Epidemiology and clinical outcomes of clinically suspected multiple endocrine neoplasia type 1 in South Korea: a nationwide cohort study
BackgroundMultiple endocrine neoplasia type 1 (MEN1) is a rare hereditary disorder characterized by multiorgan endocrine tumors, primarily affecting the parathyroid glands, pituitary, and pancreas. Despite its clinical significance, the epidemiology and outcomes of clinically suspected MEN1 in Asian...
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Frontiers Media S.A.
2025-06-01
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| Series: | Frontiers in Endocrinology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fendo.2025.1562282/full |
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| author | Kyoung Jin Kim Min Heui Yu Min Heui Yu Yoon-a Hwang Shinje Moon Namki Hong Yumie Rhee |
| author_facet | Kyoung Jin Kim Min Heui Yu Min Heui Yu Yoon-a Hwang Shinje Moon Namki Hong Yumie Rhee |
| author_sort | Kyoung Jin Kim |
| collection | DOAJ |
| description | BackgroundMultiple endocrine neoplasia type 1 (MEN1) is a rare hereditary disorder characterized by multiorgan endocrine tumors, primarily affecting the parathyroid glands, pituitary, and pancreas. Despite its clinical significance, the epidemiology and outcomes of clinically suspected MEN1 in Asian populations remain limited. This study aimed to investigate the prevalence, comorbidities, and mortality risk associated with clinically suspected MEN1 in South Korea.MethodsWe conducted a retrospective cohort study using the Korean National Health Insurance Service database (2003–2020), identifying clinically suspected MEN1 cases via two operational definitions: (1) ICD-10 MEN1 code (D44.8) with medical service records and (2) diagnoses or interventions for at least two MEN1-associated conditions (primary hyperparathyroidism, pituitary adenoma, or duodenopancreatic neuroendocrine tumors). Cases (n = 412) were matched 1:10 with controls (n = 4,120) by age, sex, and index year. Clinical characteristics, comorbidities, and mortality were analyzed using Kaplan–Meier survival analysis and multivariable Cox regression.ResultsThe incidence of clinically suspected MEN1 peaked in individuals aged 40–49 years, with a higher prevalence in females (64.6%). Parathyroid involvement was the most common manifestation (58.6%), followed by pituitary (22.3%) and duodenopancreatic tumors (19.9%). Comorbidities, including diabetes mellitus (22.6%), hypertension (38.1%), and dyslipidemia (20.6%), were significantly more prevalent in MEN1 patients than controls. Mortality was elevated among MEN1 patients (HR 3.69; 95% confidence intervals (CI) 2.56–5.31), particularly those with multiorgan involvement, although hazard ratios varied by organ combination and had wide, overlapping CIs. The mean age at death was significantly younger in MEN1 patients (60.1 years) than in controls (68.0 years).ConclusionsThis nationwide cohort study of clinically suspected MEN1 in South Korea reveals a substantial clinical burden, particularly among patients with multiorgan involvement. Enhanced clinical surveillance and early interventions are essential to improve outcomes for MEN1 patients. Future research integrating genetic testing and clinical data is needed to further guide management strategies. |
| format | Article |
| id | doaj-art-b58ac29950a7475b910cd5b9150b59a3 |
| institution | Kabale University |
| issn | 1664-2392 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Endocrinology |
| spelling | doaj-art-b58ac29950a7475b910cd5b9150b59a32025-08-20T03:31:10ZengFrontiers Media S.A.Frontiers in Endocrinology1664-23922025-06-011610.3389/fendo.2025.15622821562282Epidemiology and clinical outcomes of clinically suspected multiple endocrine neoplasia type 1 in South Korea: a nationwide cohort studyKyoung Jin Kim0Min Heui Yu1Min Heui Yu2Yoon-a Hwang3Shinje Moon4Namki Hong5Yumie Rhee6Division of Endocrinology and Metabolism, Department of Internal Medicine, Korea University College of Medicine, Seoul, Republic of KoreaDepartment of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of KoreaSeverance ENdocrinology daTa scIeNcE pLatform (SENTINEL) Team, Division of Endocrinology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of