{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,24]],"date-time":"2025-12-24T12:43:21Z","timestamp":1766580201268,"version":"build-2065373602"},"reference-count":120,"publisher":"Elsevier BV","issue":"6","license":[{"start":{"date-parts":[[2004,12,1]],"date-time":"2004-12-01T00:00:00Z","timestamp":1101859200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2004,12,1]],"date-time":"2004-12-01T00:00:00Z","timestamp":1101859200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","theclinics.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Hematology\/Oncology Clinics of North America"],"published-print":{"date-parts":[[2004,12]]},"DOI":"10.1016\/j.hoc.2004.07.006","type":"journal-article","created":{"date-parts":[[2005,4,18]],"date-time":"2005-04-18T10:53:30Z","timestamp":1113821610000},"page":"1277-1299","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":22,"title":["Diagnosis and treatment of von Willebrand disease"],"prefix":"10.1016","volume":"18","author":[{"given":"Joan","family":"Cox Gill","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.hoc.2004.07.006_bib1","first-page":"68","article-title":"Hereditar pseudohemofi","author":"von Willebrand","year":"1926","journal-title":"Finska Lak Handl"},{"key":"10.1016\/j.hoc.2004.07.006_bib2","doi-asserted-by":"crossref","first-page":"995","DOI":"10.1126\/science.3486471","article-title":"Propolypeptide of von Willebrand factor circulates in blood and is identical to von Willebrand antigen II","volume":"232","author":"Fay","year":"1986","journal-title":"Science"},{"key":"10.1016\/j.hoc.2004.07.006_bib3","doi-asserted-by":"crossref","first-page":"895","DOI":"10.1182\/blood.V70.4.895.895","article-title":"von Willebrand factor and von Willebrand disease","volume":"70","author":"Ruggeri","year":"1987","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2004.07.006_bib4","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1016\/S0046-8177(87)80332-5","article-title":"von Willebrand disease","volume":"18","author":"Zimmerman","year":"1987","journal-title":"Human Pathol"},{"key":"10.1016\/j.hoc.2004.07.006_bib5","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1016\/0049-3848(90)90181-B","article-title":"Multimeric analysis of von Willebrand factor by molecular sieving electrophoresis in sodium dodecyl sulphate agarose gel","volume":"60","author":"Raines","year":"1990","journal-title":"Thromb Res"},{"key":"10.1016\/j.hoc.2004.07.006_bib6","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1111\/j.1749-6632.1987.tb30984.x","article-title":"Endothelial cell processing of von Willebrand proteins","volume":"509","author":"Kawai","year":"1987","journal-title":"Ann NY Acad Sci"},{"key":"10.1016\/j.hoc.2004.07.006_bib7","series-title":"Platelet immunology","first-page":"255","article-title":"Homologous functions and interactions of platelets and endothelial cells","author":"Montgomery","year":"1989"},{"key":"10.1016\/j.hoc.2004.07.006_bib8","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1055\/s-0038-1649197","article-title":"Platelet interaction with collagen fibrils in flowing blood. II. Impaired adhesion-aggregation in bleeding disorders. A comparison with subendothelium","volume":"37","author":"Baumgartner","year":"1977","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.hoc.2004.07.006_bib9","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1172\/JCI110946","article-title":"Platelets have more than one binding site for von Willebrand factor","volume":"72","author":"Ruggeri","year":"1983","journal-title":"J Clin Invest"},{"key":"10.1016\/j.hoc.2004.07.006_bib10","doi-asserted-by":"crossref","first-page":"1354","DOI":"10.1182\/blood.V70.5.1354.1354","article-title":"Shear stress activation of platelet glycoprotein IIb\/IIIa plus von Willebrand factor causes aggregation: Filter blockage and the long bleeding time in von Willebrand's disease","volume":"70","author":"O'Brien","year":"1987","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2004.07.006_bib11","doi-asserted-by":"crossref","first-page":"625","DOI":"10.1182\/blood.V69.2.625.625","article-title":"Shear-induced platelet aggregation requires von Willebrand factor and platelet membrane glycoproteins Ib and IIb-IIIa","volume":"69","author":"Peterson","year":"1987","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2004.07.006_bib12","doi-asserted-by":"crossref","first-page":"1198","DOI":"10.1172\/JCI111816","article-title":"Interaction of asialo von Willebrand factor with glycoprotein Ib induces fibrinogen binding to the glycoprotein IIb\/IIIa complex and mediates platelet aggregation","volume":"75","author":"De Marco","year":"1985","journal-title":"J Clin Invest"},{"key":"10.1016\/j.hoc.2004.07.006_bib13","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1172\/JCI108788","article-title":"Stabilization of factor VIII in plasma by the von Willebrand factor: studies on post-transfusion and dissociated factor VIII and in patients with von Willebrand's disease","volume":"60","author":"Weiss","year":"1977","journal-title":"J Clin Invest"},{"key":"10.1016\/j.hoc.2004.07.006_bib14","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1016\/0049-3848(86)90253-7","article-title":"Comparison