BOARD REVIEW NEPHROLOGY Karen Estrella-Ramadan Question 1 You are seeing a 10-year-old girl who has SLE for a WCC. You diagnosed SLE when she was 8y/o, based on an initial presentation of idiopathic thrombocytopenic purpura and a positive ANA test and the subsequent development of arthritis and a positive anti-double-stranded DNA test. She is currently doing well in school and has no concerns about her memory or problem-solving skills. The only finding of note on her physical examination is a slight erythematous rash in a malar distribution. Of the following, the MOST useful screening test for other organ involvement in this girl is: Of the following, the MOST useful screening test for other organ involvement in this girl is: 1. 2. 3. 4. 5. Coombs test erythrocyte sedimentation rate magnetic resonance imaging of the brain Urinalysis Venereal Disease Research Laboratory (VDRL) testing 0% 1 0% 0% 2 3 0% 0% 4 5 10 Countdown SLE patients have high mortality from Renal and CNS disease: Renal disease occurs in 75% to 80% of children who have SLE, usually within 2 years of diagnosis. Renal manifestations are not typically apparent at diagnosis; most patients present with fever, rash, arthritis, and mucositis Typical screening besides U/A, include: urine protein-to-creatinine ratio, and serum creatinine and albumin. Renal manifestations can range from asymptomatic hematuria and proteinuria to overt nephritis (gross hematuria, azotemia, and hypertension), nephrotic syndrome, or hypertension. Patients in whom proteinuria (>1+ on a dipstick or urine protein-to-creatinine ratio >0.2 [mg/mg]) is discovered need Renal referral for assessment and possible renal biopsy. CNS: MRI if pt develops: seizures, psychosis, headaches, difficulty with concentration, or memory problems. Other organs: Renal: do a U/A to screen , initially may be subclinical Hem: hemolytic anemia pallor, do Coombs + ESR: non specific ; VDRL: false positive Question 2 An 8-year-old boy presents with cola-colored urine without blood clots. He was well until 2 days ago, when he developed a sore throat with upper respiratory tract infection symptoms. He denies any dysuria, frequency, urgency, flank pain, or trauma. On physical examination, his temperature is 37.8°C, heart rate is 84 beats/min, respiratory rate is 18 breaths/min, and blood pressure is 118/78 mm Hg. He has no costovertebral tenderness, abdominal tenderness, or edema. Labs show: Urine: Specific gravity, 1.025, pH, 6.0, 3+ blood, 3+ protein, 1+ leukocyte esterase, Nitrite, negative Microscopy shows more than 100 RBC/hpf and 5-10 WBC-hpf BUN 24 mg/dL (8.6 mmol/L) Creatinine, 0.9 mg/dL (79.6 mcmol/L) C3: 140 mg/dL (normal, 80 to 200 mg/dL) C4: 30 mg/dL (normal, 16 to 40 mg/dL) ANA: negative Of the following, the most likely diagnosis is: 1. 2. 3. 4. 5. acute pyelonephritis immunoglobulin A glomerulonephritis postinfectious glomerulonephritis Urolithiasis viral cystitis 0% 1 0% 0% 2 3 0% 4 0% 10 5 Countdown Dx: acute glomerulonephritiscola-colored urine, hematuria/proteinuria, and mild azotemia Things to differentiate: Urine color: bright red: Lower tract bleeding viral hemorrhagic cystitis or stones often accompanied by clots. Cola-colored urine without clots glomerular hematuria. Yellow but on UA dipstick + RBC, do a UA and likely + for myoglobin, Time: Very recent URI: of but not specific for immunoglobulin A (IgA) glomerulonephritis. 