clinical features

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Childhood polycystic disease is, with detailed ultrasound scanning in pregancy, becoming a prenatally diagnosed condition. However it may present at birth with grossly enlarged kidneys that may make delivery difficult, or may develop in early infancy, presenting as an abdominal mass or as poor renal function.

Often, there are concomitant liver cysts and hepatic fibrosis which may lead to portal hypertension and splenomegaly in later life.

Last reviewed 01/2018