Showing papers by "Charles A. Janeway published in 1956"

Multiple serum protein deficiencies in congenital and acquired agammaglobulinemia

Abstract: The absence of serum y-globulins in patients afflicted with severe recurring bacterial infections has been reported (1, 2). Three forms of this metabolic disturbance, agammaglobulinemia, have been described (3): 1) a physiologic or transient form occurring in infants, 2) a congenital form which is inherited as a sex-linked recessive characteristic occurring in males, and 3) an acquired form, occurring in both sexes, with the onset of infections in adolescence or adulthood. The congenital and acquired forms of the disease are due to an absence of plasma cells in the tissues of these patients (3-5) with a consequent deficiency of y-globulin and failure of antibody synthesis (4); the plasma cells appear to be the site of antibody synthesis (6). Unlike the transient form, the failure of y-globulin synthesis in the congenital and acquired groups, once established, seems to be permanent. Using rabbit and horse antisera against human y-globulin in the quantitative precipitin reaction, it had been demonstrated that the failure of y-globulin synthesis in these patients was usually not complete and that small amounts of y-globulin were often present in their sera, less than 25 mg. per cent in the congenital form and less than 100 mg. per cent in the acquired form (3). In this report, it will be shown that at least two other plasma proteins in addition to y-globulin are either absent or markedly deficient in these two forms of the disease.