Intersex - AbsoluteAstronomy.com

Intersex, in human

Human

Humans are the only living species in the Homo genus...

s and other animals, is the presence of intermediate or atypical combinations of physical features that usually distinguish female

Female

Female is the sex of an organism, or a part of an organism, which produces non-mobile ova .- Defining characteristics :The ova are defined as the larger gametes in a heterogamous reproduction system, while the smaller, usually motile gamete, the spermatozoon, is produced by the male...

from male

Male

Male refers to the biological sex of an organism, or part of an organism, which produces small mobile gametes, called spermatozoa. Each spermatozoon can fuse with a larger female gamete or ovum, in the process of fertilization...

. This is usually understood to be congenital, involving chromosomal

Chromosome

A chromosome is an organized structure of DNA and protein found in cells. It is a single piece of coiled DNA containing many genes, regulatory elements and other nucleotide sequences. Chromosomes also contain DNA-bound proteins, which serve to package the DNA and control its functions.Chromosomes...

, morphologic, genital and/or gonad

Gonad

The gonad is the organ that makes gametes. The gonads in males are the testes and the gonads in females are the ovaries. The product, gametes, are haploid germ cells. For example, spermatozoon and egg cells are gametes...

al anomalies, such as diversion from typical XX-female or XY-male presentations, e.g., sex reversal (XY-female, XX-male), genital ambiguity, or sex developmental differences. An intersex individual may have biological characteristics of both the male and the female sexes. Intersexuality as a term was adopted by medicine during the 20th century, and applied to human beings whose biological sex

Sex

In biology, sex is a process of combining and mixing genetic traits, often resulting in the specialization of organisms into a male or female variety . Sexual reproduction involves combining specialized cells to form offspring that inherit traits from both parents...

cannot be classified as clearly male or female. Intersex was initially adopted by intersex activists who criticize traditional medical approaches to sex assignment

Sex assignment

Sex assignment refers to the assigning of the biological sex at the birth of a baby. In the majority of births, a relative, midwife, or physician inspects the genitalia when the baby is delivered, sees ordinary male or female genitalia, and declares, "it's a girl" or "it's a boy" without the...

and seek to be heard in the construction of new approaches.

Some people (whether physically intersex or not) do not identify themselves as either exclusively female or exclusively male. Androgyny
Androgyny
Androgyny is a term derived from the Greek words ανήρ, stem ανδρ- and γυνή , referring to the combination of masculine and feminine characteristics...

is sometimes used to refer to those without gender-specific physical sexual characteristics or sexual preferences or gender identity, or some combination of these; such people can be physically and psychologically anywhere between the two sexes. This state may or may not include a mixture or absence of sexual preferences.

Language

Research in the late 20th century has led to a growing medical consensus that diverse intersex physicalities are normal, but relatively rare, forms of human biology. Milton Diamond

Milton Diamond

Milton Diamond is a retired professor of anatomy and reproductive biology at the University of Hawai'i at Mānoa. He has had a very long and productive career in the study of human sexuality...

, one of the most outspoken experts on matters affecting intersex people, stresses the importance of care in the selection of language related to such people.

Hermaphrodite

A hermaphrodite

Hermaphrodite

In biology, a hermaphrodite is an organism that has reproductive organs normally associated with both male and female sexes.Many taxonomic groups of animals do not have separate sexes. In these groups, hermaphroditism is a normal condition, enabling a form of sexual reproduction in which both...

is a plant or animal that has both male and female reproductive organs.

Disorders of sex development

"Disorders of sex development

Disorders of sex development

Disorders of sex development , sometimes referred to as disorders of sex differentiation, are medical terms referring to "congenital conditions in which development of chromosomal, gonadal, or anatomical sex is atypical." Lee et al...

" (DSD) is a term that has both supporters and opponents. It is defined to include congenital conditions in which development of chromosomal, gonadal, or anatomical sex is atypical.

A number of critics of traditional terminology, including the Intersex Society of North America

Intersex Society of North America

The Intersex Society of North America was a non-profit advocacy group founded in 1993 by Cheryl Chase to represent the interest of intersex people. Their objective was to end shame, secrecy, and unwanted genital surgeries...

, intersex activists, and some medical experts have moved to eliminate the term "intersex" in medical usage, replacing it with disorders of sex development in order to avoid conflating anatomy with identity. Members of the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology accepted this term in their "Consensus statement on management of intersex disorders" published in the Archives of Disease in Children and in Pediatrics.

Other intersex people, activists, supporters, and academics have contested the adoption of the terminology and its implied status as a "disorder", seeing this as offensive to intersex individuals who do not feel that there is something wrong with them, regard the DSD consensus paper as reinforcing the normativity of early surgical interventions, and criticising the treatment protocols associated with the new taxonomy. Alternatives to categorising intersex conditions as "disorders" have been suggested, including "variations of sex development". Organisation Intersex International

Organisation Intersex International

The Organisation Intersex International is a global advocacy group for people with intersex traits. It is the largest intersex support group in the world....

questions a disease/disability approach, argues for deferral of intervention unless medically necessary, when fully informed consent of the individual involved is possible, and self-determination of sex/gender orientation and identity.

History

Intersex people are treated in different ways by different cultures. In some cultures, such people were included in larger "third gender

Third gender

The terms third gender and third sex describe individuals who are categorized as neither man nor woman, as well as the social category present in those societies who recognize three or more genders...

" or gender-blending social roles along with other individuals. In most societies, intersexed people have been expected to conform to either a female or a male gender role

Gender role

Gender roles refer to the set of social and behavioral norms that are considered to be socially appropriate for individuals of a specific sex in the context of a specific culture, which differ widely between cultures and over time...

.

Whether or not they were socially tolerated or accepted by any particular culture, the existence of intersex people was known to many ancient and pre-modern cultures. An example is one of the Sumer

Sumer

Sumer was a civilization and historical region in southern Mesopotamia, modern Iraq during the Chalcolithic and Early Bronze Age....

ian creation myths from more than 4,000 years ago. The story has Ninmah, a mother goddess

Mother goddess

Mother goddess is a term used to refer to a goddess who represents motherhood, fertility, creation or embodies the bounty of the Earth. When equated with the Earth or the natural world such goddesses are sometimes referred to as Mother Earth or as the Earth Mother.Many different goddesses have...