7-21 days later: PSGN Remember Piuria: > 10WBC/hpf Hematuria: >3 RBC/hpf in2 centrifugated samples of fresh urine Proteinuria: >3.5 gr/day Evaluation of a child with suspected AGN Follow up Blood pressure and creatinine levels If HTN, or rising creatinine levels renal bx Follow serologic markers: C3 and C4, ANA and antiDNAds The combination of a normocomplementemic GN and a synpharyngitic pattern =IgA nephropathy Can also present with asymptomatic hematuria or hematuria/proteinuria. Patients who have gross hematuria have a better prognosis. Treatment usually consists of steroids, ACE Alternative diagnostic include: Alport :renal, sensorineural hearing loss, ocular defects Membranoproliferative glomerulonephritis: low C3 + low C4 PIAGN: strep infection, low C3, normal C4 Focal glomerulonephritis (HIV): normal complement Question 3 A 17-year-old sexually active girl presents for a follow-up evaluation after her third episode of a urinary tract infection. She is currently asymptomatic. The results of renal ultrasonography and voiding cystourethrography are negative. She asks you how to prevent further episodes. Of the following, you are MOST likely to advise her to 1. 2. 3. 4. 5. drink cranberry juice frequently increase her daily water intake make sure to void after intercourse self-medicate with antibiotics for 3 days when symptomatic use single-dose postcoital antibiotic prophylaxis 0% 1 0% 0% 2 3 0% 0% 4 5 10 Countdown She has…acute cystitis > common in girls, > 3 UTI in a yr Best preventive measure ( even after 5y of use): symptoms with sexual intercourse postcoital antibiotic prophylaxis (taken within 2 hours of intercourse). nitrofurantoin 50 mg, TMP-SMX] 40/200 mg, or cephalexin 500 mg Those who fail, UTI not associated with a modifiable cause or at risk of complicated UTI give: continuous TMP-SMX (40/200 mg daily at bedtime or 3 times per week) nitrofurantoin (50 to 100 mg daily at bedtime or 3 times per week), norfloxacin (200 mg daily at bedtime or 3 times per week) and cephalexin (250 mg daily). Stop meds after 6-12 months: trial, if fails, restart abx Self-medication when symptomatic with a 3-day regimen is another option Women with one to two UTI per year). IF UTI after sexually active: no imaging ~Behavior changes: “proper wiping,” avoid tight pants, avoid urge-initiated voiding, +postcoital voiding, increased fluid intake, and the daily consumption of cranberry juice Question 4 You are examining a newborn who has wrinkling of the abdominal wall skin. His mother recalls her obstetrician mentioning that her “fluid was low.” The infant was born at 37 weeks’ gestation, and his birthweight was appropriate for gestational age. Of the following, the MOST likely additional findings expected in this infant are cryptorchidism and 1. 2. 3. 4. 5. bilateral hydronephrosis Hypospadias Nephrocalcinosis polycystic kidney disease unilateral renal agenesis 0% 1 0% 0% 2 3 0% 4 0% 10 5 Countdown BL hydroureteral nephrosis + absence of abdominal wall musculature +undescended testicle= prune-belly syndrome PBS obstructive uropathy Associated with renal dysplasia BUT NOT: renal agenesis, nephrocalcinosis, cystic renal disease, hypospadias. almost exclusively in males 30% of affected patients eventually develop endstage renal disease PRUNE-BELLY SYNDROME CRIPTORCHIDISM ABSCENSE OF ABDOMINAL WALL MUSCLE-WRINKLY Question 5 A 6y/o girl presents for a WCC that was scheduled as a follow-up appointment after she had an elevated blood pressure at an urgent care facility during an evaluation for abdominal pain. Her abdominal pain has resolved. Her mother recalls the blood pressure in the urgent care center as 135/90 mm Hg. The girl has had 2 UTI with fever in the past, and her father had hypertension diagnosed at age 45 years. On physical examination, the girl’s temperature is 37.3°C, heart rate is 90 beats/min, respiratory rate is 20 breaths/min, and blood pressure is 146/86 mm Hg. A repeat blood pressure reading is 142/88 mm Hg. The four limb blood pressures are: 142/88 mm Hg in the right arm, 144/84 mm Hg in the left arm, 156/100 mm Hg in the right leg, and 160/96 mm Hg in the left leg. You find no cardiac murmurs, abdominal bruits, or edema. Femoral pulses are 2+ and symmetrical bilaterally. Renal ultrasonography shows the left kidney to be 8.5 cm with normal corticomedullary differentiation and the right kidney to be 5.5 cm with increased echogenicity. Of the following, the MOST likely cause for this patient’s elevated blood pressure is 1. 