Systematic review of literature and analysis of big data from the National Health Insurance System on primary immunodeficiencies in Korea.

Publisher: Korean Pediatric Society Country of Publication: Korea (South) NLM ID: 101761234 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2713-4148 (Electronic) Linking ISSN: 27134148 NLM ISO Abbreviation: Clin Exp Pediatr Subsets: PubMed not MEDLINE

Original Publication: Seoul, Korea : Korean Pediatric Society, [2020]-

There are very scant data on the epidemiology of primary immunodeficiency diseases (PIDs) in Korea. Here we attempted to estimate the PID epidemiology and disease burden in Korea. A systematic review was performed of studies retrieved from the PubMed, KoreaMed, and Google Scholar databases. Studies on PIDs published in Korean or English between January 2001 and November 2018 were analyzed. The number of PID patients and the healthcare costs were estimated from Health Insurance Review and Assessment Service (HIRA) Korea data for 2017. A total of 398 PID patients were identified from 101 reports. Immunodeficiencies affecting cellular and humoral immunity were reported in 11 patients, combined immunodeficiency with associated or syndromic features in 40, predominantly antibody deficiencies in 144, diseases of immune dysregulation in 58, congenital defects of phagocytes in 104, defects in the intrinsic and innate immunity in 1, auto-inflammatory disorders in 4, complement deficiencies in 36, and phenocopies of PID in none. From the HIRA reimbursement data, a total of 1,162 outpatients and 306 inpatients were treated for 8,166 and 6,149 days, respectively. In addition, reimbursement was requested for 8,200 outpatient and 1,090 inpatient cases and $1,924,000 and $4,715,000 were reimbursed in 2017, respectively. This study systematically reviewed published studies on PID and analyzed the national open data system of the HIRA to estimate the disease burden of PID, for the first time in Korea.

J Clin Immunol. 2007 Sep;27(5):517-24. (PMID: 17588141)
J Allergy Clin Immunol. 2016 Jan;137(1):327-330. (PMID: 26478010)
J Korean Med Sci. 2012 Jul;27(7):788-93. (PMID: 22787376)
Korean J Pediatr. 2016 Nov;59(Suppl 1):S57-S59. (PMID: 28018447)
J Clin Immunol. 2016 Jul;36(5):415-7. (PMID: 27048657)
J Clin Immunol. 2018 Jan;38(1):129-143. (PMID: 29226301)
J Clin Immunol. 2011 Dec;31(6):968-76. (PMID: 21956496)
J Clin Immunol. 2018 Oct;38(7):757-766. (PMID: 30151618)
Immunol Res. 2014 Oct;60(1):132-44. (PMID: 24668296)
Epidemiol Health. 2014 Jul 30;36:e2014008. (PMID: 25078381)
Immunol Res. 2018 Jun;66(3):367-380. (PMID: 29744770)
Science. 2015 Jul 24;349(6246):436-40. (PMID: 26206937)
J Clin Immunol. 2013 Jan;33(1):1-7. (PMID: 22847546)
J Allergy Clin Immunol. 2016 Oct;138(4):957-969. (PMID: 27720020)
N Engl J Med. 2019 Apr 18;380(16):1525-1534. (PMID: 30995372)
J Exp Med. 2014 Oct 20;211(11):2137-49. (PMID: 25311508)
Front Immunol. 2018 Sep 10;9:2012. (PMID: 30250467)
Clin Genet. 2015 Dec;88(6):507-15. (PMID: 25708106)
J Allergy Clin Immunol. 2016 Jun;137(6):1780-1787. (PMID: 26915675)

2016R1A6A3A11932463 National Research Foundation of Korea; Ministry of Education

Keywords: Epidemiology; Primary immunodeficiency diseases

Date Created: 20200721 Latest Revision: 20210415

20240829

PMC8024117

10.3345/cep.2019.01347

32683811