Gender identity and recalled gender related childhood play-behaviour in adult individuals with different forms of intersexuality

The concept of intersexuality subsumes a wide variety of phenomena with very specific underlying causes. In all these cases, an untypical development takes place during the prenatal sex differentiation process becoming clinically manifest, either at, or soon after birth or at the time of puberty. It subsumes conditions in which biological sexual characteristics (e.g. chromosomal sex, gonadal sex, hormonal sex, morphological sex) differ from each other and one person cannot easily be assigned to one sex. One of the main goals of medical treatment of persons with intersex-syndroms is the development of a stable gender identity. Over the last few years, sex (and gender) assignment of persons with different forms of intersexuality has become a much discussed topic. An interesting--and very obviously observable--variable that was brought in connection with sex assignment is gender related childhood play behaviour. The purpose of the presented study is to examine 37 persons with different forms of intersexuality (disturbances of androgen biosynthesis, partial and complete androgen insensitivity, gonadal dysgenesis with 46,XY and congenital adrenal hyperplasia with 46,XX) with regard to gender identity and gender role behaviour in childhood. Not all subjects in the study group had developed a clear female or male gender identity. In contrast to previous studies, some persons with CAIS did not recall distinguished female childhood play behaviour and these persons did not show a clear female gender identity. In contrast to results from other studies, the CAH-affected girls in this study did not seem to recall masculinized behaviour. Further research is needed to guarantee better psychosexual development with good quality of life in individuals with intersexuality.     

The basis of gender assignment in disorders of somatosexual differentiation

Sex assignment of patients with disorders of somatosexual differentiation is a controversial topic. The aim is to enable the patient to develop a stable gender identity during childhood, adolescence and adulthood. Enormous advances have recently been made in our knowledge of the molecular mechanisms of sexual differentiation and it is understood that long-term outcome may depend on the underlying diagnosis. There is increasing evidence that genital development is dependent on the action of androgenic steroids; moreover, both androgens and oestrogens may have an impact on other developing organs including neuronal structures such as the brain. Long-term outcome studies on the various intersexuality disorders are desperately needed in order to establish a basis for evidence-based medicine regarding sex assignment and treatment options. Premature decisions leading to irreversible interventions before an accurate diagnosis has been established must be avoided.     

Inheritance of intersex disorders.

Intersex disorders result from abnormalities of the sex chromosomes, gonads, internal and external genitalia, sex hormones and gender role. This article reviews the literature on intersex disorders, outlining the characteristics and mode of inheritance, if known, of each. For appropriate and effective management and counselling of patients and their families, physicians must have a good knowledge of the development of the genital tract and of the interaction between genetic sex and environmental influences.     

Psychological long-term outcome in intersex conditions

For decades, sex assignment in children with intersex conditions has depended more on surgical possibilities than on other criteria, since it was assumed that children are psychosexually neutral at birth. Adults with intersex conditions and professionals in the field have increasingly criticized this policy after the publication of studies suggesting that prenatal brain exposure to sex hormones determines gender development. Although prenatal brain exposure to androgens plays some part in the development of gender role behaviour, the current evidence is not in line with the idea of determination of gender identity through prenatal sex steroid exposure. Recent reviews on gender dysphoria and gender change in patients with intersex conditions show that initial gender assignment still seems to be the best predictor of adult gender identity.     

Systematization of ambiguous genitalia

Sex assignment in newborns depends on the anatomy of the external genitalia, despite this stage being the final in embryogenesis. According to the current view, the genital tubercle is the embryonic precursor of penis and clitoris. It originates from mesenchymal tissue, but mesenchymal cells are arranged across the embryonal body and do not have specific androgen receptors. The nature of the signal that initiates early derivation of the indifferent genital tubercle is unknown at present. The aims of this article are to improve surgical management of intersex disorders and investigate the development of the genital tubercle. Clinical examination of 114 females with various forms of DSD revealed ambiguous (bisexual) external genitalia in 73 patients, and 51 of them underwent feminizing surgery. Intersexuality (ambiguity) in 46,XY patients results from disruptors in the pathways of sex steroid hormones or receptors; in 46,XX females arises from excessive levels of androgens. Systematization of intersex disorders distinguishes the karyotype, gonadal morphology, and genital anatomy to provide a differential diagnosis and guide appropriate surgical management. Modified feminizing clitoroplasty with preservation of the dorsal and ventral neurovascular bundles to retain erogenous sensitivity was performed in females with severe virilization (Prader degree III-V). The outgrowth of the genital tubercle and the fusion of the urethral fold proceed in an ordered fashion; but in some cases of ambiguity, there was discordance due to different pathways. Speculation about the derivation of the genital tubercle have discussed with a literature review. The genital tubercle derives from the following 3 layers: the ectodermal glans of the tubercle, the mesodermal corpora cavernosa and the endodermal urogenital groove. According to the new hypothesis, during the indifferent stages, the 5 sacral somites have to recede from their segmentation and disintegrate: the sclerotomes form the pelvic bones, the fused myotomes follow with their genuine neurotomes and the angiotomes join to the corpora cavernosa of the genital tubercle. Sexual differentiation of external genitalia is final in gender embryogenesis, but surprisingly derivation of the indifferent genital tubercle from 5 somites occurs before gonadal and internal organs development.     

