Childhood Sex-Typed Behavior and Gender Change in Individuals with 46,XY and 46,XX Disorders of Sex Development: An Iranian Multicenter Study

AbstractContextIn 46,XY disorders of sexual development (DSD) patients, several factors may affect psychosexual development, leading to gender identity discrepancy and gender change later in life. Prenatal sexual steroid exposure and external genital virilization are considered to influence human psychosexual development, but their roles not completely understood yet.DesignA total of 144 individuals (18 to 60 years of age) with a clinical/molecular diagnosis of 46,XY DSD from a single tertiary center were enrolled. Psychosexual outcomes (gender role, gender identity, and sexual orientation) were assessed using questionnaires and psychological test. The Sinnecker score was used for genital virilization measurement. Prenatal androgen exposure was estimated according to 46,XY DSD etiology.ResultsWe found a positive association between prenatal androgen exposure and male psychosexual outcomes. Alternatively, prenatal estrogen exposure, age of gonadectomy, and the degree of external genital virilization did not influence any psychosexual outcome. There were 19% (n = 27) with gender change, which was associated with prenatal androgen exposure (P < 0.001) but not with the external genital virilization. The median age of gender change was 15 years, but most of the patients reported the desire for gender change earlier.ConclusionsPrenatal androgen exposure influenced psychosexual development in 46,XY DSD favoring male psychosexuality in all psychosexual outcomes, whereas the degree of external genital virilization did not influence these outcomes. The organizational effect of sexual steroids on psychosexuality at puberty appears to be weak in comparison with the prenatal effects. Prenatal androgen exposure also influenced female-to-male gender change frequency. All 46,XY DSD conditions with prenatal androgen exposure must be followed for gender issues in their management.

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Quality of Life in Children with Disorders of Sex Development

Hormone Research in Paediatrics ◽

10.1159/000478780 ◽

2017 ◽

Vol 88 (5) ◽

pp. 324-330 ◽

Cited By ~ 3

Author(s):

Nalini M Selveindran ◽

Syed Zulkifli Syed Zakaria ◽

Muhammad Yazid Jalaludin ◽

Rahmah Rasat

Keyword(s):

Quality Of Life ◽

Family Income ◽

Disorders Of Sex Development ◽

School Functioning ◽

Control Group ◽

Evidence Based Treatment ◽

Sex Development ◽

Longitudinal Outcome ◽

And Gender

Background/Aims: Disorders of sex development (DSD) are a heterogeneous group of rare conditions. Evidence-based treatment is challenged by a lack of clinical longitudinal outcome studies. We sought to investigate the quality of life of children with DSD other than congenital adrenal hyperplasia. Methods: The participants (aged 6–18 years) were 23 patients raised as males and 7 patients raised as females. Control data were obtained from representatives of the patients’ siblings matched for age and gender. The Pediatric Quality of Life InventoryTM Version 4.0 (PedsQL) Generic Core Scales were used as the study tool. Results: In comparison with the reference data, the patient group had significantly lower overall PedsQL (p < 0.01) and school functioning (p < 0.01) scores. Also, the total PedsQL score was significantly lower in patients with DSD who were of female social sex as compared to the controls who were females. Family income, surgical procedures, degree of virilization, and mode of puberty did not influence the PedsQL scores. Conclusion: This study revealed a poorer quality of life for patients with DSD as compared to the age-matched control group. This highlights the need for a skilled multidisciplinary team to manage this group of patients.

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Intersex

Sociology ◽

10.1093/obo/9780199756384-0232 ◽

2020 ◽

Author(s):

Morgan Carpenter

Keyword(s):

Human Rights ◽

Disorders Of Sex Development ◽

Cultural Norms ◽

Human Rights Violations ◽

Sex Characteristics ◽

Sex And Gender ◽

Sex Development ◽

High Commissioner ◽

Values And Beliefs ◽

And Gender

Intersex people are described by United Nations institutions as born with variations of sex characteristics that differ from medical and social norms for female or male bodies (see, for example, the 2019 report “Human Rights Violations against Intersex People,” by the Office of the High Commissioner for Human Rights). These variations are diverse and innate. Intersex human rights defenders and human rights institutions challenge the stigma and discrimination that intersex people face because of their physical variations, but few jurisdictions so far have tackled the human rights violations that intersex people suffer. There are multiple additional, contested, and incommensurate lenses through which intersex people are viewed. These express different values and beliefs about the same people, including their meaning, treatment, concerns, and demands. Medical lenses view intersex traits as “disorders of sex development” (DSD), and people with those traits are viewed as female or male and subjects for treatment. Anthropology and queer and gender studies have viewed intersex as an illustration of fallacies that underpin subjective cultural norms for sex and gender. Law increasingly views intersex people as members of a third sex. Historical research shows that intersex people, often termed hermaphrodites, have always existed, and often been accommodated.

