11 - Polycystic Kidney Disease

Question 1

What is PKD?

Answer 1

• Accounts for 8-10% of CKD
• Most common inherited nephropathy
• Presentation at 30-40 years of age with complications of hypertension, acute loin pain and/or haematuria or bilateral palpable kidneys
• Cysts develop anywhere in the kidney and compress surrounding parenchyma
• Impair renal function

Question 2

How many people does PKD autosomal dominant and recessive affect?

Answer 2

Autosomal dominant form affects 1 in 500 to 1000

Autosomal recessive affects 1 in 20,000 to 40,000

more common in adults

Question 3

What does PKD look like?

Answer 3

• Macroscopically - kidneys large with yellow fluid-filled cysts replacing the parenchyma
• Haemorrhage into the cysts can occur
• Microscopically - cysts are lined by cuboidal epithelium
• Ultrasound or CT scan shows bilateral enlarged kidneys with multiple cysts

Question 4

Where else may patients have cysts?

Answer 4

Liver, pancreas, ovaries, lungs and thyroid

Question 5

What else can PKD be associated with?

Answer 5

Valvular heart disease, diverticular disease and berry aneurism

Question 6

Why do adults with PKD have an increased risk of stroke and cerebral haemorrhage?

Answer 6

Berry aneurysm

Can lead to CKD
Treatment involves controlling BP
Dialysis and renal transplant needed if end-stage renal failure develops

Question 7

How can you manage a patient with PKD?

Answer 7

Control BP
Pain control - clots can cause renal colic
IV fluids to increase urine output and wash clots out of kidney
Informed about macroscopic haematuria
Avoid contact sports

Question 8

Should you scan a young child for PKD?

Answer 8

Not a useful tool unless a large number of family members with the disease are available for linkage
Screen the chld annually for elevated BP or urine dipstick abnormalities
In teens can have USS
Can repeat USS when 30 - if no cysts, APKD virtually excluded