2. 3. 4. 5. coarctation of the aorta essential hypertension renal artery stenosis renal hypoplasia/dysplasia renal scarring from prior pyelonephritis 0% 1 0% 0% 2 3 0% 4 0% 10 5 Countdown HTN, + hx of UTIs and US findings (increased ecogenicity) renal scaring from pyelonephritis (+)VUR: HTN in 20% of pts Up to 8 yrs to develop (+)HTN: 30-40% had scarring Hypertension can occur in up to 20% of patients who have (+)VUR Patients usually respond well to ACEIs caution in female patients of childbearing age due to risks of teratogenicity. If BL stenosis or on monorenal pt—DON’T USE ACEIs will worsen renal failure due to inhibition contraction of efferent arteriole that will maintain glomerular filtration Why not? Coartation of the aorta: femoral pulses are present and blood pressures are higher in the legs than in the arms Essential HTN: is a diagnosis of exclusion mild hypertension in adolescent patients who are overweight and have a positive family history of hypertension. Renal artery stenosis is rare, associated with abdominal bruits on examination and a small kidney on ultrasonography. Renal hypoplasia/dysplasia can be associated with an isolated small kidney, BUT hypertension is uncommon in this setting. Question 6 You are called to attend the delivery of a newborn male who had been monitored carefully prenatally for moderate oligohydramnios. Prenatal ultrasonography suggested bilateral cystic dysplastic kidneys, with no other congenital anomalies identified. Of the following, the MOST likely immediate lifethreatening problem in this infant is 1. 2. 3. 4. 5. bladder outlet obstruction cardiac arrhythmias liver failure renal failure respiratory failure 0% 1 0% 0% 2 3 0% 4 0% 10 5 Countdown The most immediate possibility of respiratory failure from pulmonary hypoplasia. due to decreased amniotic fluid volume, which may lead to reduced fetal respiratory excursion which impedes pulmonary development. “Potter sequence” (oligohydramnios with pulmonary hypoplasia, clubfeet, and flattened facies) Question 7 A 1-year-old girl presents for a health supervision visit. Her father underwent kidney transplantation for end-stage renal disease due to autosomal dominant polycystic kidney disease. The parents request assessment of the child's kidney function. Her serum creatinine measurement is normal for age. Of the following, the MOST correct statement about serum creatinine measurements in the pediatric patient is that 1. 2. 3. 4. 5. serum creatinine value of 0.6 mg/dL (53.1 mcmol/L) is considered normal for a 1-year-old child healthy preterm infants (<36 weeks' gestation) typically have lower serum creatinine values than term infants males and females have similar serum creatinine values up to 17 years of age newborns in the first few days after birth have lower serum creatinine concentrations than those seen in 2month-old infants serum creatinine values are related more closely to patient height than to patient age in children 0% 1 0% 0% 2 3 0% 0% 4 5 10 Countdown Kidney function is assessed best by measurement of the glomerular filtration rate (GFR The serum creatinine is the most frequently used agent to assess GFR clinically. Skeletal muscle metabolism, high values during