PKHD1 and autosomal recessive polycystic kidney disease: Fibropolycystic liver diseases, which encompass a number of genetic conditions with biliary dysgenesis such as autosomal recessive polycystic kidney disease (ARPKD), CHF, and Caroli's disease (CD), are caused by mutations in the polycystic kidney and hepatic disease 1 (PKHD1), the gene encoding for fibrocystin/polyductin (FPC) (65, 66).