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Intersex

Updated: Wikipedia source

Intersex

Intersex people are those 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". The opposite of intersex is endosex, which describes persons born with sex characteristics that are seen as typically male or female at birth. Sex assignment at birth usually aligns with a child's external genitalia. The number of births with ambiguous genitals is in the range of 1:4,500–1:2,000 (0.02%–0.05%). Other conditions involve the development of atypical chromosomes, gonads, or hormones. The portion of the population that is intersex has been reported differently depending on which definition of intersex is used and which conditions are included. Estimates often range from 0.018% (one in 5,500 births) to 1.7%. The difference centers on whether conditions in which chromosomal sex matches a phenotypic sex which is clearly identifiable as male or female, such as late onset congenital adrenal hyperplasia (1.5 percentage points) and Klinefelter syndrome, should be counted as intersex. Whether intersex or not, people may be assigned and raised as a girl or boy but then identify with another gender later in life, while most continue to identify with their assigned sex. Some estimates may be significantly higher than the 1.7% estimate, particularly when factoring in polycystic ovary syndrome (PCOS) statistics, which range from 4-20% in their own right. There is debate over whether or not PCOS is an intersex variation, and it is not officially medically recognized as one. However, proponents of its inclusion note that the medical establishment is committed to minimizing the prevalence of intersex people, both linguistically and physically, so this lack of medical recognition may not necessarily rule out the possibility of PCOS being an intersex variation. The hormonal differences associated with PCOS, as well as the noticeable differences in secondary sex characteristics that occur in some individuals with hyperandrogenic PCOS, are two major reasons that some include it under the intersex umbrella. Opponents are often uncomfortable with the idea of associating PCOS with intersexuality due to the stigma and marginalization that intersex communities experience. There are also many who feel that the inclusion of PCOS as an intersex variation would be a denial of womanhood towards women with PCOS. This stance is criticized for its lack of acknowledgment of transgender men and non-binary people with PCOS, as well as its implication that intersex women are not women. A major intersex organization, interACT, includes PCOS in their list of intersex variations, and takes the stance that people with PCOS can be considered intersex. They also clarify, however, that they are not an authority on what can "count" as intersex. Despite their stance on PCOS inclusion, interACT generally sticks to the 1.7% estimate when stating the prevalence of intersexuality. Terms used to describe intersex people are contested, and change over time and place. Intersex people were previously referred to as "hermaphrodites" or "congenital eunuchs". In the 19th and 20th centuries, some medical experts devised new nomenclature in an attempt to classify the characteristics that they had observed, the first attempt to create a taxonomic classification system of intersex conditions. Intersex people were categorized as either having "true hermaphroditism", "female pseudohermaphroditism", or "male pseudohermaphroditism". These terms are no longer used, and terms including the word "hermaphrodite" are considered to be misleading, stigmatizing, and scientifically specious in reference to humans. In biology, the term "hermaphrodite" is used to describe an organism that can produce both male and female gametes. Some people with intersex traits use the term "intersex", and some prefer other language. In clinical settings, the term "disorders of sex development" (DSD) has been used since 2006, a shift in language considered controversial since its introduction.

Intersex people face stigmatization and discrimination from birth, or following the discovery of intersex traits at stages of development such as puberty. Intersex people may face infanticide, abandonment, and stigmatization from their families. Globally, some intersex infants and children, such as those with ambiguous outer genitalia, are surgically or hormonally altered to create more socially acceptable sex characteristics. This is considered controversial, with no firm evidence of favorable outcomes. Such treatments may involve sterilization. Adults, including elite female athletes, have also been subjects of such treatment. Increasingly, these issues are considered human rights abuses, with statements from international and national human rights and ethics institutions. Intersex organizations have also issued statements about human rights violations, including the 2013 Malta declaration of the third International Intersex Forum. In 2011, Christiane Völling became the first intersex person known to have successfully sued for damages in a case brought for non-consensual surgical intervention. In April 2015, Malta became the first country to outlaw non-consensual medical interventions to modify sex anatomy, including that of intersex people.

