Cystic renal disease

Question 1

Renal cysts aetiology / epidemiology

Answer 1

common w inc age
asymptomatic and incidental discovery on US
may cause pain or haematuria
CKD may = cysts - can be premalignant

Question 2

autosomal-dominant polycystic kidney disease

Answer 2

common
multiple cysts throughout both kidneys
inc in size w advancing age = destruction of kidney tissue = loss of renal function

Question 3

gene involved autosomal-dominant polycystic kidney disease

Answer 3

mutation in PKD1 gene on short arm of chromosome 16

Question 4

clinical features autosomal-dominant polycystic kidney disease

Answer 4

acute loin pain from cyst haemorrhage or infection or urinary tract stone formation
HTN
renal failure
liver cysts
subarachnoid haemorrhage 
mitral valve prolapse

Question 5

diagnosis autosomal-dominant polycystic kidney disease

Answer 5

clinical exam = large irregular kidneys, HTN, hepatomegaly

USS = definitive

Question 6

management autosomal-dominant polycystic kidney disease

Answer 6

no rx has decreased cyst size or slow disease progression
BP should be controlled and serum creatinine regularly monitored
most pts will need dialysis / transplant