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International Birth Defects Information Systems
Sex or Gender Assignment

International Birth Defects Information Systems



Sex or Gender Assignment


Topics: | Sex or Gender Assignment | 46,XY gonadal dysgenesis | Abnormal Sex Differentiation | Androgen Insensitivity Syndrome | CAH Screening for Correct Sex Assignment | Congenital Adrenal Hyperplasia | Defining Sex | Determination of Gender | Females with XY Sexual Disorders | Gonadoblastoma | Hermaphrodite | Hermaphroditism (Cancer Risk) | Illustrations | Intersex Defined | Intersexual | Intersexuality | Male/Female A Polydimensional Continuum | Manifesto Intersex Support Group International | Mixed gonadal dysgenesis | Pseudohermaphroditism | Sex Reassignment | Sindrome de Insensibilidad a los Androgenos | SRY gene | Transgender |

Related Topics: | Hypospadias | Kallmann S | XXY Klinefelter S |

Service Related: | Support Groups | Professional Associations | Key Information Sources |

Languages: | English | Spanish | Ukrainian |

On the word "hermaphrodite"
ISNA, 2006
Visitor Comments [Support Groups]
The word "hermaphrodite" is a stigmatizing and misleading word … Victorian doctors believed that the gonads were the seat of "true sex," … in the absence of any knowledge of genetics, endocrinology, or embryology -- which categorized people as "male pseudohermaphrodite," "female pseudohermaphrodite," … It's time to eliminate this quaint Victorianism from modern medical practice …

Glossary of Medical Terms
Androgen Insensitivity Syndrome Support Group, 2007
Visitor Comments [Support Groups]
germ cells: male (sperm) and female (egg) cells
gonad: an organ that produces gametes (germ cells), especially a testis or ovary
gonadectomy: surgical removal of the gonads …
Mullerian ducts: either of two embryonic tubes that become the uterine tubes, uterus and part of the vagina in the female and that form the prostatic utricle in the male …

Determination of Gender
University of Michigan Medical School, 1999
Visitor Comments [Support Groups]
Although genetic sex (XX or XY) is determined at fertilization, the embryo's gender is not distinguishable for the first six weeks of development; this is known as the indifferent period of development …
In both sexes … development an indifferent genital tubercle … and elongates to form a phallus … without influence of androgens, the phallus becomes the clitoris … urogenital folds become the labia minora, and the labioscrotal swellings become the labia majora …
Hypospadias
Incomplete fusion of the urogenital folds creates abnormal openings of the urethra ...
If the two paramesonephric ducts fail to fuse correctly it can result in duplication of the uterus ... (double uterus and double vagina). If one paramesonephric duct fails to develop … single horn of the uterus (unicomuate uterus). Failure of the testes to descend into the scrotum (cryptorchidism) …
Rare true hermaphrodites have both ovarian and testicular tissues, usually possessing a 46,XX karyotype … Female pseudohermaphrodites are more common, possessing a 46, XX karyotype … result from exposure to excess androgens during embryologic development (as in congenital virilizing adrenal hyperplasia). Male pseudohermaphrodites have testes and a 46, XY karyotype … results from an inadequate production of androgens …

