Epidemiological Study of Hereditary Hemolytic Anemia in the Korean Pediatric Population during 1997-2016: a Nationwide Retrospective Cohort Study
- 주제(키워드) Congenital Hemolytic Anemia , Hereditary Spherocytosis , Hemoglobinopathies , Thalassemia , Glucose-6-phosphate Dehydrogenase Deficiency , Pyruvate Kinase
- 주제(기타) Medicine, General & Internal
- 설명문(일반) [Shim, Ye Jee] Keimyung Univ, Sch Med, Dept Pediat, Dongsan Med Ctr, Daegu, South Korea; [Jung, Hye Lim] Sungkyunkwan Univ, Kangbuk Samsung Hosp, Sch Med, Dept Pediat, 29 Saemunan Ro, Seoul 03181, South Korea; [Shin, Hee Young; Kang, Hyoung Jin; Choi, Jung Yoon] Seoul Natl Univ, Childrens Hosp, Canc Res Inst, Dept Pediat,Coll Med, 103 Daehak Ro, Seoul 03080, South Korea; [Hah, Jeong Ok] Daegu Fatima Hosp, Dept Pediat, Daegu, South Korea; [Lee, Jae Min] Yeungnam Univ, Dept Pediat, Coll Med, Daegu, South Korea; [Lim, Young Tak; Yang, Eu Jeen] Pusan Natl Univ, Pusan Natl Univ Childrens Hosp, Dept Pediat, Sch Med, Yangsan, South Korea; [Baek, Hee Jo] Chonnam Natl Univ, Hwasun Hosp, Dept Pediat, Med Sch, Gwangju, South Korea; [Choi, Hyoung Soo] Seoul Natl Univ, Bundang Hosp, Dept Pediat, Coll Med, Seongnam, South Korea; [Yoo, Keon Hee] Sungkyunkwan Univ, Samsung Med Ctr, Dept Pediat, Sch Med, Seoul, South Korea; [Park, Jun Eun] Ajou Univ, Dept Pediat, Sch Med, Suwon, South Korea; [Kim, Seongkoo] Catholic Univ Korea, Coll Med, Dept Pediat, Seoul St Marys Hosp, Seoul, South Korea; [Kim, Ji Yoon] Kyungpook Natl Univ, Kyungpook Natl Univ Hosp, Sch Med, Dept Pediat, Daegu, South Korea; [Park, Eun Sil] Gyeongsang Natl Univ, Gyeongsang Natl Univ Hosp, Dept Pediat, Coll Med, Jinju, South Korea; [Im, Ho Joon] Univ Ulsan, Asan Med Ctr, Dept Pediat, Coll Med,Childrens Hosp, Seoul, South Korea; [Chueh, Hee Won] Dong A Univ, Dept Pediat, Coll Med, Busan, South Korea; [Kim, Soon Ki] Inha Univ Hosp, Dept Pediat, Incheon, South Korea; [Lee, Jae Hee] Chosun Univ Hosp, Dept Pediat, Gwangju, South Korea; [Yoo, Eun Sun] Ewha Womans Univ, Seoul Hosp, Dept Pediat, Coll Med, Seoul, South Korea; [Park, Hyeon Jin; Lee, Jun Ah; Park, Meerim] Natl Canc Ctr, Ctr Pediat Canc, Dept Pediat, Goyang, South Korea; [Kang, Hyun Sik] Jeju Natl Univ, Jeju Natl Univ Hosp, Dept Pediat, Sch Med, Jeju, South Korea; [Park, Ji Kyoung] Inje Univ, Busan Paik Hosp, Dept Pediat, Coll Med, Busan, South Korea; [Lee, Na Hee] Cha Univ, Cha Bundang Med Ctr, Dept Pediat, Seongnam, South Korea; [Park, Sang Kyu] Univ Ulsan, Ulsan Univ Hosp, Dept Pediat, Coll Med, Ulsan, South Korea; [Lee, Young-Ho] Hanyang Univ, Dept Pediat, Seoul Hosp, Seoul, South Korea; [Lee, Seong Wook] Korea Univ, Ansan Hosp, Dept Pediat, Coll Med, Ansan, South Korea; [Choi, Eun Jin] Daegu Catholic Univ, Dept Pediat, Sch Med, Daegu, South Korea; [Kong, Seom Gim] Kosin Univ, Gospel Hosp, Dept Pediat, Coll Med, Busan, South Korea
- 등재 SCIE, SCOPUS, KCI등재
- OA유형 Green Published, gold
- 발행기관 KOREAN ACAD MEDICAL SCIENCES
- 발행년도 2020
- 총서유형 Journal
- URI http://www.dcollection.net/handler/ewha/000000174598
- 본문언어 영어
- Published As http://dx.doi.org/10.3346/jkms.2020.35.e279
- PubMed https://pubmed.ncbi.nlm.nih.gov/32830468
초록/요약
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.
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