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. 2022 Nov;63(11):991-998.
doi: 10.3349/ymj.2022.0143.

Population Prevalence, Cancer Risk, and Mortality Risk of Turner Syndrome in South Korean Women Based on National Health Insurance Service Data

Sung Eun Kim  1 , Sang Hyun Park  2 , Kyungdo Han  3 , Won Kyoung Cho  4 , Byung-Kyu Suh  5 , Yong-Gyu Park  2
Affiliations

Population Prevalence, Cancer Risk, and Mortality Risk of Turner Syndrome in South Korean Women Based on National Health Insurance Service Data

Sung Eun Kim et al. Yonsei Med J. 2022 Nov.

Abstract

Purpose: In South Korea, investigations into Turner syndrome (TS) prevalence and TS-associated cancer and mortality are lacking. Accurate data were estimated from the National Health Insurance Service (NHIS) and the Rare Diseases Registry (RDR) records.

Materials and methods: Data on patients with TS who were registered in the RDR between 2007 and 2017 were collected. To estimate TS-associated cancer and mortality risk, the data were compared with data of 1:3 age-matched controls.

Results: In 2017, 2054 patients with TS were identified from a total population of 26186952 South Korean women; therefore, the prevalence was 7.84 per 100000 persons. TS prevalence across 10-year interval age groups were 11.82, 23.17, 18.37, 10.49, 4.09, and 0.38 for age under 10 years, teenagers, 20s, 30s, 40s, and older than 50, respectively (per 100000 persons). The cancer risk in patients with TS was higher than that of age-matched controls over 5.3 person-years [hazard ratio (HR)=1.82, 95% confidence interval (CI) 1.01-3.27, p=0.045]. Among different types of cancer, thyroid cancer risk in patients with TS was significantly higher than that of age-matched controls (HR=2.78, 95% CI 1.06-7.26, p=0.037). We also observed that TS-associated all-cause mortality risk was higher than that of age-matched controls (HR=3.36, 95% CI 1.59-7.10, p=0.002).

Conclusion: National prevalence of TS was suggested, and an increased risk of TS-associated thyroid cancer and mortality were observed in this study.

Keywords: Prevalence; mortality risk; thyroid cancer risk; turner syndrome.

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Conflict of interest statement

The authors have no potential conflicts of interest to disclose.

Figures

Fig. 1
Fig. 1. Flow diagram of study subjects for cancer incidence and mortality.
*Cancer occurrence or death in the first year of follow-up.
See this image and copyright information in PMC

References

    1. Classic pages in obstetrics and gynecology by Henry H. Turner. A syndrome of infantilism, congenital webbed neck, and cubitus valgus. Endocrinology, vol. 23, pp. 566-574, 1938. Am J Obstet Gynecol. 1972;113:279. - PubMed
    1. Bondy CA. Care of girls and women with Turner syndrome: a guideline of the Turner Syndrome Study Group. J Clin Endocrinol Metab. 2007;92:10–25. - PubMed
    1. Gravholt CH, Andersen NH, Conway GS, Dekkers OM, Geffner ME, Klein KO, et al. Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting. Eur J Endocrinol. 2017;177:G1–G70. - PubMed
    1. Paolucci DG, Bamba V. Turner syndrome: care of the patient: birth to late adolescence. Pediatr Endocrinol Rev. 2017;14(Suppl 2):454–461. - PubMed
    1. Carvalho AB, Guerra Júnior G, Baptista MT, de Faria AP, Marini SH, Guerra AT. Cardiovascular and renal anomalies in Turner syndrome. Rev Assoc Med Bras (1992) 2010;56:655–659. - PubMed