{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T19:24:23Z","timestamp":1774466663238,"version":"3.50.1"},"reference-count":26,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2013,2,8]],"date-time":"2013-02-08T00:00:00Z","timestamp":1360281600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2014,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Pulmonary disease contributes to significant morbidity and mortality in people with ataxia telangiectasia (A\u2010T). To determine the association between age and lung function in children and young adults with A\u2010T and to identify factors associated with decreased lung function, pulmonary function tests were performed in 100 consecutive people with A\u2010T.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Children and adults ranging from 6 to 29 years of age and with the diagnosis of A\u2010T were recruited, and underwent pulmonary function tests.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The mean forced vital capacity % predicted (FVC %) in the population was 56.6\u2009\u00b1\u200920.0. Males and females between 6 and 10 years of age had similar pulmonary function. Older females were found to have significantly lower FVCs % than both older males (<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.02) and younger females (<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001). The use of supplemental gamma globulin was associated with significantly lower FVC %. A modest correlation was found between higher radiation\u2010induced chromosomal breakage and lower FVC % in males. No significant change in FVC % was found in a subset of subjects (n\u2009=\u200925) who underwent pulmonary function testing on two or more occasions over an average of 2 years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>In children and young adults with A\u2010T, older females and people who required supplemental gamma globulin had significantly lower lung function by cross\u2010sectional analysis. Stable lung function is possible over a 2\u2010year period. Recognition of groups who are at higher risk for lower pulmonary function may help direct care and improve clinical outcomes in people with A\u2010T. <jats:bold>Pediatr Pulmonol. 2014; 49:84\u2013990.<\/jats:bold> \u00a9 2013 Wiley Periodicals, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.22760","type":"journal-article","created":{"date-parts":[[2013,2,8]],"date-time":"2013-02-08T15:37:40Z","timestamp":1360337860000},"page":"84-990","source":"Crossref","is-referenced-by-count":24,"title":["Pulmonary function in children and young adults with ataxia telangiectasia"],"prefix":"10.1002","volume":"49","author":[{"given":"Sharon A.","family":"McGrath\u2010Morrow","sequence":"first","affiliation":[{"name":"Eudowood Division of Pediatric Respiratory Sciences Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Howard M.","family":"Lederman","sequence":"additional","affiliation":[{"name":"Division of Allergy and Immunology Department of Pediatrics The Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angela D.","family":"Aherrera","sequence":"additional","affiliation":[{"name":"Eudowood Division of Pediatric Respiratory Sciences Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maureen A.","family":"Lefton\u2010Greif","sequence":"additional","affiliation":[{"name":"Eudowood Division of Pediatric Respiratory Sciences Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas O.","family":"Crawford","sequence":"additional","affiliation":[{"name":"Department of Neurology and Adjunct in Department of Pediatrics The Johns Hopkins Medical Institutions Baltimore Maryland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Timothy","family":"Ryan","sequence":"additional","affiliation":[{"name":"Eudowood Division of Pediatric Respiratory Sciences Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jennifer","family":"Wright","sequence":"additional","affiliation":[{"name":"Eudowood Division of Pediatric Respiratory Sciences Johns Hopkins University School of Medicine Baltimore Maryland"},{"name":"Division of Allergy and Immunology Department of Pediatrics The Johns Hopkins University School of Medicine Baltimore Maryland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joseph M.","family":"Collaco","sequence":"additional","affiliation":[{"name":"Eudowood Division of Pediatric Respiratory Sciences Johns Hopkins University School of Medicine Baltimore 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