가능성 있는 폰빌레브란트병의 유병률에 대한 연구 (Prevalence of Possible von Willebrand Disease)

서지정보

· 발행기관 : 대한소아혈액종양학회
· 수록지 정보 : Clinical Pediatric Hematology-Oncology / 21권 / 1호 / 16 ~ 22페이지
· 저자명 : 이명숙, 김순기, 김동빈, 유기영, 김희진, 이선호, 박상규

초록

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.

영어초록

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.

참고자료

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