deamino-arginine-vasopressin and Blood-Coagulation-Disorders--Inherited

Bleeding disorders, including haemophilia, von Willebrand disease, and platelet function abnormalities pose a substantial, ongoing management challenge. Patients with these disorders not only require treatment during bleeding events but also need effective management strategies to prepare for events ranging from minor dental procedures to major surgery and childbirth. Moreover, women with bleeding disorders often require ongoing treatment to prevent menorrhagia during childbearing years. Desmopressin (DDAVP), a synthetic derivative of the antidiuretic hormone l-arginine vasopressin, has become a well-established tool for the management of patients with bleeding disorders in a variety of clinical settings. However, despite the widespread use of DDAVP, the available clinical evidence on its efficacy and safety in these settings is limited, and there has not been a recent comprehensive review of its role in the clinical management of patients with bleeding disorders. As such, this article provides a review of the mechanism of action and pharmacokinetic properties of DDAVP, followed by a concise summary of the available evidence for its use in the treatment and prevention of bleeding.

Topics: Blood Coagulation Disorders, Inherited; Deamino Arginine Vasopressin; Female; Hemorrhage; Hemostatics; Humans; Male; Surgical Procedures, Operative; Treatment Outcome

Desmopressin (1-deamino-8-D-arginine vasopressin, DDAVP) has been used in children with von Willebrand disease (VWD) and Hemophilia A for almost 35 years. This treatment has substantially lowered the number of children exposed to human plasma derived products, with a good safety profile, and at very low cost. The response to DDAVP has been shown to be associated with age, baseline factor level, and genetic mutations. A DDAVP challenge test is recommended. DDAVP has also been used to prevent and treat bleeding episodes in children with platelet function defects and other disorders associated with bleeding tendency.

Topics: Blood Coagulation Disorders, Inherited; Blood Platelets; Child; Child, Preschool; Deamino Arginine Vasopressin; Hemophilia A; Hemostasis; Humans; Platelet Function Tests; von Willebrand Diseases

The authors propose a protocol to avoid bleeding complications in patients with bleeding disorders.. When a general anesthesia or trunk nerve infiltration is indicated, clotting factor concentrates are to be used in patients with severe bleeding disorders. Desmopressin is to be used in patients with mild bleeding disorders but who are good responders and in patients with thrombopathy. An antifibrinolytic treatment and a fibrin glue are also used. Ninety-six patients underwent 107 extractions with this protocol.. Only two patients had bleeding complications requiring an additional treatment.. In case of bleeding disorders, the treatment depends on disease severity and type of anesthesia. A general treatment (clotting factor concentrates or desmopressin) is indicated with general anesthesia and with local anesthesia in severe bleeding disorders, but not absolutely necessary with local anesthesia in mild bleeding disorders. However, desmopressin can used in all good responders.

Topics: Adolescent; Adult; Aged; Anesthesia, Dental; Anesthesia, General; Anesthesia, Local; Anticoagulants; Antifibrinolytic Agents; Blood Coagulation Disorders, Inherited; Child; Deamino Arginine Vasopressin; Decision Trees; Dental Care for Chronically Ill; Female; Humans; Male; Middle Aged; Oral Hemorrhage; Postoperative Hemorrhage; Tooth Extraction; Tranexamic Acid

To assess DDAVP (1-deamino-8-d-arginine vasopressin; desmopressin) nasal spray in the management of menorrhagia in patients with inherited bleeding disorders, 39 women (aged 18-50 years) with menorrhagia were recruited and were randomized to start 2 months' therapy with placebo or DDAVP (300 micro g) spray in a double-blind crossover study. Twenty-eight and 24 completed first and second period of treatment, respectively. Menstrual loss was assessed using the pictorial blood assessment chart (PBAC) during each treatment period. The main outcome measure was comparison of PBAC scores following DDAVP and placebo treatments. The safety of DDAVP spray was also assessed by monitoring side-effects. Overall, PBAC scores were significantly lower in the second treatment period than the first (P = 0.01). After adjusting for this differences, mean PBAC scores were slightly lower (mean difference 8; 95% confidence interval of - 15.5 to 31.6) in women receiving DDAVP than when receiving placebo, although this difference was not statistically significant (P = 0.51). In conclusion, although there was an indication that menstrual bleeding was less heavy when women received DDAVP than when receiving placebo, the small sample size meant that this difference was not significant.

Topics: Administration, Intranasal; Adolescent; Adult; Blood Coagulation Disorders, Inherited; Cross-Over Studies; Deamino Arginine Vasopressin; Double-Blind Method; Female; Hemostatics; Humans; Menorrhagia; Middle Aged; Treatment Outcome; von Willebrand Diseases

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.

Topics: Adolescent; Blood Coagulation Disorders, Inherited; Child; Child, Preschool; Deamino Arginine Vasopressin; Hemophilia A; Hemostatics; Humans; Hyponatremia; Injections, Subcutaneous; Platelet Storage Pool Deficiency; Retrospective Studies; Severity of Illness Index; von Willebrand Diseases

Inherited bleeding disorders can be associated with significant morbidity and mortality. Bleeding episodes can be safely and effectively managed in the majority of patients provided the coagulation defect is identified and corrected with the appropriate replacement therapy. It is imperative that these patients are treated in centres that can provide full coagulation support, and a comprehensive care plan is formulated for each individual.

Topics: Blood Coagulation Disorders, Inherited; Deamino Arginine Vasopressin; Factor VIII; Hemophilia A; Hemostatics; Humans