Fetopathology and developmental pathology of the embryo and fetus: Adult polycystic kidneys (ADPKD)

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Adult polycystic kidneys (ADPKD)

Etiology

inherited as an autosomal dominant trait
in 90% of cases the affected gen is located on the chromosome 16
frequency of disease is 1 : 1000
most patients present in adulthood with hypertension and progressive renal failure at the age of 30 to 40 years
extrarenal symptoms inculde hepatic and pancreatic cysts and intracranial berry aneurysms, hemorrhage from ruptured aneurysm can be a cause of death in patients with ADPKD.

Macroscopic appearance

Both kidneys are enlarged and deformed by large cysts of several centimetres in size, which compress the intervening parenchyma.

Polycystická ledvina (72873)

Polycystická ledvina (72877)

Pictures

Histology

Cysts arise in any portion of the nephron and collecting system, although they show predilection for Henles loop and collecting tubules.

Autosomal dominant polycystic kidney disease in infant and fetus