KoreaDepartment of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of KoreaDepartment of Internal Medicine, Hanyang University College of Medicine, Seoul, Republic of KoreaDepartment of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of KoreaDepartment of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of KoreaBackgroundMultiple endocrine neoplasia type 1 (MEN1) is a rare hereditary disorder characterized by multiorgan endocrine tumors, primarily affecting the parathyroid glands, pituitary, and pancreas. Despite its clinical significance, the epidemiology and outcomes of clinically suspected MEN1 in Asian populations remain limited. This study aimed to investigate the prevalence, comorbidities, and mortality risk associated with clinically suspected MEN1 in South Korea.MethodsWe conducted a retrospective cohort study using the Korean National Health Insurance Service database (2003–2020), identifying clinically suspected MEN1 cases via two operational definitions: (1) ICD-10 MEN1 code (D44.8) with medical service records and (2) diagnoses or interventions for at least two MEN1-associated conditions (primary hyperparathyroidism, pituitary adenoma, or duodenopancreatic neuroendocrine tumors). Cases (n = 412) were matched 1:10 with controls (n = 4,120) by age, sex, and index year. Clinical characteristics, comorbidities, and mortality were analyzed using Kaplan–Meier survival analysis and multivariable Cox regression.ResultsThe incidence of clinically suspected MEN1 peaked in individuals aged 40–49 years, with a higher prevalence in females (64.6%). Parathyroid involvement was the most common manifestation (58.6%), followed by pituitary (22.3%) and duodenopancreatic tumors (19.9%). Comorbidities, including diabetes mellitus (22.6%), hypertension (38.1%), and dyslipidemia (20.6%), were significantly more prevalent in MEN1 patients than controls. Mortality was elevated among MEN1 patients (HR 3.69; 95% confidence intervals (CI) 2.56–5.31), particularly those with multiorgan involvement, although hazard ratios varied by organ combination and had wide, overlapping CIs. The mean age at death was significantly younger in MEN1 patients (60.1 years) than in controls (68.0 years).ConclusionsThis nationwide cohort study of clinically suspected MEN1 in South Korea reveals a substantial clinical burden, particularly among patients with multiorgan involvement. Enhanced clinical surveillance and early interventions are essential to improve outcomes for MEN1 patients. Future research integrating genetic testing and clinical data is needed to further guide management strategies.https://www.frontiersin.org/articles/10.3389/fendo.2025.1562282/fullmultiple endocrine neoplasia type 1epidemiologycomorbiditycohort studymortality |
| spellingShingle | Kyoung Jin Kim Min Heui Yu Min Heui Yu Yoon-a Hwang Shinje Moon Namki Hong Yumie Rhee Epidemiology and clinical outcomes of clinically suspected multiple endocrine neoplasia type 1 in South Korea: a nationwide cohort study Frontiers in Endocrinology multiple endocrine neoplasia type 1 epidemiology comorbidity cohort study mortality |
| title | Epidemiology and clinical outcomes of clinically suspected multiple endocrine neoplasia type 1 in South Korea: a nationwide cohort study |
| title_full | Epidemiology and clinical outcomes of clinically suspected multiple endocrine neoplasia type 1 in South Korea: a nationwide cohort study |
| title_fullStr | Epidemiology and clinical outcomes of clinically suspected multiple endocrine neoplasia type 1 in South Korea: a nationwide cohort study |
| title_full_unstemmed | Epidemiology and clinical outcomes of clinically suspected multiple endocrine neoplasia type 1 in South Korea: a nationwide cohort study |
| title_short | Epidemiology and clinical outcomes of clinically suspected multiple endocrine neoplasia type 1 in South Korea: a nationwide cohort study |
| title_sort | epidemiology and clinical outcomes of clinically suspected multiple endocrine neoplasia type 1 in south korea a nationwide cohort study |
| topic | multiple endocrine neoplasia type 1 epidemiology comorbidity cohort study mortality |
| url | https://www.frontiersin.org/articles/10.3389/fendo.2025.1562282/full |
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