDU = intravenousdrug use; LE = lower extremity.  What is the likely diagnosis in a child with Down syndrome that presents with behavioural change, urinary incontinence, and upper motor neuron symptoms? Atlantoaxial instability due to compression of the spinal cord; treatment consists of surgical fusion of the first (C1) and second (C2) cervical vertebrae Excessive mobility between vertebrae C1 and C2, which may cause a subluxation of the cervical spine and spinal cord compression. Symptoms include pain, ataxia, incontinence, quadriparesis, and quadriplegia. Spinal Spondylosis Cervical spondylosis is marked by cervical spine degeneration. It is generally associated with 2 clinical syndromes: • Cervical radiculopathy: Degeneration and osteophyte formation in the zygapophyseal (facet) and uncovertebral joints lead to intervertebral foramen narrowing and compressive nerve root symptoms. Most patients have progressive neck, shoulder, and/or arm pain plus weakness in a myotome and sensory loss in a dermatome. • Compressive cervical myelopathy: Degeneration and thickening of the lateral vertebral bodies and posterior longitudinal ligament lead to spinal canal narrowing and subsequent spinal cord compression. Musculoskeletal 664 https://t.me/usmleinnercircle Version 2 This usually presents with neck pain, lower motor neuron signs in the upper extremities, upper motor neuron signs in the lower extremities, and bowel/bladder dysfunction.  What is the likely diagnosis in an elderly patient with right-sided neck pain and sensory deficits over the posterior forearm? Physical exam reveals limited neck rotation and lateral bending. Cervical spondylosis limited neck rotation and lateral bending is due to osteoarthritis with secondary muscle spasm; sensory deficit is due to osteophyte-induced radiculopathy le Cervical Radiculopathy (C5-6) C7 (C6-7) CB (C7-T1) T1 (T1-2) . Biceps . .. . . Biceps • Thumb Brachioradialis • Triceps . . Finger flexors** Finger flexors** Laleral upper arm Index finger Dorsal forearm ne C6 . .. Middle finger Ring & little fingers In cs (C4-5) Sensory loss• Reflex affected .. .. . .. • .. . Weakness Shoulder abduction (deltoid) Elbow flexion (biceps) rC Nerve root (disc space) irc Features of cervical radiculopathy Medial forearm Elbow flexion (biceps) Forearm pronation/supination (brachioradialis) Wrist extension Elbow extension (triceps) Wristflexion Finger extension Finger flexion & extension Thumb flexion & abduction Finger abduction & adduction *In addition to neck/shoulder pain, radicular pain typically has a similar distribution to sensory loss . LE .. Not typicallyevaluatedclinically. Radiculopathy can occur due to  Disk Herniation: Abrupt onset U SM  Spinal Spondylosis: subacute, progressive Dx: Clinical; Imaging NOT required in mild symptoms T/t: NSAIDʼs and avoidance of provocative • Shoulder abduction reduces tension on the impinged nerve root, and improvement of radicular symptoms when the hand is placed on the top of the head (ie, shoulder abduction relief test) can be both diagnostic and therapeutic for short-term pain relief. MRI is recommended only for patients with severe, progressive, or bilateral neurologic deficits, or in those with high concern for malignancy or epidural abscess Cervical Spondylotic Myelopathy Musculoskeletal 665 https://t.me/usmleinnercircle Version 2 .. Epidemiology .. .. . Manifestations .. Diagnosis .. Treatment Cervical myelopathy Age >55 Degenerative cervical spine/discs --, canal stenosis--, cord compression Gait dysfunction - usually first Extremity weakness & numbness LMN signs (arms) - muscle atrophy, hyporeflexia UMN signs (legs)- Babinski, hyperreflexia t Proprioception/vibration/pain sensation MRI of cervical spine CT myelogram Nonsurgical - immobilization Surgical decompression LMN = lower motor neuron; UMN = upper motor neuron.  