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Phenotypic characterization of mononuclear infiltrate present in liver of biliary atresia
K Chen1, J S Gavaler, D H Van Thiel
1Department of Pathology, University of Pittsburgh School of Medicine, Pennsylvania 15261.
Insights
Biliary atresia in children shows immune cell patterns similar to normal liver, unlike adult hepatitis. This suggests biliary atresia is unlikely caused by viral or autoimmune liver attacks.
Area of Science:
- Hepatology
- Immunology
- Pediatric Gastroenterology
Background:
- Biliary atresia is a severe pediatric liver disease.
- Understanding the immune cell infiltrate in biliary atresia is crucial for diagnosis and treatment.
- Distinguishing biliary atresia from adult liver diseases like autoimmune hepatitis and hepatitis B is important.
Purpose of the Study:
- To compare the mononuclear cell populations in pediatric biliary atresia liver tissue with those in adult liver diseases and normal controls.
- To investigate the potential role of viral or autoimmune factors in the pathogenesis of biliary atresia based on immune cell profiles.
Main Methods:
- Analysis of liver explants from children with biliary atresia and alpha 1-antitrypsin deficiency.
- Comparison with adult liver tissues from chronic active hepatitis, hepatitis B infection, and normal controls.
- Utilizing monoclonal antibodies to identify and quantify specific mononuclear cell types in portal and lobular regions.
Main Results:
- Pediatric biliary atresia liver tissue exhibits mononuclear cell profiles more similar to normal adult liver than to adult chronic hepatitis (autoimmune or HBV).
- Children with alpha 1-antitrypsin deficiency also show immune cell patterns closer to normal liver than adult diseases, but less so than biliary atresia.
- The cellular immune landscape in biliary atresia does not resemble that typically seen in viral or autoimmune liver injury.
Conclusions:
- The immunomorphology of biliary atresia suggests it is unlikely to stem from a viral or autoimmune etiology.
- Distinct mononuclear cell infiltrates differentiate pediatric biliary atresia from adult chronic liver diseases.
- Further research into the specific immune microenvironment of biliary atresia may reveal novel therapeutic targets.
Abstract:
The liver tissue of 26 children with biliary atresia was compared to that of 20 adults with autoimmune chronic active hepatitis, 20 adults with chronic hepatitis due to hepatitis B infection, and 5 children with alpha 1-antitrypsin deficiency in terms of the number and type of mononuclear cells in portal and lobular areas of each using a panel of specific monoclonal antibodies that recognize different cell surface epitopes. All of the tissues studied were obtained at time of liver transplantation. In addition the livers of 5 adults biopsied for hepatomegaly but found to have no histologic liver disease were used as normal controls for this study. The results demonstrate that liver tissue obtained from children with end-stage biliary atresia is more like normal liver in terms of the number and type of mononuclear cells present than in either of the two types of adult liver diseases. Moreover although the liver of children with end-stage alpha 1-antitrypsin deficiency is more like that of normal liver than either of the two adult liver diseases studied in terms of the number and type of mononuclear cells within the liver, it is less similar to that of normal adult liver than is liver tissue obtained from children with biliary atresia. These findings suggest that as a morphologic basis biliary atresia is unlikely to be due to either a viral or an autoimmune attack upon the liver.