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영남 지역 폰빌레브란트병의 실태 조사

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Author(s)
Hyun Ju KimYe Jee ShimJae Min LeeYoung Tak LimEu Jeen YangKyung Mi ParkHee Won ChuehEun Sil ParkHyo Sun KimJi Kyoung ParkEun Jin ChoiSeom Gim KongJi Yoon KimSang Kyu Park
Keimyung Author(s)
Shim, Ye Jee
Department
Dept. of Pediatrics (소아청소년학)
Journal Title
Clinical Pediatric Hematology-Oncology
Issued Date
2019
Volume
26
Issue
1
Keyword
von Willebrand diseaseChildrenAdultsKoreaRetrospective studies
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.
Alternative Title
A Multicenter Study on von Willebrand Disease Realities in Yeungnam Region
Keimyung Author(s)(Kor)
심예지
Publisher
School of Medicine (의과대학)
Citation
Hyun Ju Kim et al. (2019). 영남 지역 폰빌레브란트병의 실태 조사. Clinical Pediatric Hematology-Oncology, 26(1), 46–54. doi: 10.15264/cpho.2019.26.1.46
Type
Article
ISSN
2233-4580
Source
http://www.cpho.or.kr/journal/view.html?vol=26&num=1&page=46&year=2019
DOI
10.15264/cpho.2019.26.1.46
URI
https://kumel.medlib.dsmc.or.kr/handle/2015.oak/42175
Appears in Collections:
1. School of Medicine (의과대학) > Dept. of Pediatrics (소아청소년학)
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