{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T18:08:23Z","timestamp":1787508503002,"version":"build-2736575974"},"reference-count":39,"publisher":"SAGE Publications","issue":"3","license":[{"start":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T00:00:00Z","timestamp":1761004800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T00:00:00Z","timestamp":1761004800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Neuromuscular Diseases"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Myotonic dystrophy type 1 (DM1) is characterized by a lifelong progressive muscular weakness associated with life-threatening events such as chronic respiratory failure (CRF). Early identification of patients at risk remains challenging.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To identify clinical and biological markers predictive of CRF onset and survival in a large DM1 cohort.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a retrospective cohort study of 126 DM1 adult patients followed from 2000 to 2024 at Rouen University Hospital. The primary outcome was the first forced vital capacity (FVC)\u2009\u2264\u200970% of predicted value. Prognostic factors were analyzed using Cox proportional hazards models with the start of follow-up at the time of diagnosis of DM1 and were followed until the outcome occurred, or death (censorship according to Kalbfleisch &amp; Prentice approach to competing risks).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>During a median follow-up of 12.5 years, 45 patients developed FVC\u2009\u2264\u200970%. Muscular impairment rating scale (MIRS) (HR: 1.85, 95%CI:1.30\u20132.63) and conduction\/rhythm disorders (HR: 3.13, 95%CI: 1.37\u20137.31) at diagnosis were independent predictors of FVC\u2009\u2264\u200970% adjusted on age, female sex (HR: 1.54, 95%CI: 0.82\u20132.91) and cataract at diagnosis (HR: 0.50, 95%CI: 0.22\u20131.16). CTG repeats \u2265400 was associated with a doubled risk of respiratory decline but did not reach statistical significance. The 20-year mortality rate was 32%. MIRS (HR: 2.34, 95%CI:1.22- 4.51), male sex and older age at diagnosis significantly predicted death.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Muscular impairment and cardiac rhythm\/conduction disorders at diagnosis were strong predictors of respiratory complications. MIRS, male sex and older age at diagnosis were predictors of mortality in DM1. These prognostic markers should inform clinical management strategies to improve survival in DM1 patients.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/22143602251386258","type":"journal-article","created":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T12:33:39Z","timestamp":1761050019000},"page":"344-354","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":2,"title":["Predictors of respiratory failure and survival in myotonic dystrophy type 1"],"prefix":"10.1177","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3407-7351","authenticated-orcid":false,"given":"Marie-Anne","family":"Melone","sequence":"first","affiliation":[{"name":"Thoracic Oncology and Respiratory Intensive Care"},{"name":"Normandie Univ, UNIROUEN, UR3830 GRHVN, Institute for Research and Innovation in Biomedicine, Rouen, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivana","family":"Dabaj","sequence":"additional","affiliation":[{"name":"Normandie Univ, UNIROUEN, CHU Rouen, AIMS, SysMedLab, Department of Neonatology, Pediatric Intensive Care and Neuropediatrics, Nord\/Est\/Ile de France Neuromuscular Diseases Reference Center, CHU Rouen, FILNEMUS, Rouen, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maxime","family":"Patout","sequence":"additional","affiliation":[{"name":"AP-HP, Groupe Hospitalier Universitaire APHP-Sorbonne Universit\u00e9, site Piti\u00e9-Salp\u00eatri\u00e8re, FHU UMANHYS, Service des Pathologies du Sommeil (D\u00e9partement R3S), Paris, France"},{"name":"Sorbonne Universit\u00e9, INSERM, UMRS1158 Neurophysiologie Respiratoire Exp\u00e9rimentale et Clinique, Paris, France"},{"name":"D\u00e9partement R3S (Respiration, R\u00e9animation, R\u00e9adaptation Respiratoire, Sommeil), FHU UMANHYS, Centre de R\u00e9f\u00e9rence Constitutif Maladies Pulmonaires Rares de l'Adulte Orphalung, Hypoventilations Centrales, Syndrome d'Ondine, AP-HP, Groupe Hospitalier Universitaire APHP-Sorbonne Universit\u00e9, H\u00f4pital Piti\u00e9-Salp\u00eatri\u00e8re, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andr\u00e9","family":"Gillibert","sequence":"additional","affiliation":[{"name":"Department of Biostatistics, Rouen University Hospital, Rouen, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elise","family":"Artaud-Macari","sequence":"additional","affiliation":[{"name":"Thoracic Oncology and Respiratory Intensive Care"},{"name":"Normandie Univ, UNIROUEN, UR3830 GRHVN, Institute for Research and Innovation in Biomedicine, Rouen, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucie","family":"Guyant-Marechal","sequence":"additional","affiliation":[{"name":"Department of Neurology, Rouen University Hospital, Rouen, France"},{"name":"Department of Genetics, Rouen University Hospital, Nord\/Est\/Ile de France Neuromuscular Diseases Reference Center, CHU Rouen, FILNEMUS, Rouen, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne-Laure","family":"Bedat-Millet","sequence":"additional","affiliation":[{"name":"Department of Neurology, Rouen University Hospital, Rouen, France"},{"name":"Department of Neurophysiology, Rouen University Hospital, Nord\/Est\/Ile de France Neuromuscular Diseases Reference Center, CHU Rouen, FILNEMUS, Rouen, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne-Marie","family":"Guerrot","sequence":"additional","affiliation":[{"name":"Department of Genetics, Rouen University Hospital, Nord\/Est\/Ile de France Neuromuscular Diseases Reference Center, CHU Rouen, FILNEMUS, Rouen, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mathieu","family":"Salaun","sequence":"additional","affiliation":[{"name":"Thoracic Oncology and Respiratory 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