Prevalence of von Willebrand disease in children: a multiethnic study. J Pediatr 1993 Dec;123(6):893-8
Date
12/01/1993Pubmed ID
8229521DOI
10.1016/s0022-3476(05)80384-1Scopus ID
2-s2.0-0027458101 (requires institutional sign-in at Scopus site) 423 CitationsAbstract
OBJECTIVE: Von Willebrand disease (vWD) was thought to be a rare disorder until a recent survey reported a prevalence of 0.8% in an ethnically homogenous community in northern Italy. The purpose of this study was to determine the prevalence of vWD in an ethnically heterogenous population.
METHODS: Von Willebrand factor (vWF) was measured by the ristocetin cofactor method in 600 healthy children, aged 2 to 18 years, seen for routine school physical examinations in a three-state region. Personal and family bleeding symptoms were determined by questionnaire. The diagnosis of vWD required a personal history of bleeding symptoms, an abnormal vWF activity concentration, and a family history of at least one immediate family member with bleeding symptoms.
RESULTS: A total of 315 subjects were white, 212 were black, 16 were Hispanic, 10 were from other groups, and 47 were biracial. Eight subjects (four black, four white) met the criteria for vWD, for a prevalence of 1.3%. Seven subjects with vWD had blood group O (mean vWF = 32 U/dl; range, 10 to 42 U/dl), and one had blood group A (vWF = 41 U/dl). Children who had blood group O had significantly (p < 0.001) lower vWF activities (median, 83 U/dl, range, 43 to 162 U/dl) than those from non-O blood groups (median, 98 U/dl; range, 51 to 190 U/dl). There were no significant differences in vWF activity by ethnicity. The vWF activity was significantly (p < 0.02) greater for boys than girls in both blood groups.
CONCLUSION: Von Willebrand disease is the most common congenital hemostatic disorder; its high prevalence is not limited to one ethnic group.
Author List
Werner EJ, Broxson EH, Tucker EL, Giroux DS, Shults J, Abshire TCMESH terms used to index this publication - Major topics in bold
AdolescentBlood Group Antigens
Child
Child, Preschool
Female
Humans
Male
Prevalence
Sex Factors
United States
von Willebrand Diseases