Nephrology 2

Question 0

Extrarenal findings in adult polycystic kidney disease?

Answer 0

Cerebral aneurysms

Question 1

Autosomal recessive polycystic kidney disease – extrarenal involvement? Management?

Answer 1

Liver involvement (cirrhosis with portal hypertension)

Renal transplantation

Question 2

Symptoms of medullary sponge kidney?

Answer 2

Hematuria, UTI, nephrolithiasis

Question 3

Hereditary renal diseases?

Answer 3

1. Alport’s
2. Multicystic renal dysplasia
3. Infantile PKD
4. Adult PKD
5. Medullary sponge kidney
6. Nephronophthisis-Medullary Cystic Disease Complex

Question 4

Significant versus severe versus malignant hypertension?

Answer 4

Above 95th percentile

Above 99th percentile

End organ damage

Question 5

Specific acid/base disorder in RTA?

Answer 5

Non gap hyperchloremic acidosis

Question 6

Distal RTA – characteristic feature? Causes? Clinical presentation? Treatment?

Answer 6

Inability to excrete acid

Inherited, drugs (amphotericin)

Vomiting, growth failure, nephrolithiasis, nephrocalcinosis

Small doses of oral alkaline

Question 7

Proximal RTA – characteristic feature? Causes? Clinical presentation? Treatment?

Answer 7

Impaired bicarbonate reabsorption

Heavy-metal, gentamicin, Fanconi syndrome

Vomiting, growth failure, muscle weakness

Large doses of oral alkali

Question 8

Type III RTA? Treatment?

Answer 8

Variant of type 1 with bicarbonate wasting

Large doses of oral alkali

Question 9

Type IV RTA – characteristic feature? Causes? Clinical presentation? Treatment

Answer 9

Transient acidosis in infants with hyperkalemia

Obstructive neuropathy, aldosterone deficiency

Failure to thrive

Furosemide to lower potassium, oral alkali

Question 10

Oliguria in children?

Insensible water losses in children?

Answer 10

Urine output <1 mL/kg/hr

300 mL/m²/hr

Question 11

General treatment for patient with renal failure?

Answer 11

1. Restore intravascular volume first
2. Maintain electrolytes
3. Restrict protein intake
4. Dialysis when conservative management fails

Question 12

Medical management of renal failure?

Answer 12

1. Nutritional – avoid phosphorus sodium, potassium. Take phosphate binders and vitamin D analogues
2. Blood-pressure management
3. Anemia – give EPO
4. Growth – give growth hormone
5. Electrolyte management

Question 13

Lab findings in preanal azotemia? Intrarenal failure?

Answer 13

The BUN/creatinine > 20, FEna under 20 specific gravity >1.030, urine osmolality >500,

Decreased urinary B2-Microglobulin, FEna >1%

Question 14

Child with real failure – when to dyalyse? Preferred method of dialysis in children?

Answer 14

GFR is 5-10% of normal; peritoneal dialysis

Question 15

Causes of

1. ureteropelvic junction obstruction?
2. Ureterovesical Junction obstruction?
3. Bladder outlet obstruction?

Answer 15

1. Kinks, fibrous bands, Abarrant blood vessels
2. Megaureter, ureterocele, abnormal insertion of ureter
3. Posterior urethral valves (males), prune belly syndrome

Question 16

Prune belly syndrome?

Answer 16

1. Absence of rectus muscles
2. Bladder outlet obstruction
3. Undescended testicles/Cryptorchidism in males

Question 17

Causes of renal agenesis?

Answer 17

1. Failure of mesonephric duct

2. Failure of metanephric blastema

Question 18

Vesicoureteral reflux - defect? Inheritance? Predisposes to? Outcome in most children? Outcome if severe VUR? Diagnosis? Management?

Answer 18

Urine influxing from bladder into ureters

Autosomal dominant

Pyelonephritis; most have spontaneous resolution

Reflux uropathy which may lead to ESRB

Voiding cystourethrogram

1. Low-dose prophylactic antibiotics
2. Consideration of surgical reimplantation of ureters

Question 19

Grading for vesicoureteral reflux?

Answer 19

Grade 1 – reflux into distal ureter
Grade 2 – reflux into renal pelvis and calyces without dilation
Grade 3 – Reflux into calyces with dilation
Grade 4 – dilation causes clubbed calyces
Grade 5 – gross dilation of entire collecting system

Question 20

Most common kidney stones in children? Conditions associated with urolithiasis?

Answer 20

Calcium salts, uric acid, cysteine, struvite

1. Hypercalcemia
2. hyperoxaluria (Due to Malabsorption)
3. Hyperuricosuria Lesch-Nyhan, gout, leukemia
4. Sistine urea
5. UTI – especially Proteus

Question 21

UTI – epidemiology children?

Critical features in neonates? Older infants? Young children?

Urine sample in neonates/infants versus children?

Diagnostic test?

Answer 21

younger than six months – uncircumcised boys
Older than six months – girls

Neonates – fever, irritability, jaundice
Older infants – fever, vomiting, irritability
Young children – nocturnal enuresis, daytime wetting

1. Neonates/infants – sterile catheterization
2. Older children – clean catch

Gold standard – urine culture

All children with pyelonephritis, recurrent UTI, all males, all girls younger than four with cystitis

Question 22

Urine culture for UTI – significant colony counts if collected by suprapubic aspiration? Sterile urethral catheterization? Clean catch?

Answer 22

Any group

> 10,000

50,000-100,000

Question 23

UTI treatment in symptomatic patients? Neonates? Toxic appearing children?

Duration of treatment for cystitis? Pyelonephritis? Pyelonephritis in infants?

Answer 23

1. Empiric Bactrim if symptomatic
2. If neonates – admit for IV ampicillin/gentamicin
3. If toxic appearing – admitted for IV antibiotic and hydration

7-10 days
14 days
Low-dose prophylactic antibiotics for three months to prevent renal scarring