Definition:

It is a hereditary disorder in which kidneys are completely cystic with intra-renal parenchyma almost completely obliterated.

Type:

The commonest type is adult polycystic kidney disease has autosomal dominant inheritance and manifests at any age, infantile polycystic disease is a rare developmental anomaly, invariably bilateral and has autosomal recessive inheritance.

Clinical Features:

(1) Bilateral renal swelling in a man with o without dragging pain in the loin ( sometimes pain in renal angles). (2) Haematuria. (3) Uraemic symptoms. (4) Slowly progressive hypertension. (5) Kidneys palpable with nodular surface. (6) When renal failure occurs, polyuria with urine of fixed specific gravity. (7) Urine: Low specific gravity with albumin and a few casts, (8) I.V.P. Attenuated and elongated (spider leg) calyces with terminal clubbing or cupping

Causes:

(1) Uraemia. (2) Cerebrovascular accident. (3) Cardiac failure.

Treatment:

There is no curative treatment. Management and at combating infection, uraemia and acidosis. In case of severe uncontrolled haemorrhage and abscess formation, the troubling kidney is removed.