of the in vivo survival of human factor VIII with and without von Willebrand factor in the hemophilic dog","volume":"41","author":"Fay","year":"1986","journal-title":"Thromb Res"},{"key":"10.1016\/j.hoc.2004.07.006_bib15","doi-asserted-by":"crossref","first-page":"649","DOI":"10.1111\/j.1365-2141.1982.tb02829.x","article-title":"Influence of high molecular weight factor VIII on the measurement of low molecular weight factor VIII procoagulant in different assay systems","volume":"51","author":"Muntean","year":"1982","journal-title":"Br J Haematol"},{"key":"10.1016\/j.hoc.2004.07.006_bib16","doi-asserted-by":"crossref","first-page":"1401","DOI":"10.1126\/science.3874428","article-title":"Human von Willebrand factor (VWF): isolation of complementary DNA (cDNA) clones and chromosomal localization","volume":"228","author":"Ginsburg","year":"1985","journal-title":"Science"},{"key":"10.1016\/j.hoc.2004.07.006_bib17","doi-asserted-by":"crossref","first-page":"4699","DOI":"10.1093\/nar\/13.13.4699","article-title":"Construction of cDNA coding for human von Willebrand factor using antibody probes for colony-screening and mapping of the chromosomal gene","volume":"13","author":"Verweij","year":"1985","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.hoc.2004.07.006_bib18","doi-asserted-by":"crossref","first-page":"6394","DOI":"10.1073\/pnas.82.19.6394","article-title":"Cloning and characterization of two cDNAs coding for human von Willebrand factor","volume":"82","author":"Sadler","year":"1985","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/j.hoc.2004.07.006_bib19","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1182\/blood.V69.2.454.454","article-title":"Epidemiological investigation of the prevalence of von Willebrand's disease","volume":"69","author":"Rodeghiero","year":"1987","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2004.07.006_bib20","first-page":"1","article-title":"Hemophilia in Sweden. Studies on demography of hemophilia and surgery in hemophilia and von Willebrand's disease","volume":"584","author":"Larsson","year":"1984","journal-title":"Acta Med Scand Suppl"},{"key":"10.1016\/j.hoc.2004.07.006_bib21","doi-asserted-by":"crossref","first-page":"893","DOI":"10.1016\/S0022-3476(05)80384-1","article-title":"Prevalence of von Willebrand disease in children: a multiethnic study","volume":"123","author":"Werner","year":"1993","journal-title":"J Pediatr"},{"key":"10.1016\/j.hoc.2004.07.006_bib22","doi-asserted-by":"crossref","first-page":"712","DOI":"10.1182\/blood.V56.4.712.712","article-title":"Serial studies in von Willebrand's disease: variability versus \u201cvariant\u201d","volume":"56","author":"Abildgaard","year":"1980","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2004.07.006_bib23","doi-asserted-by":"crossref","first-page":"2433","DOI":"10.1182\/blood.V88.7.2433.bloodjournal8872433","article-title":"Dominant type 1 von Willebrand disease caused by mutated cysteine residues in the D3 domain of von Willebrand factor","volume":"88","author":"Eikenboom","year":"1996","journal-title":"Blood"},{"issue":"1","key":"10.1016\/j.hoc.2004.07.006_bib24","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1055\/s-0037-1616211","article-title":"Getting at the variable expressivity of von Willebrand disease","volume":"86","author":"Levy","year":"2001","journal-title":"Thromb Haemost"},{"issue":"10","key":"10.1016\/j.hoc.2004.07.006_bib25","doi-asserted-by":"crossref","first-page":"2973","DOI":"10.1182\/blood.V98.10.2973","article-title":"Type 1 von Willebrand disease mutation Cys1149 Arg Causes intracellular retention and degradation of heterodimers: a possible general mechanism for dominant mutations of oligomeric proteins","volume":"98","author":"Bodo","year":"2001","journal-title":"Blood"},{"issue":"2","key":"10.1016\/j.hoc.2004.07.006_bib26","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1055\/s-0037-1612981","article-title":"The factor VIII\/von Willebrand factor ratio discriminates between reduced synthesis and increased clearance of von Willebrand factor","volume":"87","author":"Eikenboom","year":"2002","journal-title":"Thromb Haemost"},{"issue":"7","key":"10.1016\/j.hoc.2004.07.006_bib27","doi-asserted-by":"crossref","first-page":"2454","DOI":"10.1182\/blood-2003-02-0599","article-title":"Analysis of intracellular storage and regulated secretion of 3 von Willebrand disease-causing variants of von Willebrand factor","volume":"102","author":"Michaux","year":"2003","journal-title":"Blood"},{"issue":"4","key":"10.1016\/j.hoc.2004.07.006_bib28","doi-asserted-by":"crossref","first-page":"876","DOI":"10.1046\/j.1365-2141.2000.01944.x","article-title":"Autosomal dominant type 1 von Willebrand disease due to G3639T mutation (C1130F) in exon 26 of von Willebrand factor gene: description of five Italian families and evidence for a founder effect","volume":"108","author":"Castaman","year":"2000","journal-title":"Br J Haematol"},{"key":"10.1016\/j.hoc.2004.07.006_bib29","doi-asserted-by":"crossref","first-page":"1691","DOI":"10.1182\/blood.V69.6.1691.1691","article-title":"The effect of ABO blood group on the diagnosis of von Willebrand