Association of bilateral renal dysplasia and congenital hepatic fibrosis.

A young baby with jaundice and failure to thrive was found to have severe chronic renal failure and chronic cholestatic liver disease. She was the first child of healthy unrelated parents and died at the age of three months. Autopsy revealed bilateral renal dysplasia and congenital hepatic fibrosis. This particular association of kidney and liver disease has only rarely been observed. It must be differentiated from autosomal recessive polycystic liver and kidney disease.