Congenital

Question 1

Dysplastic Kidney (what it is, inheritance, way to differentiate from PKCD)

Answer 1

Malformation of renal parenchyma w/ cysts and abnormal tissue (cartilage and shit), NONINHERITED so not low risk in subsequent pregnancies, unilateral instead of bilateral

Question 2

PKCD (just generally what it is/location)

Answer 2

Inherited defect with bilateral enlargement of kidneys from cysts in renal cortex and medulla

Question 3

Autosomal Recessive PKCD (when and how it presents (renal and extra-renal))

Answer 3

In newborns, presents with worsening renal failure and HTN even possibly Potter sequence. Also congenital hepatic fibrosis leads to portal HTN and hepatic cysts

Question 4

Autosomal Dominant PKCD (when and how it presents (3), and 2 causes of death)

Answer 4

In young adults bc cysts take time to form
Presents as HTN with increased renin, hematuria, and worsening renal failure
Associated with berry aneurysm (#1 cause of death) and mitral valve prolapse

Question 5

2 Differences in Medullary Cystic Kidney Disease from PKCD

Answer 5

Cysts in medullary connecting duct

Fibrosis leads to shrunken kidneys