The Successful Use of Desmopressin With Tranexamic Acid in Children With Mild Type 1 Von Willebrand Disease Who Undergo Circumcision

Abstract

Background: Desmopressin (DDAVP) is a synthetic vasopressin analog, and increases endogenous von Willebrand factor (VWF) by secreting it from the vascular endothelial cell. Circumcision is one of the oldest and most commonly performed operations in the world. This study reported the specific uses of DDAVP together with tranexamic acid in children with mild type 1 von Willebrand disease (VWD) undergoing circumcision. The aim of this study was to report the successful use of DDAVP in two doses and tranexamic acid in six children with VWD undergoing circumcision.

Methods: Our study evaluated two doses of DDAVP therapy in circumcision operation of six male children diagnosed with mild type 1 VWD with a good response to DDAVP before they underwent circumcision, with a mean age of 7.3 years. The subcutaneous preparation of DDAVP was utilized. Under medical supervision, patients were subcutaneously injected DDAVP at a dose between 0.3 and 0.2 ?g/kg based on their weight. Oral tranexamic acid was instituted at a dosage of 15 mg/kg three times per day for 5 days. The first dose was given 24 hours before circumcision. The first and second doses of DDAVP were given 1 hour before the circumcision and 24 hours after the circumcision, respectively.

Results: This study included six male children aged 4 - 10 years with mild type 1 VWD, weighting 22 - 30 kg. The DDAVP dose (mean, range) based on the patients weight was 0.25 (0.23 - 0.28 ?g/kg). The calculated dose was repeated two times for each patient. Laboratory values (mean, range, U/dL) of baseline VWF:Ag, VWF:RCo and FVIII:C in mild type 1 VWD patients were 37 (32 - 42), 38 (34 - 42), and 50 (44 - 56), respectively.

Conclusion: Single infusions of DDAVP for common bleeding episodes do not usually require laboratory monitoring. There are limited published data to guide clinical practice about the optimal dosing and timing of these agents to prevent or resolve bleeding using DDAVP at minor surgeries as circumcision operation. Castaman et al showed almost all minor surgeries and deliveries occurring during follow-up were successfully managed with DDAVP in patients with type 1 VWD. We used two doses in circumcision like OBrein. Comparison of the results across studies is difficult, as surgical techniques, timing, and frequency of DDAVP dosing, and use and dosing of antifibrinolytics all varied by institution. Two doses of DDAVP allowed adequate perioperative bleeding prophylaxis management in children with mild type 1 VWD who underwent circumcision surgery with presenting bleeding complications.




Int J Clin Pediatr. 2015;4(4):181-183
doi: http://dx.doi.org/10.14740/ijcp228e