乳児胆汁うっ滞性疾患の肝内補体蛋白 (C_3,C_4,C_1q)の局在

抄録

The HRP-labeled antibody method was used for intrahepatic localization of complements (C_3, C_4, C_<1q>) in 8 infants with congenital biliary atresia (CBA) and 4 with neonatal hepatitis (NH), as well as in 7 with cholestatic disease associated with total parenteral nutrition (TPN). Normal hepatic localization of C_3 and C_4 was limited to hepatic cells, sinusoid, Disses space, vascular endothelium, Glisson's connective tissue and stellate Kupffer cell. The localization of C_<1q> was limited to hepatic cells. On the other hand, in the cholestatic disease, positive staining of the complements was also observed in the congested bile. Moreover, in CBA, the interlobular and ductular epithelial cells had positive staining of C_3, C_4, but not in NH or TPN. The present study suggests that the C_3, C_4, and C_<1q> transfer from hepatic cell into bile canaliculus and that biliary epithelial cell of CBA may possess ectopic function to produce the complements or may be the site of immunoreaction involving C_3 and C_4.