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Immunologic Incompetence in Ataxia-Telangiectasia

Am J Dis Child. 1965;110(1):69-75. doi:10.1001/archpedi.1965.02090030075010.
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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


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