title: Lymphocytic interstitial pneumonitis, elevated IgM concentration, and hepatosplenomegaly in ataxia-telangiectasia.
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.
authors.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.
authors.forename: N
authors.initials: N
authors.lastname: Tangsinmankong
authors.forename: A S
authors.initials: AS
authors.lastname: Wayne
authors.forename: M S
authors.initials: MS
authors.lastname: Howenstine
authors.forename: K R
authors.initials: KR
authors.lastname: Washington
authors.forename: C
authors.initials: C
authors.lastname: Langston
authors.forename: R A
authors.initials: RA
authors.lastname: Gatti
authors.forename: R A
authors.initials: RA
authors.lastname: Good
authors.forename: R P
authors.initials: RP
authors.lastname: Nelson
mesh_terms: 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*
chemical_list: D007075:Immunoglobulin M