*Purpose: Progressive familial intrahepatic Cholestasis (PFIC) is a heterogeneous autosomal recessive disease characterized by progressive cholestasis, pruritus leading to liver injury. Surgical therapy aims in preventing bile absorption either by external or internal biliary diversion. Three different genetic subtypes encode for defects in bile transport proteins. Since PFIC type 3 progresses to cirrhosis, this study describes the outcome of biliary diversion in the PFIC II and I patients.

*Methods: Clinical data and laboratory findings of all PFIC Patient (n=33), who were treated and managed in our hospital between 2000 and 2020, were analyzed retrospectively.

*Results: We treated 33 patients, PFIC I (n=9), II (n=17) and III (n=7). Biliary diversion was performed in 12 patients PFIC I (n = 6) and II (n =6)]. Bile acids (BA), bilirubin and liver enzymes were elevated in all patients. After biliary diversion BA, bilirubin and triglyceride levels significantly decreased only in PFIC I patients. Four PFIC I Patients did not require liver transplantation. Liver transplantation was necessary in two children because of stoma complications liver failure. Five out of six PFIC II patients, who had biliary diversion, needed a liver transplantation. One child with normal liver values underwent biliary diversion due to itching, which improved transiently after the operation and then reoccurred in the short term.

*Conclusions: Only PFIC I patients showed a significant decrease in bile acids, bilirubin levels as well as triglycerides and benefited from biliary diversion. The type of membrane transporter defect underlying the different PFIC subtypes could further explain this.

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