DELTA MEDICINE MCQ DISCUSSION - 1 ANAESTHESIA IS MEDICINE INSIDE THE OPERATION THEATRE Q. HYPOXEMIA with normal(A-a) gradient is seen with A. Hypoventilation B. V/Q mismatch C. Shunting D. ILD Ans. Hypoventilation Respiratory Failure is important topic. Respiratory failure Type 1 – Hypoxia (PaO2 < 60 mmHg) , <90% saturation Type 2 – Hypoxia + Hypercapnia, PaCO2↑↑ , Pa CO2 > 40mmHg Type 4- Hypoperfusion of respiratory muscles (seen in shock) Type 2 Respiratory Failure Hypoventilation ↓PaO2 , ↑PaCO2 Diffusion is Normal. Gradient – P(A-a)O2 -> Normal Ventilation + Diffusion issue = Type 3 Resp. Failure When a patient with ↓PaO2 , ↑PaCO2, Normal(A-a) = TYPE 2 ↓PaO2 , ↑PaCO2, ↑(A-a) =TYPE 3 Join for latest updates https://t.me/NNDOfficial 1 Issues w.r.t type 2 Respiratory failure can be at the level of Generator, Pump and Effector organ. Generator -> At CNS level ( Resp. centre only) Pump -> Chest Wall, NM system Effector organ -> Lung, Airways Type 1 Respiratory failure Hypoxemia Diffusion issue, (A-a) O2 ↑↑ When you have PaO2↓, PaCO2 Normal , (A-a)O2↑↑ = Type 1Resp. failure. If ↑PaCO2 is called as Type 3 Resp. failure. Most common Respiratory failure is Type 3. Diffusion occurs across the membrane, the membrane getting fibrosed. Hallmark cause of type 1 Respiratory failure is ILD. 3 IMP. TERMS 1. Shunt 2. V/Q Mismatch 3. Dead Space ventilation. All these come under type1 Respiratory failure. Join for latest updates https://t.me/NNDOfficial 2 V/Q mismatch – Ventilation Perfusion mismatch seen in ARDS, Pulmonary edema, Pneumonia, Lung Contusion, Atelectasis. Most common cause of type1 respiratory failure is diffusion defect. V/Q = heterogenesity / mismatch seen in ILD, Pulmonary oedema, Pneumonia, ARDS. ILD and ARDS are type 3 Respiratory failure defect. Bronchial asthma and Emphysema are Type 1 Respiratory failure defect. Shunt Physiology No ventilation V/Q = zero Intracardiac shunt Dead space ventilation V/Q= ∞ As Perfusion is zero, As seen in pulmonary embolism and severe COPD, there will be empty Bronchial airways. Join for latest updates https://t.me/NNDOfficial 3 For Anaesthetics Type 3 (Peri-operative) Respiratory Failure Residual anesthesia effects, post-operative pain, and abnormal abdominal mechanics contribute to decreasing FRC and progressive collapse of dependant lung units. Causes of post-operative atelectasis include; *Decreased FRC *Supine/obese/ ascites *Anesthesia *Upper abdominal incision *Airway secretions Join for latest updates https://t.me/NNDOfficial 4 DELTA MEDICINE MCQ DISCUSSION -2 Q. Match the Following 1.Lymphangioleiomyomatosis (LAM) 2. Burt-Hogg-Dube Syndrome 3. Pulomnary Langerhans cell Histiocytosis 4.Lymphoid Interstitial Pneumonia A. Bronchoalveolar lavage fluid CD1a + cells>5% B. Serum Vascular endothelial Growth Factor –D level>800 pg/ml C. Folliculin (FLCN) gene variants D. Sytemic rheumatic diseases Ans. 3-A, 1-B, 4-D, 2-C ILD is full blown, diffuse parenchymal lung ,fibrosis. Most common type of ILD is Idiopathic pulmonary fibrosis Idiopathic pulmonary fibrosis ----- > UIP 1) Usual interstitial pneumonia. 2) NSIP Subtype- LIP 3)COP( previously called as BOOP): Cryptogenic organizing pneumonia 4) Smoking associated ILD Desquamative interstitial pneumonia RB-ILD Respiratory Bronchiolitis LCH Langerhan cell Histiocytosis Nodules in ILD -> LCH -> Cavitating Nodules Join for latest updates https://t.me/NNDOfficial 5 Keywords for LCH Cavitating /Non-cavitating Nodules + Thin and Thick wall cysts + Young male + Upper lobe-ILD +Spontaneous Pneumothorax +Young male smoker. Microscopy -> Birbeck Granules with CD1a + Upper lobe ILD . UPPER LOBE ILD includes 1. CTDs – Ankylosing spondylitis, Sarcoidoisis, Berryliosis 2. Pulmonary LCH 3. ABPA (I+III HSR), HSP( III +IV HSR) 4. Silicosis, Coal worker Pneumoconiosis, Radiation induced Lung Disease. 5. TB Join for latest updates https://t.me/NNDOfficial 6 1. Identify Young female Tuberous sclerosis Extensive cyst formation and lung tissue destruction Pneumothorax and Chylous effusion Angiomyolipoma Everolimus (mTOR (-)) Pulmonary lymphangiomyomatosis(Rare) Ans. A young female -> Pneumothorax Haemoptysis Pneumothorax Chylous effusion CT appearance in Lymphangiomyomatosis (LAM). Cysts intervening with normal lung parenchyma, Cyst with lung destruction. Join for latest updates https://t.me/NNDOfficial 7 Prognostic Value Serum VEGF-D ↑ Bad prognosis 2. Burgg Hogg Dube Syndrome (rare) Characterized by lung thin cysts (predominantly lower zone) + fibrofollicular (pinna) + Follicular gene mutation. 3. Lymphoid interstitial Pneumonia – subtype of NSIP On HRCT, NSIP pattern in ILD – (a/w Drugs and connective tissue disease) 1) Ground glass appearance 2) Posterobasal Location 3) Septal thickening 4) Finer fibrosis Join for latest updates https://t.me/NNDOfficial 8 Subtype of NSIP – LIP (Lymphoid interstitial pneumonia) →Alveolo-interstitial infiltrates + thin walled cysts Diffuse lymphocytic infiltrates with granuloma. Subtype of NSIP Thin walled cysts -> LIP (Lymphoid interstitial pneumonia) seen in Classical of Sjogren’s Syndrome. Join for latest updates https://t.me/NNDOfficial 9 Sjogren HIV ( in salivary glands) Diffuse infiltrative Diffuse infiltrative lymphocytosis (DILS) due to CD4 lymphocytosis (DILS) due to CD8 infiltrates. infiltrating. HRCT findings in ILD : UIP Pattern Subpleural involvement Reticular involvement Bibasal involvement Cyst Destruction of Lung Parenchyma Honeycombing Traction Bronchietasis Absence of other findings, eg. Ground glass, nodules etc UIP pattern seen in RA. Cryptogenic Organizing Pneumonia (COP) pattern HRCT Findings in ILD : Organizing Pneumonia Consolidation + GGO -> Reverse Halo Sign Join for latest updates https://t.me/NNDOfficial 10 COP is mostly associated with Polymyositis / Dermatomyositis. Polymyositis associated with Anti Jo-1 Antibody (Antisynthetic antibody) seen in 1) ILD 2) Mechanic Hands 3) Reynold’s 4) Arthritis Anti T1F-1 γ and Anti NxT2 associated with Malignancy. Join for latest updates https://t.me/NNDOfficial 11 Delta – Medicine MCQ Discussion – III Development and Medicine Exploration Q.Identify the gene and disease A. Williams syndrome jag1 B. Alagille syndrome jag1 C. Williams syndrome jag2 D. Alagille syndrome notch2 Ans. Alagille syndrome jag 1 Join for latest updates https://t.me/NNDOfficial 12 Source of central key cell in cirrhosis? A. Dorsal bud B. Ventral bud C. Macrophages D. Septum transversum Ans. Septum Transversum Liver Development -> Gut Tube – Endodermal in origin At 3-4 weeks, Between foregut and Hindgut -> Ventral Bud Develops and also dorsal bud. Ventral bud develops by signal from cardiogenic mesoderm(FGF0 Signal from Septum transversum (BMP-7) Ventral bud develops into 1)Cranial portion -> Hepatic diverticula -> Hepatoblast 2) Caudal portion -> Superior part -> Biliary Tube Inferior part -> GB, Cystic duct and ventral pancreas. Dorsal bud -> gives rise to Dorsal pancreas. Bipotential Hepatoblast Gives rise to hepatocytes. Connective tissue framework of liver comes from Septum transversum. What are four structures contributes in the development of liver? 1) Hepatoblast – endodermal derivative – gives rise to hepatocytes 2) Septum transversum – mesoderm Join for latest updates https://t.me/NNDOfficial 13 Gives rise to connective tissue, capsule of the liver, Stellate cells. 3) Yolk sac- resident macrophage – Kupffer 4) Vitelline vein > Umbilical vein gives rise to sinusoids. Structural and functional unit -> Lobule -> lobe Histology:Portal tract – Bile duct, hepatic artery, portal duct. Central vein – central zone Periportal hepatocytes- Zone1 Midzonal hepatocytes – Zone 2 Centrilobular hepatocytes – Zone 3 70:30 or 60:40 70% Oxygen supply from portal vein. 30% Hepatic artery oxygen supply to liver. Hepatoblast converts to hepatocyte(require HNF-4α transcription factor) Or cholangiocyte converts to Bile duct epithelial cell Hepatocyte expresses cytokeratin 8 & 18 and AFP. Hepatoblast to become cholangiocyte, most factor is SOX-9. Jag 1 or NOTCH-2 mutation Jag1 > NOTCH-2 -> Cholestatic jaundice <6m It is typically classified by pulmonary stenosis and mental retardation -> Alagille syndrome. William’s Syndrome Chr. 7 deletion IUGR Supravalvular Aortic stenosis Hypercalcemia present Arterial narrowing Aortic Stenosis Alagille Syndrome Impairment in Jag1 Has Pulmonary stenosis Has butterfly vertebra. Hypercalcemia absent Pulmonary stenosis IUGR MR Ophthalmic Abnormality Posterior embryotoxoid Central key cell in cirrhosis -> Stellate cell/ Vit. A storage / lipocytes/ Ito cells – derivatives of septum transversum. Macrophages produce Kuffer cells. Q. Identify the bad prognostic marker in CLL. A. Sox3 B. Mutated igvh Join for latest updates https://t.me/NNDOfficial 14 C. Notch 1 D. beta 1 microglobulin Ans. NOTCH 1 CLL- Chronic leukocytic leukemia B- cells – Blood (Naïve B cells – not encountered any antigen) Monoclonal B- cell Lymphocytosis – Very few develop CLL Small blue lymphocytes proliferate in blood Lack of apoptosis of Naïve B cells. CLL is seen in elderly/ male predominant disease(<70y) Monoclonal B- cell LymphocytosisIncidental lymphocytosis CD5, CD23 +ve CD10, CD19, CD20, CD21, CD22 negative. Smudge cell seen in CLL. Rai stage and Binet stage. Rai IV – Lymphocytosis + thrombocytopenia PLT < 100x109/L Binet C – Hb<10g/dl or PLT < 100x109/L CLL adverse prognostic features:↑β2 microglobulin Unmutated IgVH CD38 expression ZAP-70 expression Deletion 17p11q P53 mutation NOTCH-1 H/P shows Smudge cells. Join for latest updates https://t.me/NNDOfficial 15 Treatment for CLL In previously untreated CLL patient check for TP53 status If TP53 is disrupted, start with ibrutinib. If TP53 is intact, we check Immunuglobin heavy chain (IGHV) mutation status. If Unmutated –IGHV , we give Ibrutinib or FCR(Fludrabine, cyclophosphamide and rituximab) If elderly patient not tolerating ibrutinib, we give Bendamixin Rituximab. If mutated IGHV and patient is not tolerant, give Ibimutuzumab or ibrutinib. Relapsed / Refractory CLL ↓ Restage with bone marrow aspirate and biopsy (exclude Richter Transformation) CT scan – CLL FISH panel and TP53 mutation study. Q Most common gene mutation in young Aortic Stenosis Ans. Notch 1 Degeneration + Calcification In Young ->Bicuspid Aortic Valve In old -> Degenerated ( sclerosed valve + calcification) Tricuspid Valve No association Bicuspid Aortic valve Associated with aortopathy (Always look for aortopathies) Investigation – CT aortogram. Q 76/M, prior smoker presents with recurrent episodes of angina and syncope. On evaluation, he is found to have a Hb of 5.4 . On detailed questioning, he gives history of episodes of melena interspersed between syncopal attacks, ongoing for the past 1 year. A colonoscopy is performed that shows characteristic vascular lesions. What’s the diagnosis? Join for latest updates https://t.me/NNDOfficial 16 Explain the pathophysiology behind it? Ans. Heydes Syndrome -> A.S + Angiodysplasia Aortic Stenosis Numbers 40/4/1 5/3/2 Severe A.S Mean Transaortic Pressure Gradient >40mmHg Angina – from it’s onset 5 years Syncope– from it’s onset 3 years Dyspnea– from it’s onset 2 years Risk for Sickle cell disease and sudden cardiac death Angina in aortic stenosis is due to LVH. Syncope –fixed cardiac output state. Dyspnea – LV failure In severe A.S, following are seen :1) Peak flow velocity > 4m/s 2) Surface area of aortic valve < 1cm2 3) Mean transaortic pressure gradient > 40mmHg. Angina – LVH (increases O2 demand) + reduced diastolic perfusion time + decreasing capillary density with recpect to wall thickness – atherosclerosis Syncope – mostly effort related due to fixed output state Dyspnoea - ↑sed LV filling pressure and fixed CO state. HEYDES Syndrome LV outflow obstruction with increased afterload. LV contractile performance maintained by concentric LVH. ↑ses transaortic valve pressure gradient. Diastolic dysfunction + LV filling pressure HIGH. Later, contractile function deteriorates CO/LV aortic pressure gradient is lost. LV failure :Low flow. Low gradual AS. Treatment-Dobutamine stress – ECHO Join for latest updates https://t.me/NNDOfficial 17 Biscuspid aortic Valve Most common congenital valve defect M>F, AD, NOTCH1, Enos defect Medial degeneration with ascending aortic aneurysm / dissection. Physical Findings:Pulse -> Severe AS -> pulsus parvus et tardus. BP -> Narrow pulse pressure. JVP -> normal Apex -> Hearing Apex Heart Sounds S1 normal, aortic ejection click maybe heard. Soft S2 / Single heart sound … severe AS has paradoxically split S2. S3 ONLY in very severe AS. S4 Into Non- dilated, non- compliant -> Hypertrophic AS Healthy atria across normal. Gallavardin phenomenon – HIGH pitch murmur radiating to Apex. Treatment of A.S -> Symptomatic Aortic Valve Replacement , asymptomatic, severe < 50% - EF. NOTCH 3 Migraine with Aura. Age- 20-40 years Lacunar syndrome – 40-50 1) Migraine with aura. 2) Subcortical ischaemic events. 