{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T20:12:01Z","timestamp":1789589521483,"version":"build-2803163510"},"reference-count":48,"publisher":"Oxford University Press (OUP)","issue":"5","license":[{"start":{"date-parts":[[2023,10,9]],"date-time":"2023-10-09T00:00:00Z","timestamp":1696809600000},"content-version":"vor","delay-in-days":38,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001349","name":"National Medical Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001349","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000274","name":"British Heart Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000274","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,9,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>The fragmentation and loss of elastic fibre in the tunica media of the aorta are pathological hallmarks of Marfan syndrome (MFS) but the dynamics of elastin degradation and its relationship to aortic size and physiological growth remain poorly understood.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods and results<\/jats:title>\n                  <jats:p>In this post hoc analysis of the AIMS randomized controlled trial, the association of plasma desmosine (pDES)\u2014a specific biomarker of mature elastin degradation\u2014with age and aortic size was analysed in 113 patients with MFS and compared to 109 healthy controls. There was a strong association between age and pDES in both groups, with higher pDES levels in the lower age groups compared to adults. During childhood, pDES increased and peaked during early adolescence, and thereafter decreased to lower adult levels. This trend was exaggerated in young individuals with MFS but in those above 25 years of age, pDES levels were comparable to controls despite the presence of aortic root dilation. In MFS children, increased aortic diameter relative to controls was seen at an early age and although the increase in diameter was less after adolescence, aortic root size continued to increase steadily with age. In MFS participants, there was an indication of a positive association between baseline pDES levels and aortic root dilatation during up to 5 years of follow-up.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>This study has shown that developmental age has a significant effect on levels of elastin turnover as measured by pDES in MFS individuals as well as healthy controls. This effect is exaggerated in those with MFS with increased levels seen during the period of physiologic development that plateaus towards adulthood. This suggests an early onset of pathophysiology that may present an important opportunity for disease-modifying intervention.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjopen\/oead095","type":"journal-article","created":{"date-parts":[[2023,10,9]],"date-time":"2023-10-09T10:28:40Z","timestamp":1696847320000},"source":"Crossref","is-referenced-by-count":12,"title":["Exaggerated elastin turnover in young individuals with Marfan syndrome: new insights from the AIMS trial"],"prefix":"10.1093","volume":"3","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0317-639X","authenticated-orcid":false,"given":"Zaid","family":"Iskandar","sequence":"first","affiliation":[{"name":"School of Medicine, University Dundee , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6207-6604","authenticated-orcid":false,"given":"Matthew","family":"Dodd","sequence":"additional","affiliation":[{"name":"Clinical Trials Unit, Department of Medical 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Heart Institute, University of Bristol, Bristol, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1266-3288","authenticated-orcid":false,"given":"Tim","family":"Clayton","sequence":"additional","affiliation":[{"name":"Clinical Trials Unit, Department of Medical Statistics, London School of Hygiene & Tropical Medicine , London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne","family":"Child","sequence":"additional","affiliation":[{"name":"Royal Brompton and Harefield Clinical Group, Guy\u2019s and St Thomas\u2019 Trust, King\u2019s College, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xu Yu","family":"Jin","sequence":"additional","affiliation":[{"name":"Oxford Heart Centre, John Radcliffe Hospital, Oxford, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jos\u00e9 Antonio","family":"Aragon-Martin","sequence":"additional","affiliation":[{"name":"National Heart & 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