Genetics for Theologians: Male/Female A Polydimensional Continuum (archived)
Roberta M. Meehan, PhD., Greeley, Colorado College Science Educator and Biological Science Writer
Visitor Comments [Support Groups]
Discussion # 1
We are discussing "male/female" … terms apply to the human condition and not as they apply to linguistics and semantics … My plan is to present a series of commentaries on my perceptions of this male/female continuum …
To begin this discussion on Chromosomes and the Continuum, I think it is important to first define a few terms … "SEX" usually refers to reproductive differences … "Gender" is one of those terms that really should be divided into about 3 or 4 different words. However, we are stuck with one word that has a variety of overlapping meanings. In biology, "gender" usually refers to man/woman -- and is thus a more restrictive term than "sex". However, "gender" can be used synonymously with "sex" when referring to humans and other higher level species ... gender is a grammatical identification and means masculine/feminine but has very little to do with male/female -- except in some cases …
SOCIETAL SEX (often called by any of several other names) is determined after birth -- i.e., how is the person raised (male or female or intersex), what are the social and cultural mores, etc. For our purposes, societal sex deals with society's identification of the individual …
Could such a person (particularly the XO/XY person) be ordained?
... As a sidelight, the Olympic Committee (as of 1991) no longer tests for chromosome compliments but does rely on visual examination to determine whether a person is male or female … There probably are quite a few priests who are XXY …
Genetic anomalies do not make human beings less human, though sometimes these anomalies make them less reproductively capable. And why should reproductive capability be a criterion for ministerial capability? Sexual dichotomy does NOT exist for the survival of the individual. Sexual dichotomy does NOT exist either for the ministerial goals of the individual or the ministerial goals of the species. Sexual dichotomy does NOT exist for the perpetuation of the Kingdom of God in any way other than to assure that all life is included. Sexual dichotomy is a biological phenomenon assuring the survival of the species; it is not a prerequisite for ministry -- which is a human (though not biological) phenomenon.

Related Conditions
Androgen Insensitivity Syndrome Support Group, June 9, 2006
Visitor Comments [Support Groups]
The pathway towards a female form seems to be the default for humans, while the path for development of a male fetus is more tortuous, and dependent on many different genes … there is a set of a fairly varied conditions in which a fetus with XY chromosomes develops as a baby girl.
Among these are:
Failure of formation of testes … (i.e. pure XY gonadal dysgenesis or Swyer syndrome, XO/XY mosaicism, testicular regression syndrome, Denys-Drash syndrome, WAGR, Campomelic dysplasia and SF - 1 mutation).
Underdevelopment of cells in testes due to unresponsiveness to hormones … Leydig cell agenesis or hypoplasia.
Inborn errors of testosterone biosynthesis …
Defects in androgen - dependent target tissues (i.e. AIS)
AIS is the most common and … the best known of these conditions and is described on other pages …
“Umbrella” Term (MPH)
These “XY female” conditions have been grouped by doctors under an umbrella term called “male pseudo - hermaphroditism” (MPH) … hangover from a time before modern genetic testing became available … However … there is no other commonly used term … male prefix … refers to the medical notion that the main determinant of the maleness is the presence of testes, and hermaphroditism refers to … outwardly female patient …
Mis - Diagnosis
… few doctors will have come across sufficient patients to enable them to fin - tune the diagnosis …
Importance of Correct Diagnosis
… very important in governing treatment decisions. For example, in non - AIS conditions the body will be fully responsive to androgens …
Failure of Formation of Testes
… also known as “dysgenic pseudo - hermaphroditism” … thought of as a failure of the development of testes in the presence of XY chromosomes …
Swyer Syndrome
… “pure XY gonadal dysgenesis,” is the name given to a condition in which individuals possess a 46XY karyotype and female external genitalia. Women with Swyer’s syndrome have no ovaries or testes, but have “streak” … Swyer individuals have a normal vagina and uterus (the so - called Mullerian structures) … menstruation does not occur naturally … Hormone replacement causes increase in breast size and underarm and public hair, and menstruation is possible if the proper hormone regime is followed. In some cases, streak gonads develop tumors which produce hormones, thereby resulting in modest breast and public hair development and minor menstrual spotting. Removal of the streak gonads is carried out to minimize the risk of cancerous growths … Since Swyer syndrome women posses a uterus they can get pregnant, through implantation of a fertilized donated egg in an in vitro fertilization procedure. As they lack ovaries, they do not ovulate. Swyer syndrome may result from a deletion along the Y chromosome, or a mutation in the SRY, the sex determining region on the Y chromosome, or from a mutation in another gene that affects the function of the sex determining region genes on the Y chromosome …
XO/XY Mosaicism
Turner syndrome is a condition in which a person has a single X sex chromosome and no Y chromosome … Turner syndrome is very distinct from AIS, and is unlikely to mistaken for AIS. However, there are patients who have a mixture of 45,X and 46 XY cells in their bodies, resulting in a condition known as 45,X/46,XY mosaicism, or "mixed gonadal dysgenesis", which has the potential to be mistaken for PAIS …
Testicular Regression Syndrome
… Vanishing Testis Syndrome …
Underdevelopment of Leydig Cells
… produce testosterone (called Leydig cells) do not develop properly. This condition is known as Leydig Cell Hypoplasia …
Inborn Errors of Testosterone Biosynthesis
There are several enzymes that are part of the usual process of testosterone synthesis …