disease","volume":"69","author":"Gill","year":"1987","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2004.07.006_bib30","doi-asserted-by":"crossref","first-page":"3578","DOI":"10.1182\/blood.V93.11.3578","article-title":"Low platelet \u03b12\u03b21 levels in type 1 von Willebrand disease correlate with impaired platelet function in a high shear stress system","volume":"93","author":"Di Paola","year":"1999","journal-title":"Blood"},{"issue":"2","key":"10.1016\/j.hoc.2004.07.006_bib31","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1002\/ajh.1090","article-title":"Population differences in von Willebrand factor levels affect the diagnosis of von Willebrand disease in African-American women","volume":"67","author":"Miller","year":"2001","journal-title":"Am J Haem"},{"issue":"4","key":"10.1016\/j.hoc.2004.07.006_bib32","first-page":"709","article-title":"Characterization of the genetic defects in recessive type 1 and type 3 von Willebrand disease patients of Italian origin","volume":"79","author":"Eikenboom","year":"2002","journal-title":"Thromb Haemost"},{"issue":"4","key":"10.1016\/j.hoc.2004.07.006_bib33","first-page":"536","article-title":"Molecular characterization of a multiethnic group of 21 patients with type 3 von Willebrand disease","volume":"84","author":"Baronciani","year":"2000","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.hoc.2004.07.006_bib34","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1097\/00005792-199701000-00001","article-title":"von Willebrand disease","volume":"76","author":"Nichols","year":"1997","journal-title":"Medicine"},{"key":"10.1016\/j.hoc.2004.07.006_bib35","doi-asserted-by":"crossref","first-page":"520","DOI":"10.1055\/s-0038-1642471","article-title":"A revised classification of von Willebrand disease","volume":"71","author":"Sadler","year":"1994","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.hoc.2004.07.006_bib36","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1182\/blood.V84.3.676.676","article-title":"Commentary: a new classification for von Willebrand disease","volume":"84","author":"Sadler","year":"1994","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2004.07.006_bib37","doi-asserted-by":"crossref","first-page":"947","DOI":"10.1172\/JCI112394","article-title":"Subunit composition of plasma von Willebrand factor. Cleavage is present in normal individuals, increased in IIA and IIB von Willebrand disease, but minimal in variants with aberrant structure of individual oligomers (types IIC, IID, and IIE)","volume":"77","author":"Zimmerman","year":"1986","journal-title":"J Clin Invest"},{"key":"10.1016\/j.hoc.2004.07.006_bib38","doi-asserted-by":"crossref","first-page":"3723","DOI":"10.1073\/pnas.86.10.3723","article-title":"Molecular basis of human von Willebrand disease: analysis of platelet von Willebrand factor mRNA","volume":"86","author":"Ginsburg","year":"1989","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/j.hoc.2004.07.006_bib39","doi-asserted-by":"crossref","first-page":"4424","DOI":"10.1016\/S0021-9258(18)42852-9","article-title":"Impaired intracellular transport produced by a subset of type IIA von Willebrand disease mutations","volume":"256","author":"Lyons","year":"1992","journal-title":"J Biol Chem"},{"key":"10.1016\/j.hoc.2004.07.006_bib40","doi-asserted-by":"crossref","first-page":"1047","DOI":"10.1056\/NEJM198005083021902","article-title":"Heightened interaction between platelets and factor VIII\/von Willebrand factor in a new subtype of von Willebrand's disease","volume":"302","author":"Ruggeri","year":"1980","journal-title":"N Engl J Med"},{"key":"10.1016\/j.hoc.2004.07.006_bib41","doi-asserted-by":"crossref","first-page":"2048","DOI":"10.1182\/blood.V79.8.2048.2048","article-title":"Expressed full-length von Willebrand factor containing missense mutations linked to type IIB von Willebrand disease shows enhanced binding to platelets","volume":"79","author":"Kroner","year":"1992","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2004.07.006_bib42","doi-asserted-by":"crossref","first-page":"1532","DOI":"10.1172\/JCI111112","article-title":"von Willebrand factor binds to platelets and induces aggregation in platelet-type but not type IIB von Willebrand disease","volume":"72","author":"Miller","year":"1983","journal-title":"J Clin Invest"},{"key":"10.1016\/j.hoc.2004.07.006_bib43","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1056\/NEJM198202113060603","article-title":"Pseudo-von Willebrand's disease. An intrinsic platelet defect with aggregation by unmodified human factor VIII\/von Willebrand factor and enhanced adsorption of its high-molecular-weight multimers","volume":"306","author":"Weiss","year":"1982","journal-title":"N Engl J Med"},{"key":"10.1016\/j.hoc.2004.07.006_bib44","doi-asserted-by":"crossref","first-page":"790","DOI":"10.1182\/blood.V60.3.790.790","article-title":"Platelet-type von Willebrand's disease: characterization of a new bleeding disorder","volume":"60","author":"Miller","year":"1982","journal-title":"Blood"},{"issue":"1","key":"10.1016\/j.hoc.2004.07.006_bib45","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1182\/blood-2003-01-0072","article-title":"Alterations in the intrinsic properties of the GPIbalpha-VWF tether