Clin Pediatr Hematol Oncol.  2014 Apr;21(1):16-22.

Prevalence of Possible von Willebrand Disease

Affiliations
  • 1Department of Pediatrics, Ulsan University Hospital, Ulsan, Korea. sang@uuh.ulsan.kr
  • 2Department of Pediatrics, Inha University Hospital, Incheon, Korea.
  • 3Korea Hemophilia Foundation, Korea.
  • 4Department of Laboratory Medicine, Samsung Medical Center, Seoul, Korea.
  • 5Department of Laboratory Medicine, Ulsan University Hospital, Ulsan, Korea.

Abstract

BACKGROUND
Definition of possible von Willebrand disease (VWD) included levels of von Willebrand factor antigen (VWF:Ag) or von Willebrand factor ristocetin cofactor activity (VWF:RCo) below 30 U/dL. The purpose of this study was to determine the prevalence of possible VWD in Ulsan, Korea. We also analyzed the influence of demographic factors such as age, gender, and blood group on subject levels of VWF.
METHODS
Between March 2011 and September 2011 we prospectively investigated 1,039 subjects (271 children, 768 adults). Blood samples were collected for the determination of VWF:Ag, VWF:RCo, and factor VIII coagulation assay (FVIII:C). A standardized questionnaire was administered to evaluate hemorrhagic symptoms at the time of first examination, using a bleeding score ranging from 0 to 3.
RESULTS
Forty five subjects met the criteria for possible VWD, for a prevalence of 4.3%. Subjects of Group O had a significantly lower mean FVIII:C, VWF:Ag, and VWF:RCo value than subjects of group A,B, or AB (P<0.001).
CONCLUSION
Our results suggest that the prevalence of VWD may be much higher than previously reported. Efforts to increase the awareness and diagnosis of VWD may help improve identification of patients with bleeding disorders and lead to early, appropriate management with safe and efficacious therapies.

Keyword

von Willebrand disease; Prevalence; Ulsan

MeSH Terms

Child
Demography
Diagnosis
Factor VIII
Hemorrhage
Humans
Korea
Prevalence*
Prospective Studies
Ulsan
von Willebrand Diseases*
von Willebrand Factor
Surveys and Questionnaires
Factor VIII
von Willebrand Factor
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