title: The limited prognostic value of liver histology in children with biliary atresia.
abstract: Background and rationale for the study. The aim of the study was to determine the prognostic value of histopathological findings with special care to the severity of liver fibrosis at the moment of hepatoportoenterostomy (HPE) in children with biliary atresia (BA). We performed analysis of 142 wedge liver biopsies taken at the time of HPE. All patients were operated by the same surgical team between 1995 and 2007. According to the outcome 6 months after HPE patients were divided into prognostic groups: group 1-bilirubin level < 2 mg% (n = 65), group 2-bilirubin level > 2 mg% (n = 77). Liver biopsies were re-evaluated according to the extended histopathological protocol and then were compared between the prognostic groups. Survival with native liver (SNL) estimates were performed in regard to severity of liver fibrosis.
  
  
  RESULTS
  Survival with native liver estimates after 2, 5 and 10 years in patients after successful operation were 96%, 91%, 75% vs. 30%, 11%, and 5% if operation failed (p < 0.001). There was no difference between groups in the following variables: fibrosis (p = 0.69), portal inflammation (p = 0.99), lobular inflammation (p = 0.95), cholangiolitis (p = 0.23), accumulation of bile pigments (zone 1:p = 0.49; zone 2:p = 0.51; zone 3:p = 0.48), bile plugs in canaliculi (p = 0.12), bile plugs in ducts (p = 0.32), bilirubinostasis in hepatocytes (p = 0.45), bile ductular proliferation (p = 0.59), ductal plate malformation (p = 0.12), focal necrosis (p = 0.44), giant cell transformation (p = 0.45), haematopoesis (p = 0.52), ductopenia (p = 0.46), microabscesses (p = 0.49), ballooning of hepatocytes (p = 0.08). The actuarial 5/10-year SNL was not dependent on severity of liver fibrosis (log-rank test p = 0.84). The severity of fibrosis corresponded neither with the age at HPE nor with the laboratory findings before operation but increased the risk of portal hypertension.
  
  
  CONCLUSIONS
  Liver histology at the time of HPE is of limited value in prognosis making in BA.
authors.affiliation: Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, The Children's Memorial Health Institute. Warsaw, Poland.
authors.forename: Piotr
authors.initials: P
authors.lastname: Czubkowski
authors.affiliation: Department of Pathology, The Children's Memorial Health Institute. Warsaw, Poland.
authors.forename: Joanna
authors.initials: J
authors.lastname: Cielecka-Kuszyk
authors.affiliation: Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, The Children's Memorial Health Institute. Warsaw, Poland.
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Rurarz
authors.affiliation: Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, The Children's Memorial Health Institute. Warsaw, Poland.
authors.forename: Diana
authors.initials: D
authors.lastname: Kamińska
authors.affiliation: Department of Pediatric Surgery and Transplantation. The Children's Memorial Health Institute. Warsaw, Poland.
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Markiewicz-Kijewska
authors.affiliation: Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, The Children's Memorial Health Institute. Warsaw, Poland.
authors.forename: Joanna
authors.initials: J
authors.lastname: Pawłowska
mesh_terms: D000367:Age Factors; D001656:Biliary Atresia / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D001706:Biopsy; D004766:Enterostomy / Q000009:adverse effects / Q000401:mortality; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008099:Liver / Q000473:pathology*; D008103:Liver Cirrhosis / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D008297:Male; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome