title: Follow-up in children with progressive familial intrahepatic cholestasis after partial external biliary diversion.
abstract: OBJECTIVE
  The aim of this study was to examine whether reversion of histological fibrosis followed partial external biliary diversion (PEBD) in patients with progressive familial intrahepatic cholestasis (PFIC); whether the duration of cholestatic episodes after PEBD influenced the evolution of fibrosis; and whether genotyping was helpful in predicting outcome of PEBD.
  
  
  METHODS
  Children with PFIC who underwent PEBD were investigated with genetic, biochemical, and anthropometric standard methods. Serial liver specimens were assessed histologically without knowledge of genotype and outcome. Findings were evaluated in the contexts of the total duration of cholestasis and the clinical outcome after PEBD.
  
  
  RESULTS
  From a total of 18 children with PFIC, 13 underwent PEBD, and 12 of these (among them 10 with identified ABCB11 mutations) were amenable for clinical and histological follow-up. When compared with baseline at PEBD, statistically significant reductions were found in histological cholestasis 1 and 3 years after PEBD, and in fibrosis 5 and >10 years after PEBD. The relative duration of cholestatic episodes after PEBD was positively correlated with the severity of fibrosis. Children homozygous for the missense mutation c.890A>G in ABCB11 responded well to PEBD.
  
  
  CONCLUSIONS
  Biliary diversion should be regarded as the first choice of surgical treatment in noncirrhotic patients with severe ABCB11 disease and may also be efficacious in other forms of PFIC.
authors.affiliation: Department of Pediatrics, CLINTEC, Karolinska Institutet, Karolinska University Hospital, Huddinge, Stockholm, Sweden. henrik.arnell@karolinska.se
authors.forename: Henrik
authors.initials: H
authors.lastname: Arnell
authors.forename: Nikos
authors.initials: N
authors.lastname: Papadogiannakis
authors.forename: Helen
authors.initials: H
authors.lastname: Zemack
authors.forename: Alex S
authors.initials: AS
authors.lastname: Knisely
authors.forename: Antal
authors.initials: A
authors.lastname: Németh
authors.forename: Björn
authors.initials: B
authors.lastname: Fischler
mesh_terms: D000293:Adolescent; D000328:Adult; D001653:Bile Ducts, Intrahepatic / Q000601:surgery*; D001662:Biliary Tract Surgical Procedures / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000473:pathology / Q000601:surgery; D005260:Female; D005355:Fibrosis; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012720:Severity of Illness Index; D016896:Treatment Outcome; D055815:Young Adult