Abstract
Progressive familial intrahepatic cholestasis (PFIC) comprises a heterogeneous group of disorders defined by specific mutations in bile salt export proteins and these disorders are further differentiated from Alagille syndrome and other cholestatic disease processes according to their clinical and diagnostic characteristics. Pruritus is a common and debilitating condition associated with cholestasis of any etiology, but pharmacological therapies are limited and often fail. Surgical procedures designed to decrease bile salt load, namely via partial external biliary diversion (PEBD) or ileal exclusion (IE), as well as technical variations and laparoscopic approaches, have been developed as have acceptable therapeutic interventions. Liver transplantation is the only treatment in advanced disease.
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Nguyen, M.V., Wang, K.S. (2017). Congenital Intrahepatic Cholestasis. In: Mattei, P., Nichol, P., Rollins, II, M., Muratore, C. (eds) Fundamentals of Pediatric Surgery. Springer, Cham. https://doi.org/10.1007/978-3-319-27443-0_79
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DOI: https://doi.org/10.1007/978-3-319-27443-0_79
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