Nephology Flashcards

1
Q

Newborn presents with:

  • bilateral renal cysts and enlarged kidneys without dysplasia.
  • history of oligohydramnios and pulmonary hypoplasia
  • Renal ultrasonography:
    • bilaterally enlarged kidneys with poor corticomedullary differentiation.
    • The renal cysts cannot be visualized on ultrasonography.

What’s the diagnosis

A

autosomal recessive polycystic kidney disease (ARPKD)

  • mutations in the gene PKHD1
    • encodes a protein expressed in the cilia of renal tubular and hepatic bile duct epithelial cells.
  • 50% chance of developing end-stage renal disease
  • Associated with congenital hepatic fibrosis, abnormalities of bile ducts
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2
Q

Newborn presents:

  • oligohydramnios
  • pulmonary hypoplasia
  • renal failure
  • Ultrasound: bilateral hydronephrosis
  • VCUG: dilated bladder and prostatic urethra

What’s the diagnosis?

A

Posterior urethral valves

  • Diagnosis: Voiding cystourethrography
  • Treatment:
    • urinary catheter to relieve the obstruction
    • ablation of the valves
  • Prognosis: Chronic kidney disease

Antenatal management: none
Buzzwords for UTZ- distended bladder, poor emptying at 30 mins, keyhole appearance

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3
Q

Gestational age nephrogenesis continues

A

until 36 weeks

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4
Q

A 3.5 kg, 6-week-old boy is diagnosed with pyloric stenosis after a 3-day history of vomiting. Serum electrolytes show sodium 132 mEq/L, potassium 2.8 mEq/L, chloride 96 mEq/L, and bicarbonate 27 mEq/L. What is the most appropriate fluid to begin intravenous resuscitation for this patient?

A

Normal saline

  • Prolonged vomiting loses gastric fluid rich in both hydrogen and chloride ions, resulting in both metabolic alkalosis and hypochloremia.
  • most of the potassium deficit is due to urinary losses triggered by secondary hyperaldosteronism.
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5
Q

when to start Na supplement in short bowel syndrome

A

Urine sodium less than 10mEq/L

less Na in distal tubles, limits ability to correct metabolic acidosis

think of contraction alkalosis, hormone: Aldosterone

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6
Q

RTA with nephrocalcinosis (renal stone)

A

RTA type 1

Distal tubule, cannot secrete H+

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7
Q

RTA that affect proximal tubules and cannot reabsorb HCO3

A

RTA type 2

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8
Q

RTA associated with aldosterone (abnormal production or sensitivity), presents with hyperkalemia and hyperchloremia

A

RTA type IV

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9
Q

It is the treatment of choice for DI in neonates

A

Hydrocholorthiazide

Why not vasopressin: difficulty in accurate dosing and variability in the absorption and action of intranasal and oral preparations- may lead to water intoxication and hyponatremia

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10
Q

Different RTAs

A
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11
Q

what are the different congenital renal anomalies

A
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12
Q

the most likely cause of renal agenesis is failure of the development of:

A

ureteric bud

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13
Q

Embryology

Where are the kidneys derived from

A

Metanephros

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14
Q

Embryology

Where does the bladder and urethra come from

A

Cloaca

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15
Q

Quick facts

VUR

A

Gold standard Dx: VCUG
Prognosis: Grade 1-3 spon resolves by 5 y/o
Antibiotic prophylaxis: yes

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