{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,2]],"date-time":"2026-08-02T22:06:13Z","timestamp":1785708373382,"version":"3.56.0"},"reference-count":36,"publisher":"Springer Science and Business Media LLC","issue":"2","license":[{"start":{"date-parts":[[2006,3,1]],"date-time":"2006-03-01T00:00:00Z","timestamp":1141171200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Curr Treat Options Cardio Med"],"published-print":{"date-parts":[[2006,3]]},"DOI":"10.1007\/s11936-006-0004-z","type":"journal-article","created":{"date-parts":[[2007,6,3]],"date-time":"2007-06-03T08:43:43Z","timestamp":1180860223000},"page":"121-127","source":"Crossref","is-referenced-by-count":19,"title":["The vascular ehlers-danlos syndrome"],"prefix":"10.1007","volume":"8","author":[{"given":"Dominique P.","family":"Germain","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","reference":[{"key":"4_CR1","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1056\/NEJM200003093421001","volume":"342","author":"M Pepin","year":"2000","unstructured":"Pepin M, Schwarze U, Superti-Furga A, Byers PH: Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med 2000, 342:673\u2013680. A major review of clinical and genetic features of vascular EDS with epidemiologic data.","journal-title":"N Engl J Med"},{"key":"4_CR2","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.anngen.2003.07.002","volume":"47","author":"DP Germain","year":"2004","unstructured":"Germain DP, Herrera-Guzman Y: Clinical and genetic features of vascular Ehlers-Danlos syndrome. Ann Genet 2004, 47:1\u20139.","journal-title":"Ann Genet"},{"key":"4_CR3","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1177\/014107680109400108","volume":"94","author":"M Jarmulowicz","year":"2001","unstructured":"Jarmulowicz M, Phillips WG: Vascular Ehlers-Danlos syndrome undiagnosed during life. J R Soc Med 2001, 94:28\u201330.","journal-title":"J R Soc Med"},{"key":"4_CR4","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1016\/S0967-2109(99)00070-8","volume":"8","author":"R Ascione","year":"2000","unstructured":"Ascione R, Gomes WJ, Bates M, et al.: Emergency repair of type A aortic dissection in type IV Ehlers-Danlos syndrome. Cardiovasc Surg 2000, 8:75\u201378.","journal-title":"Cardiovasc Surg"},{"key":"4_CR5","first-page":"687","volume":"42","author":"AG Sayin","year":"2001","unstructured":"Sayin AG, Bozkurt AK, Cangel U, et al.: A brachial aneurysm in childhood caused by Ehlers-Danlos syndrome. J Cardiovasc Surg 2001, 42:687\u2013689.","journal-title":"J Cardiovasc Surg"},{"key":"4_CR6","doi-asserted-by":"crossref","first-page":"1465","DOI":"10.1378\/chest.107.5.1465","volume":"107","author":"BA Yost","year":"1995","unstructured":"Yost BA, Vogelsang JP, Lie JT: Fatal hemoptysis in Ehlers-Danlos syndrome. Old malady with a new curse. Chest 1995, 107:1465\u20131467.","journal-title":"Chest"},{"key":"4_CR7","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1016\/0167-5273(96)02618-6","volume":"54","author":"RH Evans","year":"1996","unstructured":"Evans RH, Fraser AG: Spontaneous coronary artery rupture and cardiac tamponade in Ehlers-Danlos syndrome type IV. Int J Cardiol 1996, 54:283\u2013286.","journal-title":"Int J Cardiol"},{"key":"4_CR8","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1016\/S0196-0644(96)70066-5","volume":"28","author":"S Meldon","year":"1996","unstructured":"Meldon S, Brady W, Young JS: Presentation of Ehlers-Danlos syndrome: iliac artery pseudoaneurysm rupture. Ann Emerg Med 1996, 28:231\u2013234.","journal-title":"Ann Emerg