[ Monash University ]

Museum of Pathology

ADULT POLYCYSTIC DISEASE

ADULT POLYCYSTIC DISEASE

Clinical History

No clinical details are known.

Pathology

The specimen is an enlarged kidney, the parenchyma of which is almost completely replaced by numerous cysts up to 3cm in diameter. The cysts have thin translucent walls and some cysts contain brown mucoid material. The external surface appears lobulated due to multiple projecting cysts.

Note

During life, the cysts gradually increase in size, leading to ischaemia and pressure atrophy of intervening renal tissue, resulting in a gradual deterioration of renal function. Adult polycystic disease of the kidney is an autosomal dominant condition which in more than 50% of cases presents by the age of 40 with symptoms related to uraemia or hypertension. Ultimately, about one-third of patients die from renal failure and one-third from complications of systemic hypertension. Approximately 10% of patients die from subarachnoid haemorrhage, due to rupture of a berry aneurism. Remaining deaths are due to unrelated causes. (Compare with OA 4: childhood polycystic disease).