Renal

Question 0

Multicystic Dysplastic Kidney

Answer 0

Non functioning kidney consists of multiple unilateral non communicating cyst
Nephrectomy is preferred treatment
Increased risk of wilms tumor

Question 1

Renal Agenesis

Answer 1

Failure of kidneys to form- oligohydramnios, pulmonary hypoplasia and often death

Question 2

AR - PKD

Answer 2

Presents early in life
Symmetrically enlarged reniform
Dilated collecting ducts leading to global loss of function
Palpable abdominal mass in children

Question 3

AD - PKD

Answer 3

Presents later in adulthood
Can be detected with + FHx
Cysts can distort reniform shape
Transplant is viable

Question 4

Ureteropelvic Junction Obstruction

Answer 4

Primary: intrinsic narrowing, vascular compression
Secondary: scarring, angulation due to dilation
Leads to hydronephrosis - palpable abdominal mass, + fetal US, flank pain, hematuria; can destroy parenchyma
Surgical correction

Question 5

Vesicoureteral Reflux

Answer 5

Length of intravesical ureter is insufficient to prevent reflux
Presents as recurrent UTI or pyelonephritis – recurrent UTI and UTI < 24 mo gets US/VCUG
VCUG is preferred test for VUR
PPX abx, possible surgery

Question 7

Posterior Urethral Valves

Answer 7

Males only
Prevents normal outlet and can lead to, urethral and bladder dilation - VCUG is good Dx modality
MCC of ESRD in male children
Present with abdominal mass, abnormal voiding/VUR, hydronephrosis + bladder distention

Question 8

Hypospadias

Answer 8

Urethral meatus is ventrally located
Circumcision is contraindicated
Surgical correction
Hypospadia + Cryptoorchidism –> suspicious for ambiguous genitalia

Question 9

UTI

Answer 9

Ascending or hematogenous spread - E.coli, Proteus, Klebsiella
Risks: anatomic abn., VUR, dysf(x) voiding
Key workup in r/o sepsis in child < 2 yo
Recurrent UTI in anyone or < 24 mo - renal US +/- VCUG

Question 10

Pediatric Nephrotic Syndrome

Answer 10

Proteinuria (>3.5g/day), hypoalbuminemia, hyperlipidemia
MC present with periorbital edema, lower extremity edema, weight gain, ascites
MCD is MCC (then FSGS, MPGN, MN)
Steroids + Na restriction - refrx cases get renal bx + ImmSupp Tx
SBP is common complication from imm.supp.

Question 11

Pediatric Nephritic Syndrome

Answer 11

Hematuria, RBC casts, edema, HTN, Azotemia
-APGN: 1-2wks post infection (skin or pharyngitis), tx doesn’t prevent APGN, low C3, C3 and IgG humps below BM

• IgA neph.: 1-2days post URI, IgA deposition with normal C3 levels; IgA nephropathy + extrarenal sx (GI, joint, Skin) = HSP
• Alport Syn: T4 Collagen defect, split BM, assoc. with sensorineural hearing loss

Question 12

RTA - Type 1

Answer 12

“Distal”
Defect in collecting tubule H+ secretion; urine pH > 5.5, HypoK, higher risk of CaPO4 stones
Can present as FTT
Tx with alkalinizing agent (bicarb, citrate) correct metabolic disturbances

Question 13

RTA - Type 2

Answer 13

“Proximal”
Defect in proximal tubule bicarb reabsorption, urine pH <5.5, HypoK, Fanconi Anemia (general loss of bicarb, glucose, AA, Uric Acid, PO4) associated, increased risk of hypophosphatemic rickets
Can Present as FTT

Tx with alkalinizing agent (bicarb, citrate) correct metabolic disturbances, thiazide diuretics are useful to increase proximal bicarb uptake

Question 14

RTA - Type 4

Answer 14

“HyperK” – MC
HypoAldo/Loss of response to Aldo, impaired ammoniagenesis in proximal tubule 2/2 hyperK, poor buffering capacity in urine –> low urine pH

Tx with alkalinizing agent (bicarb, citrate) correct metabolic disturbances

Question 15

PreRenal AKI

Answer 15

2/2 renal hypoperfusion

BUN:Cr > 20, oliguria, FENa < 1%

Question 16

Intrinsic AKI

Answer 16

2/2 ATN (which itself can be a result of PreRenal AKI, toxins), sepsis, nephrotoxic meds (vanc, AGs), acute GN
Epithelial necrosis leads to tubular obstruction
Poor GFR
Epithelial casts in urine
BUN:Cr 2%

Question 17

PostRenal AKI

Answer 17

2/2 outflow obstructions (stones, BPH, neoplasia, posterior urethral valves, other anatomic abnormalities)
Only develops with bilateral obstruction
BUN:Cr > 15, FENa > 2%

Question 18

Enuresis

Answer 18

Urinary control usu. established by 2-3yo
Persistent incontinence > 5yo should be w/u
Primary (never had control), secondary (had control and lost), daytime/nighttime
Beh. Mod, nocturnal enuresis alarms, PO ddAVP (intranasal - increased risk of hyponatremic seizures), refrx patients can get imipramine or oxybutinin

Question 19

Calcium Nephrolithiasis

Answer 19

Most common stone — colorless octahedral crystal
PO4 ppt @ high pH; Oxalate ppt @ low pH
Radiopaque
Conditions that cause hyperCa (cancer, high PTH), conditions that increase oxalate absorption (Crohns), vitamin C abuse
Tx with thiazides or citrate

Question 20

Nephrolithiasis

Answer 20

Calcium, struvite (ammonium magnesium phosphate), uric acid, cystine
Increase fluid intake

SSx: colicky pain, hematuria, flank pain, n/v

Question 21

Struvite Nephrolithiasis

Answer 21

Ppt at high pH
Radiopaque
Recurrent infections with urease + bacteria (Proteus, Staph, Klebsiella) —hydrolyze urea to ammonia which alkalinizes urine
Staghorn calculi –> hydronephrosis necessitating surgical removal

Question 22

Uric acid nephrolithiasis

Answer 22

Ppt at low pH
RadiolUcent
Assoc. with hyperuricemia (gout, leukemia, tumor lysis syndrome)
Alkalinize urine – bicarbonate, acetazolamide, allopurinol
Arid climate, acidic urine, and low urine volume are risks

Question 23

Cystine nephrolithiasis

Answer 23

Ppt at low pH 
Radiopaque 
Secondary to cystinuria --- NaCN added to urine --> turns red purple --> + urine cystine 
Hexagonal crystals 
Alkalinize urine and increase volume

Question 24

Ureterolithiasis

Answer 24

Obstructive stone in the ureters or utreterovesical junction
Stabbing pain that radiates to the perineum, n/v, writhing in pain

NonCon spiral CT is best modality (US if CT not available or pregnant)