Ataxia-telangiectasia

The clinical and radiographic features of twenty-two cases of ataxia-telangiectasia are reviewed. Severity of pulmonary manifestations tended to correlate closely with severity of immunodeficiency. Observations are made upon the problem of intercurrent malignancy in ataxia-telangiectasia. The radiographic studies of patients seen at the National Institutes of Health with A-T were reviewed retrospectively and correlated with degrees of immunodeficiency determined clinically. Three fairly distinct groups with high, intermediate and low incidence of sinopulmonary disease were found. Comment is made on the problem of increased malignancy in A-T and possible clues in the detection by the radiologist.