title: Desmopressin acetate as a haemostatic elevator in individuals with combined deficiency of factors V and VIII: a clinical trial.
abstract: ESSENTIALS: Combined factor V (FV) and factor VIII (FVIII) deficiency (CF5F8D) is an autosomal recessive coagulation disorder. Desmopressin acetate (DDAVP) was intravenously infused in 20 adult patients with CF5F8D. DDAVP can enhance FVIII levels but has no effect on FV levels in patients with CF5F8D. DDAVP can be substituted for FVIII concentrates in patients with CF5F8D.
  
  
  OBJECTIVE
  Combined factor V (FV) and FVIII deficiency (CF5F8D) is a rare inherited autosomal recessive double-gene disorder most frequently seen in the Middle East. Although affected individuals have deficiency of two coagulation factors (range 5-30%), their bleeding tendencies are similar to patients who have deficiency of a single coagulation factor at the same level. The mainstay of their treatment is infusion of FVIII concentrate and fresh frozen plasma. Here, the effect of intravenous infusion of desmopressin acetate (DDAVP) on elevation of coagulation FV and FVIII was investigated through a clinical trial in May 2015.
  
  
  METHODS
  In a registered controlled trial, DDAVP (dosage 0.3 μg kg(-1) ) was intravenously infused into 20 adult patients with CF5F8D over 20 min. After an hour, blood samples were collected and plasma levels of FV and FVIII were measured.
  
  
  CONCLUSIONS
  This study revealed that DDAVP can enhance FVIII levels but has no effect on FV plasma concentration in patients with CF5F8D. Based on these findings, FVIII concentrates may be substituted for DDAVP in patients with CF5F8D.
authors.affiliation: Allergy Research Center, Ghaem Hospital, Mashhad University of Medical Sciences, Mashhad, Iran.
authors.forename: H
authors.initials: H
authors.lastname: Mansouritorghabeh
authors.affiliation: Internal Medicine Department, Ghaem Hospital, Mashhad University of Medical Sciences, Mashhad, Iran.
authors.forename: A
authors.initials: A
authors.lastname: Shirdel
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000097:blood; D001780:Blood Coagulation Tests; D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D005165:Factor V / Q000378:metabolism*; D005166:Factor V Deficiency / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D005169:Factor VIII / Q000378:metabolism*; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D006487:Hemostasis / Q000187:drug effects*; D006490:Hemostatics / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D006801:Humans; D007262:Infusions, Intravenous; D007492:Iran; D008297:Male; D008875:Middle Aged; D013997:Time Factors; D016896:Treatment Outcome; D015854:Up-Regulation; D055815:Young Adult
chemical_list: D015415:Biomarkers; D006490:Hemostatics; C078147:F8 protein, human; D005165:Factor V; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin
keywords: DDAVP; combined factor V and VIII deficiency (CF5F8D); desmopressin; factor V; factor VIII