An electric shock–like sensation down the spine with forward flexion of the neck (Lhermitte sign) is also commonly seen. • MRI scan of the cervical spine is the first test of choice, but myelography is often require D/D 1. ALS but it has assymetric involvement 2. MS but it doesnʼt cause LMN signs Vertebral Compression Fracture Etiologies . • . • . • . • . . • • . Clinical features of vertebral compression fracture Trauma (often trivial) Osteoporosis, osteomalacia Bone metastases Metabolic (eg, hyperparathyroidism) Paget disease Acute Clinical presentation Complications Back pain & decreased spinal mobility Pain increasing with standing, walking, lying on back Referred pain to abdomen/flank Spinal tenderness at affected level Chronic/gradual Painless Progressive kyphosis Loss of height Increased risk for future fractures • Hyperkyphosis--, protuberant abdomen, early satiety, weight loss, decreased reseirato~ caeacity Stenosis Musculoskeletal 666 https://t.me/usmleinnercircle Version 2 Neurogenic & vascular claudication Neurogenic claudication (pseudoclaudication) • Posture-dependent pain • Lumbar extension worsens pain (eg, walking downhill) • Lumbar flexion relieves pain (eg, walking while bent forward) • Lower extremity numbness & tingling • Lower extremity weakness • Low back pain Examination • • Frequently normal examination Diagnosis • MRI of the spine • Exertionally dependent pain • Pain relieved with rest but not with bending . forward while walking • Lower extremity cramping/lightness No significant lower extremity weakness • Possible buttock, thigh, calf, or foot pain . • Decreased pulses Normal pulses Cool extremities irc • Decreased hair growth • Pallor with leg elevation le Symptoms Vascular claudication • Ankle-brachia! index rC  Common causes of lumbar stenosis include vertebral osteoarthritis, degenerative disc disease, and thickening of the ligamentum flavum. Risk factors Bilateral pars interarticularis defects (eg, fractures) --+ anterior slippage of vertebral body Most common: L5 slips over S 1 Repetitive back extension & rotation (eg, gymnasts, divers) Adolescent growth spurt Low back pain that is worsened by extension Radiculopathy as slippage progresses Palpable step-off may be present LE Clinical features .. .. .. . . .. Spondylolisthesis In Pathophysiology ne Spondylolisthesis Diagnosis Activity modification (avoid inciting sports), pain control Neurologic deficits or symptoms >90 days: obtain MRI of spine & surgical consultation U SM Management Lumbar x-rays (typically visible on lateral views)  What is the likely diagnosis in a child that presents with several months of back pain and recent urinary incontinence with a palpable "step-off" at the lumbosacral area on physical exam? Spondylolisthesis typically caused by forward slip of the L5 vertebrae over S1 in preadolescent children Musculoskeletal 667 https://t.me/usmleinnercircle Version 2 Spondylolysis Spondylolisthesis LS vertebra Fracture of pars interarticularis .1---r,-- Spondylolysis (Fracture of the pars interarticularis) CUWorld Back Pain Diff&rantlal diagnosis of back pain Condition Degenera.tive {osteoa:rth:litis) RadlcUlopathy (eg, disc herniatioo} Spinal stenosl s .• Clinical!clues Positional Rel iell'ed With rest • Radiates to leg • Sensory &. motor findings . Positiveetr,aight-1'.eg raising test • Paln with standing, {spinal extension) • Relieve<!by spinall flexion .. Yoong mon Spondyloarthropathy .