growth The GFR in the pediatric patient increases throughout the first postnatal year before reaching adult values by approximately 18 months of age. Newborn: high values-represent maternal value, declines after 10 days Preterms have higher values 1y/o: <0.4 mg/dl Question 8 A parent requests referral to a urologist for her 7year-old son because of his bedwetting problem. He has no urinary incontinence during the day and is otherwise healthy. Physical examination reveals normal growth parameters and normal genitalia with a circumcised phallus. Dipstick urinalysis results are normal, with a specific gravity of 1.025. Of the following, the MOST appropriate next step is 1. 2. 3. 4. 5. a complete blood count psychological evaluation reassurance and a follow-up visit in 6 months referral to a urologist renal/bladder ultrasonography 0% 1 0% 0% 2 3 0% 0% 4 5 10 Countdown Nocturnal enuresis Involuntary passage of urine during sleep in children >5 y/o and occurs in approximately 15% of children at age 5 and 1% of teens at age 15. There is a male predominance and often a positive family history. Child with NO daytime wetting, NO dysuria and normal UA: renal very unlikely reassurance and a follow-up visit in 6 months Child with BOTH daytime and nighttime wetting, older than 6y/o, enuresis + encopresis, or urinary symptoms further eval assess genitourinary anatomy, exclude trauma from sexual abuse, detect lumbosacral spine anomalies, and exclude bladder dilatation or abdominal mass. Older children and children for whom nocturnal enuresis is related to social dysfunction, family stress, or poor self-esteem may benefit from therapy to target those effects. The use of a bedwetting alarm has the highest rate of success in young children. Question 9 You are examining an asymptomatic adolescent male for his annual health supervision visit. Genital examination reveals genitalia at Sexual Maturity Rating 5 and a nontender mass in his left scrotum that extends from the inguinal canal to the upper pole of the testis The mass decreases in size when he lies down. Of the following, the MOST likely diagnosis is 1. 2. 3. 4. 5. direct inguinal hernia Hydrocele Spermatocele testicular tumor varicocele 0% 1 0% 0% 2 3 0% 4 0% 10 5 Countdown Varicocele Develops slowly usually asymptomatic >15-25y/o > left side (drain to renal vein) vary in size, can extend from the testis to the inguinal canal, and feel like a "bag of worms" INCREASE in size with standing or valsalva REDUCE when lying down If a varicocele develops suddenly, does not reduce in the supine position, or is on the right side, a cause for obstruction of the spermatic vein should be sought. Pampiniform plexus Why not? Inguinal hernias: painless, intermittent groin masses that appear with straining and usually reduce spontaneously. Hydrocele: fluid within the tunica vaginalis that surrounds the testis rather than being distinct from it. They may increase in size and become obstructed, resulting in a painful scrotal mass. Transilluminates Spermatoceles are cystic lesions within the spermatic cord that are above and distinct from the testis and transilluminate. Testicular tumors are painless solid masses within the testicle that do not transilluminate. Question 10 The attending neonatologist notifies you that she is caring for a newborn boy in your practice who has multiple congenital anomalies. The baby was transferred from the newborn nursery to the intensive care unit due to cyanosis. Echocardiography reveals findings consistent with tetralogy of Fallot. The infant has a cleft palate. Chest radiographs show multiple thoracic hemivertebrae. Renal ultrasonography documents unilateral renal agenesis. On physical examination, the infant has hypoplastic thumbs. The baby is normally grown and vigorous. Of the following, the MOST likely diagnosis for this infant is 1. 