, fashioning mankind out of clay. She boasts that she will determine the fate – good or bad – for all she fashions. Enki

Enki

Enki is a god in Sumerian mythology, later known as Ea in Akkadian and Babylonian mythology. He was originally patron god of the city of Eridu, but later the influence of his cult spread throughout Mesopotamia and to the Canaanites, Hittites and Hurrians...

, the father god, retorts as follows.

Enki answered Ninmah: "I will counterbalance whatever fate – good or bad – you happen to decide." Ninmah took clay from the top of the abzu [ab = water, zu = far] in her hand and she fashioned from it first a man who could not bend his outstretched weak hands. Enki looked at the man who cannot bend his outstretched weak hands, and decreed his fate: he appointed him as a servant of the king.

... [Three men and one woman with atypical biology are formed and Enki gives each of them various forms of status to ensure respect for their uniqueness] ...

Sixth, she fashioned one with neither penis nor vagina on its body. Enki looked at the one with neither penis nor vagina on its body and gave it the name Nibru [eunuch(?)], and decreed as its fate to stand before the king."

During the Victorian era

Victorian era

The Victorian era of British history was the period of Queen Victoria's reign from 20 June 1837 until her death on 22 January 1901. It was a long period of peace, prosperity, refined sensibilities and national self-confidence...

, medical authors introduced the terms "true hermaphrodite" for an individual who has both ovarian and testicular tissue, verified under a microscope, "male pseudo-hermaphrodite" for a person with testicular tissue, but either female or ambiguous sexual anatomy, and "female pseudo-hermaphrodite" for a person with ovarian tissue, but either male or ambiguous sexual anatomy. In Europe, the term 'intersexual' was first to be used before the Second World War. The first suggestion to replace the term 'hermaphrodite' with 'intersex' came from British specialist Cawadias in the 1940s. This suggestion was taken up by specialists in the UK during the 1960s, by both those who rejected Money's framework (then emerging from the USA), and those who endorsed that approach.

Since the rise of modern medical science in Western societies, some intersex people with ambiguous external genitalia have had their genitalia surgically modified to resemble either female or male genitals. Since the advancements in surgery have made it possible for intersex conditions to be concealed, many people are not aware of how frequently intersex conditions arise in human beings or that they occur at all. Contemporary social activists, scientists and health practitioners, among others, have begun to revisit the issue. Awareness of the existence of physical sexual variation in human beings has increased.

Some groups, such as ISNA

Intersex Society of North America

, and some clinicians, such as those at University College Hospital London, have questioned the practice of performing genital corrective surgery

Intersex surgery

Intersex surgery is one of several terms referring to surgery performed to correct birth defects or early injuries of the genitalia, primarily for the purposes of making the appearance more normal and to reduce the likelihood of future problems...

on intersex children. Dialogue between what were once antagonistic groups of activists and clinicians has led to changes in medical policies and how intersex patients and their families are treated in some locations. There are intersex groups, such as OII

Organisation Intersex International

The Organisation Intersex International is a global advocacy group for people with intersex traits. It is the largest intersex support group in the world....

, who argue that the various degrees of intersex are natural human variations that should not be subject to correction.

The writer Anne Fausto-Sterling

Anne Fausto-Sterling

Anne Fausto-Sterling, Ph. D. is Professor of Biology and Gender Studies at Brown University. She participates actively in the field of sexology and has written extensively on the fields of biology of gender, sexual identity, gender identity, and gender roles.-Life and career:Fausto-Sterling...

coined the words herm (for "true hermaphrodite"), merm (for "male pseudo-hermaphrodite"), and ferm (for "female pseudo-hermaphrodite"), and proposed that these be recognized as sexes along with female and male. Her terms were "tongue-in-cheek"; she no longer advocates these terms even as a rhetorical device. The activist Cheryl Chase

Cheryl Chase (activist)

Bo Laurent, better known by her pseudonym Cheryl Chase , is an American intersex activist and the founder of the Intersex Society of North America...

criticized these terms in a letter to The Sciences
The Sciences
The Sciences was published from 1961 to 2001 by the New York Academy of Sciences. Each issue contained articles that discussed science issues with cultural relevance, illustrated with fine art and an occasional cartoon. The periodical won seven National Magazine Awards over the course of its...

, also criticizing the traditional standard of medical care. Chase announced the creation of the Intersex Society of North America

Intersex Society of North America

Sociological approaches

The first sociologist to work on 'intersexuality' was Harold Garfinkel

Harold Garfinkel

Harold Garfinkel was a Professor Emeritus at the University of California, Los Angeles. He is known for establishing and developing ethnomethodology as a field of inquiry in sociology.-Biography:...

in 1967 using a method derived from sociological phenomenology

Phenomenology (psychology)

Phenomenology is an approach to psychological subject matter that has its roots in the philosophical work of Edmund Husserl. Early phenomenologists such as Husserl, Jean-Paul Sartre, and Maurice Merleau-Ponty conducted their own psychological investigations in the early 20th century...

he called ethnomethodology

Ethnomethodology

Ethnomethodology is an ethnographic approach to sociological inquiry introduced by the American sociologist Harold Garfinkel . Ethnomethodology's research interest is the study of the everyday methods people use for the production of social order...

. He based his analysis on the everyday commonsense understandings of 'Agnes', a woman undergoing social and surgical gender reassignment. Ethnomethodology was also used in 1978 by Kessler and McKenna, who argue that, while gender can be seen as a social accomplishment, cross-cultural studies render gender as problematic as they highlight how it is usually regarded as a fact, when it can be shown to be constructed in different ways. They point to different cultural approaches to gender roles, and how 'hermaphrodites' and 'berdaches' are incorporated socially, as disruptive to fixed ideas about sex, gender, and gender-roles. They argue that what we 'know' about gender is grounded in the 'everyday social construction of a world of two genders', where gender attribution seems more important than gender differentiation.

Gender and sexual self-identification

Some people with intersex conditions self-identify as intersex, and some do not.

