Indian childhood cirrhosis: genealogic data, alpha-foetoprotein, hepatitis antigen and circulating immune complexes.

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.