title: Outcome of early hepatic portoenterostomy for biliary atresia.
abstract: BACKGROUND
  The outcome of the hepatic portoenterostomy (Kasai) procedure for biliary atresia is improved when it is performed before 90 days of age. However, it is not known whether intervention before 30 days is better than intervention between 30 and 90 days.
  
  
  METHODS
  The authors reviewed the records of all patients seen by the Pediatric Gastroenterology Service at St. Louis Children's Hospital from 1984-1999 to ascertain the outcome of patients who underwent Kasai procedure before or after 30 days of age.
  
  
  RESULTS
  Of 92 patients with biliary atresia treated at St. Louis Children's Hospital over 15 years, 9 underwent the Kasai procedure before 30 days of age. Liver transplantation was necessary in 77.8% of these patients at a mean age of 11.0 +/- 4.26 months, as compared with 53.4% at 32.14 +/- 7.14 months for the remainder of the patients who underwent the procedure after 30 days of age.
  
  
  CONCLUSIONS
  Although these data suggest that outcomes are worse for patients who undergo the procedure before 30 days of age, they may reflect a difference in the pathogenesis of biliary atresia that brings it to clinical attention earlier and may provide further evidence that biliary atresia is a phenotype for a number of distinct underlying disease processes.
authors.affiliation: Department of Pediatrics, Washington University School of Medicine, St Louis Children's Hospital, Missouri 63110, USA.
authors.forename: D
authors.initials: D
authors.lastname: Volpert
authors.forename: F
authors.initials: F
authors.lastname: White
authors.forename: M J
authors.initials: MJ
authors.lastname: Finegold
authors.forename: J
authors.initials: J
authors.lastname: Molleston
authors.forename: M
authors.initials: M
authors.lastname: Debaun
authors.forename: D H
authors.initials: DH
authors.lastname: Perlmutter
mesh_terms: D000367:Age Factors; D001656:Biliary Atresia / Q000150:complications / Q000601:surgery*; D001706:Biopsy; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008099:Liver / Q000473:pathology; D016031:Liver Transplantation; D011171:Portoenterostomy, Hepatic / Q000379:methods*; D012189:Retrospective Studies; D016896:Treatment Outcome