{"doi":"10.1002/mus.28308","title":"Mortality Trends and Causes of Death in Myotonic Dystrophy Type 1 Patients From the UK Clinical Practice Research Datalink","abstract":"INTRODUCTION/AIM: Patients with myotonic dystrophy type1 (DM1) have reduced lifespan. This study aimed to quantify mortality risks, and evaluate causes and time trends in DM1. METHODS: We identified 1021 DM1 patients and 15,104 matched DM1-free controls from the United Kingdom (UK) Clinical Practice Research Datalink. We used Cox proportional hazards regression models to assess differences in all-cause or cause-specific mortality between DM1 patients and matched controls, and computed standardized mortality ratios (SMRs) for comparisons of DM1 patients with the UK general population. RESULTS: DM1 patients were at higher risk of death compared with matched DM1-free controls (hazard ratio [HR] = 2.9, 95% confidence interval [CI] = 2.5-3.4) or the general UK population (SMR = 8.1, 95% CI = 7.3-9.1). The excess risk was primarily attributed to deaths from respiratory failure (HR = 26.7, p < 0.001), aspiration pneumonia (HR = 15.8, p < 0.001), arrythmia, and conduction defects (HR = 15.7, p < 0.001). No mortality risk difference between DM1 patients and matched DM1-free cohort was noted for all cancers combined (p = 0.52). No significant calendar time-related changes in overall survival were seen for DM1 patients (p trend = 0.19). In mortality cause-specific analysis, and compared with patients diagnosed before 1993, death from cancer was on the rise (HR = 2.35, and 5.82 for patients diagnosed 1993-2003, and 2004-2016). DISCUSSION: Most DM1 patients died of known disease complications. This highlights the need for integrated clinical approaches with more careful and frequent monitoring.","journal":"Muscle & Nerve","year":2024,"id":441631,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":9,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.6096,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2024-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":282490,"name":"Ruth M. Pfeiffer","orcid":"0000-0001-7791-2698","position":1,"is_corresponding":false},{"id":603627,"name":"Emily Pearce","orcid":"0000-0003-4222-9340","position":2,"is_corresponding":false},{"id":78619,"name":"Mark H. Greene","orcid":"0000-0003-1852-9239","position":3,"is_corresponding":false},{"id":18317,"name":"Hanns Lochmüller","orcid":"0000-0003-2324-8001","position":4,"is_corresponding":false},{"id":284135,"name":"Shahinaz M. Gadalla","orcid":"0000-0002-3255-8143","position":5,"is_corresponding":false},{"id":637753,"name":"Rotana Alsaggaf","orcid":"0000-0001-5043-8789","position":0,"is_corresponding":true}],"reference_count":24,"raw_metadata":null,"created_at":"2026-07-19T02:01:11.152920Z","pmid":"39679826","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}