Polycystic Kidney Disease

Question 1

What is PKD?

Answer 1

An autosomal dominant inherited condition where clusters of cysts develop within the kidneys

Question 2

What mutation causes PKD?

Answer 2

PKD1 and 2 but mainly 1

Question 3

Where do cysts grow?

Answer 3

Tubular portion of the nephron

Question 4

What do cysts do?

Answer 4

Compress renal architecture and vasculature

Question 5

What are risk factors for PKD?

Answer 5

FH of PKD or cerebrovascular events

Question 6

How does PKD present?

Answer 6

HTN, abdo/flank pain, headaches, LUTS, palpable

Question 7

What is the gold standard investigation?

Answer 7

Renal USS and biopsy

Question 8

What is the diagnostic criteria for <30?

Answer 8

2 unilateral or bilateral cysts

Question 9

What is the diagnostic criteria for 30-59?

Answer 9

2 cysts in each kidney

Question 10

What is the diagnostic criteria for 60+?

Answer 10

4 cysts in each kidney

Question 11

How do you treat complications?

Answer 11

HTN - medication and lifestyle, infection - antibiotics or drain

Question 12

How do you surgically treat PKD?

Answer 12

Removal - nephrectomy

Question 13

How do you manage chronic PKD?

Answer 13

Dialysis or transplant

Question 14

What is PKD associated with?

Answer 14

Berry aneurysms, HTN, kidney stones, polycystic liver disease