The role of steroidogenesis disorders in developing congenital clitoromegaly
https://doi.org/10.17749/2313-7347/ob.gyn.rep.2024.494
Abstract
Clitoromegaly is characterized by enlarged clitoris exceeding normal size typical to healthy women that represents a pressing issue in gynecological endocrinology. It emerges as an idiopathic condition or is formed along with impaired synthesis of sex steroids, accompanied by androgen excess. However, idiopathic clitoromegaly is a rather common diagnosis, since the objective cause of this symptom often remains unidentified. Investigating an alternative pathway for the synthesis of androgens and 11-oxysetroids in women with clitoromegaly is of great interest to practicing endocrinologists and gynecologists due to new opportunities for diagnosing and preventing this condition. This literature review is aimed at assessing current insights into disorders of female androgen steroidogenesis and its role in clitoromegaly pathogenesis.
About the Authors
N. V. Ivanov
Mechnikov North-Western State Medical University, Health Ministry of Russian Federation
Russian Federation
Russian Federation
Nikita V. Ivanov, MD, PhD
Scopus Author ID: 57221190481. WoS ResearcherID: JRZ-0207-2023
I. P. Serebryakova
Mechnikov North-Western State Medical University, Health Ministry of Russian Federation
Russian Federation
Russian Federation
Inna P. Serebryakova, MD, PhD
41 Kirochnaya Str., Saint Petersburg 191015
Scopus Author ID: 58679099300. WoS ResearcherID: JRZ-0285-2023
L. I. Radugina
Mechnikov North-Western State Medical University, Health Ministry of Russian Federation
Russian Federation
Russian Federation
Lyubov I. Radugina
41 Kirochnaya Str., Saint Petersburg 191015
F. M. Radugin
Saint Luke's Clinical Hospital
Russian Federation
Russian Federation
Fyodor M. Radugin, MD
46 Chugunnaya Str., Saint Petersburg 194044
Sh. K. Yusupova
Andijan State Medical Institute
Uzbekistan
Uzbekistan
Shakhnoza K. Yusupova, MD, Dr Sci Med
1 Yu. Otabekova Str., Andijan 170127
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What is already known about this subject?
► Clitoromegaly is a common symptom in endocrinological diseases: from nosologies related to disorders of sex formation to virilizing forms of adrenocortical cancer and polycystic ovary syndrome. However, the mechanisms underlying clitoromegaly development are often understudied.
What are the new findings?
► The mechanisms resulting in hyperandrogenism and development of clitoromegaly in patients associated with classical forms of congenital adrenal cortex dysfunction are discussed.
► The role of disturbed androgen synthesis in the adrenal glands and placenta in emerging hyperandrogenism in individuals with karyotype 46,XX is elucidated within the framework of understanding a role of alternative androgen steroidogenesis and mechanisms for 11-oxygenated steroid hyperproduction.
► A role of aromatase in mother's placenta for developing clitoromegaly is analyzed.
How might it impact on clinical practice in the foreseeable future?
► This review suggests expanding diagnostic approaches in management of patients with clitoromegaly to develop effective methods for its prevention and treatment.
Review
For citations:
Ivanov N.V., Serebryakova I.P., Radugina L.I., Radugin F.M., Yusupova Sh.K. The role of steroidogenesis disorders in developing congenital clitoromegaly. Obstetrics, Gynecology and Reproduction. 2024;18(5):679–692. (In Russ.) https://doi.org/10.17749/2313-7347/ob.gyn.rep.2024.494
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