Med"},{"key":"4_CR9","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1007\/s005950170228","volume":"31","author":"A Imamura","year":"2001","unstructured":"Imamura A, Nakamoto H, Inoue T, et al.: Ruptured dissecting aneurysm in bilateral iliac arteries caused by Ehlers-Danlos syndrome type IV: report of a case. Surg Today 2001, 31:85\u201389.","journal-title":"Surg Today"},{"key":"4_CR10","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/0736-4679(95)02039-X","volume":"14","author":"PJ Wimmer","year":"1996","unstructured":"Wimmer PJ, Howes DS, Rumoro DP, Carbone M: Fatal vascular catastrophe in Ehlers-Danlos syndrome: a case report and review. J Emerg Med 1996, 14:25\u201331.","journal-title":"J Emerg Med"},{"key":"4_CR11","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1016\/S0887-8994(01)00315-0","volume":"25","author":"T Kato","year":"2001","unstructured":"Kato T, Hattori H, Yorifuji T, et al.: Intracranial aneurysms in Ehlers-Danlos syndrome type IV in early childhood. Pediatr Neurol 2001, 25:336\u2013339.","journal-title":"Pediatr Neurol"},{"key":"4_CR12","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1007\/s11936-996-0018-6","volume":"6","author":"WI Schieving","year":"2004","unstructured":"Schieving WI: Cerebrovascular involvement in Ehlers-Danlos syndrome. Curr Treat Options Cardiovasc Med 2004, 6:231\u2013236.","journal-title":"Curr Treat Options Cardiovasc Med"},{"key":"4_CR13","doi-asserted-by":"crossref","first-page":"e57","DOI":"10.1161\/01.CIR.100.13.e57","volume":"100","author":"JH Koh","year":"1999","unstructured":"Koh JH, Kim JS, Hong SC, et al.: Skin manifestations, multiple aneurysms, and carotid-cavernous fistula in Ehlers-Danlos syndrome type IV. Circulation 1999, 100:e57-e58.","journal-title":"Circulation"},{"key":"4_CR14","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1097\/00041327-200206000-00002","volume":"22","author":"H Chuman","year":"2002","unstructured":"Chuman H, Trobe JD, Petty EM, et al.: Spontaneous direct carotid-cavernous fistula in Ehlers-Danlos syndrome type IV: two case reports and a review of the literature. J Neuroophthalmol 2002, 22:75\u201381.","journal-title":"J Neuroophthalmol"},{"key":"4_CR15","doi-asserted-by":"crossref","first-page":"1724","DOI":"10.1023\/A:1018869617076","volume":"42","author":"CM Gelbmann","year":"1997","unstructured":"Gelbmann CM, Kollinger M, Gmeinwieser J, et al.: Spontaneous rupture of liver in a patient with Ehlers Danlos disease type IV. Dig Dis Sci 1997, 42:1724\u20131730.","journal-title":"Dig Dis Sci"},{"key":"4_CR16","doi-asserted-by":"crossref","first-page":"2214","DOI":"10.1097\/00007890-200005270-00048","volume":"69","author":"BM Mistry","year":"2000","unstructured":"Mistry BM, Solomon H, Garvin PJ, et al.: Spontaneous rupture of the liver upon revascularization during transplantation. Transplantation 2000, 69:2214\u20132218.","journal-title":"Transplantation"},{"key":"4_CR17","first-page":"2282","volume":"91","author":"JA Solomon","year":"1996","unstructured":"Solomon JA, Abrams L, Lichtenstein GR: GI manifestations of Ehlers-Danlos syndrome. Am J Gastroenterol 1996, 91:2282\u20132288.","journal-title":"Am J Gastroenterol"},{"key":"4_CR18","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1007\/s100249900095","volume":"2","author":"MH Collins","year":"1999","unstructured":"Collins MH, Schwarze U, Carpentieri DF, et al.: Multiple vascular and bowel ruptures in an adolescent male with sporadic Ehlers-Danlos syndrome type IV. Pediatr Dev Pathol 1999, 2:86\u201393.","journal-title":"Pediatr Dev