‘Aching to be a boy’: A preliminary analysis of gender assignment of intersex persons in India in a culture of son preference

Intersexuality, particularly in the global South, remains an under-researched field of study. In my in-progress doctoral research project, I explore the cultural, social, and medical discourses that influence how key stakeholders such as healthcare providers make decisions about the sex and gender assignment of the intersex child in India. In this paper I interrogate some of these ideas around gender assignment of intersex people in India, paying particular attention to the context of son preference. I am interested in exploring how decisions of gender assignment by medical professionals are guided by ideas of son preference. Focusing on four qualitative, semi-structured, in-depth interviews across two cities with medical doctors from different specializations, this paper is a preliminary attempt to examine some of the factors that guide medical professionals in making decisions about gender assignment of intersex children and explore the dynamics of the decision-making process. Specifically, I explore the factors that inform doctors’ decision-making and locate these decision-making processes within the broader socio-cultural context of India.     

The “Normalization” of Intersex Bodies and “Othering” of Intersex Identities in Australia

Once described as hermaphrodites and later as intersex people, individuals born with intersex variations are routinely subject to so-called “normalizing” medical interventions, often in childhood. Opposition to such practices has been met by attempts to discredit critics and reasserted clinical authority over the bodies of women and men with “disorders of sex development.” However, claims of clinical consensus have been selectively constructed and applied and lack evidence. Limited transparency and lack of access to justice have helped to perpetuate forced interventions. At the same time, associated with the diffusion of distinct concepts of sex and gender, intersex has been constructed as a third legal sex classification, accompanied by pious hopes and unwarranted expectations of consequences. The existence of intersex has also been instrumentalized for the benefit of other, intersecting, populations. The creation of gender categories associated with intersex bodies has created profound risks: a paradoxically narrowed and normative gender binary, maintenance of medical authority over the bodies of “disordered” females and males, and claims that transgressions of social roles ascribed to a third gender are deceptive. Claims that medicalization saves intersex people from “othering,” or that legal othering saves intersex people from medicalization, are contradictory and empty rhetoric. In practice, intersex bodies remain “normalized” or eliminated by medicine, while society and the law “others” intersex identities. That is, medicine constructs intersex bodies as either female or male, while law and society construct intersex identities as neither female nor male. Australian attempts at reforms to recognize the rights of intersex people have either failed to adequately comprehend the population affected or lacked implementation. An emerging human rights consensus demands an end to social prejudice, stigma, and forced medical interventions, focusing on the right to bodily integrity and principles of self-determination.     

True hermaphroditism in a wild sheep: A clinical report

Intersexuality in sheep is rare, with the freemartin anomaly being the most common. We describe here a true hermaphrodite in a wild sheep. An F(1) wild sheep ewe of Argali-mouflon X Mexican desert bighorn breeding was bred to an F(1) ram of the same breeding. A single lamb was born with the external appearance of a normal female. The lamb grew faster than its female cohorts, and by 6 months of age exhibited the aggressive behavior, size, coloration and horn development associated with males. Phenotypically, the intersex had female external genitalia with an enlarged clitoris. A human chorionic gonadotrophin (hCG) response test was performed when the intersex was 1-year-old and serum testosterone, progesterone and estradiol levels were compared to the response of a normal female and male of similar age and breeding. An exploratory celiotomy revealed two gonadal-like structures associated with a female reproductive tract. Histopathology of the structures revealed spermatogenically inactive testicular vessels and ovarian tissue with primary follicles. The reproductive tract was complete with two uterine horns and a cervix. The intersexuality is attributed to an XX/XXY mosaic.     

Genetic analysis of 38XX males with genital ambiguities and true hermaphrodites in pigs

In pig, the frequency of intersexuality ranges from 0.1 to O.6%, depending on the breed. In a closed pig herd at INRA an intersex condition was observed in 0.75% of ‘females’. The present study describes 11 animals with a 38XX karyotype and the presence of testicular tissue. Phenotypically, all presented with abnormal external or/and internal genitalia. Southern blot analysis with Y-specific probes (SRY and ZFY) revealed the absence of Y material in all animals tested. By polymerase chain reaction (PCR) amplification, 10 of 11 intersex pigs lacked the SRY gene in gonad DNA. These data are compatible with an autosomally (or pseudoautosomally) determined mechanism. Moreover, analysis of familial cases seemed to indicate that 38XX male pseudohermaprodites and 38XX true hermaphrodites may represent alternative manifestations of the same genetic defect.     

