Gender Dysphoria and Gender Change in Chromosomal Females with Congenital Adrenal Hyperplasia

2005 ◽  
Vol 34 (4) ◽  
pp. 389-397 ◽  
Author(s):  
Arianne B. Dessens ◽  
Froukje M. E. Slijper ◽  
Stenvert L. S. Drop
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Gender Dysphoria ◽  
Adrenal Hyperplasia ◽  
And Gender ◽  
Gender Change

scholarly journals Effects of Pre and Post-Natal Androgen Exposure on Psychosexual Aspects and Gender Change in Congenital Adrenal Hyperplasia Due to 21-hydroxylase Deficiency

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A500-A501
Author(s):  
Rafael Loch Batista ◽  
Marlene Inacio ◽  
Mirela Costa de Miranda ◽  
Guiomar Madureira ◽  
Larissa Garcia Gomes ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
External Genitalia ◽  
Male Gender ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Deficiency ◽  
Androgen Exposure ◽  
Sex Assignment ◽  
And Gender ◽  
21 Hydroxylase Deficiency ◽  
Gender Change

Abstract Introduction: Congenital Adrenal Hyperplasia (CAH) is defined as a group of autosomal recessive disorders characterized by a deficiency of the enzyme required to synthesize cortisol by the adrenal cortex. Defects in the 21-hydroxylase enzyme make up 90% of CAH. These defects impaired cortisol synthesis leading to an ACTH increase resulting in androgen excess in both salt-wasting (SW) or simple virilizing (SV) forms. As androgens play a role in the human psychosexual development favoring male psychosexuality, this study was designed to evaluate the impact of androgen exposure on the psychosexuality of individuals with CAH due 21-hydroxylase deficiency. Methods: This retrospective cohort includes 46,XX individuals (115 female-assigned; 8 male-assigned) with a molecular diagnosis of CAH due to CYP21A2 pathogenic variants in homozygous or compounds heterozygous state. External genitalia virilization was scored using Prader scale. Phenotype, time at diagnosis, sex assignment, and gender change were assessed. The gender role at childhood was assessed through the playmates and toys profile at childhood. Gender identity was assessed by a projective psychological test (HTP). Sexual orientation was assessed by self-report sexual identity. Compliance of glucocorticoid replacement was assessed by adequate testosterone and androstenedione serum levels for age. Results: CAH was diagnosed at the neonatal time in 73% (n=78). Fifth-nine (51%) had the SW form and 49% (n=56) had the SV form. While all cases of SW were diagnosed at the neonatal time (0.12 ± 0.14 months), the mean age at diagnosis among SV was 6.03 ± 8.45 years (p=<.001). The median of Prader score was 3 in both forms. Male sex assignment was associated with more virilized external genitalia (p=.002). Gender change occurred in 6 cases (female to male), all with SV form. The prader score was higher among those who changed gender (p=.01). All of those who changed their gender had poor treatment compliance. A total of 13% (n=15) of all groups defined themselves as homosexual. There was a strong association between male toys and preference for male playmates in childhood with homosexuality and male gender identity in adulthood with both gender change from female to male and homosexuality. Conclusion: Prenatal androgen exposure favors male psychosexuality in 46,XX CAH individuals as observed by the association between highest Prader scores and all assessed psychosexual outcomes. This influence is also substantiated by post-natal androgen exposure as observed by compliance issues and late diagnosis among those who changed from female to male gender.

Gender Change from Female to Male in Classical Congenital Adrenal Hyperplasia

1996 ◽  
Vol 30 (4) ◽  
pp. 319-332 ◽  
Author(s):  
Heino F.L. Meyer-Bahlburg ◽  
Rhoda S. Gruen ◽  
Maria I. New ◽  
Jennifer J. Bell ◽  
Akira Morishima ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adrenal Hyperplasia ◽  
Gender Change

Gender Dysphoria in a 62-Year-Old Genetic Female With Congenital Adrenal Hyperplasia

2016 ◽  
Vol 45 (7) ◽  
pp. 1871-1875 ◽  
Author(s):  
Mariana Telles Silveira ◽  
Felícia Knobloch ◽  
Carolina C. P. Silva Janovsky ◽  
Claudio E. Kater
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Gender Dysphoria ◽  
Adrenal Hyperplasia

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

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Sumudu Nimali Seneviratne ◽  
Umesh Jayarajah ◽  
Shamaali Gunawardana ◽  
Malik Samarasinghe ◽  
Shamya de Silva
Keyword(s):  
Gender Identity ◽  
Gender Role ◽  
Congenital Adrenal Hyperplasia ◽  
Exploratory Analysis ◽  
Childhood And Adolescence ◽  
Healthy Children ◽  
Adrenal Hyperplasia ◽  
Related Factors ◽  
Increased Risk ◽  
And Gender

Abstract 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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