Surgery

Depending on the type of intersex condition, surgery may be performed not for protection of life or health but for aesthetic or social purposes. Unlike other aesthetic surgical procedures performed on infants, such as corrective surgery for a cleft lip (as opposed to a cleft palate), genital surgery may lead to negative consequences for sexual functioning in later life (such as loss of sensation in the genitals, for example, when a clitoris

Clitoris

The clitoris is a sexual organ that is present only in female mammals. In humans, the visible button-like portion is located near the anterior junction of the labia minora, above the opening of the urethra and vagina. Unlike the penis, which is homologous to the clitoris, the clitoris does not...

deemed too large/penile is reduced/removed), or feelings of freakishness and unacceptability, which may have been avoided without the surgery. In other cases, negative consequences may be avoided with surgery.

Opponents maintain that there is no compelling evidence that the presumed social benefits of such "normalizing" surgery outweigh the potential costs. Opponents claim this led to the degrading interpretation that females are essentially castrated males. This view overlooks the embryological origin of the penis/clitoris.

Defenders of the practice argue that it is necessary for individuals to be clearly identified as male or female in order for them to function socially. However, many intersex individuals have resented the medical intervention, and some have been so discontented with their surgically assigned gender as to opt for sexual reassignment surgery later in life. The Declaration of Montreal

Declaration of Montreal

The Declaration of Montreal on Lesbian, Gay, Bisexual, and Transgender Human Rights is a document adopted in Montreal, Quebec, Canada, on July 29, 2006, by the International Conference on LGBT Human Rights which formed part of the first World Outgames. The Declaration outlines a number of rights...

first demanded prohibition of unnecessary post-birth surgery to reinforce gender assignment until a child is old enough to understand and give informed consent

Informed consent

Informed consent is a phrase often used in law to indicate that the consent a person gives meets certain minimum standards. As a literal matter, in the absence of fraud, it is redundant. An informed consent can be said to have been given based upon a clear appreciation and understanding of the...

. This was detailed in the context of existing UN declarations and conventions under Principle 18 of The Yogyakarta Principles, which called on states to:
Intersex advocates and experts have critiqued the necessity of early interventions, citing individual's experiences of intervention and the lack of follow-up studies showing clear benefits. Specialists at the Intersex Clinic at University College London began to publish evidence in 2001 that indicated the harm that can arise as a result of inappropriate interventions, and advised minimising the use of childhood surgical procedures.

Experience of medical procedures and photography

Individuals report experiences of the trauma associated with intervention. The experiential similarities of medical interventions and child sexual abuse have been discussed.

Photographs of intersex children's genitalia are circulated in medical communities for documentary purposes; an example of this appears in the medical section 3.2.1 below. Problems associated with experiences of medical photography of intersex children have been discussed along with the ethics, control and usage.

"The experience of being photographed has exemplified for many people with intersex conditions the powerlessness and humiliation felt during medical investigations and interventions".

Gender dysphoria

The proposed revisions for DSM-5

DSM-5

The next edition of the American Psychiatric Association's Diagnostic and Statistical Manual of Mental Disorders , commonly called DSM-5 , is currently in consultation, planning and preparation...

include a change from using Gender Identity Disorder

Gender identity disorder

Gender identity disorder is the formal diagnosis used by psychologists and physicians to describe persons who experience significant gender dysphoria . It describes the symptoms related to transsexualism, as well as less severe manifestations of gender dysphoria...

to Gender Dysphoria. This revised code now specifically includes intersex people as people with Disorders of Sex Development

Disorders of sex development

. This move has been criticised by one intersex advocacy group in Australia, and criticism from the intersex community has been lodged with the appropriate DSM5 subcommittee.

Intersex in popular culture

Intersex was discussed on British TV for the first time in 1966, and became a topic of interest for broadcast TV and radio in the United States and other countries from 1989. Jeffrey Eugenides

Jeffrey Eugenides

Jeffrey Kent Eugenides is an American Pulitzer Prize-winning novelist and short story writer. Eugenides is most known for his first two novels, The Virgin Suicides and Middlesex . His novel The Marriage Plot was published in October, 2011.-Life and career:Eugenides was born in Detroit, Michigan,...

' novel Middlesex
Middlesex (novel)
Middlesex is a Pulitzer Prize-winning novel by Jeffrey Eugenides published in 2002. The book is a bestseller, with more than three million copies sold as of May 2011. Its characters and events are loosely based on aspects of Eugenides' life and observations of his Greek heritage. It is...

(2002) is narrated by an intersex character who discusses the societal experience of an intersex person. The Japanese manga series I.S., first published in 2003, features intersexual characters and how they deal with intersex-related issues and influence the lives of people around them. The 2007 Argentine film XXY

XXY (film)

XXY is a 2007 Argentine film written and directed by Lucía Puenzo. The film stars Ricardo Darín, Valeria Bertuccelli, Inés Efron and Martín Piroyansky...

centres around a young intersex person who is assisted in presenting as a girl with the assistance of medication. The film deals with discrimination, sexuality and gender identity.

Education

In secondary schools, biology and sex education instructors often place most emphasis on the most common XX and XY genotypes. Thus, people nowadays may be more likely to look towards the sex chromosomes than, for example, the histology of the gonads. However, according to researcher Eric Vilain at the University of California, Los Angeles

University of California, Los Angeles

The University of California, Los Angeles is a public research university located in the Westwood neighborhood of Los Angeles, California, USA. It was founded in 1919 as the "Southern Branch" of the University of California and is the second oldest of the ten campuses...

, "the biology of gender is far more complicated than XX or XY chromosomes". Many different criteria have been proposed, and there is little consensus.

Notable intersex people

Le Van Duyet
Le Van Duyet
Lê Văn Duyệt was a Vietnamese general who helped Nguyễn Ánh—the future Emperor Gia Long—put down the Tây Sơn rebellion, unify Vietnam and establish the Nguyễn Dynasty...

, Nguyen Dynasty general and high-ranking mandarin.
Herculine Barbin
Herculine Barbin
Herculine Barbin was a French intersex person who was treated as a female at birth but was later redesignated a male after an affair and physical examination.-Biography:...

