Follow‐up in Children With Progressive Familial Intrahepatic Cholestasis After Partial External Biliary Diversion

• DOI: 10.1097/mpg.0b013e3181df99d5
• Publication Date: 2010-07-29T21:08:14Z
• Authors: Henrik Arnell; Nikos Papadogiann...; Helen Zemack; Alex S Knisely; Antal Németh; Björn Fischler
• Keywords: Adult; Male; Adolescent; Infant, Newborn; Infant; Cholestasis, Intrahepatic; Fibrosis; Severity of Illness Index; 3. Good health; Biliary Tract Surgical Procedures; Young Adult; 03 medical and health sciences; Bile Ducts, Intrahepatic; Treatment Outcome; 0302 clinical medicine; Child, Preschool; Humans; Female; Child; Follow-Up Studies

ABSTRACT Objectives: The aim of this study was to examine whether reversion histological fibrosis followed partial external biliary diversion (PEBD) in patients with progressive familial intrahepatic cholestasis (PFIC); the duration cholestatic episodes after PEBD influenced evolution fibrosis; and genotyping helpful predicting outcome PEBD. Patients Methods: Children PFIC who underwent were investigated genetic, biochemical, anthropometric standard methods. Serial liver specimens assessed histologically without knowledge genotype outcome. Findings evaluated contexts total clinical Results: From a 18 children PFIC, 13 PEBD, 12 these (among them 10 identified ABCB11 mutations) amenable for follow‐up. When compared baseline at statistically significant reductions found 1 3 years 5 >10 relative positively correlated severity fibrosis. homozygous missense mutation c.890A>G responded well Conclusions: Biliary should be regarded as first choice surgical treatment noncirrhotic severe disease may also efficacious other forms PFIC.