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Intersex topics |
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Various criteria have been offered for the definition of intersex, including ambiguous genitalia, atypical genitalia, and differential sexual development. Ambiguous genitalia occurs in roughly 0.05% of all births, usually caused by masculinization or feminization during pregnancy, these conditions range from full androgen insensitivity syndrome to ovotesticular syndrome.
1.7% of people are born with a disorder of sexual development (DSD) as defined by the DSD consortium, such as those with Klinefelter's syndrome. The DSD was specifically made to be as inclusive to all atypical sexual development; not all conditions within the DSD affect individuals to the same extent.
Most intersex activism is based around the end of unnecessary medical interventions on intersex youth which attempt to assign an arbitrary sex and gender binary, often causing physical harm with no input from the child. Intersex conditions are usually expanded to include the DSD more generally. [1] 0.05% of births are medically treated or considered to have ambiguous genitalia. [2]
There can also be a stricter definition,[ clarification needed ] specifically for ambiguous DSD. This definition is restricted to those conditions in which typical chromosomal categorization patterns is inconsistent with phenotypic sex, or in which the phenotype is not easily classifiable as either male or female," with the prevalence of about 0.018%. [3]
The exact cut-off point between male and female in an intersex context is largely arbitrary. Likewise, the definition of biological sex is also sometimes considered to be arbitrary; as an example, some individuals with XY female (SRY inactivation) may have a uterus, ovaries, and normal menstruation, and be able to achieve pregnancy. [4] [5] These individuals would be declared to be biologically female but karyotypically male. Likewise, many intersex individuals are born completely sterile, although medical interventions have been known to remove potentially fertile gonads, which makes sex determination often arbitrary. Individuals with XX male develop male genitalia but are entirely infertile due to a lack of SRY gene expression and develop a generally feminine body. This range of possibilities is further expanded by conditions which effect genital development but not hormonal or sex gene expression. Generally, most intersex advocates, as well as parts of the medical community, advocate for broadening the definitions of sexual development and the definition of intersex. [6]
The overall causes of intersex conditions are complex, and are caused primarily by sexual development during pregnancy. Certain individuals may have a masculinized clitoris or a feminized penis, however this might change after pregnancy.[ clarification needed ] The exact differentiation of ovotestis of intersex people are often ambiguous. Other cases of intersex conditions can occur when hormones are taken during pregnancy such as estrogens or androgens, which can lead to atypical sexual development. Commonly intersex people are defined as those who are born with ambiguous genitalia, usually within the context of the OGR, or individuals with substantial atypical sexual development such as those with XX male. [7]
Most conditions under the DSD are not apparent at birth, and most are not medicalized. Certain definitions declare the need for atypical masculinization or feminization during fetal development to declare an intersex condition. [8] Under this definition, cloacal exstrophy a rare condition which is caused by the stomach internal organs developing incorrectly would not be intersex. Individuals with cloacal exstrophy who are born with XY do not develop a penis and are usually castrated and assigned female at birth. These people are medicalized like other people with intersex conditions and the OGR model. Due to this individuals with cloacal exstrophy are often considered intersex. [6] InterACT the leading organization of intersex rights in the US, states that 1.5% of children are born with an intersex condition (DSD), and 0.05% are born with full ambiguous genitalia. [2]
There is a high bias to assign intersex people with ambiguous genitalia as female at birth, as it was generally thought that it was easier to create a girl than a boy. Likewise as puberty would result in general feminization for most intersex children as well as a low libido, it was thought that they should be assigned female. This was also motivated by the fact that vaginoplasty was far more developed than phalloplasty. [9] [6]
This system was known as the optimum gender of rearing model (OGR model) which attempted to define a binary for intersex children. Some individuals who did not have any intersex conditions were raised under the OGR model, such as David Reimer who suffered a botched circumcision and was assigned female at seven months. The primary goal of the OGR was to stop gender incongruence, and to assign a gender binary for "proper" sex socialization. The model often specifically involved the falsification of medical history, such as the karyotype falsification or that internal testis were ovaries and needed to be removed for "cancer," despite no physical complications existing from their presence. [6]
Intersex advocates used a feminist perspective for criticism of the OGR as inherently sexist and cruel. The OGR modeled girls as passive and the receivers of penetration, and boys as the givers of penetration. As most intersex conditions cause vagina-like development and no phallus, medical staff were biased towards assigning female at birth. Transfeminists and queer liberationists particularly criticized the OGR model for not allowing children deviate from gender binary or expression. Likewise, feminists view bodily autonomy as a fundamental human right, which led to criticisms of the OGR taking away the bodily autonomy of intersex people. [6] [10]