bond define the kinetics of the platelet-type von Willebrand disease mutation Gly233Val","volume":"102","author":"Doggett","year":"2003","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2004.07.006_bib46","doi-asserted-by":"crossref","first-page":"2559","DOI":"10.1182\/blood.V88.7.2559.bloodjournal8872559","article-title":"Type 2M:Milwaukee-1 von Willebrand disease: an in-frame deletion in the Cys509-Cys695 loop of the von Willebrand factor A1 domain causes deficient binding of von Willebrand factor to platelets","volume":"88","author":"Mancuso","year":"1996","journal-title":"Blood"},{"issue":"2","key":"10.1016\/j.hoc.2004.07.006_bib47","first-page":"188","article-title":"Type 2M vWD resulting from a lysine deletion within a four lysine residue repeat in the A1 loop of von Willebrand factor","volume":"84","author":"Hilbert","year":"2000","journal-title":"Thromb Haemost"},{"issue":"4","key":"10.1016\/j.hoc.2004.07.006_bib48","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1046\/j.1365-2141.2003.04168.x","article-title":"A new mutation, S1285F, within the A1 loop of von Willebrand factor induces a conformational change in A1 loop with abnormal binding to platelet GPIb and botrocetin causing type 2M von Willebrand disease","volume":"120","author":"Stepanian","year":"2003","journal-title":"Br J Haematol"},{"key":"10.1016\/j.hoc.2004.07.006_bib49","first-page":"427","article-title":"Genetic cause of hereditary persistence of pro-VWF","volume":"38","author":"Rayner","year":"1990","journal-title":"Clin Res"},{"issue":"6","key":"10.1016\/j.hoc.2004.07.006_bib50","doi-asserted-by":"crossref","first-page":"2000","DOI":"10.1182\/blood.V95.6.2000","article-title":"Two novel type 2N von Willebrand disease-causing mutations that result in defective factor VIII binding, multimerization, and secretion of von Willebrand factor","volume":"95","author":"Allen","year":"2000","journal-title":"Blood"},{"issue":"6","key":"10.1016\/j.hoc.2004.07.006_bib51","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1055\/s-0038-1650401","article-title":"Characterization of type 2N von Willebrand disease using phenotypic and molecular techniques","volume":"75","author":"Nesbitt","year":"1996","journal-title":"Thromb Haemost"},{"issue":"4","key":"10.1016\/j.hoc.2004.07.006_bib52","doi-asserted-by":"crossref","first-page":"598","DOI":"10.1055\/s-0038-1650628","article-title":"Results of a screening for von Willebrand disease type 2N in patients with suspected haemophilia A or von Willebrand disease type 1","volume":"76","author":"Schneppenheim","year":"1996","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.hoc.2004.07.006_bib53","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1182\/blood.V60.1.201.201","article-title":"A variant of von Willebrand's disease with abnormal expression of factor VIII procoagulant activity","volume":"60","author":"Montgomery","year":"1982","journal-title":"Blood"},{"issue":"3","key":"10.1016\/j.hoc.2004.07.006_bib54","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1016\/S0165-5876(99)00195-0","article-title":"Von Willebrand disease as cause of unanticipated bleeding following adeno-tonsillectomy","volume":"49","author":"de Diego","year":"1999","journal-title":"International Journal of Pediatric Otorhinolaryngology"},{"issue":"3","key":"10.1016\/j.hoc.2004.07.006_bib55","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1055\/s-0037-1614035","article-title":"von Willebrand disease in a pediatric-based population\u2013comparison of type 1 diagnostic criteria and use of the PFA-100 and a von Willebrand factor\/collagen-binding assay","volume":"84","author":"Dean","year":"2000","journal-title":"Thromb Haemost"},{"issue":"9101","key":"10.1016\/j.hoc.2004.07.006_bib56","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1016\/S0140-6736(97)08248-2","article-title":"Frequency of inherited bleeding disorders in women with menorrhagia","volume":"351","author":"Kadir","year":"1998","journal-title":"Lancet"},{"issue":"5","key":"10.1016\/j.hoc.2004.07.006_bib57","doi-asserted-by":"crossref","first-page":"856","DOI":"10.1067\/mpd.2001.113042","article-title":"Bleeding disorders: a common cause of menorrhagia in adolescents","volume":"138","author":"Bevan","year":"2001","journal-title":"J Pediatr"},{"key":"10.1016\/j.hoc.2004.07.006_bib58","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1097\/00000441-196711000-00002","article-title":"Telangiectasia: its relationship to the Minot-von Willebrand syndrome","volume":"254","author":"Quick","year":"1967","journal-title":"Am J Med Sci"},{"issue":"3","key":"10.1016\/j.hoc.2004.07.006_bib59","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1002\/(SICI)1096-8652(199911)62:3<165::AID-AJH6>3.0.CO;2-C","article-title":"Use of a novel platelet function analyzer (PFA-100) with high sensitivity to disturbances in von Willebrand factor to screen for von Willebrand's disease and other disorders","volume":"62","author":"Favaloro","year":"1999","journal-title":"Am J Hematol"},{"issue":"1","key":"10.1016\/j.hoc.2004.07.006_bib60","first-page":"93","article-title":"Assessment of primary hemostasis by PFA-100 analysis in a tertiary care