{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T13:26:10Z","timestamp":1789565170304,"version":"build-2803163510"},"reference-count":21,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2017,6,21]],"date-time":"2017-06-21T00:00:00Z","timestamp":1498003200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2017,11]]},"abstract":"Introduction<\/jats:title>Due to interindividual variation in desmopressin response, non\u2010severe haemophilia A patients require desmopressin testing prior to therapeutic treatment. However, adequate response or frequency of blood sampling is not standardised in international guidelines. Consequently, various definitions and blood sampling protocols are currently applied. Interestingly, sustainability of desmopressin response is not incorporated into these definitions.<\/jats:p><\/jats:sec>Aim<\/jats:title>To study desmopressin response rates in a cohort of non\u2010severe haemophilia A patients using currently accepted desmopressin response definitions. This, in order to formulate a standardised, uniform response which includes information on sustainability and to design a standardised blood sampling protocol.<\/jats:p><\/jats:sec>Methods<\/jats:title>Currently used desmopressin responses in non\u2010severe haemophilia A patients were derived from a literature search. Actual desmopressin response rates were individualised in 105 non\u2010severe HA<\/jats:styled-content> patients from the Erasmus University Medical Centre and classified according to current varying definitions.<\/jats:p><\/jats:sec>Results<\/jats:title>Five response definitions were evaluated, three of which included only factor VIII<\/jats:styled-content> (FVIII<\/jats:styled-content>):C cut\u2010off levels and two also incorporated FVIII<\/jats:styled-content>:C\u2010fold increase over baseline. FVIII<\/jats:styled-content>:C\u2010fold increase showed no association with desmopressin response sustainability. FVIII<\/jats:styled-content>:C 1\u00a0hour after infusion (<0.30, \u22650.30\u20100.49, \u22650.50\u20100.79 and \u22650.80\u00a0IU<\/jats:styled-content>\/mL<\/jats:styled-content>) was, however, indicative of desmopressin response after 6\u00a0hours.<\/jats:p><\/jats:sec>Conclusion<\/jats:title>We suggest standardised desmopressin response based on clinically relevant FVIII<\/jats:styled-content>:C levels, e.g. 0.30 and 0.50\u00a0IU<\/jats:styled-content>\/mL<\/jats:styled-content>. In addition, patients with <0.30\u00a0IU<\/jats:styled-content>\/mL FVIII<\/jats:styled-content>:C after 1\u00a0hour (non\u2010responder) or \u22650.80\u00a0IU<\/jats:styled-content>\/mL<\/jats:styled-content> (sustained responder) do not require subsequent blood sampling. However, patients with \u22650.30\u20100.79\u00a0IU<\/jats:styled-content>\/mL FVIII<\/jats:styled-content>:C after 1\u00a0hour should undergo blood sampling after 6\u00a0hours to additionally determine response sustainability.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.13295","type":"journal-article","created":{"date-parts":[[2017,6,21]],"date-time":"2017-06-21T12:42:34Z","timestamp":1498048954000},"page":"861-867","source":"Crossref","is-referenced-by-count":7,"title":["Desmopressin in haemophilia: The need for a standardised clinical response and individualised test regimen"],"prefix":"10.1111","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0774-1496","authenticated-orcid":false,"given":"S. C. M.","family":"Stoof","sequence":"first","affiliation":[{"name":"Department of Haematology Erasmus University Medical Centre Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L. M.","family":"Sch\u00fctte","sequence":"additional","affiliation":[{"name":"Department of Haematology Erasmus University Medical Centre Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F. W. G.","family":"Leebeek","sequence":"additional","affiliation":[{"name":"Department of Haematology Erasmus University Medical Centre Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. H.","family":"Cnossen","sequence":"additional","affiliation":[{"name":"Department of Paediatric Haematology Erasmus University Medical Centre\/Sophia Children's Hospital Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. J. H. A.","family":"Kruip","sequence":"additional","affiliation":[{"name":"Department of Haematology Erasmus University Medical Centre Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,6,21]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12254"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1538-7836.2003.00190.x"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8652(199802)57:2<153::AID-AJH11>3.0.CO;2-D"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12732"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.blre.2014.03.001"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2012.02909.x"},{"key":"e_1_2_7_8_1","first-page":"71","volume-title":"Guidelines for the management of hemophilia","author":"Plasma factor level and duration of administration","year":"2012"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2000.02067.x"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2011.02572.x"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH12-06-0383"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0033-1354418"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.02145.x"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2002.03507.x"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1097\/MBC.0000000000000158"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2009.03595.x"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1537-2995.1961.tb00017.x"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.1963.03060080027008"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM196108102650605"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-103-1-6"},{"key":"e_1_2_7_21_1","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 F","year":"1989","journal-title":"Blood"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.12439"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.13295","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.13295","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T10:10:45Z","timestamp":1695550245000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.13295"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,6,21]]},"references-count":21,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2017,11]]}},"alternative-id":["10.1111\/hae.13295"],"URL":"https:\/\/doi.org\/10.1111\/hae.13295","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,6,21]]}}}