Autosomal dominant polycystic kidney disease is an important cause of renal failure. It is inherited as an autosomal dominant trait with penetrance approaching 100% in those surviving until their seventh or eighth decade. The condition most usually presents in adult life but may develop at any time, including in utero.
Both kidneys are always affected. Cysts arise from the tubules throughout the nephrons but initially, involve only a portion of them. Renal function is usually preserved until the 4th or 5th decade.
Cysts may arise in other organs, particularly the liver; intracranial berry aneurysms are found in up to 40% of patients, predisposing to subarachnoid haemorrhage.