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Original Article
A Multicenter Study on Korean von Willebrand Disease Realities
Clin Pediatr Hematol Oncol 2017;24:93-100.
Published online October 31, 2017
© 2017 Korean Society of Pediatric Hematology-Oncology and Korean Society for Pediatric Neuro-Oncology

Jae Hee Seol, M.D.1, Hyun Ju Kim, M.D.1, Soon Ki Kim, M.D.2, Ki Young Yoo, M.D.3, Ye Jee Shim, M.D.4, Hee Jo Baek, M.D.5, Young Shil Park, M.D.6, Eun Jin Choi, M.D.7 and Sang Kyu Park, M.D.1

1Department of Pediatrics, Ulsan University Hospital, Ulsan, 2Department of Pediatrics, College of Medicine, Inha University, Incheon, 3Korea Hemophilia Foundation, Seoul, 4Department of Pediatrics, Keimyung University Dongsan Medical Center, Daegu, 5Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Hwasun, 6Department of Pediatrics Kyung Hee University Hospital at Gangdong, Seoul, 7Department of Pediatrics, Daegu Catholic University Hospital, Daegu, Korea
Correspondence to: Sang Kyu Park
Department of Pediatrics, University of Ulsan College of Medicine, 877 Bangeojin Soonwhan-doro, Dong-gu, Ulsan 44033, Korea
Tel: +82-52-250-7060 Fax: +82-52-250-7068 E-mail: sang@uuh.ulsan.kr
Received September 16, 2017; Revised September 26, 2017; Accepted October 10, 2017.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
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. 
Keywords: von Willebrand disease, Korea
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April 2018, 25 (1)
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  • Sang Kyu Park