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Arterial fragility is an important feature of the disease, but its characterisation remains limited. We report the clinical history of a 41-year-old woman who presented repeated arterial accidents, which occurred in previously normal medium size arteries within a limited time span of 2 years. Molecular investigations revealed compound heterozygosity for two PLOD1 gene deletions of exons 11\u201312 and 14\u201315. Arterial fragility is an important characteristic of kyphoscoliotic EDS. It manifests as spontaneous arterial rupture, dissections and dissecting aneurysms which may occur even during early childhood. This fragility is particularly likely to manifest during surgical intervention. Early medical management and surveillance may be indicated, but its modalities remain to be defined.<\/jats:p>","DOI":"10.1136\/bcr-2018-224423","type":"journal-article","created":{"date-parts":[[2018,7,7]],"date-time":"2018-07-07T06:54:53Z","timestamp":1530946493000},"page":"bcr-2018-224423","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":18,"title":["Arterial fragility in kyphoscoliotic Ehlers-Danlos syndrome"],"prefix":"10.1136","volume":"2018","author":[{"given":"Pierrick","family":"Henneton","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne","family":"Legrand","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cecilia","family":"Giunta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Frank","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2018,7,6]]},"reference":[{"key":"2025042705273367000_2018.jul06_1.bcr-2018-224423.1","doi-asserted-by":"publisher","DOI":"10.1006\/mgme.2000.3076"},{"key":"2025042705273367000_2018.jul06_1.bcr-2018-224423.2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197205112861901"},{"key":"2025042705273367000_2018.jul06_1.bcr-2018-224423.3","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19980428)77:1<31::AID-AJMG8>3.0.CO;2-O"},{"key":"2025042705273367000_2018.jul06_1.bcr-2018-224423.4","unstructured":"Yeowell HN , Steinmann B , Syndrome E-D , et al . 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