Long-Term Colestyramine Treatment Prevents Cholestatic Attacks in Refractory Benign Recurrent Intrahepatic Cholestasis Type 1 Disease.

Benign recurrent intrahepatic cholestasis (BRIC) is an autosomal recessive cholestatic disease characterized by intermittent cholestatic episodes of variable severity and duration. BRIC type 1 has been linked to gene mutations encoded in the hepatocanalicular transporter ATP8B1.1 It has been suggested that ATP8B1 affects the regulation of the farnesoid X receptor, and thus ATP8B1 deficiency could lead to an imbalance between intestinal absorption of bile acids and hepatic secretion, causing bile acid accumulation.2 The latter aggravates hepatocellular dysfunction leading to a vicious cycle.