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 adult-type 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 adult-type polycystic kidney disease is clear, and because her asymptomatic kidney disease accords with the later onset of symptomatic polycystic kidney disease in her family.
D000328:Adult; D005260:Female; D006801:Humans; D008099:Liver / Q000473:pathology; D008107:Liver Diseases / Q000150:complications / Q000151:congenital* / Q000473:pathology; D008297:Male; D007690:Polycystic Kidney Diseases / Q000150:complications / Q000235:genetics*