3) Mood disturbances 4) Apathy 5) Cognitive impairment – Dementia CADASIL -> Arteriopathy with stroke with ischaemic leukoencephalopathy. Vascular Dementia -> CVA 1. Dementia Join for latest updates https://t.me/NNDOfficial 18 2. Cerebrovascular disease defines by one of : H/o of cerebrovascular disease. Focal signs on examination (with or without H/o) CT/MRI showing lesions that confirm CVA. 3. Temporal relationship between 1 and 2 demonstrated by one of : Onset of dementia within 3 months following stroke. Abrupt deterioration in cognitive functions. Fluctuating, stepwise progression of cognitive deficits. NOTCH 1- CLL( Biscuspid Valve) NOTCH 2- ALAGILLE NOTCH 3- CADASIL Microbleeds on Brain MRI seen in cerebral amyloid angiobleed. NOTCH pathway in SARS-COV2 (COVID-19) Shared pathogenic pathways. Resulted cardiovascular complications. IL-6 -> Tocilizumab Block. NOTCH 4 pathway. Viral entry Hemodynamic instabilities Inflammatory response Dysthymia Hypoxic response Thromboembolic events Coagulopathy Myocardial injury Endothelial damage Myocarditis Heart failure Cardiac arrest Image Identify Join for latest updates https://t.me/NNDOfficial 19 A. Deny Drasch syndrome. B. WAGR C. Beckwith Wiedman Syndrome D. Frasier syndrome Development Of Kidney Intermesoderm 1) Nephrogenic cord (urogenital ridge) – from metanephric blastema (excreting part) 2) Mesonephric duct – 5th week ureteric bud and collecting part formed. 3) Paramesophric duct 4) Gonads Pronephros – Day22 Mesonephros – 25-26days to 4 months Other structure from mesonephric duct – Trigone, urinary Bladder, Prostratic utricle, epoophoron, paroophoron, Join for latest updates https://t.me/NNDOfficial 20 gardner’s duct, seminiferous tubules, vas deferens, ejaculatory duct. 6th and 8th Cranial nerve : Epithelial mesenchymal interaction Ureteric bud and Mesenchyme of metanephric blastema. End of 8th week – Nephron 9-10th week – urine formation starts 32-36week - Nephrogenesis is complete. WT1 gene on chr. 11p WT1 gene mutation 1) WAGR- Wilm’s tumour,Aniridia, Genitourinary Abnormality, Mental retardation. 2) Deny Drasch Syndrome a) Male pseudohermaphroditism b) Wilm’s Tumour (B/L) c) Early onset renal failure. Denys Drash Syndrome Genitalia : 46, XY Features: Gonadal dysgensis, External ambiguous genitalia (without uterus) Risk of Wilm’s Tumour ↑↑ Diffuse mesangial sclerosis (0-3yrs) Frasier Syndrome Genitalia : 46, XY Features : Gonandal dysgenesis Normal external genitalia Risk of Wilm’s tumour ↑, Gonadoblastoma FSGS (10-20yrs) Beckwith Wiedman syndrome – WT2 – genomic imprinting WT2 has nothing to do with Epithelial mesenchymal interaction. Beckwith Wiedman Syndrome 1) Microcephaly 2) Organomegaly 3) Omphalocoele / umbilical hernia 4) Ear crease 5) Hemihypertrophy testes. Join for latest updates https://t.me/NNDOfficial 21 DELTA MEDICINE MCQ DISCUSSION - 4 Fact based update--Q. Which among the following CTD can produce a large vessel vasculitic involvement A. Sarcoidosis B. SLE C. RA D. IgG4 related disease Vasculitis depends on the size of the predominant vessel involved. Small vessels includes arteriole, venule, capillary vein, small intraparenchymal vessels. Large vessels includes Aorta and branches. Join for latest updates https://t.me/NNDOfficial 22 Large Vessel Vasculitis 1) Temporal arteritisRenal, coronary and pulmonary are not involved. The symptoms include headache and polymyalgia rheumatic. 2) Takayasu’s arteritis :Vessels involved are renal, pulmonary and coronary. Upper limb Claudication. Both these conditions have no predilection for strokes. Medium Vessel Vasculitis 1) Kawasaki Disease 2) PAN Small vessel Vasculitis ANCA Associated 1) GPA (Granulomatous with polyangitis) Or Wegener’s 2) EGPA (eosinophillic granulomatosis with polyangitis) 3) AGPA (Allergic granulomatosis with polyangitis) 4) Microscopic polyangitis Immune complex mediated 1) IgA vasculitis (HSP) 2) Cryoglobulinemia vasculitis 3) Anti GBM disease 4) Hypocomplementeric urticarial vasculitis (HUV) –Happens in Interface dermatitis antiC1q seen. Variable vessel Vasculitis 1) Bechet’s syndrome 2) Cogan Single organ Vasculitis 1) Cutaneous leukocytoclastic angitis 2) Cutaneous arteritis 3) Primary central nervous system vasculitis 4) Aortitis Dangerous form of vasculitis associated with mononucleiosis, ulcers and gangrene seen in medium vessels. Join for latest updates https://t.me/NNDOfficial 23 20 Vasculitis 1) CTD producing vasculitis Sarcoid, SLE, RA, IgG4 All of them produce Small vessel vasculitis (IC mediated) 2) Infections producing vasculitis Syphilis-Aortitis, HBV-PAN, HCV-Cryoglobinuria 3) Paraneoplastic vasculitis Lymphomas(produce immune complex mediated type) 4) Drugs producing vasculitis RA vasculitis is only seen in males and it generally respond to rituximab. IgG4 produces 2o aortitis. 30/1 30% patients with PAN are Hepatitis B+ve BUT only 1% patients with Hepatitis B have PAN. 90% patients with Cryoglobinuria have HCV. Purpura + Anthralgia/myalgia + ulcers -> Cryoglobinuria Drugs producing ANCA vasculitis is Hydralazine, Propiothiouracil, Levamisole adulterated with cocaine. Secondary vasculitis • Large ---- TB, syphilis, Ankylosing spondylitis. • which Medium vasculitis-?? BEHAVES LIKE PAN Ans. HBV • Small ANCA - Drugs?? Hydralazine, PTU • Small Immune complex vasculitis resembles like HCV ?? Ans. Cryo, RA/SLE/Sarcoid Paraneoplastic What is Common in these drugs given below ?? Hydralazine PTU Cocaine adulterated, Levamisole Minocycline GPS Ans. They’re p-ANCA +ve 50%, Myeloperoxidase +ve, 70% Join for latest updates https://t.me/NNDOfficial 24 Renal limited vasculitis HPA (50%) GPA(25%) EGPA(40%) p-ANCA positive can be divided into target antigen peroxidase and target Ag Non peroxidase. Common factor of the following is? Autoimmune hepatitis Primary sclerosing cholangitis RA IBD IE Cystic fibrosis Ans. P-ANCA +ve but target Ag is not Myeloperoxidase. KAWASAKI DISEASE Q. Kawasaki disease---are you astute?? A. Mimic B. Fleeting signs C. No LAB D. NO guidelines E. Devastating complications Ans. C & D KAWASAKI DISEASE • 80% ARE CHILDREN <5 YRS...boys > girls, Not seen in Adults. • Anti endothelial antibodies 10 28 56 10 % are febrile , 28% are vasculitis, 56% convulsions • VASCULITIS AFFECTING CORONARY ARTERIES(10 days to 4 weeks crucial) • UNREMITTING FEVER LASTING FOR > 5 DAYS-median 11d Join for latest updates https://t.me/NNDOfficial 25 • Most commonest manifestation in Kawasaki is myocarditis characterized by tachycardia, hyperdynamic precordium, S3, hypotension. Time is gold for Kawasaki disease. After 10 days ->25/2 2% risk of coronary events if treated, 25% risk coronary events if untreated. MUCOCUTANEOUS LYMPH NODE SYNDROME • B/L non exudative BULBAR CONJUNCTIVAL CONGESTION appearing shortly after fever(>90) • RASH...truncal polymorphous-mostly erythematous maculopapular or urticarial • Anterior CERVICAL NON SUPPURATIVE U/L LN>1.5cm(least common) • B/L symmetrical painful swelling of hands and feet with erythema of palms and sole-periungual desquamation by end of 2weeks - Red swollen cracked lip+ strawberry tongue+erythematous oropharyngeal mucosa • Entirely clinical diagnosis • Rash is never vesical/anterior cervical/no eye discharge • Perianal desquamation early/periungual desquamation Late • Cardiac manifestations around 1 week to 10 days • Tachycardia/gallop/hyperdynamic precordium • MR due to valvulitis. • Myocardial ischaemia may occur as a long term complication • THROMBOCYTOSIS AFTER 1 WEEK • ACUTE STAGE- IVIg+aspirin....single dose of 2g/kg over 10hrs preferably inside 10days • Aspirin 30-50mg/kg tapered to 3-5mg/kg and stop after repeat echo at 6-8weeks • TREATMENT WITH IVIG IN ACUTE PHASE REDUCES THE Join for latest updates https://t.me/NNDOfficial 26 RISK FOR CORONARY EVENTS FROM 25% To 2%. • Continue aspirin if there are coronary lesions • Most of them normalize by 1yr • Still arterial wall structure??? Long term data lacking Topic of Focus – IgG4 related diseases Join for latest updates https://t.me/NNDOfficial 27 Storiform fibrosis with characteristic swirling pattern. They are called tumefacetion agents they have ability to form fibrosis. Obliterative phlebitis + Eosinophillia Common in all these histology is lymphoplasmacystic infiltrates Join for latest updates https://t.me/NNDOfficial 28 IgG4---KING MAKER • Non inflammatory antigen sink • Number of cells/hpf—no much value especially in M7F Subacute Fibrosis…..IgG4/IgG ratio • Serum levels of IgG4-maybe normal in 40% / disease activity 1) It is a disease of males (M>F) >60 yrs , subacute 2) When you ever hear terms like fever, arthritis and necrotizing vasculitis, it never forms IgG4 vasculitis. 3) Lymphadenopathy undergo fibrosis ( decreased, no of IgG4) IgG4/IgG value comes down. 4)Serum IgG4 – High only in 40% Keywords Pachymeningitis + Brain parenchymal involvement -> bechet’s Disease Complains of inflammatory demyelination of CNS + sarcoid -> Mitral stenosis. Sarcoidoisis CNS Manifestations: Pachymeningitis, Basal meningeal enhancement Sarcoid granulomatosis producing stalk effect. Bilateral LMN with VIIIth Nerve Palsy IgG4 No brain parenchymal 1) Lymphocytic Hypophysitis 2) Pachymeningitis Sjogren No meningeal No brain parenchymal Ocular manifestations: Uveitis (Posterior uveitis), retinitis Risk factor for CNS ↑↑↑ swelling Submandibular > parotid> Lacrimal > orbital Thyroid minimal. Dry eye / Sicca syndrome (++) Less salivary gland swelling. Not steroid responsive. Join for latest updates https://t.me/NNDOfficial 29 Sarcoidoisis Lung involvement: Thickening of Bronchovascular Bundle, Minimal risk of NSIP. Involves upper lobe. Cardiac manifestations: Dilated cardiomyopathy, AV BLOCKs rarely pericarditis. Liver- Intrahepatic cholestasis Renal involvement: AKI (Hypercalcemiamechanism nephrogenic DI),CKD (chronic tubulointerstitial disease) Glomerular manifestation : Membranous nephropathy Arthritis Present IgG4 Thickening of Bronchovascular Bundle, Minimal risk of NSIP Sjogren NSIP and lymphoid interstitial pneumonia. Aortitis and paravertebral mass. Rarely, pericarditis. 1) PSC (primary sclerosing cholangitis) 2) Type 1 autoimmune pancreatitis 3) Retroperitoneal fibrosis Tubulo interstitial HCV, Primary biliary cirrhosis(PBC) Membranous nephropathy, C3, C4 MPGN rare. Absent Arthritis Present Tubulo interstial , Most common d- dd-RTA , rare to CKD. Common findings in all three Multiorgan immune mediated condition resembling inflammation or tumor Common binding- histology M>F 60-70 yrs MOST COMMON Autoimmune Hepatitis Submandibular sialadenitis Orbital/ periorbital lesions Join for latest updates https://t.me/NNDOfficial 30 Lymph Nodes Retroperitoneal fibrosis Chronic sinusitis and nasal obstruction with eosinophilia Riedels thyroiditis Lymphadenopathy – but biopsy difficult to establish Diagnosis IgG4 related aortitis spares the proximal vessels. Thickening of Bronchovascular Bundle / NSIP / Paravertebral mass TIN >>> Membranous IgG4 Autoimmune pancreatitis Most common Mild Abd symptoms initially but present as obstructive jaundice Type 3c DM Exocrine insufficiency Diffuse irregular NARROWING of the pancreatic duct on ERCP IgG4 sclerosing cholangitis Q. A 70 year male found to have a new onset DM. How will you manage? 