{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T16:47:03Z","timestamp":1789663623043,"version":"4.0.1"},"reference-count":25,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2023,5,31]],"date-time":"2023-05-31T00:00:00Z","timestamp":1685491200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["pericles.pericles-gcp.literatumonline.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2023,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Peri\u2010procedural management of von Willebrand disease (VWD) utilizes von Willebrand factor (VWF) concentrates or desmopressin (DDAVP) to increase VWF levels. DDAVP is safe, easily administered, and inexpensive. Currently, a consensus definition for adequate DDAVP response is lacking, and outcomes of peri\u2010procedural DDAVP use in VWD patients are seldom reported.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>This single\u2010centre retrospective review aims to characterize DDAVP\u2010responsiveness and assess clinical outcomes of peri\u2010procedural DDAVP use in VWD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients and Methods<\/jats:title><jats:p>We reviewed records for all our adult VWD patients (age \u226518 years) who underwent DDAVP challenge testing between January 2007 and January 2022. DDAVP\u2010responsiveness was assessed using six definitions. Bleeding outcomes following procedures covered by DDAVP were classified as excessive or expected bleeding.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Eighty\u2010four of 94 (89.4%) patients were DDAVP\u2010responsive by our definition (1\u2010h VWF Activity\/Factor VIII \u22650.50 IU\/mL). However, the proportion of DDAVP\u2010responders varied from 53.2% to 91.5%, depending on the literature definition used. Ninety\u2010nine procedures pre\u2010treated with DDAVP were performed during the study period. Eighty\u2010six (86.7%) procedures (31 major; 55 minor) were covered with only DDAVP\u00a0\u00b1\u00a0tranexamic acid (TXA). Excessive bleeding occurred following 4\/31 major procedures and 2\/55 minor procedures (both performed in a single patient with a bleeding score of 16). When covered with DDAVP+Factor\u00a0\u00b1\u00a0TXA, one each of 10 major and 3 minor procedures (performed in 2 patients with bleeding scores 15\u201316) resulted in post\u2010procedural bleeding.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Peri\u2010procedural DDAVP prophylaxis appears to be effective among individuals with VWD. Beyond DDAVP\u2010responsiveness, patient bleeding history and procedure invasiveness should be considered in determining suitability for DDAVP prophylaxis.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.14801","type":"journal-article","created":{"date-parts":[[2023,5,31]],"date-time":"2023-05-31T23:48:20Z","timestamp":1685576900000},"page":"1095-1103","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Desmopressin (DDAVP) use in patients with von Willebrand disease: A single\u2010centre retrospective review of test response and clinical outcomes"],"prefix":"10.1111","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4421-3599","authenticated-orcid":false,"given":"Priya","family":"Chandrakumaran","sequence":"first","affiliation":[{"name":"University of Calgary Cumming School of Medicine  Calgary Alberta Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4949-1459","authenticated-orcid":false,"given":"Julia","family":"Hews\u2010Girard","sequence":"additional","affiliation":[{"name":"University of Calgary Cumming School of Medicine  Calgary Alberta Canada"},{"name":"University of Calgary School of Nursing  Calgary Alberta Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7012-2420","authenticated-orcid":false,"given":"Man\u2010Chiu","family":"Poon","sequence":"additional","affiliation":[{"name":"University of Calgary Cumming School of Medicine  Calgary Alberta Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,5,31]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1601561"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2020003265"},{"key":"e_1_2_10_4_1","unstructured":"The diagnosis evaluation and management of von Willebrand disease [Internet]. National Heart Lung and Blood Institute. U.S. Department of Health and Human Services; 2008 [accessed 2022 Aug 15]. Available from:https:\/\/www.nhlbi.nih.gov\/health\u2010topics\/diagnosis\u2010evaluation\u2010and\u2010management\u2010of\u2010von\u2010willebrand\u2010disease"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2020.07.051"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199807233390407"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12254"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2012.077263"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2003-06-2072"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-08-109231"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-200311000-00010"},{"issue":"2","key":"e_1_2_10_12_1","first-page":"565","article-title":"von Willebrand disease: proposing definitions for future research","volume":"5","author":"Connell NT","year":"2021","journal-title":"Blood Adv"},{"issue":"2","key":"e_1_2_10_13_1","first-page":"s33","article-title":"Surgical prophylaxis in von Willebrand's disease: a difficult balance to manage","volume":"6","author":"Franchini M","year":"2008","journal-title":"Blood Transfus"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1177\/1076029617715117"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12518"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2020003264"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.13718"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/rth2.12777"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2007.01643.x"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2017-05-786699"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.1058"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01893.x"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.2000.00370.x"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.15227"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.15220"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/rth2.12064"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.14801","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,5]],"date-time":"2023-09-05T12:46:47Z","timestamp":1693918007000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.14801"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5,31]]},"references-count":25,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2023,7]]}},"alternative-id":["10.1111\/hae.14801"],"URL":"https:\/\/doi.org\/10.1111\/hae.14801","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,5,31]]},"assertion":[{"value":"2022-10-25","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-04-23","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-05-31","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}