—the 19th century memoirs
Herculine Barbin: Being the Recently Discovered Memoirs of a Nineteenth-century French Hermaphrodite
Herculine Barbin: Being the Recently Discovered Memoirs of a Nineteenth-century French Hermaphrodite is a 1980 English-language translation of Herculine Barbin's nineteenth-century memoirs, which were originally written in French. The book contains an introduction by Michel Foucault, which only...

of this French intersex person were published by Michel Foucault
Michel Foucault
Michel Foucault , born Paul-Michel Foucault , was a French philosopher, social theorist and historian of ideas...

in 1980.
Sir Ewan Forbes, 11th Baronet
Sir Ewan Forbes, 11th Baronet
Sir Ewan Forbes of Craigievar was the 11th Baronet of Craigievar from 1968 to his death, as well as a general practitioner and farmer. At birth, he was christened "Elizabeth Forbes-Sempill", and officially registered as the youngest daughter of Lord Sempill...

, formerly Elizabeth Forbes-Sempill—in 1968 the Scottish courts confirmed Ewan Forbes' intersexuality cited in the correction of his birth certificate, placing him as the male heir to the family title, making him the 11th Baronet of Craigievar.
Georgina Somerset (née Turtle), first openly intersex person in the UK; she was active in the media from the mid-1960s.
Cheryl Chase
Cheryl Chase (activist)
Bo Laurent, better known by her pseudonym Cheryl Chase , is an American intersex activist and the founder of the Intersex Society of North America...

, intersex activist.
Erik Schinegger
Erik Schinegger
Erik Schinegger is an Austrian intersexual skier. He was the world champion women's downhill skier in 1966, at which time he was recognized as female and known as Erika Schinegger.-Life:...

, alpine skier.
Jim Sinclair, autism rights activist.
Lady Colin Campbell
Lady Colin Campbell
Lady Colin Campbell, , is a British writer, biographer, autobiographer, novelist, television and radio personality, known for her biography of Diana, Princess of Wales, The Real Diana, as well as other books on the Royal Family and the international elite.Campbell was born in Jamaica, the child of...

, British aristocrat and author of Guide to Being a Modern Lady.
Edinanci Silva
Edinanci Silva
Edinanci Fernandes da Silva is a judoka from Brazil, who won the gold medal in the half heavyweight division at the Pan American Games. A resident of São Paulo, she represented the country at four consecutive Summer Olympics, starting in 1996 in Atlanta, Georgia.Edinanci was born with both male...

, judoka and Gold medalist in the woman's half-heavyweight division at the Pan-American games.
James Barry
James Barry (surgeon)
James Barry , was a military surgeon in the British Army. After graduation from the University of Edinburgh, Barry served in India and Cape Town, South Africa. By the end of his career, he had risen to the rank of Inspector General in charge of military hospitals...

, British military surgeon who gave the first successful caesarean section
Caesarean section
A Caesarean section, is a surgical procedure in which one or more incisions are made through a mother's abdomen and uterus to deliver one or more babies, or, rarely, to remove a dead fetus...

in Africa by a British surgeon.
Del LaGrace Volcano
Del LaGrace Volcano
Del LaGrace Volcano is "one of instigators of polymorphous perverse queer culture." A formally trained photographer, Del's work includes installation, performance and film and interrogates the performance of gender on several levels, especially the performance of masculinity and femininity.-Life...

, visual artist and speaker on queer and intersex issues (e.g. the Critical Sexology Seminars, London). Describes his intersexuality as self-constructed.
Santhi Soundarajan
Santhi Soundarajan
Santhi Soundarajan is an Indian athlete who competes in the middle distance track events...

, India
India
India , officially the Republic of India , is a country in South Asia. It is the seventh-largest country by geographical area, the second-most populous country with over 1.2 billion people, and the most populous democracy in the world...

n athlete who competes in the middle distance track events. She was stripped of a silver medal won at the 2006 Asian Games
Asian Games
The Asian Games, officially known as Asiad, is a multi-sport event held every four years among athletes from all over Asia. The Games were regulated by the Asian Games Federation from the first Games in New Delhi, India, until the 1978 Games. Since the 1982 Games they have been organised by the...

after failing a sex verification test, disputing her eligibility to participate in the women's competition.
Mauro Cabral, Argentine
Argentina
Argentina , officially the Argentine Republic , is the second largest country in South America by land area, after Brazil. It is constituted as a federation of 23 provinces and an autonomous city, Buenos Aires...

intersex activist, writer, Co-director of GATE (Global Action for Trans Equality)
Cr Tony Briffa JP, Australian intersex and human rights activist, former President of the AIS Support Group Australia, and Deputy Mayor of the City of Hobsons Bay
Hobsons Bay
Hobsons Bay is a bay in Port Phillip, Victoria, Australia....

in Melbourne, Australia

Discussion in media and on internet

There has been intense speculation about Caster Semenya
Caster Semenya
Mokgadi Caster Semenya is a South African middle-distance runner and world champion. Semenya won gold in the women's 800 metres at the 2009 World Championships with a time of 1:55.45 in the final....

, the South African middle-distance runner, being intersex. The way she has been dealt with by the sporting community and the media has itself been the subject of debate in the media. Tests she was subjected to included what were described as humiliating genital photography.

Noted researchers on intersex development

John Money
John Money
John William Money was a psychologist, sexologist and author, specializing in research into sexual identity and biology of gender...
Milton Diamond
Milton Diamond
Milton Diamond is a retired professor of anatomy and reproductive biology at the University of Hawai'i at Mānoa. He has had a very long and productive career in the study of human sexuality...

, professor of neurology, Univ. of Hawai'i at Manoa
Anne Fausto-Sterling
Anne Fausto-Sterling
Anne Fausto-Sterling, Ph. D. is Professor of Biology and Gender Studies at Brown University. She participates actively in the field of sexology and has written extensively on the fields of biology of gender, sexual identity, gender identity, and gender roles.-Life and career:Fausto-Sterling...

Intersex organizations

Organisation Intersex International
Organisation Intersex International
The Organisation Intersex International is a global advocacy group for people with intersex traits. It is the largest intersex support group in the world....
AIS Support Group UK & International
AIS Support Group Australia
Intersex Trust Aotearoa New Zealand (ITANZ)
Accord Alliance
Advocates for Informed Choice (AIC)

Intersex conditions and scope

Sax's strict definition of intersex is most relevant to family practice and psychological research. Other interest groups serve different communities and concerns and so broaden the definition of intersex in these fields.