2. 3. 4. 5. CHARGE syndrome Fanconi anemia trisomy 18 22q11 deletion syndrome VACTERL association 0% 1 0% 0% 2 3 0% 0% 4 5 10 Countdown CHARGE syndrome C: coloboma H: heart A: atresia choanae R: retardation of growth and/or development G: genital anomalies E: ear anomalies Fanconi anemia AR thumb hypoplasia, short stature, microcephaly, abnormal ears, epicanthal folds, site of ureteral reimplantation, congenital dislocated hips bone marrow failure more common among persons of Ashkenazi Jewish heritage VACTERL V: vertebral anomalies A: anal atresia TE: tracheoesophageal fistula R: renal anomalies C: cardiac defects L: (radial)limb anomalies The combination of tetralogy of Fallot, hypoplastic thumbs, cleft palate, and a renal abnormality 22q11 deletion syndrome Recent studies have shown that tetralogy of Fallot with pulmonary atresia belongs to a spectrum of conotruncal cardiac malformations that are often associated with monosomy 22q11. The clinical presentation of monosomy 22q11 includes patients with conotruncal anomaly face syndrome, velo-cardio-facial syndrome, and DiGeorge syndrome. More recently these syndromes have been incorporated as a group under the acronym catch 22 (cardiac defect, abnormal face, thymic hypoplasia, cleft palate, hypocalcaemia, and microdeletion 22q11) Question 11 You are evaluating a newborn in whom intrauterine renal US results are abnormal. The child appears very edematous, but requires resuscitation with fluids for initial respiratory distress and hypotension. One day later, the child appears more edematous. He has an excellent urine output. Measurements of his serum electrolytes reveal: Na: 127mEq/L K: 4.6mEq/L Cl: 92mEq/L HCO3: 27 mEq/L BUN: 10mg/dL Creatinine: 0.7 mg/dl Albumin: 0.9g/dl UA: no blood, but (4+) protein with a random urine protein to creatinine ratio of 43.5. Of the following, the MOST likely cause of this child’s clinical status is: 1. 2. 3. 4. 5. Acute glomerulonephritis Congenital nephrotic syndrome Diffuse mesangial sclerosis Focal segmental glomerulosclerosis Membranous nephropathy 0% 1 0% 0% 2 3 0% 0% 4 5 10 Countdown Congenital nephrotic syndrome Cystic formation sec to mutation in: nephrin (BM) leakage Massive proteinuria decrease oncotic pressure edema By 2 months: kidneys enlarged with loss of coticomedullary differentiation By 12 mo: decrease in size with hyperechogenicity fibrosis/ glomerular sclerosis failure Rarely in newborn period: Acute glomerulonephritis Membranous nephropathy Focal segmental glomerulosclerosis Question 12 A 3 mo old boy is admitted to the hospital for evaluation of FFT. His bwt was at %50 and length at %75. Currently, his wt is below the %5 and length is at %25, His VS and PE are otherwise normal. He appears well hydrated. Measurement of serum electrolytes reveals; Na:139, K: 4.7, Cl: 114, bicarbonate : 12, BUN: 8, creatinine: 0.3. A consulting nephrologist recommends measurement of urine pH (which is 7.5) and urine ammonium (which is 12000 mcm/L—normal is > 60000) Of the following the MOST likely cause of child’s acidosis is? 1. 2. 3. 4. 