{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,16]],"date-time":"2025-10-16T13:53:26Z","timestamp":1760622806009},"reference-count":25,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2010,8,17]],"date-time":"2010-08-17T00:00:00Z","timestamp":1282003200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Israeli Lung Association, Tel-Aviv"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2010,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>To explore the feasibility and validity of forced spirometry in patients with ataxia telangiectasia (A\u2010T).<\/jats:p><\/jats:sec><jats:sec><jats:title>Study design<\/jats:title><jats:p>Twenty\u2010eight patients (aged 3.7\u201319.3 years) performed spirometry on 47 occasions. Parameters studied were technical quality and relation to: predicted values, pulmonary illness.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Start of test criteria for correct expiratory effort was significantly prolonged (183\u2009\u00b1\u2009115\u2009ms; <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001). The rise\u2010time to peak flow in children free of respiratory symptoms (Group\u2010FRS; n\u2009=\u20098) increased by 16.2\u2009\u00b1\u200912.5\u2009ms\/year above recommended and in children having recurrent infections (n\u2009=\u20098) 30.4\u2009\u00b1\u200916.1\u2009ms\/year, <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.01. Expiration\u2010time was significantly shorter than requested (1.21\u2009\u00b1\u20090.47\u2009sec) and was ended abruptly in 57% of the patients. FEV<jats:sub>1<\/jats:sub> could not be established by 8\/20 patients. The intra\u2010subject reproducibility met criteria (4.4\u2009\u00b1\u20092.7%, 5.2\u2009\u00b1\u20092.8%, 2.9\u2009\u00b1\u20093.2%, 6.3\u2009\u00b1\u20095.3%, for FVC, FEV<jats:sub>0.5<\/jats:sub>, PEF, FEF<jats:sub>25\u201375<\/jats:sub>, respectively). Group\u2010FRS showed yearly deterioration in FVC of 2.2%, while patients with hyper\u2010reactive airways (Group\u2010HRA; n\u2009=\u200912) had a deterioration rate of 3.6%\/year. FEV<jats:sub>0.5<\/jats:sub> deterioration rate was similar in both groups (2.2 and 2.0, respectively), but baseline values in Group\u2010HRA were significantly lower than those of Group\u2010FRS (<jats:italic>P<\/jats:italic>\u2009=\u20090.029) in similar young ages, indicating airway obstruction at early ages in Group\u2010HRA. FEV<jats:sub>0.5<\/jats:sub> values deterioration also correlated with body mass index (<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.017).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Forced spirometry in A\u2010T patients is reproducible and has a distinct pattern, although curves do not meet other recommendations for acceptable criteria. The study insinuates that a rapid deterioration in lung function occurs in A\u2010T patients with recurrent respiratory infection, suggesting that early intervention may prevent further deterioration or improve their lung function. Further studies are needed to confirm our results. Pediatr Pulmonol. 2010; 45:1030\u20131036. \u00a9 2010 Wiley\u2010Liss, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.21291","type":"journal-article","created":{"date-parts":[[2010,8,17]],"date-time":"2010-08-17T19:16:29Z","timestamp":1282072589000},"page":"1030-1036","source":"Crossref","is-referenced-by-count":14,"title":["The feasibility and validity of forced spirometry in ataxia telangiectasia"],"prefix":"10.1002","volume":"45","author":[{"given":"Daphna","family":"Vilozni","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yakov","family":"Berkun","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yonit","family":"Levi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Batya","family":"Weiss","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffrey M.","family":"Jacobson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ori","family":"Efrati","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2010,8,17]]},"reference":[{"key":"e_1_2_8_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.rcl.2005.10.009"},{"key":"e_1_2_8_3_2","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.180.6.1801613"},{"key":"e_1_2_8_4_2","first-page":"587","article-title":"Endocrinological studies in ataxia telangiectasia and other immunological deficiency diseases","volume":"6","author":"Ammann AJ","year":"1970","journal-title":"Clin Exp Immunol"},{"key":"e_1_2_8_5_2","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20209"},{"key":"e_1_2_8_6_2","doi-asserted-by":"publisher","DOI":"10.1158\/0008-5472.CAN-03-2384"},{"key":"e_1_2_8_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1071-9091(98)80007-7"},{"key":"e_1_2_8_8_2","first-page":"573","article-title":"The incidence and gene frequency of ataxia telangiectasia in the United States","volume":"39","author":"Swift M","year":"1986","journal-title":"Am J Hum Genet"},{"key":"e_1_2_8_9_2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00034805"},{"key":"e_1_2_8_10_2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00035205"},{"key":"e_1_2_8_11_2","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20738"},{"key":"e_1_2_8_12_2","unstructured":"Clinical Guidelines on the Identification Evaluation and Treatment of Overweight and Obesity in Adults. 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