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Original Article
A Multicenter Study on von Willebrand Disease Realities in Yeungnam Region
Clin Pediatr Hematol Oncol 2019;26:46-54.
Published online April 30, 2019
© 2019 Korean Society of Pediatric Hematology-Oncology and Korean Society for Pediatric Neuro-Oncology

Hyun Ju Kim1, Ye Jee Shim2, Jae Min Lee3, Young Tak Lim4, Eu Jeen Yang4, Kyung Mi Park4, Hee Won Chueh5, Eun Sil Park6, Hyo Sun Kim7, Ji Kyoung Park8, Eun Jin Choi9, Seom Gim Kong10, Ji Yoon Kim11, and Sang Kyu Park1

1Department of Pediatrics, School of Medicine, University of Ulsan, Ulsan, 2Department of Pediatrics, Keimyung University College of Medicine, Daegu, 3Department of Pediatrics, Yeungnam University College of Medicine, Daegu, 4Department of Pediatrics, Pusan National University College of Medicine, Yangsan, 5Department of Pediatrics, Dong-A University College of Medicine, Busan, 6Department of Pediatrics, Gyeongsang National University College of Medicine, Jinju, 7Department of Pediatrics, Inje University Haeundae Paik Hospital, Busan, 8Department of Pediatrics, Inje University Busan Paik Hospital, Busan, 9Department of Pediatrics, School of Medicine, Catholic University of Daegu, Daegu, 10Department of Pediatrics, Kosin University College of Medicine, Busan, 11Department of Pediatrics, School of Medicine, Kyungpook National University, Daegu, Korea
Correspondence to: Sang Kyu Park
Department of Pediatrics, Ulsan University Hospital, 877 Bangeojinsunhwando-ro, Dong-gu, Ulsan 44033, Korea
Tel: +82-52-250-7060
Fax: +82-52-250-7068
E-mail: sang@uuh.ulsan.kr
ORCID ID: orcid.org/0000-0003-2458-981X
Received March 13, 2019; Revised March 26, 2019; Accepted April 9, 2019.
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 one of the most common inherited bleeding disorders. However, the number of patients who register to the Korea Hemophilia Foundation (KHF) is much lower than the expected prevalence rate and only few hospitals perform tests for diagnosis autonomously. Thus, we surveyed practical realities of VWD in Yeungnam region.
Methods: Patients with VWD (N=267) who were diagnosed at eleven university hospitals from March 1995 to March 2018 were enrolled in this study. We evaluated the medical records from each hospital retrospectively.
Results: Two hundred and twenty-eight children and 39 adults met the diagnostic criteria for VWD. Seventy-eight (57.4%) patients had the blood type O. Fifty-eight patients were definite type 1 (21.7%), 151 were possible type 1 (56.6%), and the others were type 2. Abnormal laboratory findings were the most common factor for the diagnosis in children. VWF mutations were detected in 17 patients. Patients with a family history showed age of diagnosis of 9 y, which is higher than in those with no family history (6 yr), and also showed a higher rate of significant bleeding (32.1% vs. 14.2%). VWF:RCo and VWF:Ag tests were performed in-hospital at only 1 of 11 hospitals. Twelve of 267 patients were enrolled at the KHF (4.5%).
Conclusion: A high rate of out-sourcing studies may result in inaccurate diagnosis. The registration rate to the KHF is still lower than the prevalence rate. A comprehensive nationwide registration system is necessary in order to identify the actual prevalence rate and promote the diagnosis of VWD in Korea.
Keywords: von Willebrand disease, Children, Adults, Korea, Retrospective studies
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October 2019, 26 (2)
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  • Sang Kyu Park