Nontransplant surgical interventions in progressive familial intrahepatic cholestasis

摘要: Background: Progressive familial intrahepatic cholestasis (PFIC) is a family of rare childhood diseases that was universally fatal until the development liver transplant. In last 20 years, use nontransplant surgery to treat PFIC has become standard care. There are various surgical techniques have been performed. no reviews evaluating outcome these operations. Methods: A systematic search literature for articles interventions in patients Data from studies abstracted and summarized. Results: No trials performed addressing patients. We analyzed 11 case series reports. Generally, had successful outcomes (81%) with cessation progression disease resolution symptoms. Treatment failures were often associated more advanced disease. Discussion: evidence demonstrate superiority one type intervention propose registry standardization measurements allow improved comparison results.