title: Factors influencing jaundice-free survival with the native liver in post-portoenterostomy biliary atresia patients: results from a single institution.
abstract: OBJECTIVE
  We reviewed post-portoenterostomy (PE) biliary atresia (BA) patients who became "jaundice-free" (JF; total bilirubin (T-bil) ≤1.2 mg/dL) to determine factors associated with survival with the native liver (SNL).
  
  
  METHODS
  We reviewed 76 BA patients treated by PE at our institute between 1989, when liver transplantation (LTx) became available in Japan, and 2012, prospectively. Of these, 60 who became JF and remained JF were divided into two groups, SNL (n=44) and LTx (n=16). Age and weight at PE, pre- and post-PE T-bil, AST, γ-GT, time taken to become JF, corticosteroid requirements, incidence of cholangitis, and micro-bile duct size were compared between the two groups.
  
  
  RESULTS
  The SNL patients became JF significantly earlier: 58 vs. 115 days (p<.05). Corticosteroid requirement, cholangitis, and postoperative AST/γ-GT were significantly lower in the SNL patients (p<.05). SNL was significantly higher if patients became JF ≤60 days post-PE (p<.01). LTx was performed from 0.5 to 11 years post-PE (mean=3.4). All patients who had cholangitis within 3 months of PE eventually required LTx (p<.05).
  
  
  CONCLUSIONS
  Becoming JF ≤60 days post-PE would appear to be a factor associated with SNL, while cholangitis within 3 months of PE would appear to be associated with LTx. Elevation of AST and γ-GT would also appear to be early indicators of risk for LTx during follow-up of JF patients after successful PE.
authors: Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, Tokyo 113-8421, Japan. Electronic address: h-koga@juntendo.ac.jp.
authors: Hiroyuki
authors: H
authors: Koga
authors: Momoko
authors: M
authors: Wada
authors: Hiroki
authors: H
authors: Nakamura
authors: Go
authors: G
authors: Miyano
authors: Manabu
authors: M
authors: Okawada
authors: Geoffrey J
authors: GJ
authors: Lane
authors: Tadaharu
authors: T
authors: Okazaki
authors: Atsuyuki
authors: A
authors: Yamataka
mesh_terms: D001656:Biliary Atresia / Q000150:complications / Q000601:surgery*; D002761:Cholangitis / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007565:Jaundice / Q000209:etiology*; D053208:Kaplan-Meier Estimate; D017093:Liver Failure / Q000209:etiology / Q000601:surgery; D016031:Liver Transplantation; D008297:Male; D011171:Portoenterostomy, Hepatic; D011183:Postoperative Complications; D012086:Reoperation; D016896:Treatment Outcome
keywords: Biliary atresia; Jaundice-free; Liver transplantation; Portoenterostomy; Survival with native liver