Progressive familial intrahepatic cholestasis (PFIC) refers to a group of heterogeneous mostly autosomal recessive disorders resulting from the inability to properly form and excrete bile from hepatocytes. The resulting shared phenotype is one of hepatocellular cholestasis. Clinical management targeting refractory itch and surgical interventions to interrupt the enterohepatic circulation are often pursued with variable efficacy. Recent development of the family of medications referred to as the IBAT inhibitors has introduced a novel tool in the armamentarium for the treatment of PFIC.
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