Tables

· Prevalence
Late onset congenital adrenal hyperplasia (nonclassical forms)
Late onset congenital adrenal hyperplasia (nonclassical forms)
Condition
Late onset congenital adrenal hyperplasia (nonclassical forms)
Sex specificity
Female (males are generally asymptomatic)
Approximate prevalence
One in 50–1,000 births (0.1–0.2% up to 1–2% depending on population)
Hypospadias
Hypospadias
Condition
Hypospadias
Sex specificity
Male
Approximate prevalence
One in 200–10,000 male births (0.01%–0.5%), prevalence estimates vary considerably
Klinefelter syndrome (47, XXY)
Klinefelter syndrome (47, XXY)
Condition
Klinefelter syndrome (47, XXY)
Sex specificity
Male
Approximate prevalence
One in 500–1,000 male births (0.1–0.2%)
Trisomy X or triple X syndrome (47, XXX)
Trisomy X or triple X syndrome (47, XXX)
Condition
Trisomy X or triple X syndrome (47, XXX)
Sex specificity
Female
Approximate prevalence
One in 1,000 female births (0.10%)
Turner syndrome (45, X)
Turner syndrome (45, X)
Condition
Turner syndrome (45, X)
Sex specificity
Female
Approximate prevalence
One in 2,500 female births (0.04%)
Müllerian agenesis (of vagina, i.e., MRKH Syndrome)
Müllerian agenesis (of vagina, i.e., MRKH Syndrome)
Condition
Müllerian agenesis (of vagina, i.e., MRKH Syndrome)
Sex specificity
Female
Approximate prevalence
One in 4,500 female births (0.022%)
Vaginal atresia
Vaginal atresia
Condition
Vaginal atresia
Sex specificity
Female
Approximate prevalence
One in 5,000 female births (0.02%)
45,X/46,XY mosaicism
45,X/46,XY mosaicism
Condition
45,X/46,XY mosaicism
Sex specificity
N/A, but usually male
Approximate prevalence
One in 6,666 births (0.015%)
47, XYY syndrome
47, XYY syndrome
Condition
47, XYY syndrome
Sex specificity
Male
Approximate prevalence
One in 7,000 male births (0.0142%)
Congenital adrenal hyperplasia (classical forms)
Congenital adrenal hyperplasia (classical forms)
Condition
Congenital adrenal hyperplasia (classical forms)
Sex specificity
N/A (but virilization of female infants)
Approximate prevalence
One in 10,000–20,000 births (0.01–0.02%)
48, XXXY syndrome
48, XXXY syndrome
Condition
48, XXXY syndrome
Sex specificity
Male
Approximate prevalence
One in 50,000 male births (0.002%)
49, XXXXY syndrome
49, XXXXY syndrome
Condition
49, XXXXY syndrome
Sex specificity
Male
Approximate prevalence
One in 85,000-100,000 male births (0.001%)
48, XXYY syndrome
48, XXYY syndrome
Condition
48, XXYY syndrome
Sex specificity
Male
Approximate prevalence
One in 18,000–40,000 male births (0.0025%–0.0055%)
49, XXXYY syndrome
49, XXXYY syndrome
Condition
49, XXXYY syndrome
Sex specificity
Male
Approximate prevalence
Less than one in 1000000 births
XX male or de la Chapelle syndrome
XX male or de la Chapelle syndrome
Condition
XX male or de la Chapelle syndrome
Sex specificity
Male
Approximate prevalence
One in 20,000 male births (0.005%)
Ovotesticular syndrome
Ovotesticular syndrome
Condition
Ovotesticular syndrome
Sex specificity
N/A
Approximate prevalence
One in 20,000 births (0.005%)
XY gonadal dysgenesis
XY gonadal dysgenesis
Condition
XY gonadal dysgenesis
Sex specificity
Phenotypic female
Approximate prevalence
One in 80,000 births (0.0013%)
Androgen insensitivity syndrome (complete and partial phenotypes)
