Renal/Adrenal/GU Flashcards
What is pseudohypoaldosteronism?
- what metabolic abnormalities are there?
- how do you treat?
The kidney is unresponsive to aldosterone.
- Hyponatremia, hyperkalemia, metabolic acidosis
- high renin and aldosterone levels
- high urine sodium
- Tx: salt supps, K binder
What is Bartter syndome?
- how does it present?
- what metabolic abnormalities are there?
- how do you treat?
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
What is proximal RTA?
- type?
- defect?
- is urine acidotic or alkalotic?
- associated with?
- treatment?
- 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
What is distal RTA?
- type?
- defect?
- is urine acidotic or alkalotic?
- associated with?
- treatment?
- 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
What is type IV RTA?
- defect?
- associated with?
- treatment?
- aldosterone resistance (eg, ENaC mutation, pseudohypoaldosteronism) or deficiency (CAH)
- associated with acidosis, hyperkalemia, hyponatremia (high urine sodium)
- Tx: aldosterone if needed, K+ binders, alkali
Prenatal findings of ARPKD, the most commonly inherited kidney problem?
Postnatal symptoms?
- 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)
MCC of unilateral hydronephrosis?
UPJ
MCC of unilateral hydroureter?
UVJ
Rate limiting step of androgen production?
Cholesterol into pregnenolone (cyp 11A1)
ARPKD
- most common symptom? Interestingly, what metabolic derangement is found?
- death due to what?
- 65% have hypertension, and almost all have hyponatremia
- respiratory failure or sepsis
Driver of testosterone production by leydig cells in:
- 1st trimester?
- 2nd trimester?
- placental hcg
- LH
Medications that cause secondary nephrogenic DI?
Ampho, dopamine, methicillin
MCC of abdominal mass in newborn?
- 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
Renal agenesis is when the ____ fails to develop
ureteric bud
Uosm and Una in:
- prerenal
- renal
- prerenal: high Uosm, low Una, low FeNa
- renal: low Uosm, high Una, high FeNa