Incidence of hypothyroidism in girls with Turner syndrome in Korea on the basis of real-world evidence from the Korean National Health Insurance Service database.

Abstract

Objective: Hypothyroidism is the most commonly observed autoimmune disorder in individuals with Turner syndrome. The aim of this study was to determine the risk of hypothyroidism in patients with Turner syndrome by comparing its incidence in this patient population to that in the general population.

Design: In this retrospective cohort study, patients in South Korea were followed for 10 years, and claims data from the National Health Insurance Service database were collected between 2007 and 2019.

Methods: The incidence of hypothyroidism among patients with Turner syndrome and in the general population under 65 years of age was determined. Turner syndrome was identified by at least 2 diagnosis codes, and hypothyroidism was defined by the prescription of thyroid hormone analogs lasting 180 days or more, accompanied by a hypothyroidism diagnosis code. Subscribers who had records related to medical conditions that required thyroid hormone replacement were excluded from the cohort.

Results: The cohort included 2973 patients with Turner syndrome and 21 239 127 females. Hypothyroidism developed in 11.4% of the patients with Turner syndrome and in 2.9% of the general population. The incidence rates per 10 000 person-years among patients with Turner syndrome were 86.9 (95% CI, 64.5-114.6), 101.0 (95% CI, 82.2-122.9), 139.6 (95% CI, 113.3-170.1), and 139.4 (95% CI, 112.1-171.3) in the groups aged 0-9, 10-19, 20-29, and 30-65 years, respectively, and those in the general population were 1.7 (95% CI, 1.6-1.7), 5.0 (95% CI, 5.0-5.1), 29.7 (95% CI, 29.5-29.9), and 39.1 (95% CI, 39.0-39.2), respectively. The risk of hypothyroidism in patients with Turner syndrome was approximately 45 times greater than that in the general population before 10 years of age.

Conclusions: Regular thyroid function testing and antibody screening should be initiated early in life to facilitate the early detection of hypothyroidism in patients with Turner syndrome.