1) Start OHA 2) Rule out malignancy 3)ERCP 4) USG abdomen Ans. 2) Rule out malignancy is the first thing to check, then take a CT. If CT findings shows something like sausage shaped pancreas , then we look into ERCP. Join for latest updates https://t.me/NNDOfficial 31 Treatment of IgG4 Steroids are the first line. Excellent response inside 2 weeks Steroid sparing agents ---- RITUXIMAB Join for latest updates https://t.me/NNDOfficial 32 DELTA MEDICINE MCQ DISCUSSION - 5 Clinical question Q. A 67yr old male a k/c/o NASH with no evidence of bleeding or splenomegaly or ascites presents for routine checkup Fibroscan score is 20kpa ALT 90U/L AST 77U/L WT-98Kg Urea 77mg/dl s.creat-1.6mg/dl No edema Urea-alb2+ no rbcs 1-2 epi cells On 40mg Lasix metformin and cilnidipine What is the most important cause of renal failure? A. Secondary IgA nephropathy B. Hepatorenal syndrome C. Secondary FSGS D. ATIN( acute tubulointerstitial nephritis) Explanation:Fatty Liver -80% patients have isolated fatty liver 20% NASH group – can lead to cirrhosis. This cirrhosis can lead to decompensated state and HCC. We can look into lover enzymes AST and ALT , upto 5 times the upper limit of normal. ALT > AST is normal AST> ALT , highly suggestive of cirrhosis. There is a patient with cirrhosis and NASH is the cause of it. Decompensation sign in this case for cirrhosis is ascites, There is no evidence of portal hypertension in this patient Diagnosis :- Cirrhosis, NASH, not decompensated renal failure, USG not given, Proteinuria, no hematuria. On diuretics. Join for latest updates https://t.me/NNDOfficial 33 Hepatorenal syndrome occurs in a patient with cirrhosis and portal hypertension. Hepatorenal syndrome occurs in a patient who has decompensated cirrhosis who will mandatorily have ascites. Cirrhotic patients have Portal HTN , cirrhosis related cardiomyopathy and bacterial translocation. These bacterial translocation activate innate immune system. In portal HTN , nitric oxide is being released, no splanchnic vasodilation, reduce cardiac output will result in effective hypovolemia. This hypovolemia, means that the intravascular volume is low, RAAS, Sympathetic system, AVP, ADH will be stimulated . Splanchnic vasodilation + intense intrarenal vasoconstriction. Join for latest updates https://t.me/NNDOfficial 34 GFR falls. Pre renal AKI . What is required for AKI? 1)Serum creatinine >= 0.3 mg/dl in 48 hours. 2)USG should be normal. No RBCs and proteins in urine. 3)Cirrhosis with ascites 4)Hypoalbunemia Replace albumin – no effect 5) Remove Diuretics Rule out bleeding. Rule out any pre renal manifestations. Then , we can diagnosis will be HRS. Hepatorenal Syndrome. HRS- NAKI Creatinine rise <50% in 3 months Cirrhosis with ascites. Presents with refractory ascites. Join for latest updates https://t.me/NNDOfficial 35 Treatment for HRS Mostly Transplant, make the patient fit for transplant Give Vasoconstrictors 1) Terlipressin 2) Noradrenaline 3) Midodrine oral + subcutaneous octreotide. We ruled out HRS (Hepatorenal Syndrome) Other option : ATIN Acute tubulointerstitial nephritis Cause could be due to drug induced (aminoglycosides, furosemide), infection and contrast. Patient with cirrhosis and portal HTN can have ATIN. Tubuloepithelial cast can be ATIN. Secondary IgA nephropathy is possibility in secondary cirrhosis, patient can develop IgA nephropathy It’s mentioned no RBCs Invariably have microhematuria, Hematuria and proteinuria. Secondary FSGS presents with nephrotic proteinuria without any edema, secondary to one cause (diseased kidney you remove, hyperinfiltrative injury, obesity) Ans. C. Secondary FSGS Spotter Question Large grade 3 or tense ascites or Refract??? Join for latest updates https://t.me/NNDOfficial 36 Grading of Ascites Grade 1 – mild ascites , no treatment Grade 2- Moderate ascites, symmetrical distention of the abdomen , treatment is restriction of sodium intake about (80120mmol/day) and diuretics. Grade 3- Large ascites with marked abdominal distension. Treatment is paracentesis. Ascites is a part of portal HTN, splanchnic vasodilation, hypovolemia, RAAS activation, sympathetic activation and plasma volume expansion. It is a crucial cycle. Salt and water reabsorption is the reason for ascites. The diuretic treatment in ascites should be always initiated with administration of spironolactone. Inside RAAS , aldosterone is activated , spironolactone counteract s the effects of the high circulating levels of aldosterone. That leads to increased reabsorption of sodium in the distal tubules of kidneys (i.e P cells) In ascites we start with spironolactone or eplerenone Join for latest updates https://t.me/NNDOfficial 37 Dose we start with is 100 mg/day then 100mg/week and maximum dose is 400mg. Within 3-5 days of treatment we will be actually be able to see , effects of spironolactone. Refractory ascites Refractory ascites is basically divided into diuretic resistant Ascites and diuretic intractable Ascites. Diuretic intractable Ascites means patient has diuretic related complications (example : patient has some cardiac arrythmias , renal failure and we are unable to use diuretics) Diuretic resistance ascites is that even after maximum dose of 400 mg for minimum period of week, still there is ascites. We are looking for 1kg loss over 3-4 days. Ans. Large volume paracentesis, we remove 6-8 litres of volume, we replace 8gm/l of albumin. If this is not working, we go for TIPSS. Join for latest updates https://t.me/NNDOfficial 38 TIPSS associated encephalopathy is also a problem and TIPSS cost is expensive. Join for latest updates https://t.me/NNDOfficial 39 DELTA MEDICINE MCQ DISCUSSION -6 Topics covered - Inflammatory demyelinating diseases Multiple sclerosis – v. Imp for Neet pg Neuromyelitis disorder INFLAMMATORY DEMYELINATION --MS Q. Myelin basic protein is attacked by A. Autoreactive T cells B. Antibodies produced by B cells C. Macrophages D. All the above Ans. Autoreactive T cells Lots of queries??? • Autoimmune or not?? Age?? Autoimmune, It happens in 20-40year age group , F>M , 3:1 • Demyelination or Dysmyelination?? Dysmyelination is a problem with myelin formation, demyelination is secondary attack on myelin. Only demyelination in Multiple sclerosis. • T cells are activated but how is BBB disrupted? It is T cell mediated disease, T>B cell. These genetic and environmental factors lead to Major point that is T cell activation. Disruption of blood brain barrier is caused by autoreactive T cells which cause activation of microglia which will produce IFN-1beta, it acts on astrocytes which produces VEGF-A which destroys the Blood brain barrier. ……produces gliosis Astrocyte proliferation produces gliosis. Gliosis leads on to loss of axons and neurons secondarily. ….. myelin and antibody is often seen against….. Myelin is produced by the oligodendrocytes against Myelin oligodendrocyte glycoprotein. Oligodendrocytes are seen in periphery of active plaques. Multiple sclerosis is chronic inflammatory demyelinating disorder of CNS. Most of the times, remitting relapsing type (90%) Join for latest updates https://t.me/NNDOfficial 40 HLA DR-2 and HLA DRB1 15 are important factors. Multiple sclerosis, Narcolepsy and Good Pasture’s disease have HLA DR2. Environmental factor – Vit D deficiency and EBV infection. What are other inflammatory demyelinating disorders of CNS ? Neuromyelitis optica spectrum (NMO) Anti MOG antibody (anti myelin oligodendrocytic glycoprotein disease) ADEM (Acute disseminative encephalomyelitis) Sarcoid Graft Versus Host disease What are non inflammatory demyelination disorders of CNS? Osmotic demyelination syndrome Central pontine myelinosis (due to fast sodium condition) Saltatory conduction occurs at the rate of 70mm/s from one node of ranvier to another node of ranvier. Sodium channels at the node of ranvier when you have loss of this myelin sheath, Potassium hyperpolarization , no conduction, then Loss of myelin sheath , conduction velocity is reduced to 1mm/s. Join for latest updates https://t.me/NNDOfficial 41 MULTIPLE SCLEROSIS Demyelinating disorder of CNS which spares PNS and other systems. Inflammation demyelination, gliosis and neuronal loss Perivenular cuffing by T CELL infiltrate which also affect white matter disruption of BBB,DEMYELINATION AND LOSS OF SALTATORY CONDUCTION Mbp and myelin oligodendrocyte glycoprotien are target antigens Eventually axonal destruction and cortical atrophy Only drug effective in primary progressive MS is Ocrelizumab Symptoms Sensory---large fibre v/s small fibre?? Sensory mostly predominant, large fibres symptom like numbness is mostly seen. small fibre symptom like burning pain is seen. Band like symptoms. Weakness with spasticity. UMN like symptoms with spasticity. ON (optic neuritis)-unilateral anterior recovering ON is Unilateral >> Bilateral asymmetric involvement. Join for latest updates https://t.me/NNDOfficial 42 ….. precedes visual loss Painful eye movement precedes visual loss. Involves anterior part, optic nerve is involved may get RAPD whereas optic chiasma, optic tract is involved in neuromyelitis optica. 90% patients have recovery. Uveitis/retinitis ??? There is no associated uveitis, retinitis or hearing loss. Other symptoms in Multiple sclerosis includes: Bladder dysfunction in more than 90% :-detrusor hyperreflexia, detrusor sphincter dyssynergia. Lhermittes Uhtoffs Pulfrich – Paroxysmal symptoms B/L INO, B/L trigeminal neuralgia Fatigue and depression SNHL/ulcer/ arthritis/ hypothalamic/ encephalopathy /meningismus. T2 hyperintensities in .. • Periventricular white matter: quite common • Corpus callosum ( Dawson's fingers): very typical Juxtacorical • Brainstem: esp. adjacent to CSF space • Cerebellum • Spinal cord-one segment will be involved. Join for latest updates https://t.me/NNDOfficial 43 Normal pressure, clear appearance. Cells: normal in 2/3 Protein: IgG increased in >90% OCB (oligoclonal bands) in 85-95% cases. IEF and immunofixation (IgG) - Gold standard for Dx. Oligoclonal bands need to be unique in CSF. NEUROMYELITIS OPTICA Acute attacks of optic neuritis and myelitis No correlation between optic neuritis and myelitis 40% have associated autoimmune disorder Autoantibody against aquaporin4 Disabling disease with normal MRI Steroids / plasma exchange. Join for latest updates https://t.me/NNDOfficial 44 Neuromyelitis optica is more than Tcell is B cell mediated disease, there an antibody called anti-aquaporin4 which is seen in around 80% of patients. These patients presents with area postrema syndrome typically characterized by hiccups and nausea, vomiting. B/L optic neuritis posterior severe. Severe longitudinally extensive Transverse myelitis. They can also have acute brainstem syndrome or narcolepsy. Non progressive beyond a month. Join for latest updates https://t.me/NNDOfficial 45 Keywords Case Scenario 1:- 20-40 year female, sensory motor bladder with unilateral optic neuritis Diagnosis :- multiple sclerosis Case scenario 2 : 40-60year female who is already having SLE, mostly think of NMO, patient will come with area postrema syndrome with intractable hiccup, vomiting etc. + Bilateral optic neuritis and recurrent transverse neuritis. Long segment longitudinal transverse myelitis. Case scenario 3: Young child post infection or immunization , think of ADEM, patient will be having encephalopathy + bilateral optic neuritis. Q. DRUG presently not favoured in MS due to cardiotoxicity is A. Teriflunomide B. Fingolimod C. Mitoxantrone D. Dmf Join for latest updates https://t.me/NNDOfficial 46 Ans. C Mitoxantrone Conventionally used drugs in multiple sclerosis , these included injectable drugs like Interferon beta and glatiramer acetate. In acute attacks, I.V steroids were used. Infusable drugs included monoclonal antibodies like natalizumab directed against alpha 4 integrin. Ocrelizumab which is the humanized monoclonal antibody against CD 20 Oral drugs include 1)Fingolimod 2) Dimethyl fimorate (DMF) 3)teriflunomide In Natalizumab treatment check for PMLE produced by JC virus, if antibody is present, Natalizumab is contraindicated. Ocrelizumab is the only drug approved for primary progressive MS. In ADEM, we treat the patients with steroids and plasma exchange + Rituximab. Maintenance treatment give Azathioprine, mycophenolate mofetil etc. Q. Whats the treatment