PEDIATRICS Vol. 37 No. 6 June 1966, pp. 924-933
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ATAXIA-TELANGIECTASIA: IMMUNOLOGIC AND VIROLOGIC STUDIES OF SERUM AND RESPIRATORY SECRETIONS

Joseph A. Bellanti M.D.1, Malcolm S. Artenstein M.D.1, and Edward L. Buescher M.D.1

1 Department of Virus Diseases, Walter Reed Army Institute of Research and Department of Pediatrics, Georgetown University Medical Center, Washington, D.C.

Studies of antibody function in three patients with ataxia-telangiectasia are presented. Absence of serum ggrA-immunoglobulins was found in two of the patients; the third had normal amounts of this immunoglobulin. Nasal secretions of all three possessed ggrG- and ggrM-immunoglobulins; in 2 of 3 patients a variant immunoglobulin, not clearly ggrA, was detected in nasal secretions. Following fractionation of their sera by gel filtration with Sephadex G-200, neutralizing antibody to poliovirus 1, influenza A2/63, parainfluenza 3, and echovirus 28 were found in association with the ggrM- and ggrG-immunoglobulins in two patients and with all three immunoglobulins in the third. The pattern of neutralizing antibody in nasal secretions paralleled that of serum. Since the nasal secretions lacked or were markedly deficient in ggrA-immunoglobulins, the specific viral antibody detected therein was attributed to the increased concentrations of ggrM- and ggrG-immunoglobulins found. The data suggest that although patients with ataxiatelangiectasia lack the normal complement of ggrA-immunoglobulins in nasal secretions, they do contain antiviral antibody, but in association with other immunoglobulins.

Submitted on March 4, 1965
Accepted on January 7, 1966