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polycystic kidney disease
either of two unrelated hereditary diseases in which there is massive enlargement of the kidney with cyst formation. It occurs in two forms, distinguished by age of onset and other characteristics. Autosomal dominant polycystic kidney disease (ADPKD) is the most common type, usually becoming evident during the third decade of life. Renal failure may appear by the fifth decade, with terminal failure occurring in the next ten years, although in some cases it never appears.
Although there is rarely any liver dysfunction with this disorder, sometimes cysts form in the liver. Autosomal recessive polycystic kidney disease (ARPKD) is diagnosed at birth or in the first ten years of life and is much less common than the autosomal dominant form.
Both the kidney and the liver are involved, causing renal failure and liver failure with portal hypertension. Characteristic symptoms early in the process include pain, hematuria, urinary tract infection, kidney stones, and obstructive uropathy with cessation of urination.
Copyright 2007. An Elsevier publication. All rights reserved.
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