of choice for an acute episode of Cluster headache A. 100% O2 at 6l/mt B. Oral sumatriptan C. S/c sumatriptan D. Iv dexamethasone Ans C. S/c sumatriptan CLUSTER HEADACHE(SUICIDE Headache)….5 Attacks for diagnosis Young males Unilateral periorbital headache very intense and non throbbing 15mts-3hrs(aggressive during attacks) 8-10 weeks of symptoms followed by remission 20% chronic Autonomic features PHOTOPHOBIA present……. Always unilateral Nocturnal Alcohol ACUTE ATTACK-100% 02 for 10-15mins at 10 l/mins or s/c SUMATRIPTAN Join for latest updates https://t.me/NNDOfficial 47 Steroids for short term prevention……..verapamil/LITHIUM TRIGEMINAL AUTONOMIC CEPHALGIAS (TAC) Cluster headache Paroxysmal hemicrania Sunct (Short lasting unilateral neuralgic headache with conjunctival congestion) Hemicrania continua SHORT LASTING ATTACKS OF unilateral severe HEADACHE WITH ipsilateral CRANIAL AUTONOMIC SYMPTOMS Restlesness during attacks Q. In TAC, at least one of the following symptoms or signs, ipsilateral to the headache: A. Conjunctival injection and/or lacrimation B. Nasal congestion and/or rhinorrhoea C. Eyelid oedema D. Forehead and facial sweating. E. Forehead and facial flushing F. Sensation of fullness in the ear G. Miosis and/or ptosis A, B, C are common symptoms. Cluster Headache is TAC, disease of young males, severe excruciating pain or stabbing pain by the patient + orbital pain. 1-8 attacks / day each attack can last for about, 15min-3 hours duration. Join for latest updates https://t.me/NNDOfficial 48 Autonomic features. Alcohol can act as triggers. All TACs have Migrainous features. No cutaneous triggers. Gender – M>F 3:1 Type – Stabbing, boring Severity - Excruciating Site : orbit, temple Attack frequency: 1/alternate day-8/d Duration of attack: 15-180 min Autonomic features: Yes Migrainous features: Yes; Nausea, photophobia, or phonophobia; photophobia and phonophobia are typically unilateral on the side of the pain Alcohol trigger - Yes Cutaneous triggers - No Paroxysmal hemicrania Seen equally in males and females. (M=F) Throbbing or stabbing pain. 1-20 attacks / min, short lasting duration about 2-30 mins. Orbital pain, ANS features seen, alcohol trigger not seen. Most effective drug is Indomethacin. PAROXYSMAL HEMICRANIA UNILATERAL M=F middle aged Very severe/boring/stabbing Periodicity is less striking no nocturnal preponderence SHORT LASTING ATTACKS more in number Join for latest updates https://t.me/NNDOfficial 49 Autonomic+++ Rapid response to indomethacin Short lasting Neuralgic Headache with conjunctival congestion and tearing -(SUNCT) Attack frequency is 3-200per day with 5-240seconds duration. Cutaneous triggers seen in SUNCT. SUNCT Severe unilateral orbital or temporal pain which is stabbing or throbbing 3-200 attacks/day Cutaneous triggers Lack of refractory period to triggers Acute atacks too short lived Lamotrigine for prevention Identify?? Elderly females Continous nature of unilateral headache with migrainous features Autonomic++ Responsive to indomethacin Ans. Hemicrania continua All patients with a suspected TAC, should have a MRI Brain done to rule out secondary pathology. Join for latest updates https://t.me/NNDOfficial 50 DELTA MEDICINE MCQ DISCUSSION - 7 Stay updated---PAH Q. Which among the following is not considered as a treatment option for PAH A. Trepostinil B. Nifedipine retard 20mg C. Ambrisentan D. Tadalafil Ans. Nifedipine retard 20mg Treatment Sildenafil or Tadafil-PDE5 inhibitors Bosentan 62.5mg twice a day orally BEST option. Combination therapy always superior Atrial septostomy Join for latest updates https://t.me/NNDOfficial 51 Pulmonary HYPERTENSION Whose domain??? Idiopathic pulmonary HTN is a devastating disease--- Idiopathic pulomonary HTN - only palliation and median survival of 9 months. WHO divides pulmonary hypertension into class 1,2,3,4,5 Class 1 – idiopathic pulmonary HTN (iph) Pulmonary artery hypertension • Abnormal elevation in Mean Pulmonary arterial pressure > 25mmHg • RV hypertrophy or dilatation secondary to PAH is called Cor pulmonale • BMRP2 (Bone maturation receptor protein -2) Group 1 Pulmonary arterial hypertension A. Idiopathic pulmonary arterial hypertension B. Heritable----bmpr2 C. DRUG and TOXIN D. Autoimmune??? E. HIV, schistosomiasis Rule out causes for PAH Autoimmune Infections Drugs and toxins are Join for latest updates https://t.me/NNDOfficial 52 Toxic rapeseed oil have been asked in exams, multiple times. Autoimmune conditions include limited scleroderma. Infections include HIV and schistosomiasis. Class 2- pulmonary venous hypertension which is basically a left heart tissue Class 1 is differentiated from Class 2 by Woodunits. PVR Pulmonary vascular Resistance = Right sided pressure left sided pressure / Cardiac output Class 3 related to chronic hypoxemia , mostly related to lung disease. Class 4 – chronic thromboembolic pulmonary hypertension Join for latest updates https://t.me/NNDOfficial 53 Pulmonary endarterectomy is most successful when done for--Class 4 NYHA 4 - Despite being on optimum drugs for 3 or more months – warrants Lung transplant. A combination of vasoconstriction + remodeling + thrombosis, Decreased Nitric oxide, decreased prostacycline and increased. Join for latest updates https://t.me/NNDOfficial 54 Right heart failure with pulmonary HTN A waves in JVP Palpable epigastric impulse/left parasternal heave Loud P2/ loud P2 with wide splitting of S2 RVS3 due to increased diastolic filling pressure Ascites/pedal edema/TR/apex shift/hepatomegaly Join for latest updates https://t.me/NNDOfficial 55 Gold standard is right heart catheterization. Murmurs in PAH • TR ---high pitched holosystolic blowing murmur along left sternal border increased on Inspiration (caravallo sign) • PS-ESM • Severe PAH - high pitched EDM early diastolic murmur due to PA dialatation best heard in sitting up,leaning forward and fixed exp(Graham steel murmur) PFT – FeV1, FVC normal, DLCO reduced Polysomnography- any pulmonary artery HTN is due to OSAS ECHO-RV function and TR to look for. V/Q scan in patients to rule out chronic thromboembolic PAH HRCT to rule out ILD. Join for latest updates https://t.me/NNDOfficial 56 TREATMENT— Supportive PREGNANCY MUST BE AVOIDED Oral anticoagulants LTOT Diuretics for RHF 240mg nifedipine if vasoreactive test is positive For CLASS 2 Treatment Sildenafil or Tadafil - PDE5 inhibitors Bosentan 62.5mg twice a day orally -BEST Combination therapy always superior Join for latest updates https://t.me/NNDOfficial 57 Atrial septostomy Epoprostenol via syringe pump onto permanently tunneled Catheter Trepostinil As continuous subcutaneous infusion via Microinfusion pump Beraprost is the first chemically stable and orally active prostacyclin derivative Clinical question 2 Q. A 50 yr old male develops cardiogenic shock following acute MI. His urea and creatinine begin to rise, urine analysis is normal , within a week RFT improves. Diagnosis A. CRS type 1 B. CRS type 2 C. ATN D. RPGN Ans. CRS type 1 CRS – Cardiorenal Syndrome CRS Type 1 – acute type , MI producing syndrome CRS type 2 – heart failure or cardiomyopathy, chronic disease basically results in worsening of underlying CKD. Type 3 CRS – acute renocardiac with a patient of AKI – volume overload, malabsorption disease, hyperkalemia, uremia Type 4 is CKD involved in chronic cardiac involvement. Type 5 secondary CRS involving systemic processes like cirrhosis, amyloid, sepsis, diabetes. Join for latest updates https://t.me/NNDOfficial 58 Question 2b Q. A 70yr old diabetic & HTN patient underwent angiogram for angina & after 48hrs developed fever,abdominal pain and mottled rash. He had blackening of toes, on examination BP was 180/100 and S.creat. had risen to 4.12 mg/dl A. CIN B. Heparin immediately C. kidney biopsy will show micro occlusion with clefts in vessel D. NAC useful Ans. Kidney biopsy will show micro occlusion with clefts in vessel. Symptomatic , constrast induced nephropathy is characterized by non oliguric renal failure. If you monitor the creatitine , the levels are slowly trying to increase. One week to 10 days time levels have started to decrease it is because of medullary hypoxia. Join for latest updates https://t.me/NNDOfficial 59 Mottling seen in livedo reticularis Join for latest updates https://t.me/NNDOfficial 60 Question box---HTN Q. In an uncomplicated primary hypertension detected at 42yrs what should be the preferred combination A. A+D B. B+D C. C+D D. A+B Ans A+D HTN - 24 hrs ambulatory BP 90-95% have primary HTN 5-10% have secondary HTN No target organ damage , we need to look for LVH, retinopathy, microalbunemia. In case of Target organ damage , do detailed renal and endocrine assessment. Optimal dose Filmsartan 60mg bd Benzidipine 8 mg bd Indapamide 2-5mg bd If resistance develops, stop and evaluate for secondary cause. Anybody who comes with Hypertensive emergency is to be considered secondary hypertensive. Cut-off for HTN emergency >180/120 mm Hg with end organ damage. Join for latest updates https://t.me/NNDOfficial 61 Hypertensive emergency + new onset changes in fundus is Accelerated HTN. Accelerated HTN with papilledema is called Malignant HTN. In CKD , we use A+C or A+D , D has to be metolazone. Join for latest updates https://t.me/NNDOfficial 62 DELTA MEDICINE MCQ DISCUSSION -8 Ques Q. Hypokalemic metabolic acidosis will result from excess fluid loss from A. Stomach B. lleum C. Colon D. pancreas Q. Which of the following is not a cause for inherited low renin HTN A. Conns B. Liddles syndrome C. AME D. Licorice ingestion Hypokalemia- serum Potassium <3.5 meq/l Potassium is 90% excreted by the kidney, 10% excreted by git. In hypokalemia look for non-renal loss and renal loss. Also check if there is any shift from blood to cell. Shift can be produced by insulin, beta agonist, alpha agonist. Urine spot K+ > 10meq/l Join for latest updates https://t.me/NNDOfficial 63 Urine K+/ creat > 15 meq/g is renal loss. Non renal loss is <10meq/l Hypokalemia due to non renal loss due to GI loss – colonic diarrhoea This GI loss produces NAGMA and K+ loss. Renal loss of K+ Due to Metabolic acidosis (RTA type1/2, NAGMA) and metabolic alkalosis. NAGMA – Normal anion Gap metabolic acidosis AG = Na+ - [HCO3- + Cl-] Normal AG = 8-12 meq/l NAGMA associated with Hyper K+, seen in RTA type4, early uremic acidosis and recovering DKA. Normal AG associated with Hypokalemia. Hypokalemia with Normal AG seen in RTA type 1 / 2, GI loss of K+ / HCO3- , colonic diarrhoea, ureterosigmoidostomy, Acetazolamide. NAGMA in RTA v/s GI loss of HCO3- Join for latest updates https://t.me/NNDOfficial 64 Urine spot K+ to check, Urine anion Gap UAG positive and UAG negative. Urine anion gap (UAG) = ( Na+ + K+ - Cl-) Measured cation + Unmeasured Cation = Measured Anion + Unmeasured Anion Measured cation – measured anion = Unmeasured anion – Unmeasured Cation Urine anion Gap = UA – UC UC decreases, UAG increases. UAG +ve seen in RTA. Metabolic acidosis compensated ∆AG ∆HCO3- >12 Join for latest updates https://t.me/NNDOfficial 65 OAG < ∆HCO3 OAG / ∆HCO3 > 2 PH = 7.4 PCO2= 40 HCO3- =24 Case of Metabolic acidosis. In metabolic acidosis , we check whether it is compensated or decompensated with fall in HCO3- = 8meq/l With every 1meq/l fall --- PCO2 falls by 1.2 Here, The fall is 8 x 1.2 = 9.6 Expected PCO2 = 40-9.6 = 30 approx This particular ABG is Metabolic acidosis compensated with respiratory alkalosis. AG= Na – (Cl- + HCO3-) =140- (16+102) =140- 118 = 22 It is a case of respiratory alkalosis/ almost compensated ∆AG ∆HCO3∆AG = 22-12 = 10 ∆HCO3 = normal HCO3- – patients HCO3- = 24-16=8 ∆Anion Gap /∆ HCO3-- <1 In this question, ratio is 8. 