• HLA-B27 Rellev~ with exercise Prolooged moming stiffness .. .. • Constant pain Spinal meta1tui• Worse at nlghl Not responsiveto posill0r11 cMn9es Vertebral Qlfl~mytilllli, Musculoskeletal Focal t11nd1mH1ss Fevers & nlgnt sweals • Recenl lnfactlon, lntravanoua dru:g abuse, or immune compromise 668 https://t.me/usmleinnercircle Version 2 Chronic back pain in adolescents Diagnosis Clinical features • Nocturnal pain • Systemic symptoms Malignancy • Neurologic findings if spinal cord is involved • New-onset scoliosis • Back/leg pain worse with activity • Neurologic findings (eg, weakness, hyporeflexia) Tethered cord • New-onset scoliosis (spinal dysraphism) • Lumbosacral cutaneous abnormality • Systemic symptoms • Occurrence following repetitive trauma (eg, gymnastics) • Pain with extension • Morning stiffness Inflammatory arthritis • Pain worse at rest • Sacroiliac joint tenderness rC (eg, ankylosing spondylitis) le Spondylosis & spondylolisthesis • Nocturnal pain irc Chronic multifocal osteomyelitis Management of low back pain • Maintain moderate activity Acute pain • NSAIDs or acetaminophen Chronic pain ne • Consider: muscle relaxants, spinal manipulation, brief course of opioids • Intermittent use of NSAIDs or acetaminophen • Exercise therapy (stretching/strengthening, aerobic) • Exercise therapy • Education LE Secondary prevention In • Consider: tricyclic antidepressants, duloxetine Management of acute nonspecific back pain U SM Nonpharmacologic measures First-line medication Second-line medication .. . .. .. Maintain normal, moderate activity Education (eg, activity, prognosis) Consider: heat, massage, s12inalmani12ulation, ai;y12yni;tyr!;! NSAIDs (eg, naproxen, ibuprofen) Consider acetaminophen Nonbenzodiazepine muscle relaxant (eg, cyclobenzaprine, tizanidine) Opioids, tramadol (not preferred; consider short course for severe, refracto!l'. eain) NSAIDs = nonsteroidal anti-inflammatorydrugs.  What is the recommended next step in management for a patient with chronic low back pain despite intermittent use of acetaminophen and NSAIDs? Exercise therapy if pain persists after exercise therapy and intermittent acetaminophen/ NSAIDs, patients may benefit from TCAs or duloxetine; back braces are not effective for prevention of treatment of lower back pain Musculoskeletal 669 https://t.me/usmleinnercircle Version 2 Imaging in nontraumatic acute back pain Acute back pain (<4-6 weeks) without history of trauma t Significant neurologic deficits • Urinary retention/incontinence. saddle anesthesia (CES) -Yes- Emergency MRI -Yes- MRI or consider x-ray + ESR/CRP -Yes- MRI + ESR/CRP • LE weakness involving multiple nerve roots (cord compression) I No + Suspected infection • Fever • Spinal tenderness • IVDU/immunosuppression I No + Suspected malignancy • CurrenVrecent cancer history • Systemic symptoms (eg, weight loss) • Multiple cancer risk factors I No + Uncomplicated back pain No imaging needed, continue pain management CES = cauda equina syndrome: CRP =C-reactive protein: ESR = etytlYocyte sedimentation rate: IVDU = intravenous drug use: LE = lower extremity. iC)UWorld Indications for imaging in low back pain • Osteoporosis/compression fracture X-ray • Suspected malignancy • Ankylosing spondylitis (eg, insidious onset. nocturnal pain, better with movement) • Sensory/motor deficits MRI • Cauda equina syndrome (eg, urine retention, saddle anesthesia) • Suspected epidural abscess/infection (eg, fever, intravenous drug abuse, concurrent infection, hemodialysis) Radionuclide bone scan or CT scan • Indications for MRI but patient not able to have MRI Plain film x-rays have lower sensitivity for bone metastasis, but some guidelines suggest x-ray combined with inflammatory markers to increase sensitivity for patients with moderate clinical suspicion for malignancy. Lumber Radiculopathy Musculoskeletal 670 https://t.me/usmleinnercircle Version 2 Acute lumbosacral radiculopathy • Herniated intervertebral disc (most common) • Degenerative