Persistently high levels of intersexuality in male-biased amphipod populations

In a study conducted over 30 years ago, a remarkable population of amphipod crustaceans (Gammarus minus) was discovered in which 100% of the females displayed intersex characteristics. Such high levels of intersexuality are atypical amongst gonochoristic crustaceans and current theories relating to sex allocation suggest intermediate (intersex) forms should be competitively selected against. This study set out to confirm the existence of this extraordinary population and to compare it with other G. minus populations in various spring and cave systems across Pennsylvania, Virginia and West Virginia (USA), with varying environmental characteristics in an attempt to elucidate the prevalence and cause of intersexuality in this species. Results from this study verify the continued existence of these highly abundant, intersex female populations of G. minus, with proportions ranging between 73% and 100%. These populations were also associated with highly male-biased sex ratios, which is equally unusual amongst gonochoristic crustaceans. Intersex female specimens had a reduced fecundity compared to normal females, although this result was not statistically significant. Although no feminising microsporidian parasites were observed through histological examination, the presence of a novel feminising parasitic factor cannot be ruled out. The continued vigour of these populations over three decades suggests that anthropomorphic factors are not causing the high levels of intersexuality observed. Intersexuality was absent or very low in frequency (<1%) in most other springs that we investigated. Therefore, G. minus appears to represent an interesting and novel model system for studying sex determination.     

Mind the Gaps: Intersex and (Re-productive) Spaces in Disability Studies and Bioethics

With a few notable exceptions disability studies has not taken account of intersexuality, and it is principally through the lenses of feminist and queer-theory oriented ethical discussions but not through ‘straight’ bioethics that modes valuing intersex difference have been proposed. Meanwhile, the medical presupposition that intersex characteristics are inherently disabling to social viability remains the taken-for-granted truth from which clinical practice proceeds. In this paper I argue against bioethical perspectives that justify extensive and invasive pre- and post-natal medical interference to eradicate intersex. I argue instead that to constitute the necessary conditions for the recognition of the intersexed child as a person, a life valid in its own right, clinicians must refrain from aggressive interference. Clinical specialists presuppose that intersexed children will be socially disabled and unrecognizable as persons; frustrated by the general failure of traditional interventions to assign a sex, clinicians are now pursuing prenatal technologies, including selective termination, to erase intersex.     

Congenital micropenis: long-term medical, surgical and psychosexual follow-up of individuals raised male or female

OBJECTIVES: To document long-term medical, surgical and psychosexual outcome of individuals with congenital micropenis (13 males, 5 females). METHODS: Physical measurements from childhood were collected retrospectively from medical records and at adulthood by physical examination. An adult psychosexual assessment was conducted with a written questionnaire and oral discussion. RESULTS: Adult penile length was below the normal mean in all men. Three women had vaginoplasty resulting in normal length. All men reported good or fair erections but 50% were dissatisfied with their genitalia. Dissatisfaction with body image resulted from having a small penis (66%), inadequate body hair (50%), gynecomastia (33%) and youthful appearance (33%). Ten men were heterosexual, 1 homosexual and 2 bisexual. Among women, 4 (80%) were dissatisfied with their genitalia. Three women reported average libido with orgasm and were also heterosexual. Two women had no sexual interest or experience. Finally, males were masculine and females feminine in their gender-role identity, and both groups were satisfied with their sex of rearing. CONCLUSIONS: Regarding choice of gender, male sex of rearing can result in satisfactory genito-sexual function. Female gender can also result in success, however it requires extensive feminizing surgery.     

Does surgical genitoplasty affect gender identity in the intersex infant?

There is no clear-cut answer to the question of whether surgical genitoplasty affects gender identity in the intersex infant. The debate centres around which is more important for the development of gender identity: the biological sex of a child or the sex in which a child is reared. We believe that the surgical achievement of a phenotype concordant with the sex of rearing is a tremendous help to the parents of an intersex infant. We do not consider that the 'neutral' upbringing of a child with ambiguous genitalia is a feasible option, first because of the parents' distress which prevents them from raising their child normally and second because in most cultures around the world gender variants are not treated as equals. A neutral upbringing may induce psychosocial consequences that are more pernicious than carefully considered neonatal sex attribution and concordant surgical genitoplasty.     

Is intersexuality a cost of environmental sex determination in Gammarus duebeni?