1-2 ratio , there is HAGMA, If >2 ratio, presume that there is Metabolic alkalosis. Case settings 1:Suppose, we have hypokalemia with renal loss and alkalosis. There are two possibilities 1) Normal to low BP a) Bartter syndrome(Na+-K+-2Cl-) b) Gitelman syndrome(Na+-Cl-) 2) High BP Join for latest updates https://t.me/NNDOfficial 66 Bartter Syndrome types – Na+ K+— 2Cl-, ROMK, CL-CKB,Barth. CL-CKB is type3 and is classical type of bartter. Based on Ca 2+ Hypercalciuria seen in Bartter syndrome. Hypocalciuria seen in gitelman syndrome about 4mg/kg/24 hrs. In serum Magnesium , 20% decrease in Bartter whereas, 96% decrease in gitelman. Case settings 2:Hypokalemia renal K+ loss , alkalosis , HTN. 1) Conns ( primary hyperaldosteronism) 2) Liddle (ENac gain of functional mutation) 3) Cushings 4) AME 5) GRA Liddle(AD) , AME(AR) and GRA( AD) are inherited conditions. Liddle HypoK AMEApparent mineralocorticoid Excess GRA Glucocorticoid remediable aldosteronism Alkalosis Liddle's AME GRA Gain of ENac functional mutation 11 beta hydroxy steroid dehydrogenase HTN Cortisol increased Renin Decreased Decreased Decreased Aldosterone Decreased Decreased Increased Liddle’s, AME and GRA are all low in renin, in case of hypertension. AME, Liddle and GRA have hypo K , alkalosis , HTN We reverse these to hyperkalemia, acidosis and normal to low BP. These hyperkalemia, acidosis and normal to low BP are characteristic of Pseudo hypo aldosteronism Type 1. In ENac when there is loss of functional mutation we get pseudo hypo aldosteronism Gitelman has Hypokalemia, alkalosis, normal to low BP. Join for latest updates https://t.me/NNDOfficial 67 If we reverse these as hyperkalemia, acidosis and HTN. This is pseudo hypo aldosteronism type 2 or Gordon’s syndrome. Also cause of low renin hypertension. Q. Which of the following is not a cause for inherited low renin HTN A. conns B. Liddles syndrome C. AME D. licorice ingestion Ans Conns Q. 2 yr 8 kg old child admitted with polyuria, polydipsia, vomiting, salt craving, failure to thrive. Child euvolemic And normal BP Blood urea 20 Creatinine 0.5 Na 122 K- 3 Cl 96 HCO3 28 Mg 1.5 PH 7.48 Pco2 48 HCO3 30 Urine electrolytes Urine Na - 35 Urine potassium 20 U. chloride 25 U. Calcium 40 mg/24 hr Normal 4 mg/kg ABG DIAGNOSIS ? PROBABLE ETIOLOGY ? A. BARTTERS syndrome B. Gitelman syndrome C. RTA type 1 D. Gordons syndrome Explanation :- Metabolic alkalosis, hypercalciuria 4x 8 = 32mg Ans. A. BARTTERS syndrome. Join for latest updates https://t.me/NNDOfficial 68 HTN with low renin, decreased aldosterone seen in inherited AME, liddle, Gordon. Increased aldosterone seen in conns and GRH and it is acquired . Low aldosterone can be produced by congenital adrenal hyperplasia(11OH, 17OH). RTA type 1 (inherited) Acquired form of RTA related to Sjogren’s syndrome, primary biliary cirrhosis and drug associated is Amphotericin B Type 1 RTA is due to defect in H+ ATPase or A+-K+ ATPase and alpha intercalated cells of Cortical collecting duct. Unable to acidify urine, urine pH >5.5. Severe NAGMA – osteoclastic resorption which results in medullary nephrocalcinosis. Salt and water wasting seen with RAAS activation, results in hypokalemia. Never progresses to CKD. RTA type 2 (inherited) Associated with systemic infection like Fanconi syndrome, cirrhosis and Wilson’s disease, light chain myeloma. Drugs like aminoglycosides, tenofovir, cisplatin and ifofosfamide. Never progresses to CKD. HCO3- reabsorption reduced , generalized PCT dysfunction. H+ excretion is maintained. Join for latest updates https://t.me/NNDOfficial 69 Urine PH is variable. Less severe acidosis, less severe hypokalemia, no stones. Phosphorus in urine, rickets like manifestation. Glycosuria, aminoaciduria. If you ever have doubt, look at fractional excretion of phosphorus. HYPER KALEMIA -Related to renal failure, -Unrelated to renal failure disproportionate + degree / duration of renal failure . This is what we call as RTA type 4.(acquired) It is associated with some renal dysfunction. There is decrease in aldosterone, it can be a true hypoaldosteronism or pseudohypoaldosteronism . True hypoaldosteronism can be divided into low renin, low aldosterone and high renin , low aldosterone. Low renin, low aldosterone seen in diabetic. NSAIDS will block prostaglandin, Renin inhibitors like aliskiren. High renin, low aldosterone seen in Addison’s disease, ACE inhibitors, Heparin. Pseudohypoaldosteronism is divided into PHA type 1 and PHA type 2 (Gorlin syndrome) Aldosterone is there but cannot bind, it binds to basolateral membrane at piece of collecting duct, there it is completely fibrosed. Hence, it cannot bind. Seen in sickle cell, obstruction and reflex. Drugs that block aldosterone binding spironolactone, eplerenone. Amiloride and Triamterine blocks ENac. Calcineurin Inhibitors cause fibrosis of the T cell. Structural similarity to Triamterine is trimethoprim Q. A 23yr old male smoker presents with hemoptysis and on BAL hemosiderin laden macrophages are seen....his RFT continues to worsen and is presently 3.5mg/dl. what could not explain the same A. Anti GBM Join for latest updates https://t.me/NNDOfficial 70 B. SLE C. MPA D. EGPA Ans. EGPA Young Smoker with hemoptysis and BAL hemosiderin laden macrophages Seen in Diffuse alveolar Hemorrhage (DAH). SLE can produce DAH and type 2 RPGN EGPA producing DAH and RPGN is too rare. Current case settings shows DAH + RPGN. RPGN is crescentric Glomerulonephritis, again based on clinicopathological basis Which includes renal failure, rapidly progressive renal failure, it takes around days to weeks, compartment involved is glomerulus because the patient has edema, RBCs plenty and albumin 2+ ,3+ . Urine output ↓↓↓ Biopsy shows crescents Type 3 RPGN most commonest. Pauci-immune vasculitis is seen in ANCA vasculitis which includes 1) Renal 2) MPA 3) GPA 4) EGPA Type 2 RPGN with immune complex deposition Join for latest updates https://t.me/NNDOfficial 71 Type 2 RPGN, we get in SLE, adult HSP, Vasculitis, MPGN Type 1 is linear IgG +/- C3. Type 4 and type 5 Type4 – double positive, anti GBM Positive ANCA positive. Join for latest updates https://t.me/NNDOfficial 72 DELTA MEDICINE MCQ DISCUSSION-9 Q.1 A female around 65year age has been presenting with 1-2 weeks with imbalance and Falling down. While walking she falls down. She was taken to the physician, the physician thought it was cerebellar issue or cerebritis or infection , so gave a dose of acyclovir but after a week there is no change , An MRI was suggested and it turns out MRI has prominent finding of cerebral atrophy, On routine CXR found out homogeneous mass , intensely basophillic , necrosis and cellular content being released, it’s actually getting attached to vessel wall, azzopardi effect. A. 1. Small cell carcinoma 1. X-ray showing solitary pulmonary nodule. 2. Cerebellar degeneration as a part of paraneoplastic syndrome. 3. Azzopardi effect of vessels suggestive of Small Cell Ca lung. Q.2 A CEREBELLAR DEGENERATION IS A PARANEOPLASTIC MANIFESTATION OÓF A. HODGKINS B. NHL C. CML D. CLL Ans: A Join for latest updates https://t.me/NNDOfficial 73 Paraneoplastic Syndrome Lambert Eaton Myasthenic syndrome (LEMS) – anti voltage gated Ca channel body. Associated tumour is Small cell lung cancer. Subacute cerebellar ataxia – antibodies seen are anti Yo and anti Hu , anti thyrotropin receptor Anti Hu antibody seen in small cell lung carcinoma. Anti thyrotropin antibody is seen in hodgkin’s Opismyoclonus seen with Neuroblastoma Sensory neuropathy, limbic encephalitis and encephalomyelitis seen with Small cell Lung carcinoma. Multiloculated abscess on CT, nodule on HRCT, AFB +ve , skin picture shows draining purulent sinuses Diagnosis is Nocardiosis. Join for latest updates https://t.me/NNDOfficial 74 - A.2. Nocardiosis 1. Abscesses in brain and lung. 2. Necrotic bulla on skin. 3. Filamenous AFB +ve organism. Gram stain shows regular beaded appearance, diagnosed by Nocardiosis. Mild cases managed with cotrimoxazole, in some cases may require IV antibiotics, you can give amikacin, carbapenem , minocycline, best IV drug available is amikacin. What we give is cotrimoxazole with amikacin. Join for latest updates https://t.me/NNDOfficial 75 Also, in Microbiology NEVER forget to study the differences between Nocardia Vs Actinomyces. Actinomyces is endogenous organism, nonacid fast, it is normally present in our reproductive flora, GI flora. They also produce draining infections with the typical sulfur granules. Actinomyces is responsive to penicillin whereas Nocardia is not responsive to penicillin, it is responsive to cotrimoxazole. Nocardia Aerobe Acid fast (weak) Found in soil Causes pulmonary infections in immunocompromised with CNS involvement (can mimic TB but with (- PPD) cutaneous infections after trauma in immunocompetent Treat with sulfonamides (TMP-SMX) Actinomyces Anaerobe Not acid fast Normal oral, reproductive, and Gl flora Causes oral/facial abscesses that drain through sinus tracts, forms yellow “sulfur granules;” can also cause PID with IUDS Treat with penicillin Join for latest updates https://t.me/NNDOfficial 76 Q. 3. Read history and diagnose. Question 3 A 63-year-old woman with a 4-year history of uncontrolled diabetes mellitus presented with an ulcerating rash, primarily on the shins, groin, and face; cheilitis; and glossitis. Her symptoms had been worsening for 4 years despite specialized wound care. In addition, she noted concurrent, severe weight loss, depression, abdominal pain, and intractable nausea. Diagnosis and finding? This is a case of 63 y/ F with DM at 60years, and h/o of uncontrolled DM, weight loss, abdominal pain. She has ulcerative rash which worsens at night. Alongwith, cheilitis and glossitis. Rash is typical migratory necrolitic erythema. Join for latest updates https://t.me/NNDOfficial 77 This necrolytic migratory erythema is a characteristic of glucagonoma. Must read Glucagonoma, VIPoma, Insulinoma. A. 3. Glucagonoma 1. Uncontrolled diabetes. 2. Necrotic migratory erythema as seen on examination + weight loss + abdominal pain = Glucagonoma triad 3. Chelitis, glossitis and depression could be feature of acrodermatitis Entropathica. Zn deficiency. Case of a child with repeated episodes of salt craving, hypotension, weight loss, child seems to be fainting all the time. This was thought of to be the case of childhood Addison. Triple A syndrome in Childhood Addison Absence of lacrimal gland – alacrimia Join for latest updates https://t.me/NNDOfficial 78 In Addison- Hyperpigmentation of palms and soles. Achalasia cardia- esophageal dilatation Alacrimia, Addison’s disease and Achalasia cardia is the Triple A syndrome or Allgroves Syndrome. Young child with Addison’s features, Always think of 1) Triple A syndrome 2) X- linked adrenoleukodystrophy Adult with Addison’s , always think of TB first, Autoimmune and polyglandular syndrome esp. type 1 and type 2. 34 year female , slightly overweight with ALT -140, AST-120, grade II fatty liver. No e/o cirrhosis. ? Chronic hepatitis Causes of chr. Hepatitis – hep B, C , Wilson’s , NASH. Diagnosis was made of NASH. Acute hepatitis was seen not chronic. Routine biopsy was taken. In NASH, we can see microvesicular steatosis, nucleus almost pushed to the periphery, ballooning of the parenchymal hepatocytes, infiltrates does not cross the border, it is interface hepatitis. One more finding in this patient where one cell is engulfing the other cell, this is called Emperiopolesis. It is a case of a NASH with autoimmune hepatitis overlap. Join for latest updates https://t.me/NNDOfficial 79 A.5. Nash with AIH overlap 34yr with macrovesicular steatosis. ballooning and minimal inflammation. there are rosettes in the 2nd image also to be seen interface hep both of which suggest autoimmune etiology. NASH AIH overlap A patient with retroperitoneal fibrosis, diffuse swelling of pancreas, beaded stenosis duct, on ERCP shows irregular narrowing , Bilateral submandibular swelling and proptosis. Salivary gland biopsy shows cartwheel appearance or storiform fibrosis and IHC showed IgG4 containing plasma cells. IgG4 related disease In kidney – retroperitoneal fibrosis, CTID, salivary gland and lacrimal gland similar presentation to Miculikz syndrome, Join for latest updates https://t.me/NNDOfficial 80 Riedels thyroiditis, sclerosing pancreatitis and sclerosing orbital lesions. Q. 60 yr male who underwent angioplasty for CAD , 5 weeks back. Now Decreased urine output, edema and mottled skin lesions. CBC ⬆ eosinophil C3 ⬇⬇ C4 normal WBC ⬆ LDH ⬆ CPK ⬆⬆ Amylase ⬆ H/P image shows Eosinophillia Join for latest updates https://t.me/NNDOfficial 81 Biconvex cholesterol clefts which we actually get from skin biopsy or renal biopsy. Dirty white lesions called as the Hollenhorst plaques in the retina. Together this is a case of Atheroembolic renal disease, otherwise called cholesterol embolism. A. 7. Atherosclerotic renal disease 1. Hollenhorst Plaque in fundus due to cholesterol emboli. 