Childhood Renal/Urological Disorders

Question 1

DDx for hematuria in children

Answer 1

Glomerular: glomerulonephritis
Non-glomerular: TICKS (trauma, tumor, infection, cystic disease, congenital obstruction, hyperCalciuria, stones, sickle cell)

Question 2

pediatric tubulointerstitial disease

Answer 2

proximal tubule: Fanconi’s syndrome
LOH: Bartter’s syndrome
DCT: Gitelman’s syndrome, Gordon’s syndrome
CD: Liddle’s (Type IV RTA, nephrogenic DI, Liddle’s syndrome)

Question 3

Bartter’s disease

Answer 3

renal tubular defect in LOH - like being on chronic high dose loop diuretic
Sx:
- sodium, Cl-, H20 wasting
- high aldosterone activity –> hypokalemia, metabolic alkalosis
- hypercalciuria

Question 4

Gitelman’s syndrome

Answer 4

renal tubular defect in DT - like being on chronic high dose thiazide diuretics
Sx:
- sodium chloride wasting (not as bad as Bartter’s)
- high aldosterone –> hypokalemia, metabolic alkalosis
- hypermagnesuria –> hypomagnesemia (crave vinegar)
- Hypocalciuria

Question 5

Gordon’s syndrome

Answer 5

opposite of Gitelman’s - unregulated Na-Cl cotransporter
sx:
- salt and fluid retention –> low renin hypertension
- low aldosterone –> hyperkalemia, metabolic acidosis
tx: thiazide

Question 6

Liddle’s syndrome

Answer 6

renal tubular defect of CD - gain of function mutation of ENaC channel. Autosomal dominant.
sx:
- sodium retention –> low renin hypertension
- hypokalemia and metabolic alkalosis
tx: no response to spironolactone, but tx w/ amiloride (direct ENaC antagonist

Question 7

cystic dysplasia secondary to in utero obstruction

Answer 7

kidney formed normally, but high pressure and severe hydronephrosis in utero –>

• cystic dilation of collecting system
• back pressure –> secondary abnormalities in tubules (nephrogenic DI, type 1 or type 4 RTA, renal insufficiency)

Question 8

malformations that effect both kidneys

Answer 8

bladder dysfunction
spina bifida
prune belly - weak abdom musculature, undescended testes, etc

Question 9

cloacal anomaly

Answer 9

disorder in girls

failure of urorectal septum development

Question 10

bladder outlet obstructions

Answer 10

most common is posterior urethral valves
in utero back pressure –> hydronephrosis and dysplasia
if severe –> Potter’s syndrome
tx: surgical ablation of valve, management of renal dysplasia and dysfunctional bladder