{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,15]],"date-time":"2026-05-15T15:06:08Z","timestamp":1778857568765,"version":"3.51.4"},"reference-count":27,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":9567,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1980,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Two cases of von Willebrand disease (VWD) that revealed an increased ristocetin\u2010induced platelet aggregation (RIPA) and a qualitative abnormality of the factor VIII protein are reported. The threshold concentration of ristocetin giving a 30% increase in light transmission was 0.5 mg\/ml in the proband and 0.4 mg\/ml in her father (normal: 1.16 \u00b1 SD 0.18 mg\/ml) although both patients showed reduced plasma von Willebrand factor activity (VIIIR:WF). In both patients, the amount of factor VIII related antigen (VIIIR:AG) in their platelets were normal, but an increased binding affinity of platelets to plasma factor VIII was demonstrated. The qualitative abnormality of the factor VIII protein was characterized by an increased anodal migration of VIIIR:AG in crossed immunoelectrophoresis (CIE), a delayed elution pattern as demonstrated by gel filtration on Sepharose 2B, and a decreased precipitation with concanavalin A (Con A). The response to DDAVP was also investigated.<\/jats:p>","DOI":"10.1002\/ajh.2830080308","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T05:41:10Z","timestamp":1172036470000},"page":"299-308","source":"Crossref","is-referenced-by-count":21,"title":["Von willebrand disease with an increased ristocetin\u2010induced platelet aggregation and a qualitative abnormality of the factor VIII protein"],"prefix":"10.1002","volume":"8","author":[{"given":"Hoyu","family":"Takahashi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nobuo","family":"Sakuragawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akira","family":"Shibata","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,7,11]]},"reference":[{"key":"e_1_2_1_2_2","first-page":"231","volume-title":"Progress in Hemostasis and Thrombosis","author":"Hoyer LW","year":"1976"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.0954-6820.1977.tb15737.x"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-86-5-598"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.3.5822.317"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1974.tb00484.x"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197407182910301"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01005210"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01082925"},{"key":"e_1_2_1_10_2","first-page":"44","article-title":"An atypical case of von Willebrand's disease with an isolated deficiency of factor VIII procoagulant activity","volume":"42","author":"Takahashi H","year":"1979","journal-title":"Acta Haematol Jpn"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.1910.04330140029009"},{"key":"e_1_2_1_12_2","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1111\/j.1600-0609.1970.tb01917.x","article-title":"Platelet adhesiveness in von Willebrand's disease. A study with a new modification of the glass bead filter method","volume":"7","author":"Hellem AJ","year":"1970","journal-title":"Scand J Haematol"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI107464"},{"key":"e_1_2_1_14_2","first-page":"637","article-title":"Effect of antihemophilic factor on one\u2010stage clotting tests","volume":"41","author":"Langdell R","year":"1953","journal-title":"J Lab Clin Med"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(66)90246-6"},{"key":"e_1_2_1_16_2","first-page":"318","article-title":"Platelets fixed with paraformaldehyde: A new reagent for assay of von Willebrand factor and platelet aggregating factor","volume":"85","author":"Allain JP","year":"1975","journal-title":"J Lab Clin Med"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1055\/s-0038-1651343"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/0049-3848(74)90011-5"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1977.tb00566.x"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1977.tb08812.x"},{"key":"e_1_2_1_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/0049-3848(77)90204-3"},{"key":"e_1_2_1_22_2","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1055\/s-0038-1648663","article-title":"Factor VIII\u2010related activities in normal, haemophilic and von Willebrand's disease platelet fractions","volume":"40","author":"Meucci P","year":"1978","journal-title":"Thromb Haemost"},{"key":"e_1_2_1_23_2","first-page":"132","article-title":"Factor VIII\u2010related properties in platelets from patients with von Willebrand's disease","volume":"91","author":"Ruggeri ZM","year":"1978","journal-title":"J Lab Clin Med"},{"key":"e_1_2_1_24_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1083071"},{"key":"e_1_2_1_25_2","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1055\/s-0038-1649240","article-title":"Abnormal factor VIII related antigen (FVIIIRAG) in von Willebrand's disease (vWd): Decreased precipitation by concanavalin A","volume":"37","author":"Peake IR","year":"1977","journal-title":"Thromb Haemost"},{"key":"e_1_2_1_26_2","unstructured":"TakahashiH SakuragawaN ShibataA:Decreased precipitation of factor VIII related antigen with concanavalin A in patients with a variant of von Willebrand's disease and disseminated intravascular coagulation. Tohoku J Exp Med (submitted for publication)."},{"key":"e_1_2_1_27_2","unstructured":"TakahashiH ItohM KobayashiI SakuragawaN ShibataA: Properties of the factor VIII after DDAVP (1\u2010deamino\u20108\u2010D\u2010arginine vasopressin) infusion in normal subjects.Tohoku J Exp Med(submitted for publication)."},{"key":"e_1_2_1_28_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(77)91197-7"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajh.2830080308","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.2830080308","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,13]],"date-time":"2023-11-13T04:38:37Z","timestamp":1699850317000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.2830080308"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1980,5]]},"references-count":27,"journal-issue":{"issue":"3","published-print":{"date-parts":[[1980,5]]}},"alternative-id":["10.1002\/ajh.2830080308"],"URL":"https:\/\/doi.org\/10.1002\/ajh.2830080308","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[1980,5]]}}}