{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,25]],"date-time":"2026-07-25T00:15:34Z","timestamp":1784938534959,"version":"3.55.0"},"reference-count":23,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2014,12,29]],"date-time":"2014-12-29T00:00:00Z","timestamp":1419811200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"MEXT KAKENHI","award":["24591558"],"award-info":[{"award-number":["24591558"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2015,1]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>The diagnosis of von Willebrand disease (<jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content>) is difficult due to the wide spectrum of clinical phenotypes associated with this disorder. We have analysed and characterized haemostatic function in <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> patients using a microchip\u2010based flow chamber system. Microchips coated with either collagen [platelet (<jats:styled-content style=\"fixed-case\">PL<\/jats:styled-content>)\u2010chip] or collagen\/thromboplastin [atherome (<jats:styled-content style=\"fixed-case\">AR<\/jats:styled-content>)\u2010chip] were used to evaluate platelet thrombus formation at 1000\u00a0s<jats:sup>\u22121<\/jats:sup> and fibrin\u2010rich platelet thrombus formation at 240\u00a0s<jats:sup>\u22121<\/jats:sup> respectively. Blood samples from an asymptomatic patient with <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> type 1 [von Willebrand factor (<jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>): RCo 3.2%; bleeding score (<jats:styled-content style=\"fixed-case\">BS<\/jats:styled-content> 2] displayed normal thrombus formation in both <jats:styled-content style=\"fixed-case\">PL<\/jats:styled-content>\u2010 and <jats:styled-content style=\"fixed-case\">AR<\/jats:styled-content>\u2010chips, whereas blood from a symptomatic type 1 patient (<jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>: RCo 14%, <jats:styled-content style=\"fixed-case\">BS<\/jats:styled-content> 9) had significantly delayed capillary occlusion. Nearly complete suppression of the flow pressure increase was observed in symptomatic patients with <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> type 2A (<jats:styled-content style=\"fixed-case\">BS<\/jats:styled-content> 13) and 2N (<jats:styled-content style=\"fixed-case\">BS<\/jats:styled-content> 27), whereas no flow pressure was found for the type 3 patient (<jats:styled-content style=\"fixed-case\">BS<\/jats:styled-content> 6). Fibrin\u2010rich platelet thrombus formation was only weakly increased by the <jats:italic>in vitro<\/jats:italic> addition of factor VIII (<jats:styled-content style=\"fixed-case\">FVIII<\/jats:styled-content>) to blood samples from the type 3 patient, but was normalized by the addition of <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">FVIII<\/jats:styled-content>. The <jats:italic>in vivo<\/jats:italic> effects of treatment with desmopressin or <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">FVIII<\/jats:styled-content> for the symptomatic patients were analysed using two types of microchips. The <jats:styled-content style=\"fixed-case\">PL<\/jats:styled-content>\u2010chip was highly sensitive for patients\u2019 <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>\u2010mediated platelet functions, whereas the <jats:styled-content style=\"fixed-case\">AR<\/jats:styled-content>\u2010chip allowed assessment of overall haemostatic ability, including sensitivity to both <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">FVIII<\/jats:styled-content>. The combined analysis with <jats:styled-content style=\"fixed-case\">PL<\/jats:styled-content>\u2010 and <jats:styled-content style=\"fixed-case\">AR<\/jats:styled-content>\u2010chips may be potentially useful for the diagnosis of <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> based on clinical phenotypes, and for monitoring drug effects.<\/jats:p>","DOI":"10.1111\/hae.12610","type":"journal-article","created":{"date-parts":[[2014,12,29]],"date-time":"2014-12-29T12:14:12Z","timestamp":1419855252000},"page":"71-80","source":"Crossref","is-referenced-by-count":30,"title":["Comprehensive evaluation of haemostatic function in von Willebrand disease patients using a microchip\u2010based flow chamber system"],"prefix":"10.1111","volume":"21","author":[{"given":"K.","family":"Ogiwara","sequence":"first","affiliation":[{"name":"Department of Pediatrics Nara Medical University Kashihara Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Nogami","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Nara Medical University Kashihara Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Hosokawa","sequence":"additional","affiliation":[{"name":"Research Institute Fujimori Kogyo Co. Yokohama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T.","family":"Ohnishi","sequence":"additional","affiliation":[{"name":"Research Institute Fujimori Kogyo Co. Yokohama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T.","family":"Matsumoto","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Nara Medical University Kashihara Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Shima","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Nara Medical University Kashihara Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,12,29]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00109-010-0691-5"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1053\/beha.2001.0138"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.02146.x"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-01-153296"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2012.04923.x"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.01847.x"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01737.x"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V94.3.968.415a13_968_975"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-03-0944"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0608422104"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/cphc.200700566"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2011.04464.x"},{"key":"e_1_2_8_14_1","doi-asserted-by":"crossref","first-page":"8105","DOI":"10.1016\/S0021-9258(17)40944-6","article-title":"Isolation and characterization of trypsin inhibitor from opaque\u20102 corn seeds","volume":"252","author":"Swartz MJ","year":"1977","journal-title":"J Biol Chem"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1614572"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/0925-5710(96)00470-7"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH12-06-0441"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2011.04265.x"},{"issue":"2","key":"e_1_2_8_19_1","first-page":"106","article-title":"Description of an in vitro platelet function analyzer\u2013PFA\u2010100","volume":"21","author":"Kundu SK","year":"1995","journal-title":"Semin Thromb Hemost"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2010.08333.x"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2012.077263"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V95.6.2000"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V92.12.4663"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2012.02867.x"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.12610","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.12610","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.12610","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T13:24:22Z","timestamp":1695561862000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.12610"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,12,29]]},"references-count":23,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2015,1]]}},"alternative-id":["10.1111\/hae.12610"],"URL":"https:\/\/doi.org\/10.1111\/hae.12610","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,12,29]]}}}