{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,16]],"date-time":"2026-03-16T10:53:52Z","timestamp":1773658432354,"version":"3.50.1"},"reference-count":22,"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":5611,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1991,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We describe two members of a single family, father and son, with mild factor XII deficiency associated to von Willebrand disease (vWD) with aberrant structure in whom distinct multimeric abnormalities and an abnormal proteolytic processing of von Willebrand factor (vWF) after desmopressin (DDAVP) administration were present. They had a mild bleeding history, low levels of vWF\u2010related activities, and a prolonged bleeding time. Low\u2010resolution agarose\u2010 gel electrophoresis showed a vWF with all size multimers in plasma and platelets. Higher\u2010resolution agarose gels demonstrated that the main band was present, but the relative proportion of the satellite bands was markedly reduced. The smallest oligomer was not increased. After the infusion of DDAVP to the father, a transient increase in the relative proportion of the satellite bands was seen, as described in normal individuals. No difference in the structure of vWF was observed when blood was collected with proteinase inhibitors. The analysis of native subunits of vWF and their proteolytic derived fragments, after DDAVP administration, showed a temporary augmentation of the 176 kDa fragment, as seen in normal subjects, as well as an increase of the 189 kDa fragment. This finding had not been reported previously either in normal individuals or in patients with vWD.<\/jats:p>","DOI":"10.1002\/ajh.2830360302","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T12:08:55Z","timestamp":1172059735000},"page":"163-170","source":"Crossref","is-referenced-by-count":17,"title":["Abnormal proteolytic degradation of von willebrand factor after desmopressin infusion in a new subtype of von willebrand disease (ID)"],"prefix":"10.1002","volume":"36","author":[{"given":"M. F.","family":"Lopez\u2010Fernandez","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Gonzalez\u2010Boullosa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. J.","family":"Blanco\u2010Lopez","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Perez","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Batlle","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","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1182\/blood.V70.1.173.173","article-title":"Proteolytic degradation of von Willebrand factor after DDAVP administration in normal individuals","volume":"70","author":"Battle J","year":"1987","journal-title":"Blood"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI112394"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830210207"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/0076-6879(89)69065-9"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/0049-3848(86)90188-X"},{"key":"e_1_2_1_7_2","first-page":"325","volume-title":"Coagulation and Bleeding Disorders. The Role of Factor VIII and von Willebrand Factor.","author":"Batlle J","year":"1989"},{"key":"e_1_2_1_8_2","volume-title":"Procedures for the Coagulation Laboratory.","author":"Triplett DA","year":"1981"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1462-5822.2007.00901.x"},{"key":"e_1_2_1_10_2","doi-asserted-by":"crossref","first-page":"1272","DOI":"10.1182\/blood.V59.6.1272.1272","article-title":"Multimeric composition of factor VIII\/von Willebrand factor following administration of DDAVP: Implications for pathophysiol\u2013ogy and therapy of von Willebrand disease","volume":"59","author":"Ruggeri ZM","year":"1982","journal-title":"Blood"},{"key":"e_1_2_1_11_2","first-page":"895","article-title":"Review: Von Willebrand factor and von Willebrand disease","volume":"70","author":"Ruggeri ZM","year":"1987","journal-title":"Blood"},{"key":"e_1_2_1_12_2","doi-asserted-by":"crossref","first-page":"1423","DOI":"10.1182\/blood.V66.6.1423.1423","article-title":"High\u2013resolution analysis of von Willebrand factor multimeric composition defines a new variant of type I von Willebrand disease with aberrant structure but presence of all size multimers (Type IC)","volume":"66","author":"Ciavarella G","year":"1985","journal-title":"Blood"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI110700"},{"key":"e_1_2_1_14_2","doi-asserted-by":"crossref","first-page":"1000","DOI":"10.1182\/blood.V62.5.1000.1000","article-title":"A variant of von Willebrand's disease characterized by recessive inheritance and missing triplet structure of von Willebrand factor multimers","volume":"82","author":"Mannucci PM","year":"1983","journal-title":"Blood"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830220311"},{"key":"e_1_2_1_16_2","doi-asserted-by":"crossref","first-page":"1207","DOI":"10.1182\/blood.V68.6.1207.1207","article-title":"The heterogeneity of type IIA von Willebrand's disease: studies with proteinase inhibitors","volume":"68","author":"Batlle J","year":"1986","journal-title":"Blood"},{"key":"e_1_2_1_17_2","doi-asserted-by":"crossref","first-page":"1369","DOI":"10.1182\/blood.V63.6.1369.1369","article-title":"A new variant of dominant type II von Willebrand's disease with aberrant multimeric pattern of factor VII\u2013related antigen (type IID)","volume":"63","author":"Kinoshita S","year":"1984","journal-title":"Blood"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.38.6.665"},{"key":"e_1_2_1_19_2","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1182\/blood.V68.1.269.269","article-title":"A new variant of type II von Willebrand disease with aberrant multimeric structure of plasma but not platelet von Willebrand factor (IIF)","volume":"68","author":"Mannucci PM","year":"1986","journal-title":"Blood"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830240305"},{"key":"e_1_2_1_21_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830320409"},{"key":"e_1_2_1_22_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI112843"},{"key":"e_1_2_1_23_2","first-page":"122","article-title":"Von Willebrand disease San Diego, a new variant","volume":"2","author":"Cramer AD","year":"1976","journal-title":"Lancet"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajh.2830360302","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.2830360302","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,23]],"date-time":"2023-10-23T07:21:40Z","timestamp":1698045700000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.2830360302"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1991,3]]},"references-count":22,"journal-issue":{"issue":"3","published-print":{"date-parts":[[1991,3]]}},"alternative-id":["10.1002\/ajh.2830360302"],"URL":"https:\/\/doi.org\/10.1002\/ajh.2830360302","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[1991,3]]}}}