The OGR stated that gender non-conformity was a physiological threat which affected an individual's ability to function in normal society. Most research has found this to be false and that the medical procedures practiced against intersex individuals generally leads to isolation, physiological stress and physical complications throughout life. [6] [11] [12] The definition of intersex is closely linked to the specific medical interventions on intersex people. [6] [10] [11] According to the ISNA 1.92% of the population will have some variation in sexual development throughout their lives, (0.42% excluding LOCAH). 1% of people have bodies that "differ from standard male or female," and 0.1-0.2% of births are considered for intersex genital surgeries. [13]
The DSD as a model was advocated for by intersex advocates to include all variation of atypical sexual development. Specifically the DSD exists as replacement for the OGR which was the standard model for individuals with atypical sexual development. This model stated goal was to assign a gender binary, usually female via non-consensual medicalization, often via the falsification of medical records. After the publication of individuals who had undergone the OGR model and had gone through serious physiological distress, the model was discredited. The term "disorders of sexual development" was chosen to reflect the variation of sexual development over differences which effects all individuals, this however has been controversial, with many instead opting for "differentiation" or "variation." [6]
The DSD has generally superseded the OGR in the US, although no official medical precautions exist against intersex genital mutilation in the US. Another point of contention is intersex conditions and karyotype, while many intersex individuals have atypical gene expression, many intersex individuals are born due to hormonal changes in pregnancy, either natural or induced. As an example, a case of a woman who had a virilized clitoris which was surgically altered during her birth, brought up the point that she was intersex, which was stated to be "false" by a doctor as her mother had gone on progesterone, instead of natural virilization which induced biological change. Generally those who have undergone the OGR model, or have ambiguous genitalia are considered intersex. The DSD consortium was specifically made to remedy this, and was advocated for by intersex activists by including all differentiation in sexual development. [6]
The definitions of intersex genitalia are difficult as different medical practices exist in different regions for what a "normal" penis or vagina should look like. [6]
Many intersex activists have advocated for a spectrum based approach for intersex conditions, which would differentiate various intersex conditions, including hormonal differences. Intersex conditions, even the same conditions such as ovotesticular syndrome, can vary wildly in terms of organs, genetic expression, phenotype, genotype, and karyotype. Under this model intersex conditions would be described via their own individualized effects described as effecting individuals on a spectrum. The DSD generally describes this by the description of individualized care for people with atypical sexual development, making the distinction between different conditions. [9] [14]
5α-Reductase 2 deficiency (5αR2D) is an autosomal recessive condition caused by a mutation in SRD5A2, a gene encoding the enzyme 5α-reductase type 2 (5αR2). The condition is rare, affects only genetic males, and has a broad spectrum.
Gender identity is the personal sense of one's own gender. Gender identity can correlate with a person's assigned sex or can differ from it. In most individuals, the various biological determinants of sex are congruent and consistent with the individual's gender identity. Gender expression typically reflects a person's gender identity, but this is not always the case. While a person may express behaviors, attitudes, and appearances consistent with a particular gender role, such expression may not necessarily reflect their gender identity. The term gender identity was coined by psychiatry professor Robert J. Stoller in 1964 and popularized by psychologist John Money.
XY complete gonadal dysgenesis, also known as Swyer syndrome, is a type of defect hypogonadism in a person whose karyotype is 46,XY. Though they typically have normal vulvas, the person has underdeveloped gonads, fibrous tissue termed "streak gonads", and if left untreated, will not experience puberty. The cause is a lack or inactivation of an SRY gene which is responsible for sexual differentiation. Pregnancy is sometimes possible in Swyer syndrome with assisted reproductive technology. The phenotype is usually similar to Turner syndrome (45,X0) due to a lack of X inactivation. The typical medical treatment is hormone replacement therapy. The syndrome was named after Gerald Swyer, an endocrinologist based in London.
Cloacal exstrophy (CE) is a severe birth defect wherein much of the abdominal organs are exposed. It often causes the splitting of the bladder, genitalia, and the anus. It is sometimes called OEIS complex.
Virilization or masculinization is the biological development of adult male characteristics in young males or females. Most of the changes of virilization are produced by androgens.
Sex assignment is the discernment of an infant's sex, typically made at birth based on an examination of the baby's external genitalia by a healthcare provider such as a midwife, nurse, or physician. In the vast majority of cases (99.95%), sex is assigned unambiguously at birth. However, in about 1 in 2000 births, the baby's genitalia may not clearly indicate male or female, necessitating additional diagnostic steps, and deferring sex assignment.
Intersex medical interventions (IMI), sometimes known as intersex genital mutilations (IGM), are surgical, hormonal and other medical interventions performed to modify atypical or ambiguous genitalia and other sex characteristics, primarily for the purposes of making a person's appearance more typical and to reduce the likelihood of future problems. The history of intersex surgery has been characterized by controversy due to reports that surgery can compromise sexual function and sensation, and create lifelong health issues. The medical interventions can be for a variety of reasons, due to the enormous variety of the disorders of sex development. Some disorders, such as salt-wasting disorder, can be life-threatening if left untreated.
