Immunologic Incompetence in Ataxia-Telangiectasia

THE predisposition of some children with the syndrome of ataxia-telangiectasia to suffer from frequent severe sinopulmonary infections has led to an investigation of the immunologic competence of patients with this disease. Louis-Bar,¹ Boder and Sedgwick,^2,3 and Centerwall and Miller,⁴ have described the striking clinical features of the disease which include progressive ataxia with onset in early childhood and telangiectasia involving the bulbar conjunctivae and other areas. Peterson et al⁵ recently reported thymic abnormalities and immunological incompetence in this disease. In the sera of four of five patients studied low or absent levels of γ-1A globulin were found. Fireman et al⁶ have recently reported studies on six patients and found five with no detectable γ-1A globulin and a sixth patient with a serum concentration much less than normal. Young et al⁷ have also recently reported a selective absence of γ-1A globulin in three of four