Androgen Insensitivity Syndrome Support Group (AISSG) Registered UK
Androgen Insensitivity Syndrome Support Group, October 19, 2004
Visitor Comments [Support Groups]
AIS is a condition that affects the development of reproductive and genital organs. Both men and women usually have at least one X chromosome, which contains a gene that gives their bodies the capacity to recognize and react to masculinizing hormones (androgens and the inhibition of female internal organ development. Women with AIS have a functioning Y chromosome (and therefore no female internal organs), but an abnormality in the X chromosome that renders the body partially or completely incapable of recognizing the androgens produced, therefore allowing the default external genital development along female lines ... Illness is the experience of living through the disease … are a support group providing information … Androgen Insensitivity Syndrome … We also support those affected by Swyer’s syndrome (XY gonadal dysgenesis), 5 alpha - reductase deficiency, leydig cell hypoplasia, Mayer - Rokitansky - Kuster - Hauser (MRKH) syndrome, Mullerian dysgenesis, Mullerian duct aplasia, vaginal atresia, and other related conditions …

MANAGEMENT of INTERSEXUALITY
Milton Diamond, Ph.D. et al., The UK Intersex Association, 1997
Visitor Comments [for Professionals mainly] [Support Groups]
Guidelines for dealing with individuals with ambiguous genitalia
Asking for specific guidelines on how to manage cases of traumatized or ambiguous genitalia. Below we offer our suggestions. We first, however, add this caveat: These recommendations are based on our experiences, the input of some trusted colleagues, the comments of intersexed persons of various etiologies and the best interpretation of our reading of the literature. Some of these suggestions are contrary to today’s common management procedures … Foremost, we advocate use of the terms “typical”, “usual” or “most frequent” where it is common to use the term “normal” … avoid expressions like maldeveloped or undeveloped, errors of development, defective genitals, abnormal, or mistakes of nature …
1. In all cases of ambiguous genitalia, to establish most probable cause … Genetic and endocrine evaluations are usually needed … Pelvic ultrasonography … assessment of immediate and extended family … Hospitals … House Stuff Operating Procedures to be followed in such cases … all births be accompanied by a full genital inspection. Many cases of intersex go undetected.
2. Immediately, and simultaneously with the above, advise parents of the reasons for the delay. Full and honest disclosure is best and counseling must start directly …
3. The child’s condition is nothing to be ashamed of …
4. In the most common cases, those of hypospadias and congenital adrenal hyperplasia (C.A.H.) diagnosis should be rapid and clear …
5. The sex of assignment, when based on the nature of the diagnosis rather than only considering the size of functionality of the phallus … In the majority of cases this sex of assignment will indeed be in concert with the appearance of the genitalia … In certain childhood situations, however, such assignment will be counter to the genital appearance … Rear as male - XY individuals with Androgen Insensitivity Syndrome … Grades 1 - 3) - XX individuals with Congenital Adrenal Hyperplasia - (C.A.H.) with extensively fused labia and a penile clitoris - XX individuals with Hypospadias … - XY individuals with Micropenis … Rear as female - XY individuals with Androgen Insensitivity Syndrome - (A.I.S.) (Grades 4 - 7) - XX individuals with Congenital Adrenal Hyperplasia - (C.A.H.) with hypertrophied clitoris …
6. While sex determination is ongoing, the hospital administration can wait for a final diagnosis before entering a sex of record … refer to the child as “Infant Jones” … “Baby Brown” …
7. Perform no major surgery for cosmetic reasons only … parents who will want their children to “look normal”. Explain to them that appearances during childhood, while not typical for other children, may be of less importance than functionally and post pubertal erotic sensitivity of the genitalia …
8. In rearing, parents must be consistent in seeing their child as either a boy or girl; not neuter … intersex is … not yet a commonly accepted social designation …
10. Be clear that the child is special and …
12. Maintain contact with family …
14. Counseling must include developmental sequelae to be anticipated …
16. As early as possible put the family in touch with a support group …
17. Keep genital inspection to a minimum …
18. Let the child … know that help is available if needed. Listen to the patient; even when as a child …
19. As puberty approaches be open and honest …
21. Most intersex conditions can remain without any surgery at all …
FINAL COMMENT
… many of those who have had genital surgery or been sex reassigned neonatally have complained bitterly of the treatment. Some have sex reassigned themselves …