The history of intersex surgery is intertwined with the development of the specialities of pediatric surgery, pediatric urology, and pediatric endocrinology, with our increasingly refined understanding of sexual differentiation, with the development of political advocacy groups united by a human qualified analysis, and in the last decade by doubts as to efficacy, and controversy over when and even whether some procedures should be performed.
XX male syndrome, also known as de la Chapelle syndrome, is a rare intersex condition in which an individual with a 46,XX karyotype develops a male phenotype. Synonyms for XX male syndrome include 46,XX testicular difference of sex development
Ovotesticular syndrome is a rare congenital condition where an individual is born with both ovarian and testicular tissue. It is one of the rarest DSDs, with only 500 reported cases. Commonly, one or both gonads is an ovotestis containing both types of tissue. Although it is similar in some ways to mixed gonadal dysgenesis, the conditions can be distinguished histologically.
Gonadal dysgenesis is classified as any congenital developmental disorder of the reproductive system characterized by a progressive loss of primordial germ cells on the developing gonads of an embryo. One type of gonadal dysgenesis is the development of functionless, fibrous tissue, termed streak gonads, instead of reproductive tissue. Streak gonads are a form of aplasia, resulting in hormonal failure that manifests as sexual infantism and infertility, with no initiation of puberty and secondary sex characteristics.
Partial androgen insensitivity syndrome (PAIS) is a condition that results in the partial inability of the cell to respond to androgens. It is an X linked recessive condition. The partial unresponsiveness of the cell to the presence of androgenic hormones impairs the masculinization of male genitalia in the developing fetus, as well as the development of male secondary sexual characteristics at puberty, but does not significantly impair female genital or sexual development. As such, the insensitivity to androgens is clinically significant only when it occurs in individuals with a Y chromosome. Clinical features include ambiguous genitalia at birth and primary amenhorrhoea with clitoromegaly with inguinal masses. Müllerian structures are not present in the individual.
Pseudohermaphroditism is an outdated term for when an individual's gonads were mismatched with their internal reproductive system and/or external genitalia. The term was contrasted with "true hermaphroditism", a condition describing an individual with both female and male reproductive gonadal tissues. Associated conditions includes Persistent Müllerian duct syndrome and forms of androgen insensitivity syndrome.
17β-Hydroxysteroid dehydrogenase III deficiency is a rare autosomal recessive disorder of sexual development condition that is a cause of 46,XY disorder of sex development. The impaired testosterone biosynthesis by 17β-hydroxysteroid dehydrogenase III, presents as atypical genitalia in affected males.
Disorders of sex development (DSDs), also known as differences in sex development or variations in sex characteristics (VSC), are congenital conditions affecting the reproductive system, in which development of chromosomal, gonadal, or anatomical sex is atypical. DSDs is a clinical term used in some medical settings for what are otherwise referred to as intersex traits. The term was first introduced in 2006 and has not been without controversy.
Intersex people are individuals born with any of several sex characteristics, including chromosome patterns, gonads, or genitals that, according to the Office of the United Nations High Commissioner for Human Rights, "do not fit typical binary notions of male or female bodies".
Intersex, in humans and other animals, describes variations in sex characteristics including chromosomes, gonads, sex hormones, or genitals that, according to the UN Office of the High Commissioner for Human Rights, "do not fit typical binary notions of male or female bodies". Intersex people were historically termed hermaphrodites, "congenital eunuchs", or even congenitally "frigid". Such terms have fallen out of favor, now considered to be misleading and stigmatizing.
Intersex people in the United States have some of the same rights as other people, but with significant gaps, particularly in protection from non-consensual cosmetic medical interventions and violence, and protection from discrimination. Actions by intersex civil society organizations aim to eliminate harmful practices, promote social acceptance, and equality. In recent years, intersex activists have also secured some forms of legal recognition. Since April 11, 2022 US Passports give the sex/gender options of male, female and X by self determination.
Citizens of Spain who are intersex face problems that the wider society does not encounter. Laws that provide protection against discrimination or genital mutilation for intersex people exist only in some autonomous communities rather than on a national level. The 3/2007 law is the current law in Spain which relates to legal gender change including the rights of intersex people, although a new law is about to be passed in the near future.
Sexual anomalies, also known as sexual abnormalities, are a set of clinical conditions due to chromosomal, gonadal and/or genitalia variation. Individuals with congenital (inborn) discrepancy between sex chromosome, gonadal, and their internal and external genitalia are categorised as individuals with a disorder of sex development (DSD). Afterwards, if the family or individual wishes, they can partake in different management and treatment options for their conditions.