Lymphocytic interstitial pneumonitis, elevated IgM concentration, and hepatosplenomegaly in ataxia-telangiectasia |
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| Authors: | Tangsinmankong N Wayne A S Howenstine M S Washington K R Langston C Gatti R A Good R A Nelson R P |
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| Affiliation: | Department of Pediatrics, Division of Allergy and Immunology, All Children's Hospital/University of South Florida, 802 Sixth St S, St Petersburg, FL 33701, USA. |
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| Abstract: | ![]()
An 8-year-old girl developed ataxia-telangiectasia. Western blotting of lysate revealed absence of the ATM protein, and 2 mutations in the ATM gene were found. Subsequently, the patient developed increased respiratory symptoms. Open lung biopsy revealed lymphocytic interstitial pneumonitis, which is not characteristic of ataxia-telangiectasia. There was a therapeutic response to glucocorticosteroid treatment. |
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