{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T16:36:43Z","timestamp":1776271003317,"version":"3.50.1"},"reference-count":30,"publisher":"BMJ","issue":"5","license":[{"start":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T00:00:00Z","timestamp":1772150400000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100012295","name":"NIHR Newcastle Biomedical Research Centre","doi-asserted-by":"publisher","award":["NIHR203309"],"award-info":[{"award-number":["NIHR203309"]}],"id":[{"id":"10.13039\/501100012295","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","award":["MR\/W019086\/1"],"award-info":[{"award-number":["MR\/W019086\/1"]}],"id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["J Neurol Neurosurg Psychiatry"],"accepted":{"date-parts":[[2026,1,25]]},"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Becker muscular dystrophy (BMD) is a rare X-linked neuromuscular disorder predominantly affecting males. While respiratory function has been characterised in small cohorts over limited time frames, comprehensive long-term longitudinal data remain lacking, as do established care guidelines to inform respiratory surveillance in BMD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>In this retrospective analysis, we present a large longitudinal analysis of respiratory function in patients with BMD, based on 1360 spirometry measurements from a single-centre cohort of 152\u2009patients. Analysed assessments spanned paediatric to adult ages (3.4\u201386.3 years), with a mean follow-up duration of 11 years per patient.<\/jats:p>\n                    <jats:p>Linear mixed-effects models were used to study longitudinal changes in respiratory function.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Respiratory decline in BMD appears gradual and variable, typically not affecting children (&lt;18 years of age).<\/jats:p>\n                    <jats:p>Respiratory support was infrequent (11.2%), always non-invasive and limited to nocturnal use.<\/jats:p>\n                    <jats:p>Loss of ambulation emerged as a strong predictor of faster decline in forced vital capacity percentage of predicted (FVC%) (estimate \u22120.58%\/year, p=0.002), with the requirement for assistive walking devices marking a critical transitional stage.<\/jats:p>\n                    <jats:p>Upper limb function, assessed via the PUL 2.0 entry item, correlated significantly with FVC%, particularly among non-ambulant patients (rho=0.60, p=0.02). Cardiac involvement showed a limited effect on respiratory function, likely driven by patients with more advanced cardiomyopathy.<\/jats:p>\n                    <jats:p>No consistent genotype-phenotype correlations were observed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>These findings provide important evidence to inform clinical management, supporting the recommendation of individualised respiratory monitoring strategies and contributing to the design and interpretation of clinical trials in BMD.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/jnnp-2025-337953","type":"journal-article","created":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T18:20:26Z","timestamp":1772216426000},"page":"456-463","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Respiratory function in Becker muscular dystrophy: a comprehensive longitudinal study"],"prefix":"10.1136","volume":"97","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-0241-2174","authenticated-orcid":false,"given":"Pietro","family":"Riguzzi","sequence":"first","affiliation":[{"name":"Translational and Clinical Research Institute, Newcastle University and Newcastle Hospitals NHS Foundation Trust, John Walton Muscular Dystrophy Research Centre, Newcastle upon Tyne, UK"},{"name":"Department of Neurosciences DNS, Universita degli Studi di Padova, Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emma","family":"Grover","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Newcastle University and Newcastle Hospitals NHS Foundation Trust, John Walton Muscular Dystrophy Research Centre, Newcastle upon Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2709-265X","authenticated-orcid":false,"given":"Marianela","family":"Schiava","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Newcastle University and Newcastle Hospitals NHS Foundation Trust, John Walton Muscular Dystrophy Research Centre, Newcastle upon Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Meredith K","family":"James","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Newcastle University and Newcastle Hospitals NHS Foundation Trust, John Walton Muscular Dystrophy Research Centre, Newcastle upon Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2941-7988","authenticated-orcid":false,"given":"Jordi","family":"Diaz-Manera","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Newcastle University and Newcastle Hospitals NHS Foundation Trust, John Walton Muscular Dystrophy Research Centre, Newcastle upon Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giorgio","family":"Tasca","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Newcastle University and Newcastle Hospitals NHS Foundation Trust, John Walton Muscular Dystrophy Research Centre, Newcastle upon Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John","family":"Bourke","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Newcastle University and Newcastle Hospitals NHS Foundation Trust, John Walton Muscular Dystrophy Research Centre, Newcastle upon Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Holly","family":"Borland","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Newcastle University and Newcastle Hospitals NHS Foundation Trust, John Walton Muscular Dystrophy Research Centre, Newcastle upon Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dionne","family":"Moat","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Newcastle University and Newcastle Hospitals NHS Foundation Trust, John Walton Muscular Dystrophy Research Centre, Newcastle upon Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maha","family":"Elseed","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Newcastle University and Newcastle Hospitals NHS Foundation Trust, John Walton Muscular Dystrophy Research Centre, Newcastle upon Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jassi","family":"Michell-Sodhi","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Newcastle University and 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Newcastle Hospitals NHS Foundation Trust, John Walton Muscular Dystrophy Research Centre, Newcastle upon Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emma","family":"Robinson","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Newcastle University and Newcastle Hospitals NHS Foundation Trust, John Walton Muscular Dystrophy Research Centre, Newcastle upon Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tara","family":"Reeves","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Newcastle University and Newcastle Hospitals NHS Foundation Trust, John Walton Muscular Dystrophy Research Centre, Newcastle upon Tyne, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Waldock","sequence":"additional","affiliation":[{"name":"Translational and Clinical Research Institute, Newcastle University and Newcastle Hospitals NHS 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