Androgen insensitivity syndrome (complete and partial phenotypes)
Condition
Androgen insensitivity syndrome (complete and partial phenotypes)
Sex specificity
Genetic male
Approximate prevalence
One in 22,000-64,000 male births (0.0045-0.001%)
Androgen deficiency
Androgen deficiency
Condition
Androgen deficiency
Sex specificity
N/A
Approximate prevalence
No estimate
Idiopathic intersexuality (no discernable medical cause)
Idiopathic intersexuality (no discernable medical cause)
Condition
Idiopathic intersexuality (no discernable medical cause)
Sex specificity
N/A
Approximate prevalence
One in 110,000 births (0.0009%)
Iatrogenic intersexuality (caused by medical treatment, e.g., progestogen administered to pregnant mother)
Iatrogenic intersexuality (caused by medical treatment, e.g., progestogen administered to pregnant mother)
Condition
Iatrogenic intersexuality (caused by medical treatment, e.g., progestogen administered to pregnant mother)
Sex specificity
N/A
Approximate prevalence
No estimate
5-alpha-reductase deficiency
5-alpha-reductase deficiency
Condition
5-alpha-reductase deficiency
Sex specificity
Male
Approximate prevalence
No estimate
Aromatase excess syndrome
Aromatase excess syndrome
Condition
Aromatase excess syndrome
Sex specificity
N/A
Approximate prevalence
No estimate
Aromatase deficiency
Aromatase deficiency
Condition
Aromatase deficiency
Sex specificity
N/A
Approximate prevalence
No estimate
Anorchia
Anorchia
Condition
Anorchia
Sex specificity
Male
Approximate prevalence
One in 20,000 male births (0.005%)
Persistent Müllerian duct syndrome
Persistent Müllerian duct syndrome
Condition
Persistent Müllerian duct syndrome
Sex specificity
Male
Approximate prevalence
No estimate
46,XX/46,XY
46,XX/46,XY
Condition
46,XX/46,XY
Sex specificity
N/A
Approximate prevalence
No estimate
Leydig cell hypoplasia
Leydig cell hypoplasia
Condition
Leydig cell hypoplasia
Sex specificity
Male
Approximate prevalence
One in 1,000,000 male births (0.000001%)
Gonadotropin-releasing hormone insensitivity
Gonadotropin-releasing hormone insensitivity
Condition
Gonadotropin-releasing hormone insensitivity
Sex specificity
N/A
Approximate prevalence
No estimate
Familial male-limited precocious puberty
Familial male-limited precocious puberty
Condition
Familial male-limited precocious puberty
Sex specificity
Male
Approximate prevalence
No estimate
Cytochrome P450 oxidoreductase deficiency
Cytochrome P450 oxidoreductase deficiency
Condition
Cytochrome P450 oxidoreductase deficiency
Sex specificity
N/A
Approximate prevalence
No estimate
Isolated 17,20-lyase deficiency
Isolated 17,20-lyase deficiency
Condition
Isolated 17,20-lyase deficiency
Sex specificity
N/A
Approximate prevalence
No estimate
Testicular dysgenesis syndrome
Testicular dysgenesis syndrome
Condition
Testicular dysgenesis syndrome
Sex specificity
Male
Approximate prevalence
No estimate
Penoscrotal transposition
Penoscrotal transposition
Condition
Penoscrotal transposition
Sex specificity
Male
Approximate prevalence
No estimate
Hyperandrogenism
Hyperandrogenism
Condition
Hyperandrogenism
Sex specificity
N/A
Approximate prevalence
No estimate
Hyperestrogenism
Hyperestrogenism
Condition
Hyperestrogenism
Sex specificity
N/A
Approximate prevalence
No estimate
Polyorchidism
Polyorchidism
Condition
