title: Early type I collagen deposition is associated with prognosis in biliary atresia.
abstract: BACKGROUND
  Biliary atresia (BA) is a cholestatic liver disease of children that progresses to hepatic fibrosis. BA is the main indication of pediatric liver transplantation (LTx). Histopathological markers in liver biopsies could be useful for predicting progression to end-stage disease.
  
  
  OBJECTIVE
  To establish histopathological or immunohistochemical markers in liver biopsies of BA patients and correlate those markers with prognosis.
  
  
  METHODS
  Histological analysis of biliary alterations and morphometric assessment of liver fibrosis were performed, in addition to indirect immunofluorescence assays (IF) for type I, III, IV and V collagens in initial and final liver biopsies of 36 patients with BA who underwent Kasai hepatoportoenterostomy (KPE) and LTx in the last 20years at a single center.
  
  
  RESULTS
  Histopathological markers had no correlation with evolutive time until LTx. The perisinusoidal deposition of type III and V collagens was more prominent in the initial biopsies (p<0.01), whereas deposition of type I and IV collagens indicated progression (p<0.01). Patients with large amounts of perisinusoidal type I collagen in the initial biopsies had worse progression time curves until LTx (p=0.04).
  
  
  CONCLUSIONS
  Morphometric assessment of perisinusoidal deposition of type I collagen by IF in the initial biopsy can correlate with progression time to LTx in post-surgical BA.
authors.affiliation: Department of Pediatric Surgery, University of São Paulo Medical School (FMUSP), São Paulo, Brazil. Electronic address: luis.ricardo@hc.fm.usp.br.
authors.forename: Luis Ricardo
authors.initials: LR
authors.lastname: Longo-Santos
authors.affiliation: Laboratory of Extracellular Matrix, University of São Paulo Medical School (FMUSP), São Paulo, Brazil.
authors.forename: Walcy Rosolia
authors.initials: WR
authors.lastname: Teodoro
authors.affiliation: Department of Pathology, University of São Paulo Medical School (FMUSP), São Paulo, Brazil.
authors.forename: Evandro Sobroza
authors.initials: ES
authors.lastname: de Mello
authors.affiliation: Laboratory of Extracellular Matrix, University of São Paulo Medical School (FMUSP), São Paulo, Brazil.
authors.forename: Ana Paula Pereira
authors.initials: AP
authors.lastname: Velosa
authors.affiliation: Department of Pathology, University of São Paulo Medical School (FMUSP), São Paulo, Brazil.
authors.forename: Edwin Roger
authors.initials: ER
authors.lastname: Parra
authors.affiliation: Department of Pathology, University of São Paulo Medical School (FMUSP), São Paulo, Brazil.
authors.forename: Vera Luiza
authors.initials: VL
authors.lastname: Capelozzi
authors.affiliation: Department of Pediatric Surgery, University of São Paulo Medical School (FMUSP), São Paulo, Brazil.
authors.forename: Uenis
authors.initials: U
authors.lastname: Tannuri
mesh_terms: D001656:Biliary Atresia / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism / Q000601:surgery; D015415:Biomarkers / Q000378:metabolism; D001706:Biopsy; D002648:Child; D024042:Collagen Type I / Q000378:metabolism*; D018450:Disease Progression; D005260:Female; D024022:Fibrillar Collagens / Q000378:metabolism; D005500:Follow-Up Studies; D006801:Humans; D008099:Liver / Q000378:metabolism* / Q000473:pathology; D008103:Liver Cirrhosis / Q000209:etiology / Q000378:metabolism / Q000473:pathology; D016031:Liver Transplantation; D008297:Male; D011171:Portoenterostomy, Hepatic; D011379:Prognosis; D012189:Retrospective Studies
chemical_list: D015415:Biomarkers; D024042:Collagen Type I; D024022:Fibrillar Collagens
keywords: Biliary atresia; Collagen deposition; Hepatic fibrosis; Histological markers; Kasai hepatoportoenterostomy; Liver transplantation