center","volume":"84","author":"Ortel","year":"2000","journal-title":"Thromb Haemost"},{"issue":"6","key":"10.1016\/j.hoc.2004.07.006_bib61","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1097\/00043426-200306000-00009","article-title":"Comparison of PFA-100 and bleeding time testing in pediatric patients with suspected hemorrhagic problems","volume":"25","author":"Cariappa","year":"2003","journal-title":"J Pediatr Hematol Oncol"},{"key":"10.1016\/j.hoc.2004.07.006_bib62","first-page":"152","article-title":"Determination of the von Willebrand's disease antigen (factor VIII-related antigen) in plasma by quantitative immunoelectrophoresis","volume":"85","author":"Zimmerman","year":"1975","journal-title":"J Lab Clin Med"},{"key":"10.1016\/j.hoc.2004.07.006_bib63","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1016\/0049-3848(90)90084-P","article-title":"Rapid diagnosis of von Willebrand's disease using ELISA technology","volume":"57","author":"Gilchrist","year":"1990","journal-title":"Thromb Res"},{"key":"10.1016\/j.hoc.2004.07.006_bib64","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1182\/blood.V41.5.687.687","article-title":"Ristocetin: a means of differentiating von Willebrand's disease into two groups","volume":"41","author":"Howard","year":"1973","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2004.07.006_bib65","doi-asserted-by":"crossref","first-page":"2697","DOI":"10.1172\/JCI107464","article-title":"Defective ristocetin-induced platelet aggregation in von Willebrand's disease and its correction by factor VIII","volume":"52","author":"Weiss","year":"1973","journal-title":"J Clin Invest"},{"issue":"1","key":"10.1016\/j.hoc.2004.07.006_bib66","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1055\/s-0037-1613768","article-title":"Collagen binding assay for von Willebrand factor (VWF:CBA): detection of von Willebrand's Disease (VWD), and discrimination of VWD subtypes, depends on collagen source","volume":"83","author":"Favaloro","year":"2000","journal-title":"Thromb Haemost"},{"issue":"1","key":"10.1016\/j.hoc.2004.07.006_bib67","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1046\/j.1365-2141.2002.03199.x","article-title":"Use of the collagen-binding assay for von Willebrand factor in the analysis of type 2M von Willebrand disease: a comparison with the ristocetin cofactor assay","volume":"116","author":"Riddell","year":"2002","journal-title":"Br J Haematol"},{"issue":"2","key":"10.1016\/j.hoc.2004.07.006_bib68","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1055\/s-2002-27821","article-title":"Von Willebrand factor collagen-binding (activity) assay in the diagnosis of von Willebrand disease: a 15-year journey","volume":"28","author":"Favaloro","year":"2002","journal-title":"Semin Thromb Hemost"},{"key":"10.1016\/j.hoc.2004.07.006_bib69","doi-asserted-by":"crossref","first-page":"930","DOI":"10.1182\/blood.V60.4.930.930","article-title":"Specific factor VIII-related antigen fragmentation: an in vivo and in vitro phenomenon","volume":"60","author":"Montgomery","year":"1982","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2004.07.006_bib70","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1136\/jcp.46.5.470","article-title":"Analysis of von Willebrand factor multimers using a commercially available enhanced chemiluminescence kit","volume":"46","author":"Cumming","year":"1993","journal-title":"J Clin Pathol"},{"key":"10.1016\/j.hoc.2004.07.006_bib71","doi-asserted-by":"crossref","first-page":"758","DOI":"10.1182\/blood.V67.3.758.758","article-title":"Loss of the largest von Willebrand factor multimers from the plasma of patients with congenital cardiac defects","volume":"67","author":"Gill","year":"1986","journal-title":"Blood"},{"issue":"4","key":"10.1016\/j.hoc.2004.07.006_bib72","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1056\/NEJMoa022831","article-title":"Acquired von Willebrand syndrome in aortic stenosis","volume":"349","author":"Vincentelli","year":"2003","journal-title":"N Engl J Med"},{"key":"10.1016\/j.hoc.2004.07.006_bib73","doi-asserted-by":"crossref","first-page":"1124","DOI":"10.1172\/JCI110700","article-title":"Aberrant multimeric structure of von Willebrand factor in a new variant of von Willebrand's disease (type IIC)","volume":"70","author":"Ruggeri","year":"1982","journal-title":"J Clin Invest"},{"key":"10.1016\/j.hoc.2004.07.006_bib74","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1055\/s-0038-1660152","article-title":"Multicenter comparison of von Willebrand factor multimer sizing techniques","volume":"54","author":"Mannucci","year":"1985","journal-title":"Thromb Haemost"},{"issue":"3","key":"10.1016\/j.hoc.2004.07.006_bib75","doi-asserted-by":"crossref","first-page":"716","DOI":"10.1046\/j.1365-2141.2002.03488.x","article-title":"Large experience with a factor VIII binding assay of plasma von Willebrand factor using commercial reagents","volume":"117","author":"Caron","year":"2002","journal-title":"Br J Haematol"},{"key":"10.1016\/j.hoc.2004.07.006_bib76","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1016\/0049-3848(89)90317-4","article-title":"von Willebrand factor is an acute phase reactant in man","volume":"53","author":"Pottinger","year":"1989","journal-title":"Thromb