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Sunkyu Choi  (Choi 1) 1 Article
Adrenal Gland
Big Data Articles (National Health Insurance Service Database)
Epidemiology and Long-Term Adverse Outcomes in Korean Patients with Congenital Adrenal Hyperplasia: A Nationwide Study
Jung Hee Kim, Sunkyu Choi, Young Ah Lee, Juneyoung Lee, Sin Gon Kim
Endocrinol Metab. 2022;37(1):138-147.   Published online February 28, 2022
DOI: https://doi.org/10.3803/EnM.2021.1328
  • 3,380 View
  • 149 Download
  • 8 Web of Science
  • 10 Crossref
AbstractAbstract PDFPubReader   ePub   
Background
Previous studies on the epidemiology and complications of congenital adrenal hyperplasia (CAH) were conducted in Western countries and in children/adolescents. We aimed to explore the epidemiology of CAH, as well as the risk of comorbidities and mortality, in a Korean nationwide case-control study.
Methods
CAH patients (n=2,840) were included between 2002 and 2017 from the National Health Insurance Service database and the Rare Intractable Disease program. CAH patients were compared, at a 1:10 ratio, with age-, sex-, and index year-matched controls (n=28,400).
Results
The point prevalence of CAH patients in Korea was 1 in 18,745 persons in 2017. The annual incidence rate declined between 2003 and 2017 from 3.25 to 0.41 per 100,000 persons. CAH patients were at elevated risk for cardiovascular disease (odds ratio [OR], 1.6; 95% confidence interval [CI], 1.4 to 1.9), stroke (OR, 1.7; 95% CI, 1.3 to 2.0), diabetes mellitus (OR, 2.8; 95% CI, 2.6 to 3.1), dyslipidemia (OR, 2.4; 95% CI, 2.2 to 2.6), and psychiatric disorders (OR, 1.5; 95% CI, 1.3 to 1.6). Fracture risk increased in CAH patients aged over 40 years (OR, 1.4; 95% CI, 1.1 to 1.7). CAH patients were at higher risk of mortality than controls (hazard ratio, 1.6; 95% CI, 1.3 to 2.0).
Conclusion
Our nationwide study showed a recent decline in the incidence of CAH and an elevated risk for cardiovascular, metabolic, skeletal, and psychiatric disorders in CAH patients. Lifelong management for comorbidity risk is a crucial component of treating CAH patients.

Citations

Citations to this article as recorded by  
  • Hyperandrogenism and Cardiometabolic Risk in Pre- and Postmenopausal Women—What Is the Evidence?
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    The Journal of Clinical Endocrinology & Metabolism.2024; 109(5): 1202.     CrossRef
  • Predictors of Cardiovascular Morbidities in Adults With 21-Hydroxylase Deficiency Congenital Adrenal Hyperplasia
    Suranut Charoensri, Richard J Auchus
    The Journal of Clinical Endocrinology & Metabolism.2024; 109(3): e1133.     CrossRef
  • Case report: Development of central precocious puberty in a girl with late-diagnosed simple virilizing congenital adrenal hyperplasia complicated with Williams syndrome
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    Frontiers in Endocrinology.2024;[Epub]     CrossRef
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    Endocrinology and Metabolism.2023; 38(1): 10.     CrossRef
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    Endocrine.2023; 80(3): 630.     CrossRef
  • Serum steroid profile captures metabolic phenotypes in adults with classic congenital adrenal hyperplasia
    Chang Ho Ahn, Jaeyoon Shim, Han Na Jang, Young Ah Lee, Sang-Won Lee, Man Ho Choi, Jung Hee Kim
    The Journal of Steroid Biochemistry and Molecular Biology.2023; 234: 106374.     CrossRef
  • Long‐term health consequences of congenital adrenal hyperplasia
    Riccardo Pofi, Xiaochen Ji, Nils P. Krone, Jeremy W. Tomlinson
    Clinical Endocrinology.2023;[Epub]     CrossRef
  • Multiplexed Serum Steroid Profiling Reveals Metabolic Signatures of Subtypes in Congenital Adrenal Hyperplasia
    Jaeyoon Shim, Chang Ho Ahn, Seung Shin Park, Jongsung Noh, Chaelin Lee, Sang Won Lee, Jung Hee Kim, Man Ho Choi
    Journal of the Endocrine Society.2023;[Epub]     CrossRef
  • Long-Term Outcomes of Congenital Adrenal Hyperplasia
    Anna Nordenström, Svetlana Lajic, Henrik Falhammar
    Endocrinology and Metabolism.2022; 37(4): 587.     CrossRef
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