{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T10:17:26Z","timestamp":1789899446061,"version":"4.0.1"},"reference-count":82,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2010,6,25]],"date-time":"2010-06-25T00:00:00Z","timestamp":1277424000000},"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":[[2010,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Ataxia\u2010telangiectasia (A\u2010T) is a rare autosomal recessive disorder caused by mutations in the <jats:italic>ATM<\/jats:italic> gene, resulting in faulty repair of breakages in double\u2010stranded DNA. The clinical phenotype is complex and is characterized by neurologic abnormalities, immunodeficiencies, susceptibility to malignancies, recurrent sinopulmonary infections, and cutaneous abnormalities. Lung disease is common in patients with A\u2010T and often progresses with age and neurological decline. Diseases of the respiratory system cause significant morbidity and are a frequent cause of death in the A\u2010T population. Lung disease in this population is thought to exhibit features of one or more of the following phenotypes: recurrent sinopulmonary infections with bronchiectasis, interstitial lung disease, and lung disease associated with neurological abnormalities. Here, we review available evidence and present expert opinion on the diagnosis, evaluation, and management of lung disease in A\u2010T, as discussed in a recent multidisciplinary workshop. Although more data are emerging on this unique population, many recommendations are made based on similarities to other more well\u2010studied diseases. Gaps in current knowledge and areas for future research in the field of pulmonary disease in A\u2010T are also outlined. Pediatr. Pulmonol. 2010; 45:847\u2013859. \u00a9 2010 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ppul.21277","type":"journal-article","created":{"date-parts":[[2010,6,25]],"date-time":"2010-06-25T20:27:50Z","timestamp":1277497670000},"page":"847-859","source":"Crossref","is-referenced-by-count":72,"title":["Evaluation and management of pulmonary disease in ataxia\u2010telangiectasia"],"prefix":"10.1002","volume":"45","author":[{"given":"Sharon A.","family":"McGrath\u2010Morrow","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W. Adam","family":"Gower","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cynthia","family":"Rothblum\u2010Oviatt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan S.","family":"Brody","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Langston","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leland L.","family":"Fan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maureen A.","family":"Lefton\u2010Greif","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas 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