spondylosis • Malignancy • Epidural abscess • Pain in low back radiating down posterior leg to foot • Positive straight-leg or crossed straight-leg raising test • Dermatomal sensory loss & myotomal weakness Causes Signs & symptoms • Diagnosis based primarily on clinical features • MRI recommended for: Significant/progressive or bilateral neurologic deficits 0 Evidence of bladder/bowel dysfunction, saddle anesthesia 0 Suspected malignancy or eeidural abscess • Activity modification (not bed rest) Management • First 1-2 weeks: NSAIDs le 0 irc Evaluation • After 2 weeks: consider physical therapy, oral glucocorticoids • After 4-6 weeks: obtain MRI & assess for surgical indication rC NSAIDs= nonsteroidalanti-inflammatorydrugs. Features of lumbosacral radiculopathy LS S1 S2-S4** • None • Achilles • Anocutaneous Weakness ne • Patellar Sensory loss* • Anteromedial thigh • Medial shin • Lateral shin • Dorsum of the foot • Posterior calf • Hip flexion (iliopsoas) • Hip adduction • Knee extension (quadriceps) • Foot dorsiflexion & inversion (tibialis anterior) • Foot eversion (peroneus) • Toe extension (extensor hallucis & digitorum) • Hip extension (gluteus maximus) Knee flexion (hamstrings) • Sole & lateral foot • Foot plantarflexion (gastrocnemius) • • Urinary or fecal incontinence • Perineum Sexual dysfunction LE L2-L4** Reflex affected In Nerve Root • U SM 'Radicular pain typically has a similar distribution to sensory loss. -Difficult to distinguish between individual nerve roots clinically. Achilles tendon reflex decreases with age, and its absence is common in older individuals. Normal finding)  What is the next step in management for a patient that presents with suspected lumbosacral radiculopathy (sciatica) after lifting a heavy box? Straight leg-test is positive and neurological exam is unremarkable. Trial of NSAIDs/acetaminophen Imaging in herniation is controversial as it doesn't change management. Therefore no MRI for just back pain + positive SLR alone. Musculoskeletal 671 https://t.me/usmleinnercircle Version 2 If severe/progressive neurological deficits, answer MRI. Most patients 80% will experience spontaneous resolution  Should a patient with disk herniation be recommended bed rest? No; maintain ordinary activities Commonly tested point -- do not do imaging studies in patients without focal neurological abnormalities or with simple lumbosacral strain  What is the likely diagnosis in a patient that presents with back pain after carrying heavy packages? Physical exam reveals paravertebral tenderness. Straight leg-test is negative and neurological exam is unremarkable. Lumbosacral strain most common cause of acute back pain that does not radiate below the level of the knee Lumbosacral strain Causes Clinical features Management • Strain of paraspinal muscles, tendons, intervertebral ligaments • Sudden or unbalanced muscle contraction (eg, lifting, twisting) • Risk factors: obesity, seinal deformity or degeneration, muscle weakness • Pain in lumbar area; may radiate to buttocks, hips, thighs (above knee) • Paraspinal tenderness • No neurologic deficits; negative straight-leg raising test • Moderate activity • Nonsteroidal anti-inflammatory drugs • Nonbenzodiazepine muscle relaxants Spine Trauma Prehospital Emergency department . .. . . . Management of cervical spine trauma Spinal immobilization (eg, backboard, rigid cervical collar, lateral head supports) Careful helmet removal (eg, motorcycle helmet) Supplemental oxygenation Orotracheal intubation preferred unless significant facial trauma present In-line cervical stabilization suggested unless it interferes with intubation CT of entire cervical spine  What is the first step in the management of cervical spinal trauma? Spine immobilization  