{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T15:22:45Z","timestamp":1761060165205},"reference-count":19,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2016,7,6]],"date-time":"2016-07-06T00:00:00Z","timestamp":1467763200000},"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":[[2016,9]]},"abstract":"<jats:sec><jats:title>Introduction<\/jats:title><jats:p>Despite the availability of subcutaneous desmopressin (1\u2010deamino\u20108\u2010<jats:sc>d<\/jats:sc>\u2010arginine vasopressin, <jats:styled-content style=\"fixed-case\">SC<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">DDAVP<\/jats:styled-content>) as a haemostatic agent for children with mild bleeding disorders, few publications specifically address the safety or efficacy of this mode of administration.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>Our aim was to assess whether a defined fluid restriction protocol was effective in preventing hyponatremia in children receiving perioperative <jats:styled-content style=\"fixed-case\">SC<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">DDAVP<\/jats:styled-content>, and to document adequate biological and clinical response in this setting.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We retrospectively analysed a cohort of children with mild bleeding disorders prescribed <jats:styled-content style=\"fixed-case\">SC<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">DDAVP<\/jats:styled-content> over a 5\u2010year period following institution of a \u2018two\u2010thirds maintenance\u2019 fluid restriction protocol.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sixty\u2010nine patients received <jats:styled-content style=\"fixed-case\">SC<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">DDAVP<\/jats:styled-content> following this protocol, including 15 with mild haemophilia A, 49 with von Willebrand disease (<jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content>) and five with platelet storage pool disorder. In patients who underwent formal preoperative assessment a complete or partial response was observed in 28\/29 with type 1 <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> and 14\/15 with mild haemophilia A. Perioperative <jats:styled-content style=\"fixed-case\">SC<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">DDAVP<\/jats:styled-content> provided excellent haemostasis in all patients, with no requirement for factor concentrate or blood products. Mild asymptomatic hyponatremia was detected in seven children who received multiple doses of <jats:styled-content style=\"fixed-case\">DDAVP<\/jats:styled-content> (lowest sodium 129 mmol L<jats:sup>\u22121<\/jats:sup>); however, adherence to the prescribed fluid restriction protocol was questionable in six of these cases. Symptomatic hyponatremia was not observed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Subcutaneous desmopressin was well\u2010tolerated, with no serious side\u2010effects observed, and good biological responses in preoperative trials. A two\u2010thirds maintenance fluid regimen was effective at preventing symptomatic hyponatremia in our cohort, and is now the standard protocol for fluid restriction post\u2010<jats:styled-content style=\"fixed-case\">DDAVP<\/jats:styled-content> administration in our centre.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.12949","type":"journal-article","created":{"date-parts":[[2016,7,7]],"date-time":"2016-07-07T04:12:59Z","timestamp":1467864779000},"page":"700-705","source":"Crossref","is-referenced-by-count":7,"title":["Assessment and validation of a defined fluid restriction protocol in the use of subcutaneous desmopressin for children with inherited bleeding disorders"],"prefix":"10.1111","volume":"22","author":[{"given":"J. A.","family":"Mason","sequence":"first","affiliation":[{"name":"Department of Haematology Lady Cilento Children's Hospital Brisbane QLD Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. D.","family":"Robertson","sequence":"additional","affiliation":[{"name":"Department of Haematology Lady Cilento Children's Hospital Brisbane QLD Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"McCosker","sequence":"additional","affiliation":[{"name":"Department of Haematology Lady Cilento Children's Hospital Brisbane QLD Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B. A.","family":"Williams","sequence":"additional","affiliation":[{"name":"Department of Haematology Lady Cilento Children's Hospital Brisbane QLD Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S. A.","family":"Brown","sequence":"additional","affiliation":[{"name":"Department of Haematology Lady Cilento Children's Hospital Brisbane QLD Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2016,7,6]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.24335"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.mph.0000168728.49519.4a"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830310310"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH10-11-0697"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2011.02617.x"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-08-109231"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2012.02909.x"},{"key":"e_1_2_9_9_1","first-page":"19","article-title":"Desmopressin (DDAVP) for treatment of disorders of haemostasis","volume":"8","author":"Mannuci P","year":"1986","journal-title":"Prog Hemost Thromb"},{"key":"e_1_2_9_10_1","first-page":"687","article-title":"A new pharmacological approach to the management of haemophilia and von Willebrand disease","volume":"1","author":"Mannucci P","year":"1977","journal-title":"Lancet"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01727749"},{"key":"e_1_2_9_12_1","first-page":"1033","article-title":"Intravenous and subcutaneous administration of desmopressin (DDAVP) to haemophiliacs: pharmacokinetics and factor VIII responses","volume":"58","author":"Mannucci P","year":"1987","journal-title":"Thromb Haemost"},{"key":"e_1_2_9_13_1","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1055\/s-0038-1657745","article-title":"Comparison between subcutaneous and intravenous DDAVP in mild and moderate haemophilia A","volume":"54","author":"Sio LD","year":"1985","journal-title":"Thromb Haemost"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V90.7.2515"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1179\/1607845412Y.0000000051"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2007.01519.x"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.02145.x"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830320405"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1981.tb02789.x"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH07-09-0565"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.12949","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.12949","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,16]],"date-time":"2023-09-16T23:26:55Z","timestamp":1694906815000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.12949"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,7,6]]},"references-count":19,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2016,9]]}},"alternative-id":["10.1111\/hae.12949"],"URL":"https:\/\/doi.org\/10.1111\/hae.12949","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,7,6]]}}}