For instance, the Intersex Society of North America

Intersex Society of North America

(ISNA) definition states that the following conditions "sometimes involve intersex anatomy":

5-alpha reductase deficiency
androgen insensitivity syndrome
Androgen insensitivity syndrome
Androgen insensitivity syndrome is a condition that results in the partial or complete inability of the cell to respond to androgens. The unresponsiveness of the cell to the presence of androgenic hormones can impair or prevent the masculinization of male genitalia in the developing fetus, as...
aphallia
Aphallia
Aphallia is a congenital malformity in which the phallus is absent. The word is derived from the Greek "a" for negative or no, and "phallia" for penis.-Incidence/prevalence:...
clitoromegaly
Clitoromegaly
Clitoromegaly is an abnormal enlargement of the clitoris ....
congenital adrenal hyperplasia
Congenital adrenal hyperplasia
Congenital adrenal hyperplasia refers to any of several autosomal recessive diseases resulting from mutations of genes for enzymes mediating the biochemical steps of production of cortisol from cholesterol by the adrenal glands ....
gonadal dysgenesis
Gonadal dysgenesis
Gonadal dysgenesis is a term used to describe multiple reproductive system development disorders. They are conditions of genetic origin. It is characterized by a progressive loss of primordial germ cells on the developing gonads of an embryo....

(partial & complete)
hypospadias
Hypospadias
Hypospadias is a birth defect of the urethra in the male that involves an abnormally placed urinary meatus...
Klinefelter syndrome
micropenis
Micropenis
Micropenis is an unusually small penis. A common criterion is a dorsal erect penile length of at least 2.5 standard deviations smaller than the mean human penis size. The condition is usually recognized shortly after birth...
mosaicism involving sex chromosomes
ovo-testes (formerly called "true hermaphroditism
True hermaphroditism
True hermaphroditism is a medical term for an intersex condition in which an individual is born with ovarian and testicular tissue.There may be an ovary on one side and a testis on the other, but more commonly one or both gonads is an ovotestis containing both types of tissue.It is rare—so...

")
partial androgen insensitivity syndrome
Partial androgen insensitivity syndrome
Partial androgen insensitivity syndrome is a condition that results in the partial inability of the cell to respond to androgens. 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...
progestin-induced virilisation
Progestin-induced virilisation
Maternal use of androgens or high doses of certain weakly androgenic synthetic progestogens structurally related to testosterone can masculinize the external genitalia of a female fetus during susceptible times in pregnancy....
Swyer syndrome
Swyer syndrome
Swyer syndrome, or XY gonadal dysgenesis, is a type of hypogonadism in a person whose karyotype is 46,XY. The person is externally female with streak gonads, and left untreated, will not experience puberty...
Turner syndrome
Turner syndrome
Turner syndrome or Ullrich-Turner syndrome encompasses several conditions in human females, of which monosomy X is most common. It is a chromosomal abnormality in which all or part of one of the sex chromosomes is absent...

Prevalence

The prevalence of intersex depends on which definition is used.

According to the ISNA definition above, 1 percent of live births exhibit some degree of sexual ambiguity. Between 0.1% and 0.2% of live births are ambiguous enough to become the subject of specialist medical attention, including surgery

Surgery

Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...

to disguise their sexual ambiguity.

According to Fausto-Sterling's definition of intersex, on the other hand, 1.7 percent of human births are intersex. She writes,
According to Leonard Sax the prevalence of intersex "restricted to those conditions in which chromosomal sex is inconsistent with phenotypic sex, or in which the phenotype is not classifiable as either male or female" is about 0.018%.

Signs

There are a range of variations between female and male types of genitalia; the Prader scale demonstrates this and is well illustrated here.

Ambiguous genitalia

Ambiguous genitalia appear as a large clitoris or small penis and may or may not require surgery.

Because there is variation in all of the processes of the development of the sex organs, a child can be born with a sexual anatomy that is typically female, or feminine in appearance with a larger-than-average clitoris

Clitoris

(clitoral hypertrophy), or typically male, masculine in appearance with a smaller-than-average penis

Penis

The penis is a biological feature of male animals including both vertebrates and invertebrates...

that is open along the underside. The appearance may be quite ambiguous, describable as female genitals with a very large clitoris and partially fused labia, or as male genitals with a very small penis, completely open along the midline ("hypospadic"), and empty scrotum.

Fertility is variable. According to some, the distinctions "male pseudohermaphrodite", "female pseudohermaphrodite" and especially "true hermaphrodite" are vestiges of 19th century thinking. According to others, the terms "male pseudohermaphrodite", and "female pseudohermaphrodite" are used to define the gender in terms of the histology

Histology

Histology is the study of the microscopic anatomy of cells and tissues of plants and animals. It is performed by examining cells and tissues commonly by sectioning and staining; followed by examination under a light microscope or electron microscope...

(microscopic appearance) of the gonad

Gonad

"True hermaphroditism"

With some conditions of intersex, even the chromosomal sex may not be clear. A "true hermaphrodite" is defined as someone with both male gonadal tissue (testes) and female gonadal tissue (ovarian tissue).

In 2003, researchers at UCLA published their studies of a lateral gynandromorph

Gynandromorph

A gynandromorph is an organism that contains both male and female characteristics. The term gynandromorph, from Greek "gyne" female and "andro" male, is mainly used in the field of Lepidopterology or entomology...

ic hermaphroditic zebra finch

Zebra Finch

The Zebra Finch, Taeniopygia guttata, is the most common and familiar estrildid finch of Central Australia and ranges over most of the continent, avoiding only the cool moist south and the tropical far north. It also can be found natively in Indonesia and East Timor...

, which had a testicle on the right and an ovary on the left. Its entire body was split down the middle between female and male, with hormones from both gonads running through the blood. This is an example of mosaicism or chimerism.

This extreme example of hermaphroditism is quite rare.