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Gender Development in 46,XY DSD: Influences of Chromosomes, Hormones, and Interactions with Parents and Healthcare Professionals

Scientifica ◽

10.6064/2012/834967 ◽

2012 ◽

Vol 2012 ◽

pp. 1-15 ◽

Cited By ~ 9

Author(s):

Amy B. Wisniewski

Keyword(s):

Healthcare Professionals ◽

Disorders Of Sex Development ◽

Gender Development ◽

Social Variables ◽

Sex Development ◽

Genital Ambiguity ◽

And Gender ◽

Social Components ◽

The Impact

Variables that impact gender development in humans are difficult to evaluate. This difficulty exists because it is not usually possible to tease apart biological influences on gender from social variables. People with disorders of sex development, or DSD, provide important opportunities to study gender within individuals for whom biologic components of sex can be discordant with social components of gender. While most studies of gender development in people with 46,XY DSD have historically emphasized the importance of genes and hormones on gender identity and gender role, more recent evidence for a significant role for socialization exists and is considered here. For example, the influence of parents’ perceptions of, and reactions to, DSD are considered. Additionally, the impact of treatments for DSD such as receiving gonadal surgeries or genitoplasty to reduce genital ambiguity on the psychological development of people with 46,XY DSD is presented. Finally, the role of multi-disciplinary care including access to peer support for advancing medical, surgical and psychosexual outcomes of children and adults with 46,XY DSD, regardless of sex of rearing, is discussed.

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Thoughts on the Nature of Identity: Disorders of Sex Development and Gender Identity

Child and Adolescent Psychiatric Clinics of North America ◽

10.1016/j.chc.2011.07.007 ◽

2011 ◽

Vol 20 (4) ◽

pp. 627-638 ◽

Cited By ~ 2

Author(s):

William G. Reiner ◽

D. Townsend Reiner

Keyword(s):

Gender Identity ◽

Disorders Of Sex Development ◽

Sex Development ◽

And Gender

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Neonatal Sex Assignment in Disorders of Sex Development: A Philosophical Introspection

Journal of Neonatal Surgery ◽

10.21699/jns.v6i3.604 ◽

2017 ◽

Vol 6 (3) ◽

pp. 58 ◽

Cited By ~ 7

Author(s):

V Raveenthiran

Keyword(s):

Gender Bias ◽

Personal Experience ◽

Disorders Of Sex Development ◽

Ambiguous Genitalia ◽

Third Parties ◽

Sex And Gender ◽

Sex Development ◽

Consensus Conferences ◽

Sex Assignment ◽

And Gender

Management of ambiguous genitalia is highly controversial. This condition was known previously as intersex and presently as disorders of sex development (DSD). There is no consensus regarding the choice, timing and method of sex assignment in neonates with DSD. Consensus conferences could not unify the views of various stakeholders and third parties. This article philosophically examines the nature and origin of such controversies. Misconception, bias and conflicting priorities are identified as the three cardinal sources of controversies. Conceptual duality of sexes, confused notion of sex and gender, bias towards penetrative intercourse, conflict between utopian ideals and reality, unwillingness to compromise are identified as perpetuators of controversies. Suggestions are made regarding sex assignment in various types of DSD based on the understanding of published literature and the author’s personal experience.

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Hormones, Sexual Orientation, and Gender Identity

The Oxford Handbook of Evolutionary Psychology and Behavioral Endocrinology ◽

10.1093/oxfordhb/9780190649739.013.11 ◽

2019 ◽

pp. 199-214

Author(s):

Nicholas C. Neibergall ◽

Alex J. Swanson ◽

Francisco J. Sánchez

Keyword(s):

Sexual Orientation ◽

Gender Identity ◽

Association Studies ◽

Endocrine Disrupting Chemicals ◽

Disorders Of Sex Development ◽

Biological Research ◽

Sex Development ◽

Endocrine Disrupting ◽

Specific Factors ◽

And Gender

Gender identity and sexual orientation are two characteristics that play a significant role in human development. This chapter focuses on the potential role that hormones play in their development. First, a review of the direct effects of hormones and endocrine-disrupting chemicals is provided with a focus on animal models and persons born with disorders of sex development. Second, some evidence from association studies linking characteristics that are known or suspected of being influenced by hormones is provided. Although biological research has yielded some intriguing findings—especially from neuroimaging studies—scientists have yet to conclude what specific factors contribute to their development.

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Prenatal testosterone and gender-related behaviour

Acta Endocrinologica ◽

10.1530/eje.1.02236 ◽

2006 ◽

Vol 155 (suppl_1) ◽

pp. S115-S121 ◽

Cited By ~ 106

Author(s):

Melissa Hines

Keyword(s):

Sexual Orientation ◽

Disorders Of Sex Development ◽

Mammalian Brain ◽

Critical Periods ◽

Toy Preferences ◽

Prenatal Testosterone ◽

Sex Development ◽

Play Behaviour ◽

Mental Rotations ◽

And Gender

Testosterone plays an important role in mammalian brain development. In neural regions with appropriate receptors testosterone, or its metabolites, influences patterns of cell death and survival, neural connectivity and neurochemical characterization. Consequently, testosterone exposure during critical periods of early development produces permanent behavioural changes. In humans, affected behaviours include childhood play behaviour, sexual orientation, core gender identity and other characteristics that show sex differences (i.e. differ on average between males and females). These influences have been demonstrated primarily in individuals who experienced marked prenatal hormone abnormalities and associated ambiguities of genital development (e.g. congenital adrenal hyperplasia). However, there is also evidence that testosterone works within the normal range to make some individuals within each sex more sex-typical than others. The size of testosterone-related influences, and perhaps even their existence, varies from one sex-typed characteristic to another. For instance: prenatal exposure to high levels of testosterone has a substantial influence on sex-typical play behaviour, including sex-typed toy preferences, whereas influences on core gender identify and sexual orientation are less dramatic. In addition: there appears to be little or no influence of prenatal testosterone on mental rotations ability, although mental rotations ability shows a marked sex difference. These findings have implications for basic understanding of the role of testosterone in normative gender development, as well as for the clinical management of individuals with disorders of sex development (formerly called intersex syndromes).

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