Genetic and immunological aspects of familial chronic active hepatitis (type B)

Thirteen family members of a patient with chronic active hepatitis type B were investigated. The family included both parents, 6 sons, and 5 daughters. The parents were second cousins. HBsAg, liver tests, immunological evaluation, and HLA typing were performed on all subjects. Percutaneous liver biopsies were done on the mother and 5 of the 6 sons. The mother and all 6 sons had HBs antigenemia. The mother was free from any evidence of liver disease whereas all 6 sons had abnormal liver and immunological tests. The liver biopsies of 5 sons showed chronic active hepatitis with variable degrees of progression toward cirrhosis. The 6th son could not be biopsied in view of his prolonged prothrombin time. The father and the 5 daughters were HBsAg negative and had no evidence of liver disease. Immunological abnormalities were present in all of the affected children and in the mother and 3 daughters. This is the second report in the English literature on the familial occurrence of chronic active hepatitis type B. It emphasizes the predominance of this entity in the male offspring and confirms the presence of immunological abnormalities in the relatives of such patients. There was no evidence to link the inheritance of an immunological abnormality to clear the HBsAg to the histocompatibility complex.