Gender Identity and Sexual Function in 46,XX Patients with Congenital Adrenal Hyperplasia Raised as Males

Apóstolos, Robson A. C.; Canguçú-Campinho, Ana Karina Figueira; Lago, Renata; Costa, Ana Carolina S.; Oliveira, Luciana Mattos Barros; Toralles, Maria Betânia Pereira; Barroso, Ubirajara

doi:10.1007/s10508-018-1299-z

Cited by 14 publications

(10 citation statements)

References 10 publications

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“…In the literature, the majority of 46,XX CAH patients raised as males were diagnosed late ( 4 , 10 , 11 , 12 , 13 ). Few patients are diagnosed before the age of two years ( 3 ). In our cohort, it was recommended that female patients diagnosed at <2 years of age be raised as females, particularly those diagnosed during the neonatal period.…”

Section: Discussionmentioning

confidence: 99%

“…However, the age at diagnosis, age of initiation of glucocorticoid treatment, and degree of virilization were not considered. Some studies that considered these factors proposed that severely virilized patients with 46,XX CAH may be raised as males ( 3 , 4 , 5 ). Gender determination at birth and appropriate family and social support may be important in shaping sexual identity ( 5 , 6 ).…”

Section: Introductionmentioning

confidence: 99%

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Clinical Characteristics of 46,XX Males with Congenital Adrenal Hyperplasia

Savaş‐Erdeve¹,

Aycan²,

Çeti̇nkaya³

et al. 2021

Jcrpe

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Objective: To retrospectively evaluate the follow-up data in patients with 46,XX congenital adrenal hyperplasia (CAH) who were raised male. Methods: A national database was created. The data of patients were asked to be recorded in the data form. Results: The median (range) age of diagnosis was three (0.1-18.3) years in 44 patients. Twenty nine cases were diagnosed after the age of two years. Most (95.4%) cases were stage 4-5 virilized. Hysterectomy and bilateral salpingoopherectomy, at a median age of 7.25 (2.4-25.3) years, was performed in 35 cases. Testicular prostheses were placed in 11 (25%) cases at a median age of 11.2 (2.8-17) years. The median final height was 149.2 (132.8-172) cms in 38 patients, including simple virilizing (n=18), salt-wasting (n=6), and 11-beta hydroxylase (n=12). Of the 16 patients above the age of eighteen, university education was completed in 25%. Conclusion: It was seen that most (65.9%) of the 46,XX CAH cases raised male were diagnosed after two years of age. In these cases, hysterectomy and bilateral salpingoopherectomy, genital corrective surgeries and testicular prosthesis operations were performed in a very wide age rage.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Clinical Characteristics of 46,XX Males with Congenital Adrenal Hyperplasia

Savaş‐Erdeve¹,

Aycan²,

Çeti̇nkaya³

et al. 2021

Jcrpe

View full text Add to dashboard Cite

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“…Однако здесь следует заметить, что в странах, где такая среда существует (например, в Индии), девочкам с выраженной вирилизацией НПО мужской пол и так назначался и назначается без всяких рекомендаций [25,26,27]. В других же странах, прежде всего развивающихся, такая ситуация может возникнуть у отдельных больных в связи с поздним выявлением причины вирилизации и ошибочным назначением мужского пола девочкам с ВГН [28][29][30]. При поздней диагностике и отсутствии соответствующего лечения к антенатальному воздействию андрогенов добавляется и постнатальное, что в совокупности с соответствующим воспитанием может приводить к формированию мужской половой идентичности у таких пациенток.…”

Section: выбор половой принадлежностиunclassified

“…Интересно, что в их числе был и тот, у которого в детстве проводилась феминизирующая генитопластика и имелись лишь остатки эректильных тканей фаллоса. В другом сообщении указывалось, что все четверо наблюдавшихся аналогичных больных также испытывали оргазм, хотя только двое из них имели половые контакты с женщинами [29].…”

Section: выбор половой принадлежностиunclassified

“…В статьях [26,27,29], сообщавших о больных с 46,ХХ кариотипом и выраженной вирилизацией НПО при ВГН, которым назначался мужской пол, указывалось, что у них проводились сложные этапные пластики по поводу гипоспадии. В этих работах коррекция гипоспадии осуществлялась у 10 из 12 наблюдавшихся пациентов (лишь двое имели вирилизацию V ст.).…”

Section: выбор половой принадлежностиunclassified

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Disorders of sex differentiation: state of the problem 15 years after the Chicago consensus

Shiryaev

Kagantsov

Сизонов

2020

Probl Endokrinol (Mosk)

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It is well known that the nomenclature and classification were changed in 2005 at the international consensus conference on intersex disorders, held in Chicago, where, among others, the following recommendations were proposed: (1) all children should be assigned a gender identity, and this should be done as quickly as possible, taking into account the time required for the examination. (2) all infants with congenital adrenal hyperplasia and 46,XX karyotype, including those with pronounced masculinization, must be raised as women. (3) Surgical treatment should be performed early and in cases of feminizing genitoplasty, clitoral reduction should be performed simultaneously with reconstruction of the urogenital sinus (separation of the vagina and urethra). An analysis of contemporary literature shows that all these theories, proposed 15 years ago at the Chicago meeting, failed to stand the test of time. New nomenclature and classification are constantly being revised. Currently, many groups of patients want to abolish the term sexual maturity disorders. Recommendations regarding gender reassignment and appropriate early surgical treatment have been completely ignored in some countries. All this was largely facilitated by the confrontational activities of a number of support groups.

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Gender and Sexuality in Disorders/Differences of Sex Development

Kreukels

Grift

2022

Gender and Sexuality Development

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Gender Identity and Sexual Function in 46,XX Patients with Congenital Adrenal Hyperplasia Raised as Males

Cited by 14 publications

References 10 publications

Clinical Characteristics of 46,XX Males with Congenital Adrenal Hyperplasia

Clinical Characteristics of 46,XX Males with Congenital Adrenal Hyperplasia

Disorders of sex differentiation: state of the problem 15 years after the Chicago consensus

Gender and Sexuality in Disorders/Differences of Sex Development

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