Cervical spine imaging should be performed whenever there is a high-energy mechanism of injury (eg, high-speed motor vehicle collision, fall 3 m 10 ft], trauma causing concomitant closed-head injury). Musculoskeletal 672 https://t.me/usmleinnercircle Version 2  According to NEXUS criteria, Cervical Spine imaging is necessary if any of the following is present: • Neurologic deficit • Spinal tenderness • Altered mental status • Intoxication • Distracting injury (e.g long bone fracture) A CT scan of the CS without contrast is the preferred screening test to evaluate for CS injury irc le If none of the NEXUS criteria are present, then imaging is not required; a normal neurologic examination is sufficient to rule out CS injury and remove the collar.  The presence of a single vertebral fracture (esp Cervical) in a patient with blunt trauma is an indication to image the entire spine. rC CT scan is the screening modality of choice because of its sensitivity and accuracy. Bone Pathology ne Avascular Necrosis Avascular necrosis In • Steroid use • Alcohol abuse • Systemic lupus erythematosus • Antiphospholipid syndrome • Hemoglobinopathies (eg, sickle cell) • Infections (eg, osteomyelitis, HIV) • Renal transplantation • Decom12ressionsickness • Groin pain on weight bearing • Pain on hip abduction & internal rotation LE Etiology U SM Clinical manifestations • No erythema, swelling, or point tenderness Laboratory findings Radiologic imaging • Normal white blood cell count • Normal ESR & CRP • Crescent sign seen in advanced stage • MRI is most sensitive modality CRP = C-reactive protein; ESR = erythrocyte sedimentation rate. Excessive alcohol use is a major risk factor, with the risk proportionate to the amount and duration of use. Musculoskeletal 673 https://t.me/usmleinnercircle Version 2 Osteonecrosis (avascular necrosis) Crescent Sign on Advanced X-ray MRI serpiginous low-intensity lines T1 images) Osteonecrosis of the femoral head Initially X-ray and joint movement are NORMAL  The femoral head has 2 main sources of blood - the ascending arteries and the foveal artery, which lies within the ligamentum teres. The foveal artery is patent early in life, but may become obliterated in older patients. For this reason, aseptic necrosis of the femoral head is uncommon in children but the risk rises in older patients. Musculoskeletal 674 https://t.me/usmleinnercircle Version 2 Osteomalacia Clinical features of osteomalacia Diagnosis le 0 Cortical thinning & reduced bone density 0 Bilateral symmetric pseudofractures (Looser zones) irc Symptoms/signs • Malabsorption • Intestinal bypass surgery • Celiac disease • Chronic liver disease • Chronic kidney disease • May be asymptomatic • Bone pain & muscle weakness • Muscle cramps • Difficulty walking, waddling gait • t Alkaline phosphatase, t PTH • ! Serum calcium & phosphorus, ! urinary calcium • ! 25(0H)D levels • Imaging rC Causes Rickets 25(0H)D = 25-hydroxycholecalciferol; PTH = parathyroidhormone. Clinical manifestations of rickets U SM Enlarged--...... epiphyses LE In ne Pseudofracture (osteomalacia) Pagetʼs Disease Musculoskeletal 675 https://t.me/usmleinnercircle Version 2 . . Laboratory testing Imaging Treatment Most patients are asymptomatic Bone pain & deformity • Skull: Headache, hearing loss Clinical features Pathogenesis Paget disease of bone . • Spine: Spinal stenosis, radiculopathy . . . . . . . . . Long bones: Bowing, fracture, arthritis of adjacent joints Giant cell tumor, osteosarcoma Osteoclast dysfunction Increased bone turnover Elevated alkaline phosphatase Elevated bone turnover markers (eg, PINP, urine hydroxyproline) Calcium & phosphorus are usually normal X-ray: Osteolytic or mixed lyticlsclerotic lesions Bone scan: Focal increase in uptake