Res"},{"key":"10.1016\/j.hoc.2004.07.006_bib77","doi-asserted-by":"crossref","first-page":"713","DOI":"10.7326\/0003-4819-97-5-713","article-title":"Factor VIII activity and thyroid function","volume":"97","author":"Rogers","year":"1982","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.hoc.2004.07.006_bib78","first-page":"205","article-title":"High levels of plasma von Willebrand factor in hyperthyroidism","volume":"25","author":"Cuianu","year":"1987","journal-title":"Med Interne"},{"key":"10.1016\/j.hoc.2004.07.006_bib79","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1111\/j.1365-2141.1981.tb02689.x","article-title":"Newborn factor VIII complex: elevated activities in term infants and alterations in electrophoretic mobility related to illness and activated coagulation","volume":"47","author":"Johnson","year":"1981","journal-title":"Br J Haematol"},{"key":"10.1016\/j.hoc.2004.07.006_bib80","first-page":"793","article-title":"Neonatal von Willebrand's disease: diagnostic difficulty at birth","volume":"134","author":"Weinger","year":"1980","journal-title":"Am J Dis Child"},{"issue":"2","key":"10.1016\/j.hoc.2004.07.006_bib81","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1046\/j.1365-2141.2000.01830.x","article-title":"Type 1 von Willebrand disease - a clinical retrospective study of the diagnosis, the influence of the ABO blood group and the role of the bleeding history","volume":"108","author":"Nity-Whalley","year":"2000","journal-title":"Br J Haematol"},{"issue":"6","key":"10.1016\/j.hoc.2004.07.006_bib82","doi-asserted-by":"crossref","first-page":"2089","DOI":"10.1182\/blood-2002-09-2892","article-title":"Von Willebrand disease type 1: a diagnosis in search of a disease","volume":"101","author":"Sadler","year":"2003","journal-title":"Blood"},{"issue":"3","key":"10.1016\/j.hoc.2004.07.006_bib83","first-page":"1065","article-title":"Inconsistency of association between type 1 von Willebrand disease phenotype of genotype in families identified in an epidemiological investigation","volume":"82","author":"Castaman","year":"1999","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.hoc.2004.07.006_bib84","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/S0308-2261(21)00013-8","article-title":"Coagulation factor therapy","volume":"5","author":"Rizza","year":"1976","journal-title":"Clin Haematol"},{"issue":"1","key":"10.1016\/j.hoc.2004.07.006_bib85","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1046\/j.1351-8216.2003.00809.x","article-title":"Successful treatment for patients with von Willebrand disease undergoing urgent surgery using factor VIII\/VWF concentrate (Humate-P)","volume":"10","author":"Thompson","year":"2004","journal-title":"Haemophilia"},{"issue":"6","key":"10.1016\/j.hoc.2004.07.006_bib86","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1046\/j.1351-8216.2003.00816.x","article-title":"Successful treatment of urgent bleeding in von Willebrand disease with factor VIII\/VWF concentrate (Humate-P): use of the ristocetin cofactor assay (VWF:RCo) to measure potency and to guide therapy","volume":"9","author":"Gill","year":"2003","journal-title":"Haemophilia"},{"issue":"2","key":"10.1016\/j.hoc.2004.07.006_bib87","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1182\/blood.V99.2.450","article-title":"Treatment of von Willebrand disease with a high-purity VIII\/von Willebrand factor concentrate: a prospective, multicenter study","volume":"99","author":"Mannucci","year":"2002","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2004.07.006_bib88","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1055\/s-0037-1612977","article-title":"Efficacy and safety of the factor VIII\/von Willebrand factor concentrate, haemate-P\/humate-P: ristocetin cofactor unit dosing in patients with von Willebrand disease","volume":"87","author":"Lillicrap","year":"2002","journal-title":"Thromb Haemost"},{"issue":"Suppl 1","key":"10.1016\/j.hoc.2004.07.006_bib89","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1046\/j.1365-2516.2001.00096.x","article-title":"Use of ristocetin cofactor activity in the management of von Willebrand disease","volume":"7","author":"Ewenstein","year":"2001","journal-title":"Haemophilia"},{"issue":"3","key":"10.1016\/j.hoc.2004.07.006_bib90","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1046\/j.1365-2516.2001.00498.x","article-title":"Hemophilia Research Society of North America. Management of von Willebrand disease: a survey on current clinical practice from the haemophilia centres of North America","volume":"7","author":"Cohen","year":"2001","journal-title":"Haemophilia"},{"key":"10.1016\/j.hoc.2004.07.006_bib91","doi-asserted-by":"crossref","first-page":"1279","DOI":"10.1001\/jama.1985.03350330077026","article-title":"Hematologic management of hemophilia A for surgery","volume":"253","author":"Kasper","year":"1985","journal-title":"JAMA"},{"key":"10.1016\/j.hoc.2004.07.006_bib92","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1056\/NEJM196609012750904","article-title":"Treatment of hemophilia with glycine-precipated factor VIII","volume":"275","author":"Abildgaard","year":"1966","journal-title":"N Engl J