title: Assessment and validation of a defined fluid restriction protocol in the use of subcutaneous desmopressin for children with inherited bleeding disorders.
abstract: BACKGROUND
  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.
  
  
  OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Haematology, Lady Cilento Children's Hospital, Brisbane, QLD, Australia. jane.mason@health.qld.gov.au.
authors.forename: J A
authors.initials: JA
authors.lastname: Mason
authors.affiliation: Department of Haematology, Lady Cilento Children's Hospital, Brisbane, QLD, Australia.
authors.forename: J D
authors.initials: JD
authors.lastname: Robertson
authors.affiliation: Department of Haematology, Lady Cilento Children's Hospital, Brisbane, QLD, Australia.
authors.forename: J
authors.initials: J
authors.lastname: McCosker
authors.affiliation: Department of Haematology, Lady Cilento Children's Hospital, Brisbane, QLD, Australia.
authors.forename: B A
authors.initials: BA
authors.lastname: Williams
authors.affiliation: Department of Haematology, Lady Cilento Children's Hospital, Brisbane, QLD, Australia.
authors.forename: S A
authors.initials: SA
authors.lastname: Brown
mesh_terms: D000293:Adolescent; D025861:Blood Coagulation Disorders, Inherited / Q000188:drug therapy* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003894:Deamino Arginine Vasopressin / Q000009:adverse effects / Q000627:therapeutic use*; D006467:Hemophilia A / Q000188:drug therapy / Q000473:pathology; D006490:Hemostatics / Q000009:adverse effects / Q000627:therapeutic use*; D006801:Humans; D007010:Hyponatremia / Q000209:etiology; D007279:Injections, Subcutaneous; D010981:Platelet Storage Pool Deficiency / Q000188:drug therapy / Q000473:pathology; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014842:von Willebrand Diseases / Q000188:drug therapy / Q000473:pathology
chemical_list: D006490:Hemostatics; D003894:Deamino Arginine Vasopressin
keywords: desmopressin; haemophilia; hyponatremia; paediatric; von Willebrand disease