Pathoma: Congenital [Kidney Problems]

Question 1

__________ is the most common kidney abnormality.

Answer 1

Horseshoe kidney

Question 2

Horseshoe kidneys usually get stuck on ___________.

Answer 2

the inferior mesenteric artery

Question 3

In unilateral renal agenesis, the existent kidney usually ___________.

Answer 3

gets hypertrophied; the excess filtration increases the risk of renal failure

Question 4

Why is there lung hypoplasia in bilateral renal agenesis?

Answer 4

Because the lungs need fluid to stretch out, so without kidneys (and the fluid they would filter out), the lungs don’t develop

Question 5

What is the Potter sequence?

Answer 5

Oligohydramnios leads to (1) lung hypoplasia, (2) flat face, (3) low-set ears, and (4) malformation of the hands

Question 6

Dysplastic kidney is not ___________.

Answer 6

inherited

Question 7

Polycystic kidney disease is an ____________.

Answer 7

inherited defect

Question 8

PKD is __________.

Answer 8

bilateral

Question 9

Autosomal recessive PKD is associated with two other pathophysiologies: ___________.

Answer 9

hepatic fibrosis and cysts

Question 10

Autosomal recessive PKD usually presents in ___________.

Answer 10

infancy

Question 11

The autosomal dominant form of PKD presents in __________.

Answer 11

young adulthood (ADult)

Question 12

What other pathophysiologies are associated with autosomal dominant PKD?

Answer 12

Berry aneurysms
Hepatic cysts
Mitral valve prolapse

Question 13

What inheritance pattern is medullary cystic kidney disease?

Answer 13

Autosomal dominant