{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T07:16:07Z","timestamp":1790838967328,"version":"4.1.0"},"reference-count":0,"publisher":"Georg Thieme Verlag KG","issue":"02","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Thromb Haemost"],"published-print":{"date-parts":[[2018]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>A reduced von Willebrand factor (VWF) synthesis or survival, or its increased proteolysis, alone or in combination, contributes to the development of von Willebrand disease (VWD).<\/jats:p><jats:p>We describe a new, simple mechanistic model for exploring how VWF behaves in well-defined forms of VWD after its 1-desamino-8-D-arginine vasopressin (DDAVP)-induced release from endothelial cells. We aimed to ascertain whether the model can consistently predict VWF kinetic changes. The study involved 9 patients with VWD types Vicenza (a paradigmatic form with a reduced VWF survival), 8 type 2B, 2 type 2A-I, 1 type 2A-II (associated with an increased VWF proteolysis), and 42 normal controls, whose VWF levels were measured after a 24-hour-long DDAVP test. The rate constants considered were: k\n          0, associated with the VWF release phase; k\n          1, illustrating the phase of conversion from high- to low-molecular-weight VWF multimers; and k\n          e, associated with the VWF elimination phase. The amount of VWF released (D) was also measured.<\/jats:p><jats:p>\n          k\n          e and D were significantly higher in O than in non-O blood group controls; k\n          1 was also higher, but less markedly so. All the parameters were accelerated in type Vicenza, especially k\n          e (p\u2009&lt;\u20090.0001), which explains the significant reduction in VWF half-life. In types 2B and 2A-II, k\n          1 was one order of magnitude higher than in controls, which explains their loss of large VWF multimers. All parameters except k\n          e were lower in type 2A-I.<\/jats:p><jats:p>The proposed mechanistic model clearly describes the altered biochemical pathways in well-characterized VWD, prompting us to suggest that it might help clarify elusive forms of VWD too.<\/jats:p>","DOI":"10.1160\/th17-05-0375","type":"journal-article","created":{"date-parts":[[2018,1,29]],"date-time":"2018-01-29T23:56:28Z","timestamp":1517270188000},"page":"309-319","source":"Crossref","is-referenced-by-count":6,"title":["A Mechanistic Model to Quantify von Willebrand Factor Release, Survival and Proteolysis in Patients with von Willebrand Disease"],"prefix":"10.1160","volume":"118","author":[{"given":"Myriam","family":"Ferrari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federico","family":"Galvanin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Massimiliano","family":"Barolo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Viviana","family":"Daidone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Padrini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabrizio","family":"Bezzo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Casonato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2018,1,29]]},"container-title":["Thrombosis and Haemostasis"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1160\/TH17-05-0375.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,5,6]],"date-time":"2019-05-06T20:10:06Z","timestamp":1557173406000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1160\/TH17-05-0375"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018]]},"references-count":0,"journal-issue":{"issue":"02","published-online":{"date-parts":[[2018,1,29]]},"published-print":{"date-parts":[[2018,2]]}},"URL":"https:\/\/doi.org\/10.1160\/th17-05-0375","relation":{},"ISSN":["0340-6245","2567-689X"],"issn-type":[{"value":"0340-6245","type":"print"},{"value":"2567-689X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018]]}}}