Ovotestes

Though naturally-occurring true hermaphroditism in humans is unknown, there is, on the other hand, a spectrum of forms of ovotestes

Ovotestis

An ovotestis is a gonad with both testicular and ovarian aspects. In humans, ovotestes are an anatomical abnormality associated with gonadal dysgenesis.- In gastropods :...

. The varieties include having two ovotestes or one ovary and one ovotestis, often in the form of streak gonads. Phenotype is not determinable from the ovotestes; in some cases, the appearance is "fairly typically female"; in others, it is "fairly typically male," and it may also be "fairly in-between in terms of genital development."

Intersex activist Cheryl Chase

Cheryl Chase (activist)

Bo Laurent, better known by her pseudonym Cheryl Chase , is an American intersex activist and the founder of the Intersex Society of North America...

is an example of someone with ovotestes.

Other diagnostic signs

In order to help in classification, methods other than a genitalia inspection can be performed:

For instance, a karyotype

Karyotype

A karyotype is the number and appearance of chromosomes in the nucleus of an eukaryotic cell. The term is also used for the complete set of chromosomes in a species, or an individual organism.p28...

display of a tissue sample may determine which of the causes of intersex is prevalent in the case.

Management

Clinical management of intersex can be categorized into one of the following two:

Treatments: Restore functionality (or potential functionality)
Enhancements: Give the ability to identify with “mainstream” people, e.g., breast enlargement surgery

However, there are other categorisation systems of management of intersexed, which falls into neither category.

In any case, the most common procedure is surgery.

Surgery

The exact procedure of the surgery depends on what is the cause of a less common body phenotype in the first place. There is often concern as to whether surgery should be performed at all. A traditional approach to the management of intersexuality has been socially motivated surgery. However, some (Alice Dreger) say that surgical treatment is socially motivated and, hence, ethically questionable; without evidence, doctors regularly assume that intersexed persons cannot have a clear identity. This is often taken further with parents of intersexed babies advised that without surgery their child will be stigmatized. Further, since almost all such surgeries are undertaken to fashion female genitalia for the child, it is more difficult for the child to present as male if they later select a male gender identity. 20-50% of surgical cases result in a loss of sexual sensation (Newman 1991, 1992).

As convention, surgery is performed at birth. Intersex advocates such as Anne Fausto-Sterling in her Sexing the Body argue surgery on intersexed babies should wait until the child can make an informed decision, and label operation without consent as genital mutilation

Genital mutilation

Genital mutilation can refer to:*Clitoridectomy*Female genital mutilation, also known as female circumcision*Genital modification and mutilation*Infibulation...

Typical sex development

The common pathway of sexual differentiation

Sexual differentiation

Sexual differentiation is the process of development of the differences between males and females from an undifferentiated zygote...

, where a productive human female has an XX chromosome pair, and a productive male has an XY pair, is relevant to the development of intersexed conditions.

During fertilization, the sperm adds either an X (female) or a Y (male) chromosome to the X in the ovum. This determines the genetic sex of the embryo. During the first weeks of development, genetic male and female fetuses are "anatomically indistinguishable," with primitive gonads beginning to develop during approximately the sixth week of gestation. The gonads, in a "bipotential state," may develop into either testes (the male gonads) or ovaries (the female gonads), depending on the consequent events. Through the seventh week, female and male fetuses appear identical.

At around eight weeks of gestation, the gonads of an XY embryo differentiate into functional testes, secreting testosterone. Ovarian differentiation, for XX embryos, does not occur until approximately Week 12 of gestation. In normal female differentiation, the Müllerian duct system

Müllerian duct

Müllerian ducts are paired ducts of the embryo that run down the lateral sides of the urogenital ridge and terminate at the Müllerian eminence in the primitive urogenital sinus. In the female, they will develop to form the Fallopian tubes, uterus, cervix, and the upper two-third of the vagina; in...

develops into the uterus

Uterus

The uterus or womb is a major female hormone-responsive reproductive sex organ of most mammals including humans. One end, the cervix, opens into the vagina, while the other is connected to one or both fallopian tubes, depending on the species...

, Fallopian tubes, and inner third of the vagina.
In males, the Müllerian duct-inhibiting hormone MIH

MIH

Mih or MIH may refer to:* Media independent handover, a technology standardized by IEEE 802.21 for handing off IP sessions among alternative wireless access technologies.* Mullerian inhibiting hormone, an alternative name for Anti-Müllerian hormone....

causes this duct system to regress. Next, androgens cause the development of the Wolffian duct system

Wolffian duct

The mesonephric duct is a paired organ found in mammals including humans during embryogenesis....

, which develops into the vas deferens

Vas deferens

The vas deferens , also called ductus deferens, , is part of the male anatomy of many vertebrates; they transport sperm from the epididymis in anticipation of ejaculation....

, seminal vesicles, and ejaculatory ducts.
By birth, the typical fetus has been completely "sexed" male or female, the hormones and genital development remaining consistent with the genetic sex.

Conditions

The final body appearance does not always correspond with what is dictated by the genes. In other words, there is sometimes an incongruity between genotypic (chromosomal) and phenotypic sex. Citing medical research regarding other factors that influence sexual differentiation, the Intersex Society of North America

Intersex Society of North America

challenges the XY sex-determination system

XY sex-determination system

The XY sex-determination system is the sex-determination system found in humans, most other mammals, some insects and some plants . In this system, females have two of the same kind of sex chromosome , and are called the homogametic sex. Males have two distinct sex chromosomes , and are called...

's assumption that chromosomal sex is the determining factor of a person's "true" biological sex.

