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We investigated the prevalence of cardiovascular risk factors and cardiovascular events in adults with LOPD compared to an individually matched control cohort.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Adults with LOPD were individually matched to control subjects, with the same sex, smoking status, age (\u00b1\u20095 years) and BMI (\u00b1\u20095\u00a0kg\/m\n                      <jats:sup>2<\/jats:sup>\n                      ). Assessments included measurement of height, weight, waist-hip circumference, 30\u00a0min blood pressure measurement, electrocardiogram, and blood and urine laboratory analysis. Medical history of cardiovascular risk factors and events were collected via questionnaires and patient records.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Seventy-eight patients (median age 55.9 years, BMI 25.1\u00a0kg\/m\n                      <jats:sup>2<\/jats:sup>\n                      ) and 78 control subjects (median age 55.3 years, BMI 25.6\u00a0kg\/m\n                      <jats:sup>2<\/jats:sup>\n                      ) were included. Patients had a higher resting heart rate (76 vs. 66 BPM,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001), larger waist circumference (93 vs. 90\u00a0cm,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.011) and waist-to-hip ratio (0.91 vs. 0.86,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.005). A greater proportion of patients than controls had a history of hypertension (32% vs. 14%;\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.026). More patients than controls (19 vs. 9%) experienced a cardiovascular event in the past, but this difference was not statistically significant.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Patients with LOPD showed a less favorable cardiovascular risk profile compared to an individually matched control cohort. The prevalence of cardiovascular events did not significantly differ between the cohorts, although our study is likely underpowered to detect subtle differences due to the low number of events.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s13023-026-04400-8","type":"journal-article","created":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T16:15:13Z","timestamp":1779898513000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Unfavorable cardiovascular risk profile without increased event prevalence in late-onset Pompe disease: an individually matched cohort study"],"prefix":"10.1186","volume":"21","author":[{"given":"Harmke A.","family":"van Kooten","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danny","family":"van Zoest","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle","family":"Michels","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadine A. 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Written informed consent was obtained from all participants in this study; all forms are on file and available in case of request. All individual patient data was de-identified. This study was performed in accordance with the ethical standards of the Helsinki Declaration.","order":1,"name":"Ethics","label":"Ethical approval","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"HvK, DvZ and EB declare no relevant conflicts of interest. MM received research grants from Bristol Meyers Squibb and Cytokinetics and speaker honoraria from Bristol Meyers Squibb and Sanofi. She participated in advisory board meetings for Bayer, Alnylam and Bristol Meyers Squibb. Apart from speaker honorarium from Bristol Meyers Squibb, all payments were made to Erasmus MC University Medical Center. NvdB has participated in advisory board meetings for Sanofi, Bayer and Amicus Therapeutics and has received speaker honoraria from Sanofi and Recordati Rare Disease Foundation (RRDF). All payments were made to Erasmus MC University Medical Center. AvdP has received funding for research and clinical trials or advisory fees from Sanofi, Amicus Therapeutics, Denali Therapeutics, and Spark Therapeutics, under agreements with Erasmus MC University Medical Center and the relevant industry. JL received funding for research and\/or participated in clinical trials from Clinuvel, Alnylam, Ultragenyx, Moderna, and Disc medicine. All payments were made to Erasmus MC University Medical Center. PvD has received research grants from the Prinses Beatrix Spierfonds and participated as a scientific advisor\/steering committee member for Annexon, Argenx, Hansa, Octapharma, and Sanofi. He received speaker honoraria from Argenx and Grifols. All payments were made to Erasmus MC University Medical Center. MW participates in industry sponsored clinical trials from Moderna, Ultragenyx, Clinuval, Mitshubishi, under agreements between the industry and Erasmus MC University Medical Center.","order":2,"name":"Ethics","label":"Conflict of interest","group":{"name":"EthicsHeading","label":"Declarations"}}],"article-number":"256"}}