A Single Word: Stop!
Deborah E. Brown, Intersex Support Group International
Visitor Comments [Support Groups]
If there were only one word I could give to parents of a child born with ambiguous genitalia (intersexual, hermaphrodite, Androgen Insensitivity Syndrome, et al), that word would be: Stop! Repeat after me, ''STOP!'' When the doctors say, "Your child could never function as a male/female, so we suggest that we cut..." Say ''STOP!'' When an expert surgeon is brought in to tell you that your child has malformed genitals, that she/he needs adjustment, and now is the best time to do it. Say ''STOP!'' … As much as every parent wants to bring home a girl, or a boy, your child may not be distinctly either. Often immediate corrective procedures are necessary for the life of the child. When well meaning clinicians attempt to make a package deal, say ''STOP!'' Early invasive treatments literally change the destiny, the identity, the 'me' of your child. It is OK to wait. Gender assignment (mutilation) is NOT COSMETIC SURGERY! It is OK to wait and see who the 'me' your child turns out to be, and then bring your child into the decision loop … For too long, the unyielding demands of people claiming the name of Christ have over - ridden the real love and understanding of our Creator and Lord. Acknowledged in scriptures, both old and new testaments, persons born with ambiguous or no - existent genitalia have been promised special blessing, ( Isaiah 56:3 - 5), as well as understanding of their peculiar relationship in a two - gendered society, (Matthew 19:11 - 12) … As intersexuals we have been created unique by God. Deus Unicus Creatus

A Reassessment of “sex assignment”
Susan J. Bradley et al., Hospital for Sick Children, Toronto, Ontario, Canada, PEDIATRICS Vol. 102 No. 1 July 1998, p. e9
Visitor Comments [for Professionals mainly]
Experiment of Nurture: Ablatio Penis at 2 Months, Sex Reassignment at 7 Months, and a Psychosexual Follow - up in Young Adulthood
Guidelines of psychosexual management for infants born with physical intersex conditions … sex assignment should be to the gender that carries the best prognosis for good reproductive function, good sexual function, normal - looking external genitalia and physical appearance, and a stable gender identity … decision regarding sex assignment should be made as early as possible … there should be minimal uncertainty and ambiguity on the part of parents … J. Money used these guidelines in a case of a biologically normal male infant (one of a pair of monozygotic twins) whose penis was accidentally ablated during a circumcision at the age of 7 months. The decision to reassign the infant boy to the female sex and to rear him as a girl was made at 17 months, with surgical castration and initial genital reconstruction occurring at 21 months … the girl was described as having may “tomboyish” behavioral traits, a female gender identity had apparently differentiated. Thus, it was concluded that gender identity is sufficiently incompletely differentiated at birth as to permit successful assignment of a genetic male as a girl, in keeping with the experiences of rearing. Subsequent follow - up by other investigators reported that by early adolescence the patient had rejected the female identity and began to live as a male at the age of 14 years. In adulthood, the patient recalled that he had never felt comfortable as a girl and his mother reported similar recollections. At age 25, the patient married a woman and adopted her children. The patient reported exclusive sexual attraction to females. The present case report is a long - term psychosexual follow - up on a second case of ablatio penis in a 46,XY male. During an electrocautery circumcision at the age of 2 months, the patient sustained a burn of the skin of the entire penile shaft, and the penis eventually sloughed off. At age 7 months, the remainder of the penis and the testes were removed. By age 7 months, if not earlier, the decision was made to reassign the patient as a female and to raise the infant as a girl … At ages 16 and 26, the patient was living socially as a woman and denied any uncertainty about being a female … she was attracted predominantly to women in fantasy … she was in a relationship with a man and wished to be able to have intercourse. The patient’s self - described sexual identity was “bisexual” … began … lesbian relationship …