Pathol"},{"key":"4_CR19","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1055\/s-2007-994321","volume":"13","author":"AM Athanassiou","year":"1996","unstructured":"Athanassiou AM, Turrentine MA: Myocardial infarction and coronary artery dissection during pregnancy associated with type IV Ehlers-Danlos syndrome. Am J Perinatol 1996, 13:181\u2013183.","journal-title":"Am J Perinatol"},{"key":"4_CR20","first-page":"303","volume":"15","author":"B Debie","year":"2005","unstructured":"Debie B, Hamer F, Dahan K, et al.: Rupture of the renal artery and renal parenchyma in a pregnant woman with vascular form of Ehlers-Danlos syndrome. Prog Urol 2005, 15:303\u2013305.","journal-title":"Prog Urol"},{"key":"4_CR21","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1111\/j.1365-2133.1996.tb01143.x","volume":"135","author":"FM Pope","year":"1996","unstructured":"Pope FM, Narcisi P, Nicholls AC, et al.: COL3A1 mutations cause variable clinical phenotypes including acrogeria and vascular rupture. Br J Dermatol 1996, 135:163\u2013181.","journal-title":"Br J Dermatol"},{"key":"4_CR22","unstructured":"HGMD. Human Gene Mutation Database Cardiff: Collagen III alpha I. http:\/\/archive.uwcm.ac.uk\/uwcm\/ mg\/hgmd0.html."},{"key":"4_CR23","doi-asserted-by":"crossref","first-page":"1335","DOI":"10.1056\/NEJM199405123301902","volume":"330","author":"J Shores","year":"1994","unstructured":"Shores J, Berger KR, Murphy EA, et al.: Progression of aortic dilatation and the benefit of long-term beta-adrenergic blockade in Marfan\u2019s syndrome. N Engl J Med 1994, 330:1335\u20131341.","journal-title":"N Engl J Med"},{"key":"4_CR24","doi-asserted-by":"crossref","first-page":"1530","DOI":"10.1161\/01.CIR.0000121741.50315.C2","volume":"109","author":"P Boutouyrie","year":"2004","unstructured":"Boutouyrie P, Germain DP, Fiessinger JN, et al.: Increased carotid wall stress in vascular Ehlers-Danlos syndrome. Circulation 2004, 109:1530\u20131535.","journal-title":"Circulation"},{"key":"4_CR25","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1007\/s00595-003-2634-z","volume":"34","author":"Y Sugawara","year":"2004","unstructured":"Sugawara Y, Ban K, Imai K, et al.: Successful coil embolization for spontaneous arterial rupture in association with Ehlers-Danlos syndrome type IV: report of a case. Surg Today 2004, 34:94\u201396.","journal-title":"Surg Today"},{"key":"4_CR26","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1177\/153857440203600510","volume":"36","author":"S Sultan","year":"2002","unstructured":"Sultan S, Morasch M, Colgan MP, et al.: Operative and endovascular management of extracranial vertebral artery aneurysm in Ehlers-Danlos syndrome: a clinical dilemma\u2014case report and literature review. Vasc Endovascular Surg 2002, 36:389\u2013392.","journal-title":"Vasc Endovascular Surg"},{"key":"4_CR27","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1007\/s10016-001-0229-y","volume":"16","author":"DP Germain","year":"2002","unstructured":"Germain DP: Clinical and genetic features of vascular Ehlers-Danlos syndrome. Ann Vasc Surg 2002, 16:391\u2013397.","journal-title":"Ann Vasc Surg"},{"key":"4_CR28","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1016\/j.jvs.2005.03.053","volume":"42","author":"GS Oderich","year":"2005","unstructured":"Oderich GS, Panneton JM, Bowers TC, et al.: The spectrum, management and clinical outcome of Ehlers-Danlos syndrome type IV: a 30-year experience. J Vasc Surg 2005, 42:98\u2013106. A major review of clinical and surgical management of vascular EDS.","journal-title":"J Vasc