Autosomal-recessive inheritance of benign recurrent intrahepatic cholestasis

Benign recurrent intrahepatic cholestasis BRIO is a rare disorder characterized by recurrent episodes of cholestasis without permanent liver damage. Familial and sporadic cases have been described. Based on existing evidence, both autosomal-recessive and autosomal-dominant inheritance have been considered. We describe a large Dutch pedigree with 4 patients, strongly suggesting autosomal-recessive inheritance. © 1995 Wiley-Liss, Inc.