Desmopressin acetate as a haemostatic elevator in individuals with combined deficiency of factors V and VIII: A clinical trial

Mansouritorghabeh, H. and Shirdel, A. (2016) Desmopressin acetate as a haemostatic elevator in individuals with combined deficiency of factors V and VIII: A clinical trial. Journal of Thrombosis and Haemostasis, 14 (2). pp. 336-339.

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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. Summary: Objectives: 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. Patients and 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. Results and conclusion: 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. © 2016 International Society on Thrombosis and Haemostasis.

Item Type: Article
Additional Information: Cited By :7 Export Date: 16 February 2020 CODEN: JTHOA Correspondence Address: Mansouritorghabeh, H.; Allergy Research Center, Ghaem Hospital, Mashhad University of Medical SciencesIran; email: Mansouritorghabeh@mums.ac.ir
Uncontrolled Keywords: Combined factor V and VIII deficiency (CF5F8D) DDAVP Desmopressin Factor V Factor VIII blood clotting factor 8 fresh frozen plasma argipressin1 deamino biological marker blood clotting factor 5 F8 protein, human hemostatic agent adult Article bleeding blood clotting factor 5 deficiency blood transfusion clinical article consanguineous marriage drug efficacy drug response female hemophilia A hot flush human injury male menorrhagia prevalence priority journal protein blood level risk reduction tooth extraction treatment outcome blood blood clotting test complication drug effects Factor V Deficiency hemostasis intravenous drug administration Iran metabolism middle aged time factor upregulation young adult Biomarkers Blood Coagulation Tests Deamino Arginine Vasopressin Hemostatics Humans Infusions, Intravenous Time Factors Up-Regulation
Subjects: QU Biochemistry
Divisions: Mashhad University of Medical Sciences
Depositing User: mr lib4 lib4
Date Deposited: 03 Mar 2020 11:09
Last Modified: 03 Mar 2020 11:09
URI: http://eprints.mums.ac.ir/id/eprint/13078

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