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Definition

An autosomal dominant disorder characterized by progressive formation and enlargement of cysts in both kidneys, typically leading to end-stage renal disease in adult life. Cysts also occurs in the liver and other organs. It represents approximately 15% of the cases of autosomal dominant polycystic kidney disease. PKD2 is clinically milder than PKD1 but it has a deleterious impact on overall life expectancy.

Acronym

PKD2

Synonyms

ADPKD2
Adult polycystic kidney disease type 2
Autosomal dominant polycystic kidney disease 2
PKD-2
Polycystic kidney disease adult type II

Keywords

Ciliopathy

 
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