title: Positive C4d staining of the portal vein endothelium in the liver of patients with biliary atresia: a role of humoral immunity in ongoing liver fibrosis.
abstract: OBJECTIVE
  This study aimed to clarify the role of complement activation in fibrogenesis in BA.
  
  
  METHODS
  In total, 27 paraffin-embedded liver biopsy samples were immunohistochemically analyzed using C4d polyclonal antibody, vascular cell adhesion molecule-1 (VCAM-1), and CD45. The liver samples were obtained from 25 patients during Kasai operation, and two additional specimens were obtained from 2 patients by needle biopsy later at the time of liver function deterioration. The degree of liver fibrosis was histologically graded 1-3.
  
  
  RESULTS
  Among the 25 samples, 9 showed C4d-positive immunostaining localized on the endothelia of a few portal veins in the portal tract. The degree of fibrosis was correlated with C4d staining (p = 0.025). The age at Kasai operation correlated with the degree of fibrosis and the C4d positivity. Two needle biopsy samples were positive for C4d. Among 13 samples submitted for VCAM-1 staining, 2 negative samples were C4d negative and all positive C4d samples were VCAM-1 positive with CD45 mononuclear cell infiltration.
  
  
  CONCLUSIONS
  These findings suggest that ongoing cirrhosis could be a result of progressive "vasculopathy" of the portal vein caused by humoral and cell-mediated immune interaction.
authors: Department of Pediatric Surgery, Ibaraki Children's Hospital, 3-3-1 Futabadai, Mito, Ibaraki, 311-4145, Japan.
authors: Sorahiko
authors: S
authors: Fujisawa
authors: Toshihiro
authors: T
authors: Muraji
authors: Naoya
authors: N
authors: Sakamoto
authors: Naoki
authors: N
authors: Hosaka
authors: Satoshi
authors: S
authors: Matsuda
authors: Hajime
authors: H
authors: Kawakami
authors: Misako
authors: M
authors: Hirai
authors: Toshihiro
authors: T
authors: Yanai
mesh_terms: D000367:Age Factors; D001656:Biliary Atresia / Q000150:complications / Q000276:immunology* / Q000473:pathology; D001706:Biopsy; D015935:Complement C4b / Q000276:immunology*; D004727:Endothelium / Q000473:pathology / Q000648:ultrastructure; D005260:Female; D005455:Fluorescent Antibody Technique / Q000379:methods; D005500:Follow-Up Studies; D006801:Humans; D056724:Immunity, Humoral / Q000276:immunology*; D007223:Infant; D008099:Liver / Q000276:immunology* / Q000473:pathology; D008103:Liver Cirrhosis / Q000150:complications / Q000276:immunology* / Q000473:pathology; D008297:Male; D015588:Observer Variation; D010446:Peptide Fragments / Q000276:immunology*; D011169:Portal Vein / Q000276:immunology* / Q000473:pathology / Q000648:ultrastructure; D012720:Severity of Illness Index
chemical_list: D010446:Peptide Fragments; D015935:Complement C4b; C032261:complement C4d