5. Inborn error of metabolism Lactic acidosis Type I (distal renal tubular) acidosis Type II (proximal renal tubular) acidosis Type IV (renal tubular acidosis) 0% 1 0% 0% 2 3 0% 0% 4 5 10 Countdown Step 1 Calculate anion gap help you narrow your differential HIGH: severe CRF Ketoacidosis: diabetic, alcoholic, starvation Lactic acidosis Poisonings: salycilates, methanol, ethylene glycol NORMAL: Renal tubular acidosis Diarrhea Carbonic anhydrase inhibitors Hyperalimentation with TPN RTA 3 types Type 1: distal Type 2: proximal Type 4: distal NO TYPE 3 Type 2-proximal Inability to reabsorb HCO3 Very very alkaline urine If distal is normal, will compensate a little by excreting H+ and the urine is ~6 K: is low or normal CAUSES • Fanconi sd • Acetazolamide, 6 mercaptopurine • Heavy metals: lead, copper, mercury, cadmium • Disorders in protein, CHO, AA • Multiple myeloma Type2 Type 1-distal Inability to excrete hydrogen, as well as ammonium secretion Urine very very alkalotic~>7.5 with a low urine ammonium hypokalemic CAUSES: RENAL STONES-obstruction / hypercalciuria Amphotericin Toluene (glue sniffing) Lithium SLE Sjogren Chronic active hepatitis Type 1 Type 4-distal Affects Na/K/H exchange Hyperkalemic Hyperchloremic CAUSES: Obstructive uropathy Interstitial renal disease Multicystic renal disease Type 1 pseudohypoaldosteronism Diabetic nephropathy 21-OH deficiency Renal transplant Type 4 Type 1 Don’t forget…. Anion gap: Na+(Cl-HCO3) In RTA lost of bicarbonate will actually increase absorption of chloride—therefore, there is a balance in the equation ALL RTA HAVE: HYPERCHLOREMIA NORMAL ANION GAP TYPE 1: HYPO-K TYPE 2: NORMAL K TYPE 4: HYPER K Question 13 A 6y/o F had been noted by her gral pediatrician to have symptomatic, persistent hematuria for 2 yrs. Her initial UA and microscopic analysis in your office show moderate blood, with 25-50 RBC/hpf. Results of her renal US were normal, as was her urine calcium-tocreatinine ratio. Serum electrolytes were normal in 2 occasions. Over the next 2 yrs, you ordered 4 concurrent UA, all of which showed moderate blood with 25-50 RBC/hpf, without proteinuria. She comes to clinic after being lost to follow up for 3 yrs. Of the following, the clinical symptom or laboratory result MOST consistent with progression of renal disease in this child will be? 1. 2. 3. 4. 5. Abdominal pain Leukocyturia Proteinuria Recurrent gross hematuria Urinary RBC casts 0% 1 0% 0% 2 3 0% 0% 4 5 10 Countdown Hematuria + proteinuria= renal disease Proteinuria-important facts False positives: highly concentrated, alkaline urine Fixed proteinuria (day/night) requires further eval Transient hematuria: in fever, strenuous exercise and CHF Benign orthostatic proteinuria: higher during growth spurt Random urine protein/creatinine ratio > 0.5 24 hrs urine protein excretion > 3.5 gr/day Females When lying down: none, when up: positive Microalbuminuria: DM > 300: nephropathy Question 14 You are evaluating a 10y/o boy who has intermittent urinary incontinence. Voiding cystourethrography detects a urethral stricture. Of the following, the most likely cause of this body’s urethral stricture is? 1. 2. 3. 4. 5. Carcinoma Chronic narrowing Congenital narrowing Intermittent urolithiasis Trauma 0% 1 0% 0% 2 3 0% 4 0% 10 5 Countdown Urethral strictures •Mostly secondary to trauma: pelvic fx •> rare: instrumentation, infection(gonorrhea), congenital, complication of balanitis, cancer •SYMPTOMS: Straining to urinate, decrease in size and force of urine stream, persistent sense of bladder fullness, urine dribbling, and freq and urgency of urination •<2cm: endoscopic dilatation Question 15 A 10y/o F presents to the ER with a 1 day hx of brown urine. She reports no dysuria, urgency or abdominal or flank pain. Her VS: T 98.8, BP: 165/97, HR: 84, RR: 20. On PE, moderate periobital edema is evident, but here are no other abnormalities. UA reveals: moderate blood, and 4+ protein. The serum C3 concentration is low and the C4 concentration is normal. Of the following, the most likely cause of this girl’s hematuria is? 1. 2. 3. 4. 