X/Y	Name	Description >- \| XX	Congenital adrenal hyperplasia Congenital adrenal hyperplasia Congenital adrenal hyperplasia refers to any of several autosomal recessive diseases resulting from mutations of genes for enzymes mediating the biochemical steps of production of cortisol from cholesterol by the adrenal glands .... (CAH)	adrenal gland Adrenal gland In mammals, the adrenal glands are endocrine glands that sit atop the kidneys; in humans, the right suprarenal gland is triangular shaped, while the left suprarenal gland is semilunar shaped... s produce abnormally high levels of virilizing hormones. In people without a Y chromosome (i.e., XX), this can range from partial masculation that produces a large clitoris, to virilisation and male appearance. The latter applies in particular to Congenital adrenal hyperplasia due to 21-hydroxylase deficiency Congenital adrenal hyperplasia due to 21-hydroxylase deficiency Congenital adrenal hyperplasia due to 21-hydroxylase deficiency , in all its forms, accounts for over 95% of diagnosed cases of congenital adrenal hyperplasia, and "CAH" in most contexts refers to 21-hydroxylase deficiency... , which is the most common form of CAH. Individuals born with XX chromosomes affected by 17α-hydroxylase deficiency Congenital adrenal hyperplasia due to 17 alpha-hydroxylase deficiency Congenital adrenal hyperplasia due to 17α-hydroxylase deficiency is an uncommon form of congenital adrenal hyperplasia resulting from a defect in the gene for the enzyme CYP17A1. It produces decreased synthesis of both cortisol and sex steroids, with resulting increase in mineralocorticoid production... are born with female internal and external anatomy, but, at puberty, neither the adrenals nor the ovaries can produce sex-hormones, inhibiting breast development and the growth of pubic hair. See below for XY CAH 17α-hydroxylase deficiency. >- \| XX	Progestin-induced virilisation Progestin-induced virilisation Maternal use of androgens or high doses of certain weakly androgenic synthetic progestogens structurally related to testosterone can masculinize the external genitalia of a female fetus during susceptible times in pregnancy....	progestin Progestin A progestin is a synthetic progestogen that has progestinic effects similar to progesterone. The two most common uses of progestins are for hormonal contraception , and to prevent endometrial hyperplasia from unopposed estrogen in hormone replacement therapy... , a drug that was used in the 1950s and 1960s to prevent miscarriage. These individuals normally have internal and external female anatomy, with functional ovaries and will therefore have menstruation. They develop, however, some male secondary characteristics and they frequently have unusually large clitorises. In very advanced cases, such children have initially been identified as males. >- \| XX	Freemartin Freemartin A freemartin or free-martin is an infertile female mammal which has masculinized behavior and non-functioning ovaries. Genetically and externally the animal is female, but it is sterilized in the womb by hormones from a male twin, becoming an infertile partial intersex... ism	cattle Bovinae The biological subfamily Bovinae includes a diverse group of 10 genera of medium to large sized ungulates, including domestic cattle, the bison, African buffalo, the water buffalo, the yak, and the four-horned and spiral-horned antelopes... and affects most females born as a twin to a male. It is rare or unknown in other mammals, including humans. In cattle, the placenta Placenta The placenta is an organ that connects the developing fetus to the uterine wall to allow nutrient uptake, waste elimination, and gas exchange via the mother's blood supply. "True" placentas are a defining characteristic of eutherian or "placental" mammals, but are also found in some snakes and... e of fraternal twins usually fuse at some time during the pregnancy, and the twins then share their blood supply. If the twins are of different sexes, male hormones produced in the body of the fetal bull find their way into the body of the fetal heifer (female), and masculinize her. Her sexual organs do not develop fully, and her ovaries may even contain testicular tissue. When adult, such a freemartin is very like a normal female in external appearance, but she is infertile, and behaves more like a castrated Castration Castration is any action, surgical, chemical, or otherwise, by which a male loses the functions of the testicles or a female loses the functions of the ovaries.-Humans:... male (a steer). The male twin is not significantly affected, although (if he remains entire) his testes may be slightly reduced in size. The degree of masculinization of the freemartin depends on the stage of pregnancy at which the placental fusion occurs in about ten percent of such births no fusion occurs and both calves develop normally as in other mammals. >- \| XY	Androgen insensitivity syndrome Androgen insensitivity syndrome Androgen insensitivity syndrome is a condition that results in the partial or complete inability of the cell to respond to androgens. The unresponsiveness of the cell to the presence of androgenic hormones can impair or prevent the masculinization of male genitalia in the developing fetus, as... (AIS)	Cases with typically female appearance and genitalia are said to have complete androgen insensitivity syndrome (CAIS). People with CAIS have a vagina Vagina The vagina is a fibromuscular tubular tract leading from the uterus to the exterior of the body in female placental mammals and marsupials, or to the cloaca in female birds, monotremes, and some reptiles. Female insects and other invertebrates also have a vagina, which is the terminal part of the... and no uterus Uterus The uterus or womb is a major female hormone-responsive reproductive sex organ of most mammals including humans. One end, the cervix, opens into the vagina, while the other is connected to one or both fallopian tubes, depending on the species... , cervix Cervix The cervix is the lower, narrow portion of the uterus where it joins with the top end of the vagina. It is cylindrical or conical in shape and protrudes through the upper anterior vaginal wall... , or ovaries, and are infertile. The vagina may be shorter than usual, and, in some cases, is nearly absent. Instead of female internal reproductive organs, a person with CAIS has undescended or partially descended testes, of which the person may not even be aware. In mild and partial androgen insensitivity syndrome (MAIS and PAIS), the body is partially receptive to androgens, so there is virilization to varying degrees. PAIS can result in genital ambiguity, due to limited metabolization of the androgens produced by the testes. Ambiguous genitalia may present as a large clitoris, known as clitoromegaly Clitoromegaly Clitoromegaly is an abnormal enlargement of the clitoris .... , or a small penis, which is called micropenis Micropenis Micropenis is an unusually small penis. A common criterion is a dorsal erect penile length of at least 2.5 standard deviations smaller than the mean human penis size. The condition is usually recognized shortly after birth... or microphallus; hypospadias and cryptorchidism Cryptorchidism Cryptorchidism is the absence of one or both testes from the scrotum. It is the most common birth defect regarding male genitalia. In unique cases, cryptorchidism can develop later in life, often as late as young adulthood. About 3% of full-term and 30% of premature infant boys are born with at... may also be present, with one or both testes undescended, and hypospadias appearing just below the glans on an otherwise typical male penis, or at the base of the shaft, or at the perineum and including a bifid (or cleft) scrotum. >- \| XY	5-alpha-reductase deficiency 5-alpha-reductase deficiency 5-Alpha-reductase deficiency is an autosomal recessive intersex condition caused by a mutation of the 5-alpha reductase type 2 gene.