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Indian childhood cirrhosis: genealogic data, alpha-foetoprotein, hepatitis antigen and circulating immune complexes
Insights
Indian Childhood Cirrhosis (ICC) likely results from inherited liver vulnerability combined with hepatitis virus infection. This leads to progressive liver damage and mortality in young children.
Area of Science:
- Pediatrics
- Hepatology
- Immunology
Background:
- Indian Childhood Cirrhosis (ICC) is a major cause of child mortality in India.
- The exact etiology of ICC remains unclear, necessitating further investigation into genetic and infectious factors.
Purpose of the Study:
- To investigate the inheritance pattern, immunological abnormalities, and potential role of hepatitis B virus in Indian Childhood Cirrhosis.
- To identify factors contributing to the progressive liver damage observed in ICC patients.
Main Methods:
- Pedigree analysis was performed on 100 ICC patients.
- Serum levels of alpha-foetoprotein (AFP), immunoglobulins, complement components (C3), and hepatitis B markers (HBsAg, HBsAb) were analyzed.
- Lymphocyte response to HBsAg and presence of circulating immune complexes were assessed.
Main Results:
- Autosomal recessive inheritance was suggested by pedigree analysis.
- Elevated AFP, immunoglobulins, and decreased C3 levels were observed in patients.
- Hepatitis B surface antigen (HBsAg) was more frequent in patients and relatives, with impaired lymphocyte response to HBsAg.
Conclusions:
- ICC may arise from inherited hepatocyte vulnerability triggered by hepatitis virus infection.
- Immunological responses and hepatitis B virus infection appear to play a role in the pathogenesis of ICC.
- These factors contribute to progressive hepatic damage and mortality in affected children.
Abstract:
Indian childhood cirrhosis is a significant cause of morbidity and mortality in young children in India. One hundred patients with ICC, 66 boys and 34 girls, were studied. Pedigree analysis yielded a segregation ratio of 0-2196, suggestive of an autosomal recessive inheritance. Serum alpha1-antitrypsin level was normal. Serum alpha-foetoprotein (AFP) concentration was increased in all the patients, parents and in some siblings. Serum immunoglobulins G, A, M, and D were elevated. Haemolytic complement and C3 were low. Electrophoretically altered complement components were detected in 36% of patients. There was an inverse relationship between C3 concentration and immunoconglutinin titre. Circulatingimmune complexes were detected in the sera of six out of ten patients who had significant proteinuria. Hepatitis B surface antigen (HBsAg) was present in the serum, ascitic fluid, saliva, urine and faeces of ICC patients more frequently than in controls. HBsAb was detected less often. Lymphocyte response to HBsAg was impaired. The first-degree relatives had a higher incidence of HBsAg and HBsAb than healthy controls. It is suggested that ICC occurs in infants with an inherited hepatocyte vulnerability and that one of the precipitating causes of liver cell necrosis is infection with hepatitis virus(es). The consequent immunologic epiphenomena contribute to progressive hepatic damage ending in death.