{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T00:33:38Z","timestamp":1783384418482,"version":"3.54.6"},"reference-count":27,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2024,12,16]],"date-time":"2024-12-16T00:00:00Z","timestamp":1734307200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000196","name":"Canada Foundation for Innovation","doi-asserted-by":"publisher","award":["CFI\u2010JELF 38412"],"award-info":[{"award-number":["CFI\u2010JELF 38412"]}],"id":[{"id":"10.13039\/501100000196","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000024","name":"Canadian Institutes of Health Research","doi-asserted-by":"publisher","award":["FDN\u2010167281"],"award-info":[{"award-number":["FDN\u2010167281"]}],"id":[{"id":"10.13039\/501100000024","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2025,2]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Introduction\/Aim<\/jats:title><jats:p>Patients with myotonic dystrophy type1 (DM1) have reduced lifespan. This study aimed to quantify mortality risks, and evaluate causes and time trends in DM1.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We identified 1021 DM1 patients and 15,104 matched DM1\u2010free controls from the United Kingdom (UK) Clinical Practice Research Datalink. We used Cox proportional hazards regression models to assess differences in all\u2010cause or cause\u2010specific mortality between DM1 patients and matched controls, and computed standardized mortality ratios (SMRs) for comparisons of DM1 patients with the UK general population.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>DM1 patients were at higher risk of death compared with matched DM1\u2010free controls (hazard ratio [HR]\u2009=\u20092.9, 95% confidence interval [CI]\u2009=\u20092.5\u20133.4) or the general UK population (SMR\u2009=\u20098.1, 95% CI\u2009=\u20097.3\u20139.1). The excess risk was primarily attributed to deaths from respiratory failure (HR\u2009=\u200926.7, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001), aspiration pneumonia (HR\u2009=\u200915.8, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001), arrythmia, and conduction defects (HR\u2009=\u200915.7, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). No mortality risk difference between DM1 patients and matched DM1\u2010free cohort was noted for all cancers combined (<jats:italic>p<\/jats:italic>\u2009=\u20090.52). No significant calendar time\u2010related changes in overall survival were seen for DM1 patients (<jats:italic>p<\/jats:italic> trend\u2009=\u20090.19). In mortality cause\u2010specific analysis, and compared with patients diagnosed before 1993, death from cancer was on the rise (HR\u2009=\u20092.35, and 5.82 for patients diagnosed 1993\u20132003, and 2004\u20132016).<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Most DM1 patients died of known disease complications. This highlights the need for integrated clinical approaches with more careful and frequent monitoring.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.28308","type":"journal-article","created":{"date-parts":[[2024,12,16]],"date-time":"2024-12-16T13:35:13Z","timestamp":1734356113000},"page":"229-236","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Mortality Trends and Causes of Death in Myotonic Dystrophy Type 1 Patients From the UK Clinical Practice Research Datalink"],"prefix":"10.1002","volume":"71","author":[{"given":"Rotana","family":"Alsaggaf","sequence":"first","affiliation":[{"name":"Clinical Genetics Branch, Division of Cancer Epidemiology &amp; Genetics, National Cancer Institute National Institutes of Health  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruth M.","family":"Pfeiffer","sequence":"additional","affiliation":[{"name":"Biostatistics Branch, Division of Cancer Epidemiology &amp; Genetics, National Cancer Institute National Institutes of Health  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily E.","family":"Pearce","sequence":"additional","affiliation":[{"name":"Clinical Genetics Branch, Division of Cancer Epidemiology &amp; Genetics, National Cancer Institute National Institutes of Health  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark H.","family":"Greene","sequence":"additional","affiliation":[{"name":"Clinical Genetics Branch, Division of Cancer Epidemiology &amp; Genetics, National Cancer Institute National Institutes of Health  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanns","family":"Lochmuller","sequence":"additional","affiliation":[{"name":"Children's Hospital of Eastern Ontario Research Institute, Division of Neurology, Department of Medicine, The Ottawa Hospital, and Brain and Mind Research Institute University of Ottawa  Ottawa Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3255-8143","authenticated-orcid":false,"given":"Shahinaz M.","family":"Gadalla","sequence":"additional","affiliation":[{"name":"Clinical Genetics Branch, Division of Cancer Epidemiology &amp; Genetics, National Cancer Institute National Institutes of Health  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,12,16]]},"reference":[{"key":"e_1_2_11_2_1","volume-title":"GeneReviews(R)","author":"Bird T. 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