Surg"},{"key":"4_CR29","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1097\/00006123-200209000-00004","volume":"51","author":"WI Schieving","year":"2002","unstructured":"Schieving WI, Link MJ, Piepgras DG, et al.: Intracranial aneurysm surgery in Ehlers-Danlos syndrome type IV. Neurosurgery 2002, 51:607\u2013611.","journal-title":"Neurosurgery"},{"key":"4_CR30","doi-asserted-by":"crossref","first-page":"e1","DOI":"10.1016\/j.jpedsurg.2003.10.027","volume":"39","author":"JR Fuchs","year":"2004","unstructured":"Fuchs JR, Fishman SJ: Management of spontaneous colonic perforation in Ehlers-Danlos syndrome type IV. J Pediatr Surg 2004, 39:e1-e3.","journal-title":"J Pediatr Surg"},{"key":"4_CR31","doi-asserted-by":"crossref","first-page":"424","DOI":"10.1177\/000313480306900513","volume":"69","author":"JE Wiedeman","year":"2003","unstructured":"Wiedeman JE, Ritter EM: Use of porcine small intestinal submucosa in Ehlers-Danlos syndrome type IV. Am Surg 2003, 69:424\u2013426.","journal-title":"Am Surg"},{"key":"4_CR32","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1097\/00006565-199508000-00010","volume":"11","author":"J Kinanne","year":"1995","unstructured":"Kinanne J, Priebe C, Caty M, Kupperman N: Perforation of the colon in an adolescent girl. Pediatr Emerg Care 1995, 11:230\u2013232.","journal-title":"Pediatr Emerg Care"},{"key":"4_CR33","first-page":"187","volume":"122","author":"DP Germain","year":"1995","unstructured":"Germain DP: Ehlers-Danlos syndromes. Clinical, genetic and molecular aspects. Ann Dermatol Venereol 1995, 122:187\u2013204.","journal-title":"Ann Dermatol Venereol"},{"key":"4_CR34","doi-asserted-by":"crossref","first-page":"491","DOI":"10.1111\/j.1365-2141.2004.05220.x","volume":"127","author":"A Paepe De","year":"2004","unstructured":"De Paepe A, Malfait F: Bleeding and bruising in patients with Ehlers-Danlos syndrome and other collagen vascular disorders. Br J Haematol 2004, 127:491\u2013500.","journal-title":"Br J Haematol"},{"key":"4_CR35","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1002\/ajh.10247","volume":"72","author":"H Yasui","year":"2003","unstructured":"Yasui H, Adachi Y, Minami T, et al.: Combination therapy of DDAVP and conjugated estrogens for a recurrent large subcutaneous hematoma in Ehlers-Danlos syndrome. Am J Hematol 2003, 72:71\u201372.","journal-title":"Am J Hematol"},{"key":"4_CR36","first-page":"268","volume":"91","author":"B Rochelson","year":"1991","unstructured":"Rochelson B, Caruso R, Davenport D, Kaelber A: The use of prophylactic desmopressin (DDAVP) in labor to prevent hemorrhage in a patient with Ehlers-Danlos syndrome. N Y State J Med 1991, 91:268\u2013369.","journal-title":"N Y State J Med"}],"container-title":["Current Treatment Options in Cardiovascular Medicine"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s11936-006-0004-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s11936-006-0004-z\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s11936-006-0004-z","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,2,14]],"date-time":"2024-02-14T19:09:01Z","timestamp":1707937741000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s11936-006-0004-z"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,3]]},"references-count":36,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2006,3]]}},"alternative-id":["4"],"URL":"https:\/\/doi.org\/10.1007\/s11936-006-0004-z","relation":{},"ISSN":["1092-8464","1534-3189"],"issn-type":[{"value":"1092-8464","type":"print"},{"value":"1534-3189","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,3]]}}}