Manifesto
Intersex Support Group International
Visitor Comments [Support Groups]
Intersex Support Group International was established BY and FOR persons whose bodies were born with a physiological or genetic characteristic which has been diagnosed as Intersexual … That consequence is not God’s will …

"Intersex" - Frequently Asked Questions
Intersex Society of North America
Visitor Comments [Support Groups]
What is intersexuality ( hermaphroditism )?
Our culture conceives sex anatomy as a dichotomy: humans come in two sexes … However, developmental embryology … Anatomic sex differentiation occurs on a male/female continuum … Genetic sex, or the organization of the “sex chromosomes” … However, something like 1/500 of the population have a karyotype other than XX or XY. Since genetic testing was instituted for women in the Olympic Games, a number of women have been disqualified as “not women,” after winning. However, none of the disqualified women is a man; all have atypical karyotypes … … sex chromosomes determine the differentiation of the gonads into ovaries, testes, ovo - testes, or nonfunctioning streaks …
What is androgen insensitivity syndrome?
Androgen Insensitivity Syndrome, or AIS, is a genetic condition, inherited (except for occasional spontaneous mutations), occurring in approximately 1 in 20,000 individuals … cells are unable to respond to androgen, or “male hormones … Some individuals have partial androgen insensitivity … As in typical male fetuses, the MIH causes the fetal mullerian ducts to regress, so the fetus lacks uterus, fallopian tubes, and cervix plus upper part of vagina. However, because cells fail to respond to testosterone, the genitals differentiate in the female, rather than the male pattern, and Wolffian structures (epididymis, vas deferens, and seminal vesicles) are absent. The newborn AIS infant has genitals of normal female appearance, undescended or partially descended testes, and usually a short vagina with no cervix … At puberty, the estrogen produced by the testes produces breast growth … Vaginoplasty surgery is frequently performed on AIS infants or girls to increase the size of the vagina … Because AIS is a genetic defect located on the X chromosome, it runs in families …
What is partial androgen insensitivity syndrome?
Extent of androgen insensitivity in 46 XY individuals is quite variable, even in a single family … typically results in “ambiguous genitalia.” The clitoris is large or, alternatively, the penis is small and hypospadic …
What is Progestin Induced Virilization?
Caused by prenatal exposure to exogenous androgens, most commonly progestin … administered to prevent miscarriage in the 50’s and 60’s and it is converted to an androgen (virilizing hormone) by the prenatal XX persons metabolism. If the timing is right, the genitals are virilized with effects ranging from enlarged clitoris to the development of a complete phallus and the fusing of the labia … In other words, XX people affected in - utero by virilizing hormones can be born into a continuum of sex phenotype which ranges from “female with larger clitoris” to “male with no testes” …
What is Adrenal Hyperplasia?
The most prevalent cause of intersexuality amongst XX people … about 1 in 20,000 births. It is caused when an anomaly of adrenal function (usually 21 - hydroxylase or 11 - hydroxylase deficiency) causes the synthesis … sex phenotype varies along the same continuum, with the possible added complication of metabolic problems which upset serum sodium balance …
What is Klinefelter syndrome?
Most men inherit a single X chromosome from their mother, and a single Y chromosome from their father. Men with Klinefelter syndrome inherit an extra X chromosome from either father or mother; their karyotype is 47 XXY. Klinefelter is quite common, occurring in 1/500 to 1/1,000 male births. The effects of Klinefelter are quite variable … absence of sperm in the ejaculate, causing infertility. Except for small testes … don’t virilize (develop facial and body hair, muscles, deep voice, larger penis and testes) … Physicians recommend that boys … be given testosterone at puberty …
What is hypospadias?
Refers to a urethral meatus (“pee - hole”) which is located along the underside …
Is there a risk of gonadal tumors?
Dysgenetic testicular tissue (testicular tissue that has developed in an unusual way) is at risk of developing tumors, and not merely because it is undescended. That is, the risk persists even after successful orchiopexy … Undescended testes in women with AIS are at risk of developing tumors … Testosterone replacement in men with dysgenetic testes may increase the probability of gonadal tumors developing … Tumors are not likely in the absence of a Y chromosome …
Hormone replacement therapy and osteoporosis
Sex hormones (principally testosterone or estrogen) are necessary to maintain healthy adult bones …
Where can I read some of the earliest first person writings of intersexuals?
Opinions of intersex specialists Grumbach of UCSF and Gearhart of Hopkins (surgery is necessary to prevent parents from treating child as an outcast) are contrasted with personal experience of Chase and Holmes (surgery experienced as mutilation, causing sexual dysfunction) … “Once a dark secret, “ BMJ 1994; 308:542 (19 February) … A woman with XY karyotype and “testicular feminization” (androgen insensitivity syndrome) briefly relates how damaging she has found the secrecy surrounding her condition …

