Urology/ Nephro Flashcards
most common cause of congenital obstructive uropathy
ureteropelvic junction (UPJ) obstruction
Most common causes of UTIs in boys
vesiculoureteral reflux
When is hypertonic 3% saline indicated
- Serum sodium concentration < 120 mEq/L
- patients with associated neurologic manifestations such as headaches, seizures, behavioral changes, obtundation, coma, and respiratory arrest
Alport Syndrome
pathophys & clinical clues
Inherited disorder of basement membrane collagen
persistent microscopic hematuria (with RBC casts) with proteinuria
family history of renal disease and deafness
X-linked dominant AS
Increased urinary excretion of ____, ___, ___ is associated with decreased risk of renal stone formation
citrate, magnesium, pyrophosphate
Urinary metabolic evaluation process
24-hour urine collection, needs to be at home without IVF
hyper_____ is the most commonly identified metabolic cause of renal stones
hypercalciuria
Boy with proteinuria on UA x2 instances, 6 months apart. What is the next evaluative step?
First-Morning urinalysis
(Urine protein/Creatinine ratio >0.2 in first-morning sample is abnormal.)
Then Renal US if proteinuria is confirmed
(don’t really do 24-hour urine collection for proteinuria in adults or kids)
Cut off for urine dipstick to make protein appear positive
300 mg/day
Normal
What is benign proteinuria?
Orthostatic proteinuria (when active), and disappears when patient is supine/asleep for at least 2 hours (first-morning sample)
Potter syndrome
Autosommal Recessive PKD
- oligohydramnios
- pulmonary hypoplasia
- facial appearance (pseudoepicanthus, flattened ears and nose, recessed chin)
- skeletal abnormalities (hemivertebrae, sacral agenesis)
- ophthalmologic malformations (cataracts, lens prolapse)
- limb abnormalities (club feet, hip dislocation)
Ultrasonographic findings of Potter Syndrome
Large echogenic kidneys with decreased corticomedullary differentiation
hepatic echogenicity is possible
Causes of asymptomatic gross hematuria in adolescent boys
Usually there is an obvious underlying cause
Trauma
Familial nephritis
HSP
Ddx and Workup of gross hematuria in children
detailed H&P, urinalysis, workup of infection, renal US
Ddx: meatal/prineal irritation, calciuria (crystalluria), nephrolithiasis, cystitis, congenital renal anomalies, bladder mass
Most common extra-renal manifestation of ADPKD, other manifestations, and pathophys
cerebral aneurysm
Others include: hepatic, pancreatic and seminal vesicle cyst; cardiac valve disease (MVP, Aortic Regurge); colonic diverticulal abd wall hernia
Pathophys: defective epithelial cell differentiation or extracellular matrix function associated with genetic abnormality in ADPKD
First dx test to check if children have ADPKD (if they have positive family hx)
Renal US (to look for cysts): need 3 unilateral or bilateral kidney cysts. PPV 100%, sensitivity 82-96%.
If there is a murmur: need echo
Most common manifestation of ARPKD and how to diagnose
liver abnormalities (congenital hepatic fibrosis, portal HTN, perilobular fibrosis and ‘Caroli Syndrome’)
Dx: antenatal US at 24wks gestation
Causes of UTI in prepubertal girl >2yo
posterior urethral valves
+ foreign body or labial adhesions
Most common pathogen causing lower urinary tract infections
E. Coli
Bartter Syndrome
Syndrome caused by defect in NaCl reabsorption in medullary thick ascending limb of loop of Henle
Similar to chronic furosemide therapy
Presents as growth restriction, hypoK, metabolic alkalosis and polyuria
volume depletion from salt wasting
Gitelman Syndrome
Syndrome cause by mutation in gene coding for thiazide-sensitive NaCl transporter in DISTAL TUBULE
Causes hypo K, polyuria, polydipsia
Presents in late in childhood or adulthood with muscle cramps
Reduced urinary calcium and hypomagnesemia
** volume depletion** from salt wasting