Mason J A, Robertson J D, McCosker J, Williams B A, Brown S A
Department of Haematology, Lady Cilento Children's Hospital, Brisbane, QLD, Australia.
Haemophilia. 2016 Sep;22(5):700-5. doi: 10.1111/hae.12949. Epub 2016 Jul 6.
Despite the availability of subcutaneous desmopressin (1-deamino-8-d-arginine vasopressin, SC-DDAVP) as a haemostatic agent for children with mild bleeding disorders, few publications specifically address the safety or efficacy of this mode of administration.
Our aim was to assess whether a defined fluid restriction protocol was effective in preventing hyponatremia in children receiving perioperative SC-DDAVP, and to document adequate biological and clinical response in this setting.
We retrospectively analysed a cohort of children with mild bleeding disorders prescribed SC-DDAVP over a 5-year period following institution of a 'two-thirds maintenance' fluid restriction protocol.
Sixty-nine patients received SC-DDAVP following this protocol, including 15 with mild haemophilia A, 49 with von Willebrand disease (VWD) 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 VWD and 14/15 with mild haemophilia A. Perioperative SC-DDAVP 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 DDAVP (lowest sodium 129 mmol L(-1) ); however, adherence to the prescribed fluid restriction protocol was questionable in six of these cases. Symptomatic hyponatremia was not observed.
Subcutaneous desmopressin was well-tolerated, with no serious side-effects observed, and good biological responses in preoperative trials. A two-thirds maintenance fluid regimen was effective at preventing symptomatic hyponatremia in our cohort, and is now the standard protocol for fluid restriction post-DDAVP administration in our centre.
尽管皮下注射去氨加压素（1-去氨基-8-D-精氨酸加压素，SC-DDAVP）可作为轻度出血性疾病患儿的止血剂，但专门论述这种给药方式安全性或有效性的出版物却很少。
我们的目的是评估特定的液体限制方案是否能有效预防接受围手术期SC-DDAVP治疗的儿童发生低钠血症，并记录在这种情况下充分的生物学和临床反应。
我们回顾性分析了一组在采用“三分之二维持量”液体限制方案后的5年期间内，被处方使用SC-DDAVP的轻度出血性疾病患儿。
按照该方案，69例患者接受了SC-DDAVP治疗，其中包括15例轻度甲型血友病患者、49例血管性血友病（VWD）患者和5例血小板贮存池病患者。在接受正式术前评估的患者中，1型VWD患者28/29例以及轻度甲型血友病患者14/15例观察到完全或部分反应。围手术期SC-DDAVP在所有患者中均提供了良好的止血效果，无需使用凝血因子浓缩剂或血液制品。7例接受多剂去氨加压素的儿童检测到轻度无症状低钠血症（最低血钠129 mmol/L）；然而，其中6例患者对规定的液体限制方案的依从性存疑。未观察到有症状的低钠血症。
皮下注射去氨加压素耐受性良好，未观察到严重副作用，且术前试验中有良好的生物学反应。三分之二维持量的液体方案在我们的队列中有效预防了有症状的低钠血症，目前是我们中心DDAVP给药后液体限制的标准方案。