Med"},{"issue":"3","key":"10.1016\/j.hoc.2004.07.006_bib93","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1046\/j.1365-2516.2001.00500.x","article-title":"High-dose DDAVP intranasal spray (Stimate) for the prevention and treatment of bleeding in patients with mild haemophilia A, mild or moderate type 1 von Willebrand disease and symptomatic carriers of haemophilia A","volume":"7","author":"Leissinger","year":"2001","journal-title":"Haemophilia"},{"key":"10.1016\/j.hoc.2004.07.006_bib94","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1111\/j.1365-2141.1981.tb02789.x","article-title":"Response of factor VIII \/ von Willebrand factor to DDAVP in healthy subjects and patients with haemophilia A and von Willebrand's disease","volume":"47","author":"Mannucci","year":"1981","journal-title":"Br J Haematol"},{"key":"10.1016\/j.hoc.2004.07.006_bib95","doi-asserted-by":"crossref","first-page":"6","DOI":"10.7326\/0003-4819-103-1-6","article-title":"Response of patients with mild and moderate hemophilia A and von Willebrand's disease to treatment with desmopressin","volume":"103","author":"de la Fuente","year":"1985","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.hoc.2004.07.006_bib96","doi-asserted-by":"crossref","first-page":"2515","DOI":"10.1182\/blood.V90.7.2515","article-title":"Desmopressin (DDAVP) in the treatment of bleeding disorders: the first 20 years","volume":"90","author":"Mannucci","year":"1997","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2004.07.006_bib97","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1016\/S0022-3476(83)80526-5","article-title":"DDAVP: a useful alternative to blood components in moderate hemophilia A and von Willebrand disease","volume":"102","author":"Warrier","year":"1983","journal-title":"J Pediatr"},{"issue":"8","key":"10.1016\/j.hoc.2004.07.006_bib98","doi-asserted-by":"crossref","first-page":"1714","DOI":"10.1046\/j.1538-7836.2003.00359.x","article-title":"Increased clearance of von Willebrand factor antigen post-DDAVP in Type 1 von Willebrand disease: is it a potential pathogenic process?","volume":"1","author":"Brown","year":"2003","journal-title":"J Thromb Haemost"},{"issue":"6","key":"10.1016\/j.hoc.2004.07.006_bib99","doi-asserted-by":"crossref","first-page":"2032","DOI":"10.1182\/blood-2003-06-2072","article-title":"Biologic response to desmopressin in patients with severe type 1 and type 2 von Willebrand disease: results of a multicenter European study","volume":"103","author":"Federici","year":"2004","journal-title":"Blood"},{"issue":"11","key":"10.1016\/j.hoc.2004.07.006_bib100","doi-asserted-by":"crossref","first-page":"874","DOI":"10.1097\/00043426-200311000-00010","article-title":"Desmopressin (DDAVP) responsiveness in children with von Willebrand disease","volume":"25","author":"Revel-Vilk","year":"2003","journal-title":"J Pediatr Hematol Oncol"},{"key":"10.1016\/j.hoc.2004.07.006_bib101","doi-asserted-by":"crossref","first-page":"1997","DOI":"10.1182\/blood.V74.6.1997.1997","article-title":"Consistency of responses to repeated DDAVP infusions in patients with von Willebrand's disease and hemophilia A","volume":"74","author":"Rodeghiero","year":"1989","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2004.07.006_bib102","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1111\/j.1600-0609.1982.tb00565.x","article-title":"The effect of intranasal DDAVP on coagulation and fibrinolytic activity in normal persons","volume":"29","author":"Nilsson","year":"1982","journal-title":"Scand J Haematol"},{"issue":"Suppl 1","key":"10.1016\/j.hoc.2004.07.006_bib103","first-page":"244","article-title":"Safety and efficacy of Stimate (desmopressin acetate) nasal spray, 1.5 mg\/mL, in patients with mild hemophilia A [abstract]","volume":"PS998","author":"Cornell","year":"1997","journal-title":"Thromb Haemost"},{"issue":"5","key":"10.1016\/j.hoc.2004.07.006_bib104","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1067\/mpd.2002.123626","article-title":"Evaluation of high concentration intranasal and intravenous desmopressin in pediatric patients with mild hemophilia A or mild-to-moderate type 1 von Willebrand disease","volume":"140","author":"Gill","year":"2002","journal-title":"J Pediatr"},{"key":"10.1016\/j.hoc.2004.07.006_bib105","doi-asserted-by":"crossref","first-page":"1449","DOI":"10.1182\/blood.V72.5.1449.1449","article-title":"Desmopressin: a nontransfusional form of treatment for congenital and acquired bleeding disorders","volume":"75","author":"Mannucci","year":"1988","journal-title":"Blood"},{"key":"10.1016\/j.hoc.2004.07.006_bib106","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1002\/ajh.2830310310","article-title":"Hyponatremia and seizures in young children given DDAVP","volume":"31","author":"Smith","year":"1989","journal-title":"Am J Hematol"},{"issue":"2","key":"10.1016\/j.hoc.2004.07.006_bib107","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1177\/0310057X0002800214","article-title":"Severe hyponatraemia secondary to desmopressin therapy in von Willebrand's disease","volume":"28","author":"Bertholini","year":"2000","journal-title":"Anaesth Intensive