2. Peripheral esosinophilia 3. Small blood vessel in kidney showing cholesterol clefts. Classical concentric onion skin fibrosis, male patient with jaundice and bloody diarrhea. Seen in PSC (Primary sclerosing cholangitis) 2/3rd patient’s with PSC have ulcerative colitis. PSC is a risk factor for cholangiocarcinoma. PSC is a fibrosing destruction of the intra and extra hepatic bile duct, PBC (primary biliary cirrhosis) is the autoimmune destruction of the intrahepatic bile duct. PBC is seen in females. PSC is seen more commonly in males. PBC has antibody called as AMA + anti mitochondrial. PSC has no antibody, sometimes we see, P-ANCA +ve. Treatment option is Transplant. Join for latest updates https://t.me/NNDOfficial 82 Test being done on Skin, nodules seen on X ray and CT. A. 9 ABPA 1. Forearm allergen testing 2. Finger in glove sign on x-ray 3. ILD feature on CT 1- Allergic Bronchopulmonary Aspergillosis 2- Finger in glove appearance, fleeting opacities, tram track lines, central bronchiectasis with normal tapering bronchi, high attenuated mucus plugs Join for latest updates https://t.me/NNDOfficial 83 Central bronchiectasis with mosaic pattern with mucoid impact , centrilobular nodules – tree in bud appearance. Patient was with asthma and was given repetitative inhalers, recurrent asthmatic episodes and despite treatment, we think of ABPA. 1. Predisposing asthma or cystic fibrosis. Join for latest updates https://t.me/NNDOfficial 84 2. Obligatory criteria a. IgE> 1000 IU/mL and (type 1& 3 Hypersensitivity) b. Positive immediate skin test or increased IgE antibody to Aspergillus. Q.10. Renal biopsy specimen reveals chronic calcific pancreatitis as well as bipolar disease on lithium . Following an episode of diarrhea he develops, severe oliguric AKI, on biopsy. On routine H/E staining patient has lot of fibrosis, some degree of underlying CTID. Birefringent oxalate crystals. Acute oxalate nephropathy following diarrhea. Chronic calcific pancreatitis (CCP) can produce malabsorption, that malabsorption is the reason for diarrhea. CCP also produce enteric hyperoxaluria, reason for oxalate stones. Supportive (>=2) criteria a. Eosinophillia > 500 b. Precipitins or increased IgG antibody to Aspergillus. c. Consistent radiographic opacities. Join for latest updates https://t.me/NNDOfficial 85 Bluish skin lesions are kaposi sarcoma like lesions. Patient is already on ATT. They have diagnosed HIV and started on HART, after 3 months patient started to get really worse and Having a significant pleural effusion. Ans. Immune reconstitution inflammatory syndrome (IRIS) Skin lesion showing kaposi sarcoma. HIV Patient diagnosed with pulmonary TB worsened on Treatment due to IRIS. Two types of IRIS 1) Unmasking iris 2) Paradoxical iris Paradoxical iris happens when CD4<50, high viral load in the background of TB or any infection. After some time, CD4 increases viral load decreases. Worsening of TB Start with steroids. Unmasking IRIS refers to the flare-up of an underlying, previously undiagnosed infection soon after antiretroviral therapy (ART) is started. Join for latest updates https://t.me/NNDOfficial 86 Risk factors for HIV associated IRIS Risk Host related Low CD4 count at start of ART Ol or TB prior to ART initiation Paucity of immune response at Ol diagnosis (as in cryptococcus IRIS) Pathogen related Degree of dissemination of OI/burden of infection High pre-ART HIV viral load Treatment related Shorter duration of OI treatment prior to starting ART Rapid suppression of HIV viral load Small blue round pattern, salt and pepper chromatin, angioinvasion. Organized Islands of small blue cells. The lady presented with thymic carcinoid associated SVC obstruction. Paraneoplastic hyperpigmentation, Paraneoplastic ACTH excess. It is paraneoplastic cushings syndrome. A.12 Paraneoplastic Cushing Cushingoid features. Hyperpigmentation ACTH related. Mass in thymus. Thymic carcinoid. Paraneoplastic cushings. Join for latest updates https://t.me/NNDOfficial 87 Thoracic TumoursLung/Mediastinal carcinoids Lung SCLC / Adenok Thymic tumours Medullary Thyroid K Image of Thymic carcinoid Q. 13Diagnose A 2 year old female child presented with one episode of seizure Elder sibling died at 3 yr of age with similar history O/E- febrile and confused Hb - 8g/dl Sr. Creatinine-2.5 mg/dl Sr. LDH - 1400 IU Join for latest updates https://t.me/NNDOfficial 88 ALT-48 IU; AST-50 IU Coagulation parameters: Plts-50 X 109 /L aPTT-28 sec/ 30 sec PT - 12 sec/ 13 sec TT- 16 sec/ 16 sec Fibrinogen – 250/ 240 mg/l. D-dimer – Negative Questions: 1. What is your diagnosis? 2. What investigation will you advice to confirm the diagnosis? Patient is having Anaemia (schistocytes or Helmet RBCs) – Microangiopathy hemolytic anaemia (MAHA), Thrombocytopenia and Renal failure. Diagnosis : HUS 1.Can HUS patient come with seizure? It’s extremely unlikely unless and until you are having a HTN related encephalopathy or PRES (posterior reversiblity encephalopathy syndrome) 2. HUS related problem in the Brain can be a TTP TTP is MAHA +thrombocytopenia + neurological symptoms If it is a TTP always look for ADAMTS-13 level. Pentad of TTP – Microangiopathy hemolytic anemia + thrombocytopenia + renal failure + fever and neurological symptoms. There is no DIC – Fibrinogen levels is normal. PLTs and Clotting factors are being trapped in TTP Join for latest updates https://t.me/NNDOfficial 89 Classical Apple green Birefringence seen under the Congo red stain. H/P image shows group of plasma cells present on the bone marrow, eccentric nucleus. Enlargement of the anterior shoulder due to deposits in the periarticular soft tissue. (Shoulder Pad sign) Case of AL Amyloidoisis. Various Organs with AL Amyloidoisis and their peculiarity: Heart- RCMP, Hepatomegaly without any particular reason, Adult Nephrotic Syndrome, ANS findings which are not explainable like peripheral neuropathy and orthostatic Hypotension. Join for latest updates https://t.me/NNDOfficial 90 20 yr /M who has recurrent asthmatic episodes, CXR – Normal, CT was taken. He started developing skin lesions. Skin lesions – non cytopenic palpable purpura , Mostly Vasculitis P. Smear -Eosinophillic CT shows Bilateral infiltrates Case of Churg Strass Syndrome / Allergic Granulomatosis with Polyangitis (AGPA) / Eosinophillic Granulomatosis with Polyangitis (EGPA) Any kind of Neuropathy, we can get in Churgg strauss maybe a central or peripheral neuropathy. Join for latest updates https://t.me/NNDOfficial 91 A stable patient of Cardiac failure. Look at Lead II and Lead I, slightly wide QRS complex ?Bundle Branch Block pattern When we look at V1 and V2 , we get positive R wave. We are having RVH/RBBB pattern V5 and V6 are tall, and V1, V2,V3 – Notching pattern Lead I and Avl is tall. Axis is more towards the left side. This patient has got a LBBB morphology in Lead I and AvL. RBBB morphology in the precordial leads. We are having a chest leads with RBBB morphology. Limb leads with LBBB morphology. A.16 Masquerading Bundle branch block • The precordial leads show a Right Bundle Branch Block (RBBB) pattern while the limb leads resemble a Left Bundle Branch Block (LBBB), Join for latest updates https://t.me/NNDOfficial 92 • This finding on an ECG is almost invariably associated with severe underlying heart disease. It is a marker of poor cardiac outcomes. Q.17 Physician on Call→ A 24 year old male patient presented with CVA Right hemiplegia with bradycardia and hypertension (220/110mmHg). He was being treated under neurosurgery. Despite starting multiple anti-hypertensives , patients BP remained persistently high. You, as a physician, now have been called for advice regarding control of hypertension. Explanation : Young stroke with Bleed on CT, On ECG V5,V6 showing Tall leads, it is LVH with strain, with target organ damage with disproportionate change to degree and duration of Hypertension. Patient with 2o HTN that has lead down to the bleed, it is extremely difficult to control reflux. A. 17. COA Young patient with persistent high BP/resistant BP indicates secondary cause. Most common cause renal parenchymal>coarctation of aorta. CT shows ICH (putamen?) consistent with R hemiplegia CXR shows rib notching, figure of 3 features of coarctation. Join for latest updates https://t.me/NNDOfficial 93 45/F, gradually progressive renal failure over 6 months, recurrent E. coli progressive nephritis. H/O extensive Psoriasis on adalimumab therapy for over 12 months Long standing hyperuricemia, on febuxostat (1 year) HISTOLOGICAL DIAGNOSIS ?? Tubulointerstitial membrane seen with white arrows, few slightly dilated tubules. Doesn’t give any history of CKD.(no fibrosis, some dilated tubules) Tubulointerstitial nephritis- it’s a case of granulomatous tubulointerstitial nephritis. Patient was started on Anti-TNF alpha – Join for latest updates https://t.me/NNDOfficial 94 Before starting Anti –TNF alpha , patient should do mantoux test. In this patient , there is reactivation of Latent TB. A. 18 Granulomatous interstitial nephritis Latent TB reactivation precipitated by adalimumab. Before TNF alpha rule out latent TB. Image shows dilated intestinal obstruction, Dilated bronchiBronchiectasis. Case of Cystic fibrosis. Question 20 A 25 year female presented with sudden onset of pallor and jaundice H/o small joint pain & photosensitivity O/E- malar rash+, mod splenomegaly ANA 4+, anti-ds-DNA 4+ Sr. LDH - 1700 IU Complete hemogram Hb-5.2 g% RBC-1.2 x 10% c.mm MCV-150 fl MCH-157 pg MCHC-170 g% Join for latest updates https://t.me/NNDOfficial 95 TLC-22 x 10%/L Plts - 40 x 10%/L Questions: Q1: Interpret the complete hemogram and give your diagnosis. Q2: What problem will you face in blood bank? Explanation: Sudden pallor and Jaundice- Hemolysis Malar rash –SLE Thrombocytopenia SLE patients with Hemolysis, think of Warm antibody Autoimmune Hemolytic anaemia. Smear shows agglutination,almost like acrocyanosis (periphery you are getting cold). Case of Cold agglutinin Disease(Rare) A.20 Cold Agglutination Haemolytic anemia or Cold Ab AIHA Problems in Blood bank because of auto agglutination. Q.21 20 yr male presented with severe headache. BP 160/120. No Papilledema or renal bruit. Blood urea 30 mg/dl S. Creatinine 0.9 mg/dl S.Na 140, S.K 2.5 S.Chloride 86 Urine Na 20 Urine Cl 60 Urine K 30 Join for latest updates https://t.me/NNDOfficial 96 ABG PH 7.46 PCO2 43 HCO3 28 PO2 98 Renin : 0.2 ng/ml/hr Aldosterone 20 ng/dl ABG interpretation? Probable etiology? Explanation : Secondary HTN with endocrine cause Possible Causes Hypokalemia Conns Liddles Cushings AME GRA Hyperkalemia Gordons We rule out Gordons. Potassium-2.5 Hypokalemia with Alkalosis and HTN. PH-7.46 i.e Alkalosis, HCO3-28, PCO3 – Increased Primary Metabolic alkalosis ↑HCO3 should increase PCO2↑ 1meq should be 0.6↑ For 4meq 4x 0.6=2.4 Normal value of PCO2 -40 It should become 40+2.4 = 43. It is primary metabolic alkalosis compensated. Aldosterone levels >20ng/dl are high. So, we rule out liddle, Cushing and AME. In liddle, there is pseudohypoaldosteronism. (epithelial Na channel mutation,Intravascular volume is high, You are suppressing RAAS, renin low, aldosterone low) Cushing will have high cortisol with low aldosterone. AME is due to apparent minerelocoids, the cortisol is behaving like a mineralocorticoid. Join for latest updates https://t.me/NNDOfficial 97 Hypokalemia with Increased Aldosterone It can either be Conns and GRA (Glucocorticoid remediable aldosteronism) ACTH is taking charge of aldosterone pathway. If you steroid, it will inhibit ACTH. Renin levels-0.2 low ↑Aldosterone levels and ↓renin levels. Diagnosis could be Conns or GRA. CT abdomen is taken to rule out correct cause. In conns , adrenals will have adenoma,hyperplasia, mass in adrenals. GRA is autosomal dominant condition where CT is normal, no mass in adrenals. What is