{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T06:08:49Z","timestamp":1773382129261,"version":"3.50.1"},"reference-count":30,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2023,8,4]],"date-time":"2023-08-04T00:00:00Z","timestamp":1691107200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2023,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Understanding real\u2010world profiles from neuromuscular databases is helpful for optimizing clinical care and planning research studies. The Canadian Neuromuscular Disease Registry (CNDR) has respiratory data from a population of boys with Duchenne Muscular Dystrophy (DMD).<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>To describe cross\u2010sectional respiratory profiles from a national DMD real\u2010world dataset. To explore the relationship between forced vital capacity percent predicted (FVC%) and disease severity parameters: scoliosis, ambulation and ventilation status.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Descriptive statistics summarized the respiratory profiles. The CNDR registry enrolls and collects DMD clinic data from 36 Canadian centers.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>There were 414 participants enrolled. The age ranged from 2 to 36 years old. Pulmonary function test data were available for 323 participants. The use of ventilatory support was seen in a significant proportion (19.5%) of subjects by age 14\u201316 years and was used by the majority (69.2%) by age 20\u201322 years. FVC% declined at a rate of 3.19% per year with every 1\u2010year increase in age. FVC% declined annually by 2.47% in nonambulatory participants versus by 0.96% in ambulatory participants. FVC% did not significantly change over age with the presence of scoliosis or use of ventilatory technology.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The data from this large cohort are valuable for understanding real\u2010world patterns of clinical care and disease progression. There is a significant association between the loss of ambulation and the rate of FVC% decline. Further longitudinal studies are needed to better understand the impact of disease parameters on pulmonary function decline and the need for ventilatory support.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.26554","type":"journal-article","created":{"date-parts":[[2023,8,4]],"date-time":"2023-08-04T11:40:38Z","timestamp":1691149238000},"page":"2725-2732","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Duchenne muscular dystrophy respiratory profiles from real world registry data"],"prefix":"10.1002","volume":"58","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7716-3807","authenticated-orcid":false,"given":"Mona","family":"Hnaini","sequence":"first","affiliation":[{"name":"Department of Pediatrics Schulich School of Medicine &amp; Dentistry Western University  London Ontario 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