Gender dysphoria and XX congenital adrenal hyperplasia: how frequent is it? Is male-sex rearing a good idea?

Jesus, Lisieux Eyer de; Costa, Eduardo Corrêa; Dekermacher, Samuel

doi:10.1016/j.jpedsurg.2019.01.062

Cited by 23 publications

(15 citation statements)

References 35 publications

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“…There is a paucity of data on the influence of socio-cultural factors on gender development in CAH [ 2 ]. A recent review reporting gender dysphoria rates among girls with CAH patients found wide variation (6.3 to 27.2%) across different settings, and highlighted the need to explore hereto unexplored cultural biases, and factors such as illiteracy, religious dogma and unavailability of specialized health care providers which may play a significant role in the observed variations [ 2 ]. Higher rates of male rearing in genetic females with CAH have been reported in cultures strongly favouring males on religious, financial or societal grounds, and patients from underdeveloped countries with late diagnosis and non-accessibility to the correct treatment.…”

Section: Discussion/conclusionmentioning

confidence: 99%

“…Classical congenital adrenal hyperplasia (C-CAH) is the commonest disorder/ difference of sex development (DSD) under the 46, XX DSD subgroup. Females with classical CAH often have virilised genitalia at birth due to excessive fetal adrenal androgen production, but are conventionally reared as girls, mainly due to future female reproductive potential [ 1 , 2 ]. However, it is postulated that in-utero “virilisation” of the brain may also occur, which can affect gender-role behaviour (GRB) and gender-identity (GI) in childhood and adulthood [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

“…Gender-related issues in CAH are complex. Socio-cultural background, and delayed diagnosis/ inadequate treatment, can influence GRB/GI in girls with CAH [ 2 , 17 ]. Data is available however, mainly from high-income countries in Europe and North America and there is a lack of data from Low-Middle Income Countries (LMIC), where socio-cultural background and medical care will differ.…”

Section: Introductionmentioning

confidence: 99%

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Gender-role behaviour and gender identity in girls with classical congenital adrenal hyperplasia

et al. 2021

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Introduction Girls with classical congenital adrenal hyperplasia (CAH) are exposed to excess fetal adrenal androgens in-utero, and often born with masculinised genitalia. They are conventionally reared as females, but show more “boyish” gender-role behaviour (GRB) and gender-identity (GI) issues in childhood and adolescence. Male-rearing is also reported mainly due to delayed treatment and/or socio-cultural factors. We compared GRB/GI in girls with CAH with healthy age matched children, and explored for associations with socio-demographic and diagnosis/treatment related factors. Methods GRB and GI were assessed using the Gender Identity Questionnaire for children (GIQC) in 27 girls with classical CAH at a specialised clinic, and compared with 50 age-matched healthy controls, with exploratory-analysis based on socio-demographic and diagnosis/treatment-related factors. Results Girls with CAH had lower total GIQC scores compared to healthy children (3.29 vs. 4.04, p = < 0.001) with lower GRB score (3.39 vs. 4.23, p < 0.001), and tendency for lower GI score (3.19 vs. 3.5, p = 0.08). Exploratory analysis showed no differences based on diagnosis/treatment factors including age, degree of virilisation at diagnosis and surgical procedures. and only subtle changes based on ethnicity and maternal education. Discussion/conclusion Girls with CAH managed at a specialised centre showed more masculinised GRB and tendency for ambiguous GI, which did not vary upon diagnosis/treatment related factors, suggesting that prenatal androgen exposure was the likely contributor. Clinicians should be vigilant about the increased risk of gender-related problems in girls with CAH, irrespective of sociocultural background and despite early treatment.

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Section: Discussion/conclusionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Gender-role behaviour and gender identity in girls with classical congenital adrenal hyperplasia

et al. 2021

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“…В следующем исследовании [36] был проведен аналитический дескриптивный обзор литературы за 30-летний период (с 1988 по 2018 гг.). Было показано, что больные с 46,ХХ и ВГН, воспитывавшиеся в женском поле, часто имели желание быть мальчиками, демонстрировали мужское поведение и во взрослом состоянии становились гомо-или бисексуалами, но это не представляло собой гендерную дисфорию (ГД).…”

Section: выбор половой принадлежностиunclassified

“…Было отмечено, что у женщин она может быть более гибкой и изменчивой, чем у мужчин, а также, возможно, более подверженной культурным и социальным факторам. К тому же следует учесть и полученные de Jesus et al [36] данные о том, что нетрадиционная сексуальная ориентация у женщин с ВГН не сопровождается ГД с желанием смены пола.…”

Section: выбор половой принадлежностиunclassified

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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Disorders or Differences of Sex Development

Cools

Hannema²

2023

Endocrinology

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Gender dysphoria and XX congenital adrenal hyperplasia: how frequent is it? Is male-sex rearing a good idea?

Cited by 23 publications

References 35 publications

Gender-role behaviour and gender identity in girls with classical congenital adrenal hyperplasia

Gender-role behaviour and gender identity in girls with classical congenital adrenal hyperplasia

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

Disorders or Differences of Sex Development

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