{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T20:35:09Z","timestamp":1791837309261,"version":"4.3.4"},"reference-count":10,"publisher":"SAGE Publications","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JND"],"published-print":{"date-parts":[[2020,9,18]]},"abstract":"<jats:p>Background: Few studies have examined respiratory dysfunction in patients with Becker muscular dystrophy (BMD). Objective: This study aimed to examine the characteristics of respiratory dysfunction in patients with BMD. Methods: The present retrospective study assessed respiratory parameters of adult BMD patients using medical records and compared these parameters with various patient characteristics to identify correlations. BMD patients aged 17 years and older who had been diagnosed genetically and\/or pathologically were included in the analysis. Results: Of the source population of 133 patients, respiratory function was assessed in 85. Two of these patients had no symptoms, and eight had died. Mean % forced vital capacity (% FVC) was 94.2+\/\u201321.7% (median, 96.1%; range, 5.1\u2013134.1%). In 16 (19%) of the 85 patients, % FVC was &lt;80%. Of these, seven were non-ambulant. Age, ambulation, and cardiac function did not significantly differ between patients with or without respiratory dysfunction, whereas age at onset was significantly lower in patients with respiratory dysfunction (7.7+\/\u20134.7 years vs. 14.4+\/\u201311.9 years; p\u200a=\u200a0.001). One non-ambulant patient was a continuous NPPV user, and one patient had been recommended NPPV use but refused. Autopsy of one patient revealed that the diaphragm and intercostal muscles were less affected than proximal skeletal muscles. Conclusion: BMD patients are at risk of developing respiratory dysfunction due to dystrophic changes in respiratory muscles. Respiratory function should be carefully and periodically monitored in these patients.<\/jats:p>","DOI":"10.3233\/jnd-190438","type":"journal-article","created":{"date-parts":[[2020,7,7]],"date-time":"2020-07-07T11:13:18Z","timestamp":1594120398000},"page":"425-431","source":"Crossref","is-referenced-by-count":6,"title":["Respiratory Dysfunction in Becker Muscular Dystrophy Patients: A Case Series and Autopsy Report"],"prefix":"10.1177","volume":"7","author":[{"given":"Madoka","family":"Mori-Yoshimura","sequence":"first","affiliation":[{"name":"Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Ogawahigashi, Kodaira, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasushi","family":"Oya","sequence":"additional","affiliation":[{"name":"Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Ogawahigashi, Kodaira, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hirohumi","family":"Komaki","sequence":"additional","affiliation":[{"name":"Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Ogawahigashi, Kodaira, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuhiko","family":"Segawa","sequence":"additional","affiliation":[{"name":"Department of Cardiology, National Center Hospital, National Center of Neurology and Psychiatry, Ogawahigashi, Kodaira, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Narihiro","family":"Minami","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, National Center Hospital, National Center of Neurology and Psychiatry, Ogawahigashi, Kodaira, Tokyo, Japan"},{"name":"Medical Genome Center, National Center of Neurology and Psychiatry, Ogawahigashi, Kodaira, Tokyo, Japan"},{"name":"Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Ogawahigashi, Kodaira, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuko","family":"Saito","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, National Center Hospital, National Center of Neurology and Psychiatry, Ogawahigashi, Kodaira, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ichizo","family":"Nishino","sequence":"additional","affiliation":[{"name":"Medical Genome Center, National Center of Neurology and Psychiatry, Ogawahigashi, Kodaira, Tokyo, Japan"},{"name":"Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Ogawahigashi, Kodaira, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuji","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Ogawahigashi, Kodaira, Tokyo, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","reference":[{"key":"10.3233\/JND-190438_ref1","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1136\/jmg.25.1.9","article-title":"Mild and severe muscular dystrophy associated with deletions in Xp21 of the human X chromosome","volume":"25","author":"Davies","year":"1988","journal-title":"J Med Genet"},{"key":"10.3233\/JND-190438_ref2","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1016\/S1474-4422(03)00585-4","article-title":"Dystrophin and mutations: One gene, several proteins, multiple phenotypes","volume":"2","author":"Muntoni","year":"2003","journal-title":"Lancet Neurol"},{"key":"10.3233\/JND-190438_ref3","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1016\/S0140-6736(02)07815-7","article-title":"The muscular dystrophies","volume":"359","author":"Emery","year":"2002","journal-title":"Lancet."},{"key":"10.3233\/JND-190438_ref4","first-page":"121","article-title":"Improvement of survival in Duchenne Muscular Dystrophy: Retrospective analysis of 835 patients","volume":"31","author":"Passamano","year":"2012","journal-title":"Acta Myol"},{"key":"10.3233\/JND-190438_ref5","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1016\/0888-7543(88)90113-9","article-title":"An explanation for the phenotypic differences between patients bearing partial deletions of the DMD locus","volume":"2","author":"Monaco","year":"1988","journal-title":"Genomics"},{"key":"10.3233\/JND-190438_ref6","doi-asserted-by":"crossref","first-page":"193","DOI":"10.3233\/JND-170225","article-title":"Characteristics of Japanese Patients with Becker Muscular Dystrophy and Intermediate Muscular Dystrophy in a Japanese National Registry of Muscular Dystrophy (Remudy): Heterogeneity and Clinical Variation","volume":"5","author":"Mori-Yoshimura","year":"2018","journal-title":"J Neuromuscul Dis"},{"key":"10.3233\/JND-190438_ref7","doi-asserted-by":"crossref","first-page":"1610","DOI":"10.1007\/s00415-011-5979-z","article-title":"Genotype and phenotype characterization in a large dystrophinopathic cohort with extended follow-up","volume":"258","author":"Magri","year":"2011","journal-title":"J Neurol"},{"key":"10.3233\/JND-190438_ref8","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1016\/j.braindev.2018.02.012","article-title":"Identification of sleep hypoventilation in young individuals with Becker muscular dystrophy: A pilot study","volume":"40","author":"Nakamura","year":"2018","journal-title":"Brain Dev"},{"key":"10.3233\/JND-190438_ref9","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1136\/jnnp-2013-306350","article-title":"Dystrophin levels and clinical severity in Becker muscular dystrophy patients","volume":"85","author":"van den Bergen","year":"2014","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"1-2","key":"10.3233\/JND-190438_ref10","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1016\/S0022-510X(03)00041-8","article-title":"Protein and gene analyses of dysferlinopathy in a large group of Japanese muscular dystrophy patients","volume":"211","author":"Tagawa","year":"2003","journal-title":"J Neurol Sci"}],"container-title":["Journal of Neuromuscular Diseases"],"original-title":[],"link":[{"URL":"https:\/\/content.iospress.com\/download?id=10.3233\/JND-190438","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T23:49:29Z","timestamp":1777506569000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/full\/10.3233\/JND-190438"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,9,18]]},"references-count":10,"journal-issue":{"issue":"4"},"URL":"https:\/\/doi.org\/10.3233\/jnd-190438","relation":{},"ISSN":["2214-3599","2214-3602"],"issn-type":[{"value":"2214-3599","type":"print"},{"value":"2214-3602","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,9,18]]}}}