{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,18]],"date-time":"2026-01-18T11:21:34Z","timestamp":1768735294341,"version":"3.49.0"},"reference-count":27,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2020,7,20]],"date-time":"2020-07-20T00:00:00Z","timestamp":1595203200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["1 R34 HL 113390\u201001"],"award-info":[{"award-number":["1 R34 HL 113390\u201001"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2020,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>Respiratory compromise in congenital muscular dystrophy (CMD) occurs, in part, from chest wall contractures. Passive stretch with hyperinsufflation therapy could reduce related costo\u2010vertebral joint contractures. We sought to examine the impact of hyperinsufflation use on lung function and quality of life in children with CMD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Study Design<\/jats:title><jats:p>We conducted a randomized controlled trial on hyperinsufflation therapy in children with CMD at two centers. An individualized hyperinsufflation regimen of 15\u2009minutes twice daily using a cough assist device over a 12 months period was prescribed. We measured lung function, quality of life, and adherence. To demonstrate reproducibility, pulmonary function was measured twice on the same day. A mixed\u2010effects regression model adjusting for confounders was used to assess the effects of hyperinsufflation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We enrolled 34 participants in the study; 31 completed the trial (n\u2009=\u200917 treatment group and n\u2009=\u200914 controls). Participants in the treatment group demonstrated a relative gain in lung volume measured at 4 and 8 months, but not at 12 months. The control group required increases in the maximum insufflation pressures to achieve maximum lung volumes while the treatment group did not. Adherence was best early in the study, peaking at the first visit and decreasing at subsequent visits. Caregiver\u2010reported quality of life was higher in the treatment group.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Hyperinsufflation therapy is effective in increasing and sustaining lung volume over time. Adherence, however, was inconsistent and difficult to maintain. Further research should determine if improved adherence leads to sustained benefits of hyperinsufflation.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.24954","type":"journal-article","created":{"date-parts":[[2020,7,13]],"date-time":"2020-07-13T13:54:42Z","timestamp":1594648482000},"page":"2471-2478","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Randomized trial of lung hyperinflation therapy in children with congenital muscular dystrophy"],"prefix":"10.1002","volume":"55","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0478-7205","authenticated-orcid":false,"given":"Hemant","family":"Sawnani","sequence":"first","affiliation":[{"name":"Division of Pulmonary Medicine Cincinnati Children's Hospital Medical Center  Cincinnati Ohio"},{"name":"Department of Pediatrics University of Cincinnati College of Medicine  Cincinnati Ohio"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Oscar H.","family":"Mayer","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Medicine Children's Hospital of Philadelphia  Philadelphia Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Avani C.","family":"Modi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Cincinnati College of Medicine  Cincinnati Ohio"},{"name":"Division of Behavioral Medicine and Clinical Psychology, Center for Treatment Adherence and Self\u2010Management Cincinnati Children's Hospital Medical Center  Cincinnati Ohio"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John E.","family":"Pascoe","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Medicine Cincinnati Children's Hospital Medical Center  Cincinnati Ohio"},{"name":"Department of Pediatrics University of Cincinnati College of Medicine  Cincinnati Ohio"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keith","family":"McConnell","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Medicine Cincinnati Children's Hospital Medical Center  Cincinnati Ohio"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joseph M.","family":"McDonough","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Medicine Children's Hospital of Philadelphia  Philadelphia Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anne M.","family":"Rutkowski","sequence":"additional","affiliation":[{"name":"Cure CMD Foundation and Kaiser SCPMG  Los Angeles California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Md Monir","family":"Hossain","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Cincinnati College of Medicine  Cincinnati Ohio"},{"name":"Division of Epidemiology and Biostatistics Cincinnati Children's Hospital 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