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

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          Abstract

          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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          Most cited references38

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          Sexual orientation in women with classical or non-classical congenital adrenal hyperplasia as a function of degree of prenatal androgen excess.

          46,XX individuals with classical congenital adrenal hyperplasia (CAH) due to deficiency of the enzyme, 21-hydroxylase, show variable degrees of masculinization of body and behavior due to excess adrenal androgen production. Increased bisexuality and homosexuality have also been reported. This article provides a review of existing reports of the latter and presents a new study aimed at replicating the previous findings with detailed assessments of sexual orientation on relatively large samples, and at extending the investigation to the mildest form, non-classical (NC) CAH. Also, this is the first study to relate sexual orientation to the specific molecular genotypes of CAH. In the present study, 40 salt-wasters (SW), 21 SV (simple-virilizing), 82 NC, and 24 non-CAH control women (sisters and female cousins of CAH women) were blindly administered the Sexual Behavior Assessment Schedule (SEBAS-A, 1983 ed.; H. F. L. Meyer-Bahlburg & A. A. Ehrhardt, Privately printed). Most women were heterosexual, but the rates of bisexual and homosexual orientation were increased above controls not only in women with classical CAH, but also in NC women, and correlated with the degree of prenatal androgenization. Classifying women by molecular genotypes did not further increase the correlation. Diverse aspects of sexual orientation were highly intercorrelated, and principal components analysis yielded one general factor. Bisexual/homosexual orientation was (modestly) correlated with global measures of masculinization of non-sexual behavior and predicted independently by the degree of both prenatal androgenization and masculinization of childhood behavior. We conclude that the findings support a sexual-differentiation perspective involving prenatal androgens on the development of sexual orientation.
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            The development of sex role stereotypes in the third year: relationships to gender labeling, gender identity, sex-typed toy preference, and family characteristics.

            The onset and development of preschoolers' awareness of sex role stereotypes, gender labeling, gender identity, and sex-typed toy preference were explored in 26-, 31-, and 36-month-old children. Gender labeling, gender identity, sex-typed toy preferences, and awareness of adult sex role differences were observed in significantly more 26-month-old children than would have been expected by chance. Verbal gender labeling was observed in a majority of 26-month-olds, while verbal and nonverbal gender identity were observed in a majority of 31-month-olds. Nonverbal gender labeling and awareness of adult sex role differences were observed in a majority of children by 36 months. No evidence of awareness of sex differences in children's toys was found in the majority of children at any age. Awareness of sex role differences in children's toys was not related to awareness of adult sex role differences. Brighter children were more aware of adult sex role differences. Sex-typed toy preference was not related to awareness of sex role differences but was related to gender identity. Predictors of sex role development included the mothers' employment, and the father's personality traits, attitudes toward women, and sex-typed activities in the home. Implications for theories of early sex role development are discussed.
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              Early androgen exposure and human gender development

              During early development, testosterone plays an important role in sexual differentiation of the mammalian brain and has enduring influences on behavior. Testosterone exerts these influences at times when the testes are active, as evidenced by higher concentrations of testosterone in developing male than in developing female animals. This article critically reviews the available evidence regarding influences of testosterone on human gender-related development. In humans, testosterone is elevated in males from about weeks 8 to 24 of gestation and then again during early postnatal development. Individuals exposed to atypical concentrations of testosterone or other androgenic hormones prenatally, for example, because of genetic conditions or because their mothers were prescribed hormones during pregnancy, have been consistently found to show increased male-typical juvenile play behavior, alterations in sexual orientation and gender identity (the sense of self as male or female), and increased tendencies to engage in physically aggressive behavior. Studies of other behavioral outcomes following dramatic androgen abnormality prenatally are either too small in their numbers or too inconsistent in their results, to provide similarly conclusive evidence. Studies relating normal variability in testosterone prenatally to subsequent gender-related behavior have produced largely inconsistent results or have yet to be independently replicated. For studies of prenatal exposures in typically developing individuals, testosterone has been measured in single samples of maternal blood or amniotic fluid. These techniques may not be sufficiently powerful to consistently detect influences of testosterone on behavior, particularly in the relatively small samples that have generally been studied. The postnatal surge in testosterone in male infants, sometimes called mini-puberty, may provide a more accessible opportunity for measuring early androgen exposure during typical development. This approach has recently begun to be used, with some promising results relating testosterone during the first few months of postnatal life to later gender-typical play behavior. In replicating and extending these findings, it may be important to assess testosterone when it is maximal (months 1 to 2 postnatal) and to take advantage of the increased reliability afforded by repeated sampling.
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                Author and article information

                Journal
                J Neonatal Surg
                J Neonatal Surg
                J Neonat Surg
                Journal of Neonatal Surgery
                EL-MED-Pub
                2226-0439
                10 August 2017
                Jul-Sep 2017
                : 6
                : 3
                : 58
                Affiliations
                Department of Pediatric Surgery, Sri Ramasamy Memorial (SRM) Medical College SRM University, Chennai, India
                Author notes
                [* ] Correspondence: V. Raveenthiran, 200. Fifth Street Viduthalai Nagar Sunnambu Kolathur Chennai 600117, India E-mail: vrthiran@ 123456yahoo.co.in
                Article
                10.21699/jns.v6i3.604
                5593477
                5f4c4ec2-962e-400f-9543-5c5343e284d9
                Copyright: © 2017 Raveenthiran

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 23 April 2017
                : 11 June 2017
                Categories
                Athena's Pages

                disorders of sex development,intersex,ambiguous genitalia,congenital adrenal hyperplasia,androgen insensitivity syndrome,gonadal dysgenesis,gender dysphoria,sex reassignment

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