Three possible causes of intersexuality in Gammarus duebeni , a crustacean with environmental sex determination, were investigated. Intersexuality appears to be a cost of the flexible sex-determining mechanism of this species: the occurrence of intersexes is influenced by photoperiod, which also cues sex determination. Intersexuality is heritable: intersex mothers produce more intersex offspring than do true females. Parasitism by a feminizing microsporidian is ruled out as a significant cause of intersexuality.     

Divergence or disorder?: the politics of naming intersex

The conditions once known under the umbrella terms intersex and hermaphroditism are now generally being called disorders of sex development in medical settings. The terms might seem synonymous, but in fact there are significant differences with controversial consequences. Hermaphroditism, an older term that can still be found in many medical writings, is vague, demeaning, and sensationalistic, conjuring mythic images of monsters and freaks. In the 1990s, activists advocated intersex to describe discordance between the multiple components of sex anatomy, but that word alienated many parents of affected children, as it suggests a self-conscious alternative gender identity and sexuality. Disorders of sex development also refers to intersex, but it deemphasizes the identity politics and sexual connotations associated with intersex, avoids the degradation associated with hermaphrodite, and instead highlights the underlying genetic or endocrine factors that cause prenatal sex development to take an unusual path. I argue that using disorder is problematic, because it implies medical conditions in need of repair, when some intersex anatomies, though atypical, do not necessarily need surgical or hormonal correction. I advocate a less pathologizing new term, divergence of sex development, that might reduce some of the conflict over nomenclature and satisfy intersex people, their parents, and their doctors.     

<Emphasis Type="Bold">Intersex Activists in Israel: Their Achievements and the Obstacles They Face</Emphasis>

This article focuses on the dynamic between the medical policy on intersex bodies and intersex activists in Israel. Recently, in many countries changes have taken place in medical guidelines regarding intersex patients and laws that regulate medical practices and prohibit irreversible surgeries for intersex babies for cosmetic reasons and without the patient’s consent. In Israel, intersex activists are limited by several factors. On the one hand, they are influenced by the achievements of intersex activism around the world but on the other, the pathologizing medical discourse and socio-medical practices, which include early diagnosis, early irreversible surgeries, and secrecy surrounding intersexed bodies, present obstacles to achieving bodily autonomy for intersex individuals and social recognition of different sex development. Nevertheless, intersex activists are attempting to find different social and media spaces in which to achieve public acknowledgement and future bodily autonomy for intersexed people and seeking medical professionals’ cooperation. Recently, the Israeli Ministry of Health published a new circular for intersex/DSD patients, and while it does not clearly forbid irreversible surgeries, it provides information about the complexities of intersex people and their experience.     

Intersexuality in the amphipod Gammarus duebeni results from incomplete feminisation by the vertically transmitted parasitic sex ratio distorter Nosema granulosis

We investigated the effects of parasitism and temperature on the production of intersexes in the amphipod Gammarus duebeni from a field population at Cumbrae, Scotland. There was significant temporal variation in intersex frequency which ranged from 0.5 to 5.2% in monthly field collections. Prevalence of Nosema granulosis, a feminising microsporidian parasite, also varied temporally and there was a significant correlation between parasite prevalence and intersex frequency in the field. Intersexes (16.3 ± 0.4 mg) were larger than true females (14.8 ± 0.1 mg) but produced fewer eggs thus demonstrating a cost of intersexuality. Intersexes were less likely to be paired than true females. In a breeding experiment, only females infected by N. granulosis produced intersex offspring. Temperature had no effect on intersex frequency. Intersexes therefore appear to be the result of incomplete feminisation by N. granulosis in this population. These results contrast with previous studies of G. duebeni from different populations which found that intersexes were the result of abnormal development under environmental sex determination. We suggest that intersexuality may be induced by both environmental and parasitic factors in populations of G. duebeni. This revised version was published online in August 2006 with corrections to the Cover Date.     

Sociocultural aspects of disorders of sex development

Disorders of sex development (DSD) is a congenital condition in which the development of chromosomes, gonads, hormones, and reproductive structures are atypical. DSD brings with it a psychological impact on the affected individual and their families. The consensus statement on management of DSD strongly advised an integrated and multidisciplinary approach in providing care to the affected individuals. Studies have been conducted focusing on medical intervention, and more recently, there is increasing attention paid to psychological aspects of DSD. However, studies reporting cultural aspects of DSD are lacking. This review provides an overview on how culture impacts the affected individuals in coping with DSD and making decisions with regard to gender assignment or reassignment, help‐seeking behavior for medical treatments, attitudes toward medical treatment, religious beliefs, and values concerning marriage and fertility. The involvement of social scientists is needed to study sociocultural aspects of DSD from more diverse cultures, to help affected individuals and their families in gaining better social acceptance. Birth Defects Research (Part C) 108:380–383, 2016. © 2016 Wiley Periodicals, Inc.