Bisphosphonates PIN P = procollagen type I N-terminal propeptide. Urine hydroxyproline reflects collagen breakdown 24-5: A, Radiograph of the humerus in Paget disease. Note the cortical thickening of the bone and the ragged appearing lytic areas throughout the bone matrix. B, Bone scan showing "hot spots" in Paget disease. (A from Katz D, Math K. Groskin S: Radiology Secrets, Philadelphia, Hanley & Belfus, 1998, p 311, Fig. 1; B from Bouloux P: Self-Assessment Picture Tests Medidne, Vol. 4, London, Mosby-Wolfe, 1997, p 82, Fig. 163.) Most common cause of an asymptomatic elevation of alkaline phosphatase in an elderly patient. Osteoporosis  What is the recommended screening protocol for osteoporosis? One-time dual-energy x-ray absorptiometry DEXA scan) for all women  65 also recommended for younger women who have an equivalent risk of osteoporotic fracture Musculoskeletal 676 https://t.me/usmleinnercircle Version 2 Osteoporosis risk factors Nonmodifiable Advanced age Post menopause Low body weight White or Asian ethnicity Malabsorption disorders Hypercortisolism, hyperthyroidism, hyperparathyroidism Inflammatory disorders (eg, rheumatoid arthritis) . . . . . . Smoking Excessive alcohol intake Sedentary lifestyle Medications (eg, glucocorticoids, anticonvulsants) Vitamin D deficiency, inadequate calcium intake Estrogen deficiency (eg, premature menopause, hysterectomy/oophorectomy) Chronic liver or renal disease le . . . . . . . . Modifiable  In patients with osteoporosis, the risk for fragility fracture is highest in those with a history of prior irc fragility fracture. ne DEXA is not done when you suspect fracture. rC • Osteoporosis is often asymptomatic but increases the risk for fractures, most commonly involving the hip, vertebrae, and distal radius. Osteopenia can limit the detection of occult fractures on x-rays, and when they are suspected, advanced imaging with MRI or CT scan should be pursued. Medications associated with osteoporotic fractures Possible mechanism Anticonvulsants that induce cytochrome P450 r Vitamin D catabolism In Medication (phenobarbital, phenytoin, carbamazepine) Aromatase inhibitors ! Estrogen LE Medroxyprogesterone GnRH agonists ! Testosterone & estrogen Proton pump inhibitors ! Calcium absorption U SM Glucocorticoids Musculoskeletal ! Bone formation Unfractionated heparin 677 https://t.me/usmleinnercircle Version 2 Clues to secondary causes of bone loss Malabsorption Hyperthyroidism Hypereortisolism Hyperparathyroidism Inflammatory disorder ( eg, rheumatoid arthritis) Hypogonadism Multiplemyeloma .. .. .. .. .. .. .. Secondarycauses of osteoporosis Diarrhea, weight loss ! 25-Hydroxyvitamin D, l urine calcium excretion Endocrine Weight loss, heat intolerance, tremor Goiter Central obesity/cushingoid habitus Metabolic/nutritional Hyperglycemia Hypercalcemia, hypercalciuria Gastrointestinal/hepatic Kidney stones Joint pain, morning stiffness Renal 1 ESR, CRP ~: amenorrhea, weight loss/anorexia Medications c]: j libido, erectile dysfunction, loss of body hair Anemia Hypercalcemia, T creatinine Other CRP = C-reactive protein; ESR = erythrocyte sedimentation rate. .. .. .. .. .. .. . .. .. Hyperthyroidism Hyperparathyroidism Hypercortisolism Hypogonadism Calcium &/or vitamin D deficiency Eating disorder Malabsorption (eg, celiac disease, Crohn disease) Chronic liver disease Chronic kidney disease Renal tubular acidosis Glucocorticoids Phenytoin, carbamazepine Proton pump inhibitors Inflammatory disorder (eg, rheumatoid arthritis) Multiple myeloma Alcohol use disorder Immobilization Prevention of glucocorticoid-induced osteoporosis .. .. . General measures High-risk patients . . Initial evaluation Lifestyle measures Antiresorptive therapy .. . . . .. . . Calcium & vitamin D supplementation