{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2022,4,4]],"date-time":"2022-04-04T12:47:30Z","timestamp":1649076450874},"reference-count":44,"publisher":"Hindawi Limited","license":[{"start":{"date-parts":[[2015,1,1]],"date-time":"2015-01-01T00:00:00Z","timestamp":1420070400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/3.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Case Reports in Hematology"],"published-print":{"date-parts":[[2015]]},"abstract":"<jats:p>von Willebrand disease type 3 (VWD3) is a rare but the most severe form of von Willebrand disease; it is due to almost complete lack of von Willebrand factor activity (VWF:RCo). It is inherited as autosomal recessive trait; whilst heterozygote carriers have mild, or no symptoms, patients with VWD3 show severe bleeding symptoms. In the laboratory, this is characterised by undetectable VWF:Ag, VWF:RCo, and reduced levels of factor VIII &lt; 0.02\u2009IU\/dL. The bleeding is managed with von Willebrand\/FVIII factor concentrate replacement therapy. In this rare but challenging case we report on the successful excision and repair of an ascending aortic aneurysm following adequate VWF\/FVIII factor concentrate replacement using Haemate-P.<\/jats:p>","DOI":"10.1155\/2015\/703803","type":"journal-article","created":{"date-parts":[[2015,4,19]],"date-time":"2015-04-19T21:03:28Z","timestamp":1429477408000},"page":"1-8","source":"Crossref","is-referenced-by-count":0,"title":["Successful Aortic Aneurysm Repair in a Woman with Severe von Willebrand (Type 3) Disease"],"prefix":"10.1155","volume":"2015","author":[{"given":"Victoria","family":"Campbell","sequence":"first","affiliation":[{"name":"Department of Haematology, Imperial College Healthcare NHS Trust, London W2 1NY, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kevin","family":"Marriott","sequence":"additional","affiliation":[{"name":"Department of Haematology, Imperial College Healthcare NHS Trust, London W2 1NY, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rex","family":"Stanbridge","sequence":"additional","affiliation":[{"name":"Department of Cardiothoracic Surgery, Imperial College Healthcare NHS Trust, London W2 1NY, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Abdul","family":"Shlebak","sequence":"additional","affiliation":[{"name":"Department of Haematology, Imperial College Healthcare NHS Trust, London W2 1NY, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"98","reference":[{"key":"1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.02146.x"},{"key":"2","doi-asserted-by":"publisher","DOI":"10.1053\/beha.2001.0139"},{"key":"3","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13064"},{"key":"4","doi-asserted-by":"publisher","DOI":"10.1007\/s002770050184"},{"key":"5","doi-asserted-by":"publisher","DOI":"10.1053\/beha.2001.0133"},{"key":"6","doi-asserted-by":"publisher","DOI":"10.1172\/jci115376"},{"key":"7","doi-asserted-by":"publisher","DOI":"10.1172\/jci109795"},{"issue":"4","key":"8","doi-asserted-by":"crossref","first-page":"520","DOI":"10.1055\/s-0038-1642471","volume":"71","year":"1994","journal-title":"Thrombosis and Haemostasis"},{"key":"9","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1986.tb02966.x"},{"key":"10","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1984.tb02876.x"},{"key":"11","volume":"307, article 127","year":"1982","journal-title":"The New England Journal of Medicine"},{"issue":"7","key":"12","doi-asserted-by":"crossref","first-page":"2433","DOI":"10.1182\/blood.V74.7.2433.2433","volume":"74","year":"1989","journal-title":"Blood"},{"key":"13","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.02070.x"},{"issue":"3","key":"14","first-page":"411","volume":"101","year":"1983","journal-title":"Journal of Laboratory and Clinical Medicine"},{"key":"15","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.02088.x"},{"key":"16","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2011.02534.x"},{"key":"17","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2010.02445.x"},{"key":"18","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2007.02562.x"},{"key":"19","doi-asserted-by":"publisher","DOI":"10.1160\/th10-09-0616"},{"key":"20","doi-asserted-by":"publisher","DOI":"10.1160\/th11-02-0057"},{"key":"21","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.2002.00688.x"},{"key":"22","doi-asserted-by":"publisher","DOI":"10.1160\/th10-10-0631"},{"key":"23","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.2002.00673.x"},{"key":"24","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2007.01643.x"},{"key":"25","doi-asserted-by":"publisher","DOI":"10.1056\/nejmra040403"},{"key":"26","doi-asserted-by":"publisher","DOI":"10.1046\/j.1351-8216.2003.00809.x"},{"key":"27","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.11124"},{"issue":"11","key":"28","first-page":"1279","volume":"88","year":"2003","journal-title":"Haematologica"},{"key":"29","doi-asserted-by":"publisher","DOI":"10.1046\/j.1351-8216.2003.00816.x"},{"key":"30","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.1998.0040s2033.x"},{"key":"38","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12497"},{"key":"39","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01901.x"},{"key":"34","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2007.01570.x"},{"key":"36","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2006.01430.x"},{"key":"37","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2010.02206.x"},{"issue":"2","key":"31","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1055\/s-0037-1612977","volume":"87","year":"2002","journal-title":"Thrombosis and Haemostasis"},{"key":"32","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2007.02588.x"},{"key":"33","doi-asserted-by":"publisher","DOI":"10.1182\/blood.v99.2.450"},{"key":"35","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2005.01435.x"},{"key":"40","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12237"},{"key":"41","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2011.02603.x"},{"issue":"12","key":"42","doi-asserted-by":"crossref","first-page":"3130","DOI":"10.1182\/blood.V79.12.3130.bloodjournal79123130","volume":"79","year":"1992","journal-title":"Blood"},{"key":"43","doi-asserted-by":"publisher","DOI":"10.1055\/s-2006-949668"},{"key":"44","doi-asserted-by":"publisher","DOI":"10.1097\/mbc.0b013e3283254570"}],"container-title":["Case Reports in Hematology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/downloads.hindawi.com\/journals\/crihem\/2015\/703803.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/downloads.hindawi.com\/journals\/crihem\/2015\/703803.xml","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/downloads.hindawi.com\/journals\/crihem\/2015\/703803.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,9,1]],"date-time":"2020-09-01T02:30:35Z","timestamp":1598927435000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.hindawi.com\/journals\/crihem\/2015\/703803\/"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015]]},"references-count":44,"alternative-id":["703803","703803"],"URL":"https:\/\/doi.org\/10.1155\/2015\/703803","relation":{},"ISSN":["2090-6560","2090-6579"],"issn-type":[{"value":"2090-6560","type":"print"},{"value":"2090-6579","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015]]}}}