Congenital Hepatic Fibrosis and Asymptomatic Familial Adult-Type Polycystic Kidney Disease in a 19-Year-Old Woman

Congenital hepatic fibrosis has been associated with a variety of renal malformations, but rarely adulttype polycystic kidneys. The case of a 19-yr-old woman with congenital hepatic fibrosis associated with asymptomatic familial adult-type (autosomal dominant) polycystic kidney disease is described. A literature review revealed seven other reports of this association. Our patient differs because the association between congenital hepatic fibrosis and adulttype polycystic kidney disease is clear, and because her asymptomatic kidney disease accords with the later onset of symptomatic polycystic kidney dis-, ease in her family.