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  • Title: Role of immunologic costimulatory factors in the pathogenesis of biliary atresia.
    Author: Kobayashi H, Li Z, Yamataka A, Lane GJ, Miyano T.
    Journal: J Pediatr Surg; 2003 Jun; 38(6):892-6. PubMed ID: 12778387.
    Abstract:
    BACKGROUND: The authors studied the patterns of expression of immunologic costimulatory molecules (B7-1, B7-2, and CD40) in biliary atresia (BA) patients to confirm any correlation with clinical course/outcome. METHODS: Based on clinical status 2 years postoperatively, 24 BA patients were divided into group I (n = 8, normal liver function), group II (n = 10, anicteric with moderate liver dysfunction), and group III (n = 6, icteric with severe liver dysfunction). Liver biopsies obtained at portoenterostomy and from 6 age-matched controls, were analyzed immunohistochemically using antibodies against B7-1, B7-2, and CD40. RESULTS: There was no expression of B7-1, B7-2, or CD40 in any control liver specimen. In all BA specimens, B7-1, B7-2, and CD40 were expressed strongly in bile ductules in portal tracts. In groups with liver dysfunction, B7-1, B7-2, and CD40 were expressed strongly on the surfaces of Kupffer and dendritic cells and in hepatocyte cytoplasm. Positive staining cells were significantly fewer in patients with better clinical outcome. B7-1 was found in vascular and sinusoidal endothelial cells only in cases of postoperative portal hypertension. CONCLUSIONS: Costimulatory factors expressed on bile ductules, hepatocytes, and vascular endothelial cells appear to mediate autoimmune processes causing progressive liver fibrosis and portal hypertension in BA.
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