Lymphocytic interstitial pneumonitis, elevated IgM concentration, and hepatosplenomegaly in ataxia-telangiectasia.
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.
Tangsinmankong
N
N
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.
Wayne
A S
AS
Howenstine
M S
MS
Washington
K R
KR
Langston
C
C
Gatti
R A
RA
Good
R A
RA
Nelson
R P
RP
D001260:Ataxia Telangiectasia / Q000150:complications* / Q000276:immunology; D002648:Child; D005260:Female; D006529:Hepatomegaly / Q000209:etiology*; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood*; D017563:Lung Diseases, Interstitial / Q000209:etiology*; D013163:Splenomegaly / Q000209:etiology*
D007075:Immunoglobulin M