ABSTRACT
Background: von Willebrand Diseases (vWD) is the most commonly inherited bleeding disorder. It is defined as a deficiency or abnormality of von Willebrand Factor (vWF) causing impaired hemostasis. Studies in a predominantly pediatric population reveal that the prevalence of vWD is 0.8-1.3 percent. Objective: To determine the prevalence of von Willebrand Diseases and to establish its clinico-hematologic profile. Methods: Ninety-nine patients with bleeding manifestations referred to the National Hemophilia Center from all over the Philippines were studied. Patients who fulfilled the inclusion criteria of at least 2 symptoms consistent with vWD, underwent initial screening tests: a complete blood count with actual platelet, blood typing, bleeding time, prothrombin time and activated partial thromboplastin time. Laboratory tests to diagnose vWD were done. Results: Thirty four patients (34.34 percent) with bleeding manifestations had vWD. Patients with vWD and those without were comparable as to age, sex distribution, family history of bleeding, blood type and bleeding manifestations. Among the patients with vWD, 11 (32.35 percent) had Type I vWD and 23 (67.65 percent) had Type 2 vWD. The mean FVIII, vWF: Ag and vWF: RCo were decreased. Conclusion: The study suggests that there is a high proportion of vWD among patients with bleeding tendency in the Philippines.
Subject(s)
Humans , Male , Female , Adult , Young Adult , Adolescent , Child , von Willebrand Diseases , HemorrhageABSTRACT
OBJECTIVES: We evaluated the efficacy and safety of recombinant activated factor VII (rFVIIa) in children aged < 18 years old with grade II or grade III Dengue hemorrhagic fever (DHF) who required blood component therapy for controlling bleeding episodes. STUDY DESIGN: Patients were randomized to the rFVIIa group or placebo group in a ratio of 2:1. rFVIIa or placebo (100 microg/kg body weight) was given by intravenous bolus injection. When bleeding was not effectively controlled, a second dose of rFVIIa or placebo (100 microg/kg) was given 30 min after the first dose. RESULTS: Nine and 16 patients received placebo and rFVIIa, respectively. The demographics, bleeding manifestations and grade of DHF were similar for the rFVIIa and placebo groups. Apart from petechiae and ecchymosis, one to four additional bleeding sites were found in each patient, including hematemesis (n = 15), epistaxis (n = 14), gum bleeding (n = 12), melena (n = 7), hypermenorrhea (n = 4), hematochezia (n = 2) and hematuria (n = 2). The mean total dose of rFVIIa (138.4 +/- 50.9 microg/kg) and placebo (145.4 +/- 53.7 microg/kg) were comparable. The efficacy of bleeding control at 2 h after the first dose was completely ceased (rFVIIa 75.0% versus placebo 44.4%), decreased (rFVIIa 18.7% versus placebo 11.2%), and unchanged or worsened (rFVIIa 6.3% versus placebo 44.4%). Some patients with active bleeding received platelet concentrates 3-12 h after the first dose of rFVIIa or placebo. The subsequent efficacy of bleeding control at 6, 12 and 24 h was comparable between the two groups. The cumulative use of red blood cells (rFVIIa 31.3% versus placebo 33.3%) and plasma (rFVIIa 25% versus placebo 22%) during the 24-h period was not significantly different between the two groups. In contrast, platelet concentrate requirement in the rFVIIa group (6.3%) was lower than the placebo (33.3%). No clinical evidence of thromboembolic complications or mortality as a result of bleeding was observed. CONCLUSION: rFVIIa appears to be a useful adjunctive treatment to blood component transfusion for controlling active bleeding in children with DHF especially when platelet concentrate is not readily available.
