계명대학교 의학도서관 Repository

Epidemiological Study of Hereditary Hemolytic Anemia in the Korean Pediatric Population during 1997–2016: a Nationwide Retrospective Cohort Study

Metadata Downloads
Author(s)
Ye Jee ShimHye Lim JungHee Young ShinHyoung Jin KangJung Yoon ChoiJeong Ok HahJae Min LeeYoung Tak LimEu Jeen YangHee Jo BaekHyoung Soo ChoiKeon Hee YooJun Eun ParkSeongkoo KimJi Yoon KimEun Sil ParkHo Joon ImHee Won ChuehSoon Ki KimJae Hee LeeEun Sun YooHyeon Jin ParkJun Ah LeeMeerim ParkHyun Sik KangJi Kyoung ParkNa Hee LeeSang Kyu ParkYoung-Ho LeeSeong Wook LeeEun Jin ChoiSeom Gim Kong
Keimyung Author(s)
Shim, Ye Jee
Department
Dept. of Pediatrics (소아청소년학)
Journal Title
Journal of Korean Medical Science
Issued Date
2020
Volume
35
Issue
33
Keyword
Congenital Hemolytic AnemiaHereditary SpherocytosisHemoglobinopathiesThalassemiaGlucose-6-phosphate Dehydrogenase DeficiencyPyruvate Kinase
Abstract
Background:
Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA.

Methods:
We collected the data of a newly diagnosed pediatric HHA cohort (2007–2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997–2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey.

Results:
A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia.

Conclusion:
In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA.
Keimyung Author(s)(Kor)
심예지
Publisher
School of Medicine (의과대학)
Citation
Ye Jee Shim et al. (2020). Epidemiological Study of Hereditary Hemolytic Anemia in the Korean Pediatric Population during 1997–2016: a Nationwide Retrospective Cohort Study. Journal of Korean Medical Science, 35(33), e279. doi: 10.3346/jkms.2020.35.e279
Type
Article
ISSN
1598-6357
Source
https://jkms.org/search.php?where=aview&id=10.3346/jkms.2020.35.e279&code=0063JKMS&vmode=FULL
DOI
10.3346/jkms.2020.35.e279
URI
https://kumel.medlib.dsmc.or.kr/handle/2015.oak/42863
Appears in Collections:
1. School of Medicine (의과대학) > Dept. of Pediatrics (소아청소년학)
공개 및 라이선스
  • 공개 구분공개
파일 목록

Items in Repository are protected by copyright, with all rights reserved, unless otherwise indicated.