Renal/Adrenal/GU Flashcards

1
Q

What is pseudohypoaldosteronism?

  • what metabolic abnormalities are there?
  • how do you treat?
A

The kidney is unresponsive to aldosterone.

  • Hyponatremia, hyperkalemia, metabolic acidosis
  • high renin and aldosterone levels
  • high urine sodium
  • Tx: salt supps, K binder
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2
Q

What is Bartter syndome?

  • how does it present?
  • what metabolic abnormalities are there?
  • how do you treat?
A

Autosomal recessive defects in Na, K, Cl transporters

  • severe polyhydramnios prior to delivery, severe polyuria after. Type IV presents with deafness.
  • Hyponatremia, hypokalemia, metabolic alkalosis (high bicarb)
  • Tx: salt, water, indomethacin for type IV
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3
Q

What is proximal RTA?

  • type?
  • defect?
  • is urine acidotic or alkalotic?
  • associated with?
  • treatment?
A
  • Type II
  • unable to resorb HCO3, so it gets excreted in the kidneys
  • happens proximally, so kidney is still able to acidify urine distally
  • associated with polyuria and Fanconi (everything is lost - phosphate, glucose, amino acids)
  • Tx: bicarb
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4
Q

What is distal RTA?

  • type?
  • defect?
  • is urine acidotic or alkalotic?
  • associated with?
  • treatment?
A
  • Type I
  • unable to excrete H+ in exchange from K+, so body becomes acidic and hypokalemic
  • urine is v alkalotic
  • associated with nephrocalcinosis and hypokalemia
  • Tx: bicarb
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5
Q

What is type IV RTA?

  • defect?
  • associated with?
  • treatment?
A
  • aldosterone resistance (eg, ENaC mutation, pseudohypoaldosteronism) or deficiency (CAH)
  • associated with acidosis, hyperkalemia, hyponatremia (high urine sodium)
  • Tx: aldosterone if needed, K+ binders, alkali
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6
Q

Prenatal findings of ARPKD, the most commonly inherited kidney problem?
Postnatal symptoms?

A
  • prenatally: oligo, bilateral enlarged kidneys with snowstorm appearance, small cysts confined to the collecting duct
  • postnatally: respiratory distress, abd distention, hepatic fibrosis, severe HTN, and hyponatremia
  • can also have Potter sequence (wide set eyes, small chin, arthrogryposis, club feet, resp failure)
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7
Q

MCC of unilateral hydronephrosis?

A

UPJ

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8
Q

MCC of unilateral hydroureter?

A

UVJ

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9
Q

Rate limiting step of androgen production?

A

Cholesterol into pregnenolone (cyp 11A1)

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10
Q

ARPKD

  • most common symptom? Interestingly, what metabolic derangement is found?
  • death due to what?
A
  • 65% have hypertension, and almost all have hyponatremia

- respiratory failure or sepsis

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11
Q

Driver of testosterone production by leydig cells in:

  • 1st trimester?
  • 2nd trimester?
A
  • placental hcg

- LH

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12
Q

Medications that cause secondary nephrogenic DI?

A

Ampho, dopamine, methicillin

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13
Q

MCC of abdominal mass in newborn?

A
  • hydronephrosis
  • also MCKD, but can involute even prenatally so may not be present at birth
  • can be associated with other urinary tract abnormalities such as VUR
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14
Q

Renal agenesis is when the ____ fails to develop

A

ureteric bud

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15
Q

Uosm and Una in:

  • prerenal
  • renal
A
  • prerenal: high Uosm, low Una, low FeNa

- renal: low Uosm, high Una, high FeNa

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16
Q

If definite cysts are noted before 20 weeks’ gestation, what pathology is most likely?

A

MCDK

17
Q

When does UOP start in the fetus? What percent of amniotic fluid is urine at 20 weeks?

A

UOP starts at 9 weeks, fetal urine is 90% of amniotic fluid at 20 weeks

18
Q

MCC kidney tumor

A

Mesoblastic nephroma/hamartoma

19
Q

Where are the cysts confined to in ARPKD?

A

collecting duct