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KMID : 0387820170240020093
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Clinical Pediatric Hematology-Oncology
2017 Volume.24 No. 2 p.93 ~ p.100
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A Multicenter Study on Korean von Willebrand Disease Realities
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Seol Jae-Hee
Kim Hyun-Ju
Kim Soon-Ki
Yoo Ki-Young
Shim Ye-Jee
Baek Hee-Jo
Park Young-Shil
Choi Eun-Jin
Park Sang-Kyu
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Abstract
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Background: von Willebrand disease (VWD) is the most common inherited bleeding dis-order with a prevalence of up to 1%. However in Korea, only 126 VWD patients were registered in Korea Hemophilia Foundation (KHF). The aim of this study was to determine the status of VWD patients in Korea. We analyzed VWD patients by age, gender, blood group, family history and bleeding history.
Methods: One hundred twenty-six VWD patients registered in the KHF by December 2016, and 74 patients diagnosed at six university hospitals were enrolled in this study. We evaluated the medical records from the KHF and the questionnaires from six university hospitals retrospectively.
Results: Seventeen patients misdiagnosed and ten patients duplicated were excluded. One hundred nine patients registered in the KHF and 64 patients diagnosed at six university hospitals met the criteria for VWD. The blood type O accounts for 72 (51.8%). VWF mutation was detected in 30 patients (17.3%). Median age at diagnosis was 10.5 yr. The bleeding score of adults was higher than that of children (P＜0.001). The most common bleeding symptom was epistaxis (48.5%). The distribution of VWD types was: 67% of type 1, 30.1% of type 2, and 2.9% of type 3.
Conclusion: Even though only six hospitals responded to the survey, 64 patients not registered in the KHF were diagnosed with VWD. Our results suggest the prevalence of Korean VWD might be higher than previously reported. A nationwide registration system is warranted in order to accurately identify the national prevalence of VWD.
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KEYWORD
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von Willebrand disease, Korea
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