Abstract
Intractable pruritus due to an accumulation of bile acids in the circulation is the most debilitating symptom of Alagille syndrome (AGS), leading to a markedly impaired quality of life.[1] [2] Some patients are refractory to conventional medical therapy.[1] [3] In these patients, effective treatment requires surgical intervention, either biliary diversion[1] [4] [5] or liver transplantation.[2] Liver transplantation is also considered to treat AGS-related symptoms even in the absence of liver failure or cirrhosis,[1] [2] [4] although only 10% of AGS patients progress to end-stage liver disease.
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