your clinical diagnosis? What is your first line therapy for this entity? Explanation: First patient presented with CVT, we did CT venogram and MR angio. MR angio is showing filling defect at the level of sigmoid sinus and tranverse sinus. Second patient with renal artery stenosis. Both these patients have corresponding skin lesions. Third patient was COOMBs test positive. Skin lesions with mottling called livedo reticularis is present. One of the patient had young stroke. Which is the condition where you can find livedo reticularis, young stroke, Renal artery stenosis, Coombs test +ve, Venous thrombosis? Ans. APLA Join for latest updates https://t.me/NNDOfficial 98 1/3rd patients with SLE are going to be APLA positive. Renal Artery Stenosis is not common in APLA. More common in APLA is TMA. APLA can present as DVT(Most common), Young’s stroke arterial or venous, hepatic vein thrombosis or Budd Chiari syndrome. It can present with uncertain neurological features like chorea, migraine etc. It can present with thrombotic microangiopathy, sometimes present with adrenal involvement. When 3 or more organ systems are involved inside one week, that is called catastrophic APLA. Catastrophic APLA is important for Plasmapheresis with IV Ig. Pleural effusion, yellow nails and Lymohedema. Classical yellow nail syndrome. Join for latest updates https://t.me/NNDOfficial 99 Patient presented with Hypotension and JVP is elevated, lungs are clear. Soft heart sounds, fluid present. ECHO shows pericardial effusion. ECG shows Electrical alternans. Beck’s triad – Hypotension, muffling heart sounds, JVP elevated. Seen in cardiac Tamponade. All the differences between constrictive pericarditis and cardiac tamponade very IMP. She has no other major symptoms except for tiredness and fatiguability. ESR is 50, CBC- Normal. Hb-10.6 ANA – weak +ve ANA profile was done Join for latest updates https://t.me/NNDOfficial 100 Explanation:Rash involves the nasolabial folds, ANA weakly positive. There is strong suspicion of Dermatomyositis. In ANA profile, we look for Anti-Jo antibody, Or anti synthetase antibody. We also look for ILD – NSIP, Also search for malignancy, paraneoplastic manifestations. This a patient who presented with acute abdomen, 35 y/M Past 1-2 weeks he is having this rash which looks like palpable purpura, PLT count is normal. He has developed Rapidly progressive renal failure with serum creatine of 5.6. High coloured urine with Nil urine output. Skin Biopsy showed vessel wall granulocytes; Granulocytes were strongly IgA positive. This is actually a case of IgA vasculitis New name for IgA vasculitis is HSP (Henoch Scholein Purpura). A.26 case of Adult onset IgA vasculitis or Adult onset HSP. Join for latest updates https://t.me/NNDOfficial 101 Delta – Medicine MCQ Discussion -10 Neurology Question Q. 27. Diagnose A 32 year old male has migraine with aura. He developed left sided hemiparesis. Family history revealed father and grandfather suffering from migraine and dementia. For suspected acute stroke MRI is done and showed the image on the right. Your consultant asks you to get a skin biopsy. What is the likely clinical diagnosis? What is the underlying genetic abnormality? White matter hyperintensity T2 flair image Skin Biopsy in CADASIL • Generalized arteriopathy: Skin biopsy 96% sensitive 100% specific Granular Osmiophilic Material (GOM) deposits Join for latest updates https://t.me/NNDOfficial 102 Ans. Diagnosis : CADASIL, NOTCH-3 mutation Explanation: Young male presented with migraine with aura, F/h/o migraine and dementia , alongwith white matter changes. White matter Hyperintensities especially involving the temporal pole. Event neither a atherosclerotic event nor a amyloid event, this an arteriopathy with NOTCH-3 mutation on chromosome19. It’s a case of CADASIL (Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy D/Ds for CADASIL Amyloid angiopathy – (microhaemorrhages seen on MRI ) In elderly patient, always think of Binswanger disease that is because of hypertension , there will be small lacunar strokes and white matter hyperintensities. KEY POINTS Clinical suspicion : Stroke due to unknown reason, frequent lacunar infarction episodes and familial distribution of the disease. MRI features : White matter Hyperintensities, Lacunar infarcts and Cerebral Microbleeds. Skin Biopsy : GOM Molecular Genetic analysis : NOTCH 3 Mutation. Q. A 56 yr old gentlemen-a known patient with HIV disease He presents with increasing abdominal distension and abdominal pain On examination there is 9x8 cm mass in umbilical and epigastric quadrant Biopsy shows CD10 +ve and CD5 +ve, Join for latest updates https://t.me/NNDOfficial 103 CD 20, CD 21 and CD22 +ve. Explanation : H/P image shows blue round tumour cells with white macrophages with debris. Stars in the sky, with tangible body macrophages- Starry Sky appearance. Sky- Small Blue tumour cells. We identify tumour cells, by Germinal cell malignancy or post germinal cell malignancy. C/O Burkitt’s lymphoma seen in HIV positive patient. Q 2nd image is post renal transplant on triple immunosuppression since 9months with fatigue and near normal graft function Explanation : 9 months Post Transplant patient, with the person on immunosuppresants presents with fatigue and normal serum creat. levels. 2nd image shows monomorphic infiltrate, no alteration of architecture. C/O Post Transplant Lymphoproliferative disease LINK Between Burkitt’s and Post transplant lymphoproliferative disorder is EBV. Both are produced by Epstein Barr virus. Join for latest updates https://t.me/NNDOfficial 104 Q.29 Causative Agent? Patient is on treatment for testicular cancer. Flagellate rash Treatment regime responsible is BEP(Bleomycin, etoposide and cisplatin) Bleomycin is responsible for Flagellate rash. Q30 Identify : These are opportunistic infections in HIV patient Image- Perivascular Retinal H’ge seen in CMV. White matter hyperintensities especially on occipital pole of T2 Flair- seen in PMLE produced by JC virus. Oral thrush by candidiasis. Thrust lesion on palm, Bacillary angiomatosus produced by Bartonella henselae. Join for latest updates https://t.me/NNDOfficial 105 Q.31 She has eye pain. She has no other major symptoms except for tiredness and fatiguability, ESR is 50, CBC normal On evaluation ANA was weak positive ds DNA, smith ro la negative. Explanation : Physical finding : Nasal dipping. ANCA +ve, ANCA associated vasculitis, a case of Wegener’s Granulomatosis. Upper respiratory tract infections-Sinusitis, serous otitis media. Lower respiratory tract infections – DAH and nodule cavitate Kidney- type 3 RPGN Q. 32 What is your clinical diagnosis? Name the therapy related complication from the picture? Adrenal mass and fibrosis seen on CT. Join for latest updates https://t.me/NNDOfficial 106 No fat cells in BM. Case of Aplastic anemia- nail change, lacy reticular pattern skin change, tongue change all with aplastic anemia, points to Dyskeratosis congenita. Part of Busulfan toxicity, we get fibrosis. Don’t confuse with Diamond Blackman, it is just pure red cell aplasia. Q.33 A. Connect and Diagnose B. Which is the most common cause of morbidity and mortality in these patients? Telengiectasia, Severe raynauds, sclerodactyly,, calcinosis cutis Case of crest syndrome. Most common cause of morbidity and mortality in these patients is PAH (Pulmonary artery Hypertension) Q.34 Findings and what’s the common general exam finding in this Autosomal dominant disease ? Join for latest updates https://t.me/NNDOfficial 107 Image clearly shows, iliac horns, prominent iliac crest on Xray and Pn electron microscopy thickened GBM and moth eatan appearance of GBM in autosomal dominant disease, LMX 1B gene. Case of Nail patella syndrome. Q. 35 Correlate A 39yr old female admitted with 2 weeks history of intermittent fever with progressively worsening headache…. On admission is lethargic vitals stable, BP of 100/70 and no papilledema. CSF is showing pleocytosis with mild neutrophil predominance… routine evaluation was normal except for altered TFT.. seen ahead are the images. Join for latest updates https://t.me/NNDOfficial 108 2 weeks History 39 year old female with fever and headache. ?Lymphoma. Typical leptomeningeal enhancement, Hyperintensity of pituitary small and is enhancing. Case of Disseminated sarcoidosis. Join for latest updates https://t.me/NNDOfficial 109 Q.36 Correlate 24 yr old male hypertensive for 10 yrs not evaluated on drugs presenting with 3months history of recurrent chest pain and palpitation. Very likely to be Secondary Hypertension, Serum potassium 2meq/l Aldosterone – ↑↑ Renin- ↓↓ ?Conns Both kidneys and adrenals aare looking normal, inheritance shows likely AD pattern. There is some kind of stent in the descending thoracic aorta. Stent on aorta - artherosclerosis related, some kind of a guide wire. When you think of Conns having a normal adrenal gland, always bring your DDs to GRA (Glucocorticoid remediable aldosteronism). In GRA, we get AD pattern, atherosclerotic aneurysm. Join for latest updates https://t.me/NNDOfficial 110 Spotter question Roundworm/ Ascaris Hookworm/ Ancyclostoma Ileal perforation in Typhoid. Join for latest updates https://t.me/NNDOfficial 111 Perivascular Haemorrhage, Hemolysis, High coloured Urine. Seen in Black water Fever in Malaria. Q.38 Correlate A 20yr old male with no comorbidies acutely presented with pain and swelling right upper limb of 1 day duration… some diagnosis was made and iv plus oral drugs given.. he was discharged on day4… from day5 onwards in the left upper limb he began to notice new lesions.. identify He presented with acute DVT, echogenic thrombus also seen. For DVT, he was started on heparin. Heparin induced thrombocytopenia with thrombosis (HITT) syndrome. Remove heparin or don’t give warfarin, Some people give fondaparinaux(not approved). We give is indirectly acting thrombin inhibitors like lepirudin, Bivalrudin or argatoxaban. Join for latest updates https://t.me/NNDOfficial 112 Q. 39. Correlate A 20 yr old male with 4 months history of difficulty in eating and writing with right upper limb now slowly starting to involve left as well. No issues with sensorium or memory or walking or getting up from bed....can’t hold cricket bat now. Has excessive sweating of upper limb but no fasciculations or urinary symptoms On examination images of limbs shown DTR normal. LL normal. He was evaluated and asked 2b on follow up now presented with involuntary jerky movements of right UL for which EEG was done Ans. 20 yr boy with 4 months history of distal weakness (Right side to left side), His higher mental functions are normal, Cranial nerves are normal, he has excessive sweating of upper limb but no fasciculations or urinary symptoms. Atrophic wasted Upper limb, DTR normal, LL normal but suddenly during follow up, he presents with jerky moments. For Jerky Moments EEG was done. In neutral position MRI is normal, On flexion position MRI , the spinal cord is impinging on the vertebral body somewhere around C7 – T1. Join for latest updates https://t.me/NNDOfficial 113 This is case of Monomelic amyotrophy, also called as HIRAYAMA DISEASE. EEG shows generalized activity, polyspike and then wave pattern, this person had juvenile myoclonic epilepsy with the background of Hirayama. A male patient with malar rash and oral ulcers. He is ANA ds DNA positive. At presentation he was having normal Wbc, normal Hb, Normal PLT. He was admitted for fever. This fever was actually thought to be Dengue fever, DENGUE NS1 was positive. Into the second week of his infection , he started having Downhill course, his WBC count reduced and he started to have fatiguability, peripheral smear shows a bicytopenia, His LDH, Triglycerides were high, Ferritin was very very high, ESR was low. Join for latest updates https://t.me/NNDOfficial 114 Activated macrophages are engulfing RBCs, platelets, lymphocytes. This is called the HLH syndrome (Hemophagocytic Lymphohistocytosis) new name for Macrophage activation Syndrome. Q. 41. Diagnose A 52 year old man Mr A known case of major depressive disorder with poly substance abuse and poorly controlled