Weight-bearing exercise Minimization of dose & duration of glucocorticoid therapy Periodic DXA scans Clinical features: 0 Estimated 10-year risk for major fracture ~20% 0 Medium-/high-doseglucocorticoids (eg, ~7.5 mg/day prednisone) 0 Preexisting osteoporosis (eg, prior osteoporotic fracture) Oral bisphosphonatesadvised Postmenopausal osteoporosis DXAscan Serum chemistry panel (ie, electrolytes, calcium, renal & hepatic markers) Complete blood count 25-Hydroxyvitamin D level measurement TSH in symptomatic patients Regular weight-bearing exercise & fall prevention Avoidance of tobacco & excessive alcohol intake Adequate dietary intake of calcium & vitamin D Supplemental vitamin D often needed to correct deficiencies Bisphosphonate (eg, alendronate, risedronate) • Alternate: zoledronic acid, denosumab Osteomyelitis  What is the next step in management for a patient with suspected vertebral osteomyelitis that has a significantly elevated ESR with normal spinal X-ray? MRI Musculoskeletal 678 https://t.me/usmleinnercircle Version 2 followed by CT-guided bone biopsy if positive Pathogenesis .. .. .. . .. . Vertebral Evaluation of vertebral osteomyelitis osteomyelitis Staphylococcus aureusmostcommon Fever,back pain & focal spinal tenderness Hematogenous seeding (eg, distant infection, IVDU, intravenous catheter) Contiguousspread from local infection Penetrating injury,invasive procedure/iatrogenic Blood cultures Subacute-chronic pain & midline spinal tenderness Complications Management Possible radiculopathy, LE weakness Elevated CRP/ESR MRI fESR/CRPbut normal x-rays le Diagnosis ESR/CRP Plain spinal x-rays +/- Fever Blood culture, CT-guided bone biopsy/culture • Abscess/epidural extension/neurologic deficits .. T • Vertebral collapse irc Clinical features Intravenousantibiotics Drainage of any associated abscesses CRP = C-reactive protein; ESR = erythrocyte sedimentation rate; IVDU = intravenous drug use; LE = lower extremity. rC CT-guided needle aspiration/biopsy ne Clinically suspected osteomyelitis Order labs ESRICRP,\.WlCs, In bloodcultures Onset of symptoms? < 2 weeks Patient MRI +-wnh metatimplant CT 1/ MRI contramdicated U SM Scmtigraphy LE i Nuclear study > 2 weeks i No findings typical of osteomyelitis X-ray No other imaging available T Ultrasound .. Typical findings of osteomyelitis Osteomyelitis Bone biopsy feasible? ..~---Yes--- Start empiric antibiotics -7 I Empiric antibiotics only Swnch to + Specific antibiotics culture results : _-- - - - -- - - - -- - - - - - Musculoskeletal Possible Surgery - --- - - -- - - --- - - -' 679 https://t.me/usmleinnercircle Version 2 MRI is the most sensitive diagnostic study in the work-up, but biopsy is the best confirmatory test  What is the empiric treatment for osteomyelitis in adults? IV vancomycin + antipseudomonals (cephalosporins or fluoroquinolones) Treat after bone biopsy is done!  What is the likely diagnosis in a patient with a recent UTI that presents with fever, back pain, focal tenderness over the L4L5 vertebrae, and paravertebral muscle spasm? Vertebral osteomyelitis most likely due to hematogenous spread of the UTI; initial workup includes CBC, blood cultures, ESR/CRP, and plain spinal X-rays A high index of suspicion for progenitor vertebral osteomyelitis should be present when examining patients with history of IV drug use or recent distant site infection (UTI). Fever and leukocytosis are unreliable findings! May be normal in vertebral osteomyelitis  What is the likely causative organism in a patient that develops osteomyelitis after stepping on a rusty nail? The nail pierced the patient's shoes and pierced the heel. Pseudomonas aeruginosa Staph aureus and Pseudomonas are respon

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