5. Focal segmental glomerulosclerosis Immunoglobulin A nephropathy Lupus nephritis Membranous proliferative glomerulonephritis Postinfectious acute glomerulosclerosis 0% 1 0% 0% 2 3 0% 4 0% 10 5 Countdown Gross hematuria + hypertension+ periorbital edema+ decreased C3 and normal C4 PIAGN Self-limited C3 will be back to normal in 8wks HTN sec to Na + water retention up to 3 months Now…lets go for a case Case A 4mo M infant presents with a hx of poor wt gain and irritability. FT, NSVD, Bwt:3.5kg. He was BF for 3 months and appeared to have satisfactory wt gain initially. He was then switched to formula feeds, which he has been taking well. Mom says his diapers are always soaking wet. His development has been normal and his vaccines are UTD. On PE, you find an infant that is thin with wt <5% and ht <25%. He appears to be irritable, with midly sunken anterior fontanel, an umbilical hernia and mild bilateral tibial curvature. Lab studies Serum Hb: 10.8; hct: 32.9% BUN 17, creat: 0.5 Na: 148 K: 3.7 Cl: 114 Bicarb: 20 Calcium: 9.8 Phosphorus: 5.5 Alk Phos: 360 Mg: 1.9 Glucose: 72 Urine S. gravity: 1.005 Urine pH: 6.8 Urine osm: 198 The serum osm on this pt is? 1. 2. 3. 4. 5. 296 302 306 300 None of the above 0% 1 0% 0% 2 3 0% 4 0% 10 5 Countdown Serum osmolarity = 2Na + [(BUN/3) + (glucose/18)] Normal: 285-295 The most likely cause for this infant’s failure to thrive is? 1. 2. 3. 4. 5. Metabolic acidosis because of renal tubular acidosis an urinary concentration defect Nephrogenic diabetes insipidus Bartter syndrome with polyuria Gittelman syndrome Liddle disease 0% 1 0% 0% 2 3 0% 0% 4 5 10 Countdown Calculate anion gap Diabetes insipidus: Serum: HIGH Na + HIGH osmolarity Urine: LOW Na+ LOW osmolarity In this case, baby developed early because initially on breast milk which has less sodium, less protein and less osmolar load, compared to formula which has higher solutes. While baby on breast milk less water loss Bartter syndrome: defect in Na-K-2Cl cotransporter on the loop of Henle Loss of Na, K, Cl Gitelman syndrome: older children, defect in Na-CL cotransport in the distal convulated tubule Loss of Na, K, Cl, Mg Hypokalemic, hypochloremic metabolic alkalosis Hypokalemic, hypochloremic metabolic alkalosis Liddle syndrome: adolescents and adults, upregulation of Na channel in principal cell of cortical convulated tubule Excessive Na absorption with hypoK due to exchange and loss in urine HTN/ deafness/ blindness Other laboratory investigations that may be helpful include: 1. 2. 3. 4. 5. Spot urine calcium-to creatinine ratio Serum vasopressin levels Nasal vasopressin (DDAVP) test 1 and 3 All of the above 0% 1 0% 0% 2 3 0% 4 0% 10 5 Countdown Diabetes insipidus = ADH problem Nephrogenic: insensibility to ADH Central: no production of ADH Do a vasopressin test: Give 1 dose of DDAVP intranasal and collect urine over the next 5hrs Nephrogenic: unable to concentrate urine= persists low urine osm (<200) Central: + response with concentrated urine The disease is most commonly inherited as? 1. 2. 3. 4. 5. AR AD X-linked recessive X-linked dominant None of the above * Remember the case 0% 0% 1 2 0% 0% 0% 3 4 5 0% 10 6 Countdown Congenital Nephrogenic DI 90% X-linked recessive Common 10% symptoms: Hypernatremia FTT on a baby switched from Breast Milk to formula sec to mutation in aquaporin 2 This condition is most appropriately treated with? 1. 2. 3. 4. 5. Low-solute diet Thiazide diuretics + amiloride + indomethacin Thiazide diuretics + indomethacin All of the above 1 and 3 0% 1 0% 0% 2 3 0% 4 0% 10 5 Countdown Treatment GOAL avoid dehydration 1. 2. Decrease solute load Free water replacement, change to breast milk ADD diuretics: Thiazides Add K-sparing diuretic: amiloride Add prostaglandin synthesis inhibitors: indomethacin acts on mesangial cells in the glomerulus increasing GFR