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Clin Pediatr Hematol Oncol. 2019 Apr;26(1):46-54. Korean. Multicenter Study. https://doi.org/10.15264/cpho.2019.26.1.46
Kim HJ , Shim YJ , Lee JM , Lim YT , Yang EJ , Park KM , Chueh HW , Park ES , Kim HS , Park JK , Choi EJ , Kong SG , Kim JY , Park SK .
Department of Pediatrics, School of Medicine, University of Ulsan, Ulsan, Korea. sang@uuh.ulsan.kr
Department of Pediatrics, Keimyung University College of Medicine, Daegu, Korea.
Department of Pediatrics, Yeungnam University College of Medicine, Daegu, Korea.
Department of Pediatrics, Pusan National University College of Medicine, Yangsan, Korea.
Department of Pediatrics, Dong-A University College of Medicine, Busan, Korea.
Department of Pediatrics, Gyeongsang National University College of Medicine, Jinju, Korea.
Department of Pediatrics, Inje University Haeundae Paik Hospital, Busan, Korea.
Department of Pediatrics, Inje University Busan Paik Hospital, Busan, Korea.
Department of Pediatrics, School of Medicine, Catholic University of Daegu, Daegu, Korea.
Department of Pediatrics, Kosin University College of Medicine, Busan, Korea.
Department of Pediatrics, School of Medicine, Kyungpook National University, Daegu, Korea.
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.

Copyright © 2019. Korean Association of Medical Journal Editors.