Polycystic Kidney Disease (3)

Question 1

What is this?

What type of inheritance is it?

What is it associated with?

Answer 1

➊ Genetic condition, causing multiple cysts to form in kidneys, therefore significantly impairing its function

➋ Can be autosomal dominant or recessive, but the dominant type is more common

➌ Hepatic cysts (most common extra-renal manifestation) and Cerebral berry aneurysms

N.B. These anuerysms most commonly form at the junction between the anterior communicating artery and anterior cerebral artery.

Question 2

How can it present?

What investigation is done?

Answer 2

➊ • HTN
• Flank pain and haematuria with cyst rupture
• Flank pain, fever and urinary symptoms with cyst infection

➋ Renal US

Question 3

How is it managed?

What are the complications?

Answer 3

➊ • Manage CKD and HTN
• Tolvaptan (ADH receptor antagonist), which slows down cyst development and progression of renal decline

➋ • Cyst rupture
• Cyst infection
• CKD
• Subarachnoid haemorrhage due to berry aneurysm