A FAMILY OF SELECTIVE IMMUNODEFICIENCY WITH NORMAL IMMUNOGLOBULINS - POSSIBLE AUTOSOMAL-DOMINANT INHERITANCE

We report here our findings in two Japanese siblings who experienced r ecurrent bacterial and viral infections since early infancy. Recent sy mptoms included diarrhoea, conjunctivitis, rashes, headache, sore thro at, joint pain, vomiting and vertigo, all similar to those seen in tox ic shock syndrome, except for shock. These symptoms improved following gammaglobulin treatment. Staphylococcus aureus with coagulase type TV was continuously isolated from nasal smears producing toxic shock syn drome toxin-l (TSST-1). Serum antibodies did not or only poorly respon ded to TSST-1, diphtheria toroid, varicella virus and rubella virus, w hereas total and subclass levels of serum immunoglobulin and in vitro DNA synthesis of lymphocytes stimulated by TSST-1, Staph. aureus, vari cella vaccine and mitogens were normal. In the family, ten other membe rs in three generations (five males : five females) including the moth er had similar clinical symptoms. Thus, the disease may be inherited i n an autosomal dominant fashion.