-Normal function:... (5-ARD)	Y chromosome Y chromosome The Y chromosome is one of the two sex-determining chromosomes in most mammals, including humans. In mammals, it contains the gene SRY, which triggers testis development if present. The human Y chromosome is composed of about 60 million base pairs... , making their bodies unable to convert testosterone to dihydrotestosterone (DHT). DHT is necessary for the development of male genitalia in utero, and plays no role in female development, so its absence tends to result in ambiguous genitalia at birth; the effects can range from infertility with male genitalia to male underdevelopment with hypospadias to female genitalia with mild clitoromegaly. The frequency is unknown, and children are sometimes misdiagnosed as having AIS. Individuals can have testes, as well as vagina and labia, and a small penis capable of ejaculation that looks like a clitoris at birth. Such individuals are usually raised as girls. The lack of DHT also limits the development of facial hair. >- \| XY	Congenital adrenal hyperplasia Congenital adrenal hyperplasia Congenital adrenal hyperplasia refers to any of several autosomal recessive diseases resulting from mutations of genes for enzymes mediating the biochemical steps of production of cortisol from cholesterol by the adrenal glands .... (CAH)	Congenital adrenal hyperplasia due to 17 alpha-hydroxylase deficiency Congenital adrenal hyperplasia due to 17 alpha-hydroxylase deficiency Congenital adrenal hyperplasia due to 17α-hydroxylase deficiency is an uncommon form of congenital adrenal hyperplasia resulting from a defect in the gene for the enzyme CYP17A1. It produces decreased synthesis of both cortisol and sex steroids, with resulting increase in mineralocorticoid production... , CAH inhibits virilization, unlike cases without a Y chromosome. >- \| XY	Persistent Müllerian duct syndrome Persistent Mullerian duct syndrome Persistent Müllerian duct syndrome refers to the presence of a uterus and sometimes other Müllerian duct derivatives in a male animal... (PMDS)	>- \| Other	Unusual chromosomal sex	Turner syndrome Turner syndrome Turner syndrome or Ullrich-Turner syndrome encompasses several conditions in human females, of which monosomy X is most common. It is a chromosomal abnormality in which all or part of one of the sex chromosomes is absent... (XO Turner syndrome Turner syndrome or Ullrich-Turner syndrome encompasses several conditions in human females, of which monosomy X is most common. It is a chromosomal abnormality in which all or part of one of the sex chromosomes is absent... ), Triple X syndrome Triple X syndrome Triple X syndrome is a form of chromosomal variation characterized by the presence of an extra X chromosome in each cell of a human female. The condition always produces females, with an XX pair of chromosomes, as well as an additional chromosome, resulting in the formation of XXX. A mosaic form... (XXX), Klinefelter's Syndrome Klinefelter's syndrome Klinefelter syndrome, 46/47, XXY, or XXY syndrome is a condition in which human males have an extra X chromosome. While females have an XX chromosomal makeup, and males an XY, affected individuals have at least two X chromosomes and at least one Y chromosome... , (XXY/XXXY Klinefelter's syndrome Klinefelter syndrome, 46/47, XXY, or XXY syndrome is a condition in which human males have an extra X chromosome. While females have an XX chromosomal makeup, and males an XY, affected individuals have at least two X chromosomes and at least one Y chromosome... ), XYY syndrome XYY syndrome XYY syndrome is an aneuploidy of the sex chromosomes in which a human male receives an extra Y-chromosome, giving a total of 47 chromosomes instead of the more usual 46. This produces a 47,XYY karyotype... (XYY XYY syndrome XYY syndrome is an aneuploidy of the sex chromosomes in which a human male receives an extra Y-chromosome, giving a total of 47 chromosomes instead of the more usual 46. This produces a 47,XYY karyotype... ), de la Chapelle syndrome XX male syndrome XX male syndrome is a rare sex chromosomal disorder. Usually it is caused by unequal crossing over between X and Y chromosomes during meiosis in the father, which results in the X chromosome containing the normally-male SRY gene... (XX male), Swyer syndrome Swyer syndrome Swyer syndrome, or XY gonadal dysgenesis, is a type of hypogonadism in a person whose karyotype is 46,XY. The person is externally female with streak gonads, and left untreated, will not experience puberty... (XY female Swyer syndrome Swyer syndrome, or XY gonadal dysgenesis, is a type of hypogonadism in a person whose karyotype is 46,XY. The person is externally female with streak gonads, and left untreated, will not experience puberty... ), and there are many other individuals who do not follow the typical patterns (such as individuals with four or even more sex chromosomes). >- \| Other	Mosaicism and chimerism	A mix can occur, where some of the cells of the body have the common XX or XY, while some have one of the less usual chromosomal contents above. Such a mixture is caused by either mosaicism or chimerism. In mosaicism, the mixture is caused by a mutation in one of the cells of the embryo after fertilization, whereas chimerism is a fusion of two embryos. In alternative fashion, it is simply a mixture between XX and XY, and does not have to involve any less-common genotypes in individual cells. This, too, can occur both as chimerism and as a result of one sex chromosome having mutated into the other. However, not all cases of mosaicism and chimerism involve intersex.

Complications

In the cases where nonfunctional testes are present, there is a risk that these develop cancer. Therefore, doctors either remove them by orchidectomy or monitor them carefully. This is the case for instance in androgen insensitivity syndrome

Androgen insensitivity syndrome

Androgen insensitivity syndrome is a condition that results in the partial or complete inability of the cell to respond to androgens. The unresponsiveness of the cell to the presence of androgenic hormones can impair or prevent the masculinization of male genitalia in the developing fetus, as...

External links

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Language

Hermaphrodite

Disorders of sex development

History

Sociological approaches

Gender and sexual self-identification

Surgery

Experience of medical procedures and photography

Gender dysphoria

Intersex in popular culture

Education

Notable intersex people

Discussion in media and on internet

Noted researchers on intersex development

Intersex organizations

Intersex conditions and scope

Prevalence

Signs

Ambiguous genitalia

"True hermaphroditism"

Ovotestes

Other diagnostic signs

Management

Surgery

Typical sex development

Conditions

Complications

See also

External links