Article

Complete androgen insensitivity syndrome: Long-term medical, surgical, and psychosexual outcome

Department of Pediatrics, Johns Hopkins University, Baltimore, Maryland, United States
Journal of Clinical Endocrinology &amp Metabolism (Impact Factor: 6.31). 09/2000; 85(8):2664-9. DOI: 10.1210/jc.85.8.2664
Source: PubMed

ABSTRACT Controversy concerning the most appropriate treatment guidelines for intersex children currently exists. This is due to a lack of long-term information regarding medical, surgical, and psychosexual outcome in affected adults. We have assessed by questionnaire and medical examination the physical and psychosexual status of 14 women with documented complete androgen insensitivity syndrome (CAIS). We have also determined participant knowledge of CAIS as well as opinion of medical and surgical treatment. As a whole, secondary sexual development of these women was satisfactory, as judged by both participants and physicians. In general, most women were satisfied with their psychosexual development and sexual function. Factors reported to contribute to dissatisfaction were sexual abuse in one case and marked obesity in another. All of the women who participated were satisfied with having been raised as females, and none desired a gender reassignment. Although not perfect, the medical, surgical, and psychosexual outcomes for women with CAIS were satisfactory; however, specific ways for improving long-term treatment of this population were identified.

1 Follower
 · 
308 Views
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Die Suche nach den neurobiologischen Grundlagen sexueller und transsexueller Entwicklungen beschäftigt die Wissenschaft seit mehr als fünf Jahrzehnten. Das Wissen um die Sexualdifferenzierung neuronaler Strukturen konnte seitdem erheblich erweitert werden. Die vorliegende Arbeit stellt exemplarisch genetische, neuroendokrinologische, neurostrukturelle und neurofunktionelle Befunde vor, die in einem Zusammenhang mit transsexuellen Entwicklungen stehen können. In der Zusammenschau liefern die dargestellten Forschungsergebnisse Hinweise dafür, dass es neurobiologische Muster zu geben scheint, die einen Einfluss auf geschlechts­atypische Verhaltensweisen haben und in Interaktion mit psychologischen und sozialen Einflüssen die Wahrscheinlichkeit für eine transsexuelle Entwicklung erhöhen. Das Verständnis um die Bedingungen transsexueller Entwicklungen wird durch dieses zunehmende neurobiologische Wissen maßgeblich erweitert. Eine offene und multidisziplinäre Diskussion ist notwendig, um die neurobiologischen Befunde sinnvoll in die Theorie und Praxis transsexueller Entwicklungen zu integrieren.
    Zeitschrift für Sexualforschung 10/2011; 24(3):199-227. DOI:10.1055/s-0031-1283716 · 0.33 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: 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.
    02/2015; 6(1):3. DOI:10.1186/s13293-015-0022-1
  • [Show abstract] [Hide abstract]
    ABSTRACT: The sexual differentiation of the brain is primarily driven by gonadal hormones during fetal development. Leading theories on the etiology of gender dysphoria (GD) involve deviations herein. To examine whether there are signs of a sex-atypical brain development in GD, we quantified regional neural gray matter (GM) volumes in 55 female-to-male and 38 male-to-female adolescents, 44 boys and 52 girls without GD and applied both univariate and multivariate analyses. In girls, more GM volume was observed in the left superior medial frontal cortex, while boys had more volume in the bilateral superior posterior hemispheres of the cerebellum and the hypothalamus. Regarding the GD groups, at whole-brain level they differed only from individuals sharing their gender identity but not from their natal sex. Accordingly, using multivariate pattern recognition analyses, the GD groups could more accurately be automatically discriminated from individuals sharing their gender identity than those sharing their natal sex based on spatially distributed GM patterns. However, region of interest analyses indicated less GM volume in the right cerebellum and more volume in the medial frontal cortex in female-to-males in comparison to girls without GD, while male-to-females had less volume in the bilateral cerebellum and hypothalamus than natal boys. Deviations from the natal sex within sexually dimorphic structures were also observed in the untreated subsamples. Our findings thus indicate that GM distribution and regional volumes in GD adolescents are largely in accordance with their respective natal sex. However, there are subtle deviations from the natal sex in sexually dimorphic structures, which can represent signs of a partial sex-atypical differentiation of the brain. Copyright © 2015 Elsevier Ltd. All rights reserved.
    Psychoneuroendocrinology 01/2015; 55C:59-71. DOI:10.1016/j.psyneuen.2015.01.016 · 5.59 Impact Factor

Preview

Download
4 Downloads
Available from