{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T11:33:28Z","timestamp":1785411208785,"version":"3.56.0"},"reference-count":30,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2014,1,10]],"date-time":"2014-01-10T00:00:00Z","timestamp":1389312000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2014,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Vascular abnormalities and glycogen accumulation in vascular smooth muscle fibres have been described in Pompe disease. Using carotid\u2010femoral pulse wave velocity (cfPWV), the gold standard methodology for determining aortic stiffness, we studied whether aortic stiffness is increased in patients with Pompe disease. Eighty\u2010four adult Pompe patients and 179 age\u2010 and gender\u2010matched volunteers participated in this cross\u2010sectional case\u2010controlled study. Intima media thickness and the distensibility of the right common carotid artery were measured using a Duplex scanner. Aortic augmentation index, central pulse pressure, aortic reflexion time and cfPWV were assessed using the SphygmoCor\u00ae system. CfPWV was higher in patients than in volunteers (8.8 versus 7.4 m\/s, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). This difference was still present after adjustment for age, gender, mean arterial blood pressure (MAP), heart rate and diabetes mellitus (<jats:italic>p<\/jats:italic>\u2009=\u20090.001), and was shown by subgroup analysis to apply to the 40\u201059 years age group (<jats:italic>p<\/jats:italic>\u2009=\u20090.004) and 60+ years age group (<jats:italic>p<\/jats:italic>\u2009=\u20090.01), but not to younger age groups (<jats:italic>p<\/jats:italic>\u2009=\u20090.99). Except for a shorter aortic reflexion time (<jats:italic>p<\/jats:italic>\u2009=\u20090.02), indirect indicators of arterial stiffness did not differ between patients and volunteers. Relative to volunteers (20 %), more Pompe patients had a history of hypertension (36 %, <jats:italic>p<\/jats:italic>\u2009=\u20090.005), and the MAP was higher than in volunteers (100 versus 92 mmHg, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). This study shows that patients with non\u2010classic Pompe disease have increased aortic stiffness and blood pressure. Whether this is due to glycogen accumulation requires further investigation. To reduce the potential risk of cardiovascular diseases, we recommend that blood pressure and other common cardiovascular risk factors are monitored regularly.<\/jats:p>","DOI":"10.1007\/s10545-013-9667-2","type":"journal-article","created":{"date-parts":[[2014,1,9]],"date-time":"2014-01-09T13:30:24Z","timestamp":1389274224000},"page":"391-397","source":"Crossref","is-referenced-by-count":13,"title":["Increased aortic stiffness and blood pressure in non\u2010classic Pompe disease"],"prefix":"10.1002","volume":"37","author":[{"given":"Stephan C. A.","family":"Wens","sequence":"first","affiliation":[{"name":"Department of Neurology Erasmus University Medical Center Mailbox 2040 Rotterdam 3000 CA The Netherlands"},{"name":"Center for Lysosomal and Metabolic Diseases Erasmus MC Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esther","family":"Kuperus","sequence":"additional","affiliation":[{"name":"Department of Neurology Erasmus University Medical Center Mailbox 2040 Rotterdam 3000 CA The Netherlands"},{"name":"Center for Lysosomal and Metabolic Diseases Erasmus MC Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco U. S.","family":"Mattace\u2010Raso","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Erasmus MC Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle E.","family":"Kruijshaar","sequence":"additional","affiliation":[{"name":"Center for Lysosomal and Metabolic Diseases Erasmus MC Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esther","family":"Brusse","sequence":"additional","affiliation":[{"name":"Department of Neurology Erasmus University Medical Center Mailbox 2040 Rotterdam 3000 CA The Netherlands"},{"name":"Center for Lysosomal and Metabolic Diseases Erasmus MC Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kees C. A. G. 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