Studies on the pathogenesis of biliary atresia

C. L. Witzleben, B. E. Buck, L. Schnaufer, W. J. Brzosko

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23 Scopus citations


Liver biopsy and the extrahepatic biliary system from five patients who underwent portoenterostomy for biliary atresia were studied by light microscopy to delineate the character and distribution of the anatomic lesions, and by culture and immunofluorescence techniques for evidence of two viruses (cytomegalovirus and hepatitis B) which have been suspected of playing a role in the genesis of biliary atresia. The liver biopsies showed typical findings, with variable giant cell transformation, cholestasis, and prominent bile duct proliferation. In the extrahepatic biliary system, in all five cases there was lumen obliteration in the common hepatic duct, and in three cases there was also comparable obliteration in the common bile duct. Proximal to the areas of obliteration, no lumen dilation was evident. In the areas of obliteration there was evidence of a resolving inflammatory process. Where the lumen was present, it tended to be small, especially in the hepatic and common ducts. In the porta hepatitis, there appeared to be various stages of an evolving lesion, the earliest stage of which included extensive active epithelial injury with developing fibrosis, and the latest, more advanced fibrosis with reduction in duct lumen area. In the earlier stage, a dense inflammatory infiltrate, with small numbers of polymorphonuclear leukocytes, numerous lymphocytes, plasma cells, and eosinophils, was present. In the latter stage mononuclear cells predominated, polymorphonuclear leukocytes were scant, but eosinophils were often still quite prominent. These histologic findings demonstrate the presence of active ductal epithelial injury, at least in the porta hepatis, in this age period. There was no evidence of cytomegalovirus in any case or of hepatitis B in three of five cases. In two cases, one of the less specific techniques showed suggestive evidence of hepatitis B in the liver. These latter findings suggest that other viruses need to be sought, or cases should be studied at an earlier stage for the presence of these viruses, or both.

Original languageEnglish (US)
Pages (from-to)525-532
Number of pages8
JournalLaboratory Investigation
Issue number5
StatePublished - Dec 1 1978

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Molecular Biology
  • Cell Biology


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