Type 2 diabetes melitus is found unconscious and incontinent of urine in his room and admitted On arrival, the patient was afebrile and hemodynamically stable with a respiratory rate of 18/min-lethargic and oriented to self with dry mucous membranes Urine toxicology screen was negative The likely diagnosis for Mr. A? Join for latest updates https://t.me/NNDOfficial 115 Ans.52y/M with Depression, T2DM found unconscious, lethargic/dry. s. creat-1.5 -> Mild renal failure, due to increased serum calcium that can be due volume depletion. Bicarbonate – 34, Alkalosis. Low PTH, slightly low magnesium and phosphorus. Hypercalcemia with high PTH –> Hyperparathyroidism PTH related peptide kind of state can cause Hypercalcemia. In paraneoplastic, PTH is suppressed. Patient has got depression with poly substance abuse. Could be a case of Calcium carbonate abuse or calcium tablet abuse with >4g/day calcium. Hypercalcemia with metabolic alkalosis. This is called as the milk alkali syndrome. Join for latest updates https://t.me/NNDOfficial 116 Q. 41. Correlate 24 yr old male with repeated childhood history of enterocolitis now gets admitted for a 6 month history of profuse watery diarrhea and weight loss..no blood in stools or fever.. he has edema and mild ascites as well.. on examination he looks pale and slightly wasted. CBC shows hb of 9g/dl with markedly low ferritin. RFT normal. LFT normal except for low serum albumin. USG abd normal. CT abd..only mild bowel thickening. Anti TTG negative. Explanation : 24yr adult with childhood enterocolitis, presenting with watery diarrhea, edema ascites, pale and wasted. Low albumin and iron deficiency anaemia. H/P is villous atrophy. PET normal. Endoscopy is normal with mild bowel thickening. ?celiac disease Ruled out with anti TTG negative. In celiac, serology has to be positive. Immunoelectrophoresis – kappa , lambda no staining. IgA alone staging- heavy chain stained. There is IgA Heavy chain proliferation. Diagnosis –alpha heavy chain disease Immunoproliferative Small intestinal disease (IPSID) Join for latest updates https://t.me/NNDOfficial 117 Hypotonia, obesity, short stature, severe mental retardation and acromichria. Case of Prader Willi Syndrome. Q.43 What is the diagnosis? Constrast urography with Excretory urogram. Seen in middle aged females, associated with no CKD, sporadic stain. Retention of constrast by dilated collecting duct. Join for latest updates https://t.me/NNDOfficial 118 Paint brush/ Bouquet of flower appearance seen in Medullary Sponge kidney. In medullary sponge kidney, 50 % people have distal RTA. Q.44 Correlate This patient had fever for one week and was evaluated at local hospital, suddenly developed bilateral lower limb weakness. Eschar present s/o scrub typhus started with Doxy. MRI was showing Hyperintensity, even after giving constrast there is not much of enhancement on post constrast image. Pre constrast and Post constrast are looking the same with not much of enhancement. Join for latest updates https://t.me/NNDOfficial 119 GBS was ruled out based on 1) Premonitory sensory symptoms like tingling, numbness, parathesia. 2) Urinary complaints 3) Definite level of sensory loss 4) H/o of scrub typhus. Case of Acute scrub typhus induced transverse myelitis. Q. 45. Correlate This is a patient who has some ? Illness with multiple ulcers for 10yrs now presenting with acute epigastric and hypochondrial pain. Tense ascites and hepatomegaly were present..No jaundice or spleen … mild edema lower limbs present. On evaluation mildly altered ALT/AST…other parameters in LFT and RFT normal..Hb was only 7.8 and ESR was 87mm/hr.. Explanation : Acute abdominal, ascites, hepatomegaly, no jaundice and mild edema of lower limb, ALT/AST – mild alteration. Hb- 7.8, ESR- 87 mm/hr. Images are CT abdomen and CT venogram Patient having recurrent ulcers for 10 years not HIV positive. Oral ulcers, genital ulcers, young male – he is underlying case of Bechet’s syndrome. Whenever there is acute abd pain, ascites and hepatomegaly without jaundice. Always think of Liver and post liver. Pre liver will be having definite splenomegaly and upper GI bleed. Post liver most probably hepatic vein defect that is filling defect. Join for latest updates https://t.me/NNDOfficial 120 It is a case of Hepatic venous thrombosis. Most dreaded complication in Bechets syndrome is venous thrombosis presenting as Budd- Chiari. Q. 46 Correlate Fried egg appearance Explanation : Patient diagnosed with leukemia, TRAP +ve, Monomorphic cells with typical fried egg appearance, Lymphophocytes with hairy appearance. Diagnosed with Hairy cell leukemia. Patient with hairy cell leukemia and erythematous vesicular neck lesions with febrile neutrophillic dermatoses. Skin biopsy shows neutrophillic infiltrate. This is a Case of Sweet’s syndrome. Q.47 Correlate Travel package, Multiple Hypodense lesions seen in the liver and the spleen Enlarged, and abscess. Arthritis. Join for latest updates https://t.me/NNDOfficial 121 Multiple air opacities within Bronchogram. ?Necrotizing pneumonia +/-abscess Case of Meliodosis caused Burkholderia pseudomallei. Third generation Ceftazidine and carbapenems for treatment. Q. 48 Correlate Hyperinflated lung ?emphysema H/P image shows Protein unable to migrate from Endoplasmic reticulum to golgi body, it’s actually been held up inside the endoplasmic reticulum as a globule which is eosinophillic. Clear case of alpha 1 antitrypsin deficiency. Panacinar emphysema. Join for latest updates https://t.me/NNDOfficial 122 RAS-Renal artery stenosis Image shows café au lait, Lisch nodule and renal artery stenosis Diagnosis : Neurofibromatosis Similarly Check images in Tuberous sclerosis - Ash leaf nodes, shagreen patches, adenoma sebaceum. Join for latest updates https://t.me/NNDOfficial 123 1 2 Image 1- Hypodensity on the constrast CT, uniform. No rim of cortical enhancement. Renal infarct Vs Pyelonephritis. In renal infarct, there would be a rim while in pyelonephritis, no rim. Rim sign present in renal infarct whereas absent in pyelonephritis. Case of Acute pyelonephritis one side and emphysematous on the other side. In Image 2On this contrast enhanced CT, medulla is enhancing, but the cortex is not enhancing much. Generally, the cortex enhances more than the medulla. Reverse rim sign –cortex no enhancement, medulla enhancement. Reverse rim sign is seen in acute cortical necrosis. In reverse rim sign, capsular perforators are spared. Join for latest updates https://t.me/NNDOfficial 124 Spotter Images Consolidation surrounded by ground glass opacification. This is known as the Halo sign. Seen in condition like Invasive aspergillosis. Ground glass opacification surrounded by consolidation called as Reverse Halo sign, seen in Cryptogenic organizing Pneumonia (previously called as BOOP) Join for latest updates https://t.me/NNDOfficial 125 Opacity with crescent of Air – Monod sign (seen in aspergilloma) No fibrotic change, only ground glass opacification, NSIP. Join for latest updates https://t.me/NNDOfficial 126 Extensive ground glass opacification with Septal thickening Seen in Pulmonary alveolar proteinosis. UIP is characterized by loss of lung architecture, cystic honey combing, traction bronchiectasis. Split pleura sign Join for latest updates https://t.me/NNDOfficial 127 Most important sign to differentiate between empyema and Lung abscess is split pleural sign. Q 52 Correlate Patient has come to us with right heart failure when we evaluate the history , regurgitation across here and sign over neck called lancis Sign where we see a wave, v wave and JVP together , case of tricuspid regurgitation. Cabergoline induced right heart failure. Microscopic appearance of pituitary adenoma, maybe prolactinoma. Join for latest updates https://t.me/NNDOfficial 128 Q. 53 Cause? The drug which causes this rare adverse effect acts on this cell. Name the cell. Osteonecrosis of the jaw, the cell is osteoblast, drug which causes it is bisphosphonates. Q.54 Correlate Elderly patient with HTN for a long time, memory destruction. MRI shows atrophy , T2 Flair shows hyperintensities. This is called Binswager disease. Join for latest updates https://t.me/NNDOfficial 129 Q. 55. Identify underlying condition Livedo reticularis, Porphyria cutanea tarda, chronic plaque psoriasis, oral lichen planus. Image shows ulcer and purpura, agglutinated. Oral Lichen Planus Most important skin lesions in HCV are lichen planus, porphyria cutanea tarda. Diagnosis Hepatitis C virus. (HCV) We, can get Vasculitis – Cryoglobinuria Join for latest updates https://t.me/NNDOfficial 130 Q.56. Correlate H’gic edematous ulcerative mucosa. X ray image shows Toxic Megacolon. Reason for this Toxic Megacolon was ulcerative colitis. For treatment, he was given steroids and he recovered. Later on, at a point in time, he started developing severe proteinuria for which biopsy was done. Nodule like lesion seen on biopsy. Patient also had Macroglossia. Glomeruli: -Begins in mesangium extending into capillary walls Join for latest updates https://t.me/NNDOfficial 131 glomerulus is flooded by confluent masses or interlacing ribbons of amyloid. -Homogenous amorphous eosinophilic deposits: H&E On Congo red stain, Amyloid deposits seen. Toxic Megacolon seen in Ulcerative colitis, with long standing renal involvement. Diagnosis is 20 Amyloidoisis (AA type) characterized by macroglossia and amorphous eosinophillic deposits which are better stained with Congo red. DDs for Nodules in Glomerulus 1. MPGN 2. Amyloidoisis 3. Lichen deposition disease 4. Diabetic nephropathy Q. A 12 year old child was brought on wheelchair for both upper and lower limb weakness. Which classical sign given by an Indian neurologist is shown in the images? Join for latest updates https://t.me/NNDOfficial 132 C B A Abductor sign (ImageA), abductors are preserved in Duchenne Muscular Dystrophy. Gower’s sign (Image B)- Using arms to get up. Poly hill Sign (Image C) - Deltoid, Infraspinatus and abductors are spared. They may become more prominent and rest would be wasted. String of Beads appearance in fibromuscular Dysplasia. Q. ECG depicted : Join for latest updates https://t.me/NNDOfficial 133 ECG Same patient with two points in time, first time and after 6 months. First time when you see, R wave > S wave, there is definitely an RVH, RBBB (having m pattern). In RBBB, we expect to get right axis deviation Most prominent are AvL, I ; we get leftward deviation. So, RBBB with left fascicular Block. When you have right Bundle branch with one of the two fascicles being blocked, it is actually called as the Bifascicular Block. Typical case of Bifascicular Block. Same patient, Repeat ECG is done after 6 months The ECG is completely reversed has become a LBBB morphology with Left axis deviation. LBBB is itself, a bifascicular. To check whether it is Trifascicular, we look for PR interval. If PR interval is almost normal, there is no trifascicular block only bifascicular block. If PR is prolonged, it is trifascicular block. In lead II, P is being missed after QRS complex. Is it First degree AV block? Every P wave is followed by QRS complex. It’s not first degree AV Block? Is it Second degree AV Block? Is it MObitz or Advanced degree AV block. Join for latest updates https://t.me/NNDOfficial 134 Criteria for MObitz 1. At a time only one beat should be missed. 2. More than one (2 or more) impulse should be conducted down. If both these criteria is not fulfilled, it’s Advanced. Inside Mobitz – it’s type1 or type2 Progressive prolongation of PR interval. For every 2 beats one beat is missed, Mobitz type2 AV block. P --- > QRS – 10 (For whatever PR interval is prolonged) 20 Degree AV Block means some beats are being missed. Whether it’s Mobitz or advanced? In Mobitz, both these criteria are being fulfilled. 1. At a time only one beat should be missed. 2. More than one (2 or more) impulse should be conducted down. If both these criteria is not fulfilled, it’s Advanced. 30 Degree means P and R have no relationship, complete asynchrony between P and R. In this ECG, it’s 20 Degree AV Block Mobitz type2. Join for latest updates https://t.me/NNDOfficial 135
