{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T00:39:21Z","timestamp":1787791161228,"version":"build-2784847793"},"reference-count":44,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2013,8,19]],"date-time":"2013-08-19T00:00:00Z","timestamp":1376870400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Funda\u00e7\u00e3o de Amparo a Pesquisa do Estado de S\u00e3o Paulo, S\u00e3o Paulo, Brazil","award":["06946-5\/2008"],"award-info":[{"award-number":["06946-5\/2008"]}]},{"DOI":"10.13039\/501100002322","name":"CAPES","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100002322","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2014,3]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Ataxia telangiectasia (AT) is a genetic syndrome caused by a mutation of chromosome 11. The clinical features are cerebellar ataxia, telangiectasia, and progressive loss of muscular coordination, including an inefficient cough secondary to progression of neurological disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To evaluate the effects of inspiratory muscle training (IMT) on ventilation, lung volume, dyspnoea, respiratory muscle strength, and quality of life in patients with AT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A longitudinal study was conducted with 11 AT patients and nine healthy volunteers. Ventilometry, subjective sensation of dyspnoea, maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and quality of life were assessed before and after a 24\u2010week IMT program. The IMT load used was set at 60% of the MIP, and the training was performed for 20\u2009min daily.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients with AT had lower height and weight and also had lower respiratory muscle strength and lung volume compared with healthy volunteers. Furthermore, patients with AT showed a significant improvement when pre\u2010 and post\u2010IMT were compared for ventilatory pattern: Vt (476.5\u2009\u00b1\u2009135\u2009ml vs. 583.3\u2009\u00b1\u200966\u2009ml, <jats:italic>P<\/jats:italic>\u2009=\u20090.015) and f (23.3\u2009\u00b1\u20096\u2009rpm vs. 20.4\u2009\u00b1\u20094\u2009rpm, <jats:italic>P<\/jats:italic>\u2009=\u20090.018), and VC (1,664\u2009\u00b1\u2009463\u2009ml\/kg vs. 2,145\u2009\u00b1\u2009750\u2009ml\/kg, <jats:italic>P<\/jats:italic>\u2009=\u20090.002). IMT also significantly improved the sensation of dyspnoea (median 0.5; minimum 0; maximum 1.0; <jats:italic>P<\/jats:italic>\u2009=\u20090.022) and respiratory muscle strength: MIP (\u221222.2\u2009\u00b1\u20092\u2009cmH<jats:sub>2<\/jats:sub>O vs. \u221238\u2009\u00b1\u20099\u2009cmH<jats:sub>2<\/jats:sub>O, <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001) and MEP (29\u2009\u00b1\u20097\u2009cmH<jats:sub>2<\/jats:sub>O vs. 40\u2009\u00b1\u20098\u2009cmH<jats:sub>2<\/jats:sub>O, <jats:italic>P<\/jats:italic>\u2009=\u20090.001). The health and vitality domains of the SF\u201036 also showed significant improvement (<jats:italic>P<\/jats:italic>\u2009=\u20090.009 and <jats:italic>P<\/jats:italic>\u2009=\u20090.014, respectively) post\u2010IMT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>IMT was effective in improving ventilatory pattern, lung volume, respiratory muscle strength, and the health and vitality domains for quality of life in patients with AT. IMT may be an effective adjunct therapy to drug treatment for patients with AT. <jats:bold>Pediatr Pulmonol. 2014; 49:238\u2013244.<\/jats:bold> \u00a9 2013 Wiley Periodicals, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.22828","type":"journal-article","created":{"date-parts":[[2013,8,19]],"date-time":"2013-08-19T06:08:44Z","timestamp":1376892524000},"page":"238-244","source":"Crossref","is-referenced-by-count":29,"title":["Effects of inspiratory muscle training on lung volumes, respiratory muscle strength, and quality of life in patients with ataxia telangiectasia"],"prefix":"10.1002","volume":"49","author":[{"given":"Erika","family":"F\u00e9lix","sequence":"first","affiliation":[{"name":"Department of Pediatrics, School of Medicine Federal University of Sao Paulo (UNIFESP) Sao Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Cristina","family":"Gimenes","sequence":"additional","affiliation":[{"name":"Pulmonary Function and Clinical Exercise Physiology Unit, Division of Respiratory Diseases, Department of Medicine Federal University of Sao Paulo (UNIFESP) Sao Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beatriz Tavares","family":"Costa\u2010Carvalho","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, School of Medicine Federal University of Sao Paulo (UNIFESP) Sao Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,8,19]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1186\/gm12"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2004.02492.x"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1385\/CRIAI:20:1:87"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.21291"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.21095"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.21276"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20209"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2007.00338.x"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.21277"},{"key":"e_1_2_7_11_1","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1002\/ppul.20738","article-title":"Pulmonary function in adolescents with ataxia\u2013telagiectasia","volume":"43","author":"Mcgrath\u2010Morrow S","year":"2008","journal-title":"Pediatr Pulmonol"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.2006.094268"},{"key":"e_1_2_7_13_1","first-page":"162","article-title":"Hodgkin disease in ataxia\u2013telangiectasia patients with poor outcomes","volume":"3","author":"Sandoval C","journal-title":"Med Pediatr Oncol"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(00)70106-5"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-510X(01)00617-7"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2005.02.035"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2003.009894"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1080\/10790268.2008.11753645"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.120.3.765"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181fef115"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0031-1271972"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1006\/clim.1999.4799"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.2471\/BLT.07.043497"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1526-0542(03)00051-4"},{"key":"e_1_2_7_25_1","first-page":"456","article-title":"ATS consensus statement. 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