Polyorchidism
Sex specificity
Male
Approximate prevalence
No estimate
Aphallia
Aphallia
Condition
Aphallia
Sex specificity
Male
Approximate prevalence
No estimate
Cryptorchidism
Cryptorchidism
Condition
Cryptorchidism
Sex specificity
Male
Approximate prevalence
One in 33–100 male births (3–1%)
Cloacal exstrophy (born with XY chromosomes)
Cloacal exstrophy (born with XY chromosomes)
Condition
Cloacal exstrophy (born with XY chromosomes)
Sex specificity
Male
Approximate prevalence
One in 400,000 live births (0.0025%)
Condition
Sex specificity
Approximate prevalence
Late onset congenital adrenal hyperplasia (nonclassical forms)
Female (males are generally asymptomatic)
One in 50–1,000 births (0.1–0.2% up to 1–2% depending on population)
Hypospadias
Male
One in 200–10,000 male births (0.01%–0.5%), prevalence estimates vary considerably
Klinefelter syndrome (47, XXY)
Male
One in 500–1,000 male births (0.1–0.2%)
Trisomy X or triple X syndrome (47, XXX)
Female
One in 1,000 female births (0.10%)
Turner syndrome (45, X)
Female
One in 2,500 female births (0.04%)
Müllerian agenesis (of vagina, i.e., MRKH Syndrome)
Female
One in 4,500 female births (0.022%)
Vaginal atresia
Female
One in 5,000 female births (0.02%)
45,X/46,XY mosaicism
N/A, but usually male
One in 6,666 births (0.015%)
47, XYY syndrome
Male
One in 7,000 male births (0.0142%)
Congenital adrenal hyperplasia (classical forms)
N/A (but virilization of female infants)
One in 10,000–20,000 births (0.01–0.02%)
48, XXXY syndrome
Male
One in 50,000 male births (0.002%)
49, XXXXY syndrome
Male
One in 85,000-100,000 male births (0.001%)
48, XXYY syndrome
Male
One in 18,000–40,000 male births (0.0025%–0.0055%)
49, XXXYY syndrome
Male
Less than one in 1000000 births
XX male or de la Chapelle syndrome
Male
One in 20,000 male births (0.005%)
Ovotesticular syndrome
N/A
One in 20,000 births (0.005%)
XY gonadal dysgenesis
Phenotypic female
One in 80,000 births (0.0013%)
Androgen insensitivity syndrome (complete and partial phenotypes)
Genetic male
One in 22,000-64,000 male births (0.0045-0.001%)
Androgen deficiency
N/A
No estimate
Idiopathic intersexuality (no discernable medical cause)
N/A
One in 110,000 births (0.0009%)
Iatrogenic intersexuality (caused by medical treatment, e.g., progestogen administered to pregnant mother)
N/A
No estimate
5-alpha-reductase deficiency
Male
No estimate
Aromatase excess syndrome
N/A
No estimate
Aromatase deficiency
N/A
No estimate
Anorchia
Male
One in 20,000 male births (0.005%)
Persistent Müllerian duct syndrome
Male
No estimate
46,XX/46,XY
N/A
No estimate
Leydig cell hypoplasia
Male
One in 1,000,000 male births (0.000001%)
Gonadotropin-releasing hormone insensitivity
N/A
No estimate
Familial male-limited precocious puberty
Male
No estimate
Cytochrome P450 oxidoreductase deficiency
N/A
No estimate
Isolated 17,20-lyase deficiency
N/A
No estimate
Testicular dysgenesis syndrome
Male
No estimate
Penoscrotal transposition
Male
No estimate
Hyperandrogenism
N/A
No estimate
Hyperestrogenism
N/A
No estimate
Polyorchidism
Male
No estimate
Aphallia
Male
No estimate
Cryptorchidism
Male
One in 33–100 male births (3–1%)
Cloacal exstrophy (born with XY chromosomes)
Male
One in 400,000 live births (0.0025%)

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