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Ovotesticular Disorder of Sex Development in Korean Children: A Single-Center Analysis over a 30-Year Period

Authors
 Hye In Kim  ;  Inha Lee  ;  Sang Hwa Kim  ;  Yong Seung Lee  ;  Sang Won Han  ;  Bo Hyon Yun 
Citation
 JOURNAL OF PEDIATRIC AND ADOLESCENT GYNECOLOGY, Vol.34(5) : 626-630, 2021-10 
Journal Title
JOURNAL OF PEDIATRIC AND ADOLESCENT GYNECOLOGY
ISSN
 1083-3188 
Issue Date
2021-10
Keywords
Disorders of sex development ; Ovotesticular disorder ; True hermaphrodite
Abstract
Study objective: To present clinical features that characterize ovotesticular disorder of sex development (OT-DSD) in the Korean population. Among the patient cohort who were initially suspected to have OT-DSD, the actual OT-DSD patients and those of other disorder of sex development were compared. Design: Retrospective medical chart review of patients who were initially suspected to have OT-DSD from 1984 to 2018 on the basis of clinical examination. Setting: Tertiary care university hospital. Participants: Of 26 patients with initial diagnosis of OT-DSD, 3 were excluded because of incomplete records, and finally, 23 patients were subjected to analysis. Various examinations were performed before the surgical confirmation of gonad histopathology. Interventions: Medical records were reviewed for clinical, anatomical, biochemical, and cytogenic characteristics, gender assignment, medical treatment, and histopathologic diagnosis. Main outcome measures: Characteristics of OT-DSD in a Korean population. Results: Among 23 patients suspected to have OT-DSD, 13/23 (56.5%) were diagnosed as OT-DSD after histopathologic confirmation. Of the remaining 10 patients, 5/23 (21.7%) were diagnosed with mixed gonadal dysgenesis, 3 with Turner variant, 1 with 46,XX disorder of sex development, and 1 with Mayer-Rokitansky-Küster-Hauser syndrome. Among the 13 OT-DSD cases, 9 patients presented with the 46,XX karyotype, 1 with the 46,XY, and 3 with the 46,XX/XY karyotype. Nine patients were assigned as male and 4 as female at birth. The most common gonad histology was ovotestis 10/26 (38%), followed by ovary and testis. Conclusion: OT-DSD is one of the rarest disorders with various clinical presentations. A patient with ambiguous genitalia must be examined with a multidisciplinary approach with clinical suspicion for OT-DSD. Standardized procedure of evaluation and treatment is crucial.
Full Text
https://www.sciencedirect.com/science/article/pii/S1083318821001492
DOI
10.1016/j.jpag.2021.02.105
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Obstetrics and Gynecology (산부인과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Urology (비뇨의학교실) > 1. Journal Papers
Yonsei Authors
Kim, Hye In(김혜인) ORCID logo https://orcid.org/0000-0001-7961-5988
Yun, Bo Hyon(윤보현) ORCID logo https://orcid.org/0000-0001-5703-797X
Lee, Yong Seung(이용승) ORCID logo https://orcid.org/0000-0003-3778-9888
Lee, Inha(이인하) ORCID logo https://orcid.org/0000-0003-4869-6281
Han, Sang Won(한상원) ORCID logo https://orcid.org/0000-0003-0941-1300
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/185387
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