Natural history of autosomal dominant polycystic kidney disease.
At least two different genes, which have been mapped to chromosomes 4 and 16, cause autosomal dominant polycystic kidney disease, a disorder with renal and extrarenal manifestations. Although gene-linkage testing is possible, the disease is diagnosed mainly through ultrasonography. Renal disease is characterized clinically by hypertension, acute and chronic pain, and variable progression to end-stage renal disease. Extrarenal manifestations include liver cysts, which may lead to complications; ruptured intracranial aneurysms; cardiac valvular disease; colonic diverticula; and inguinal hernias. Disease management is directed at minimizing and treating the complications of this illness.
Fick
G M
GM
University of Colorado Health Sciences Center, Denver 80262.
Gabow
P A
PA
D003560:Cysts / Q000503:physiopathology; D008040:Genetic Linkage; D006801:Humans; D006973:Hypertension / Q000503:physiopathology; D007676:Kidney Failure, Chronic / Q000503:physiopathology; D008107:Liver Diseases / Q000503:physiopathology; D016891:Polycystic Kidney, Autosomal Dominant / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*; D014463:Ultrasonography