Care"},{"issue":"1","key":"10.1016\/j.hoc.2004.07.006_bib108","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1046\/j.1365-2516.2000.00367.x","article-title":"Adverse events during use of intranasal desmopressin acetate for haemophilia A and von Willebrand disease: a case report and review of 40 patients","volume":"6","author":"Dunn","year":"2000","journal-title":"Haemophilia"},{"key":"10.1016\/j.hoc.2004.07.006_bib109","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1016\/S0140-6736(89)90915-X","article-title":"Desmopressin and thrombosis","volume":"2","author":"Mannucci","year":"1989","journal-title":"Lancet"},{"issue":"3","key":"10.1016\/j.hoc.2004.07.006_bib110","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1055\/s-0037-1613040","article-title":"Successful surgical hemostasis in patients with von Willebrand disease following infusion of Koate DVI","volume":"87","author":"Srivastava","year":"2002","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.hoc.2004.07.006_bib111","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1056\/NEJM200008173430702","article-title":"High plasma levels of factor VIII and the risk of recurrent venous thromboembolism","volume":"343","author":"Kyrle","year":"2000","journal-title":"N Engl J Med"},{"issue":"3","key":"10.1016\/j.hoc.2004.07.006_bib112","first-page":"378","article-title":"Venous thrombosis following the use of intermediate purity FVIII concentrate to treat patients with von Willebrand's disease","volume":"88","author":"Makris","year":"2002","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.hoc.2004.07.006_bib113","doi-asserted-by":"crossref","first-page":"149","DOI":"10.7326\/0003-4819-100-1-149","article-title":"Hepatitis B vaccine","volume":"100","year":"1984","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.hoc.2004.07.006_bib114","first-page":"H117","article-title":"Persistence of antibody response to hepatitis A vaccine [abstract]","volume":"124","author":"Vandamme","year":"1994","journal-title":"ICAAC"},{"key":"10.1016\/j.hoc.2004.07.006_bib115","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1111\/j.1365-2141.1971.tb07061.x","article-title":"Epsilon-aminocaproic acid therapy for dental extractions in haemophilia and Christmas disease: a double-blind controlled trial","volume":"20","author":"Walsh","year":"1971","journal-title":"Br J Haematol"},{"key":"10.1016\/j.hoc.2004.07.006_bib116","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1136\/bmj.2.5809.311","article-title":"Tranexamic acid in control of haemorrhage after dental extraction in haemophilia and Christmas disease","volume":"2","author":"Forbes","year":"1972","journal-title":"Br Med J"},{"issue":"2","key":"10.1016\/j.hoc.2004.07.006_bib117","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1046\/j.1365-2516.2000.00370.x","article-title":"Optimising local therapy during oral surgery in patients with von Willebrand disease: effective results from a retrospective analysis of 63 cases","volume":"6","author":"Federici","year":"2000","journal-title":"Haemophilia"},{"key":"10.1016\/j.hoc.2004.07.006_bib118","doi-asserted-by":"crossref","first-page":"840","DOI":"10.1056\/NEJM198903303201305","article-title":"Hemostatic effect of tranexamic acid mouthwash in anticoagulant-treated patients undergoing oral surgery","volume":"320","author":"Sindet-Pedersen","year":"1989","journal-title":"N Engl J Med"},{"key":"10.1016\/j.hoc.2004.07.006_bib119","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1055\/s-0038-1647265","article-title":"1-Desamino-8-D-arginine vasopressin (DDAVP) infusion in type IIB von Willebrand's disease: shortening of bleeding time and induction of a variable pseudothrombocytopenia","volume":"64","author":"Casonato","year":"1990","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.hoc.2004.07.006_bib120","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1002\/(SICI)1096-8652(199602)51:2<158::AID-AJH11>3.0.CO;2-E","article-title":"1-Desamino-8-arginine-vasopressin corrects the hemostatic defects in type 2B von Willebrand's disease","volume":"51","author":"McKeown","year":"1996","journal-title":"Am J Hematol"}],"container-title":["Hematology\/Oncology Clinics of North America"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0889858804000887?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0889858804000887?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T11:37:52Z","timestamp":1760182672000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0889858804000887"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,12]]},"references-count":120,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2004,12]]}},"alternative-id":["S0889858804000887"],"URL":"https:\/\/doi.org\/10.1016\/j.hoc.2004.07.006","relation":{},"ISSN":["0889-8588"],"issn-type":[{"type":"print","value":"0889-8588"}],"subject":[],"published":{"date-parts":[[2004,12]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Diagnosis and treatment of von Willebrand disease","name":"articletitle","label":"Article Title"},{"value":"Hematology\/Oncology Clinics of North America","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.hoc.2004.07.006","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2004 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}