title: Ileal exclusion in children with progressive familial intrahepatic cholestasis.
abstract: OBJECTIVE
  Children with progressive familial intrahepatic cholestasis (PFIC) rarely benefit from medical treatment and most patients require surgical intervention. Partial external biliary diversion (PEBD) is presently the treatment of choice but for those who cannot benefit from PEBD, an alternative surgical procedure--ileal exclusion (IE)--was introduced. The aim of this study was to analyze our experience with IE in children with PFIC.
  
  
  METHODS
  This procedure was performed in 9 patients (6 girls, 3 boys) at the median age of 11 years (range 8-21). In 4 children, it was the primary operation (group 1), and in 5, IE was performed after PEBD (group 2). All of the patients were screened for ABCB11 and ATP8B1 mutations, and in 3 cases, PFIC type 2 was confirmed.
  
  
  RESULTS
  Median follow-up after IE surgery was 8.5 years (range 3-14). In group 1, 1 patient had to be converted to PEBD and the remaining 3 children experienced alleviation in pruritus and decrease in bilirubin and bile acids concentrations 2 and 5 years after IE. After 10 years, only 2 children were still accessible for follow-up. In both, pruritus varied and elevated serum bile acids were observed. Of the 5 patients who underwent IE after PEBD, 1 eventually required liver transplantation, 1 developed varying degree of pruritus, and 3 female patients, operated on because of aesthetic reasons, had excellent outcomes.
  
  
  CONCLUSIONS
  IE is an alternative rescue option to PEBD and should be offered cautiously, only to patients who cannot benefit from PEBD.
authors.affiliation: *Department of Gastroenterology, Hepatology, and Eating Disorders †Department of Pediatric Surgery and Organ Transplantation, Children's Memorial Health Institute, Warsaw, Poland.
authors.forename: Irena
authors.initials: I
authors.lastname: Jankowska
authors.forename: Piotr
authors.initials: P
authors.lastname: Czubkowski
authors.forename: Piotr
authors.initials: P
authors.lastname: Kaliciński
authors.forename: Hor
authors.initials: H
authors.lastname: Ismail
authors.forename: Adam
authors.initials: A
authors.lastname: Kowalski
authors.forename: Józef
authors.initials: J
authors.lastname: Ryżko
authors.forename: Joanna
authors.initials: J
authors.lastname: Pawłowska
mesh_terms: D000293:Adolescent; D001647:Bile Acids and Salts / Q000097:blood / Q000235:genetics*; D001663:Bilirubin / Q000097:blood / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000150:complications / Q000235:genetics / Q000601:surgery*; D013505:Digestive System Surgical Procedures; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007082:Ileum / Q000601:surgery*; D007223:Infant; D008099:Liver / Q000473:pathology* / Q000601:surgery; D016031:Liver Transplantation; D008297:Male; D009154:Mutation; D011537:Pruritus / Q000209:etiology / Q000235:genetics / Q000517:prevention & control*; D016896:Treatment Outcome
chemical_list: D001647:Bile Acids and Salts; D001663:Bilirubin