Intersex Defined
Intersex Support Group International, 2002
Visitor Comments [Support Groups]
Following statistics are appropriate, and are stated in terms of the frequency per 1000 live births … The statistics presented here are our best guess for North America … Dr. Anne Fausto - Sterling, Professor of Medical Science, Brown University.
Disorders of Gonadal Differentiation
Gonadal dysgenesis 6.5/1000 live births
Turner (45,X) 0.2/1000
Klinefelter (XXY, XXXY, etc) 0.5/1000
47,XXX 1/1000
46,XY complete gonadal dysgenesis ???
46,XY partial gonadal dysgenesis???
Congenital adrenal hyperplasia 0.05/1000
Hypospadias 3.33/1000 males ...
Microphallus 0.20
Adult cryptorchism 0.28 ...
Dysgenetic testicular tissue (testicular tissue that has developed in and unusual way) is at risk of developing tumors, and not merely because it is undescended. That is, the risk persists even after successful orchiopexy (surgically bringing undescended testes down into scrotal sac) … Undescended testes in women with AIS are at risk of developing tumors … Gonadal tumors are less likely in cases of sex - reversal (46XX male, 46XX true hermaphrodite) … Tumors are not likely in the absence of a Y chromosome (or Y genes involved in testicular determination, which may be present on the X chromosome in sex - reversal). When there is a Y chromosome or Y genes are surmised to be present, the gonads are at elevated risk, and should be carefully monitored … Hormone replacement therapy and osteoporosis Sex hormones (principally testosterone or estrogen) are necessary to maintain healthy adult bones …

Hermaphroditism
David A. Hatch, M.D., 2003
Visitor Comments [Illustrated]
If abnormalities of cell division occur a fetus with mosaicism may result. The baby may have both cells with 46 XY chromosomes and cells with 46 XX chromosomes. This condition is called hermaphroditism after the greek god Hermes and goddess Aphrodite. Because both male and female cells exist within the same fetus, both male and female structures develop. Typically, the penis is not completely virilized (hypospadias). One or both testes may not be palpable or they may be palpable, but undescended. Whenever you see an infant with both hypospadias and an undescended testis (cryptorchidism), you must consider the diagnosis of hermaphroditism …
Whenever you suspect hermaphroditism you should confirm the diagnosis by ordering a karyotype. In addition, the child should have an abdominal ultrasound …

The International Foundation for Androgynous Studies Inc (Archived)
The International Foundation for Androgynous Studies Inc., 2003
Congenital or inherited abnormalities which can lead to genital ambiguity or the intersex condition …

Hermaphroditism
MEDLINE plus, Health Information , October 11, 2007
Alternative names: hermaphrodite; intersexual; pseudohermaphroditism
Definition: A term referring to being of both sexes (intersexual)
True hermaphroditism requires the presence of both ovarian (female) and testicular (male) reproductive tissue and is relatively rare and poorly understood. Pseudohermaphroditism is more common and from a medical standpoint hermaphroditism suggests two factors:
- ambiguous external genitalia
- genitalia that may not match the genetic make-up of the person (example: female genitalia in an XY, genetically male, individual.)
The following conditions can produce ambiguous genitalia
- congenital adrenal hyperplasia
- fetal exposure to progestins (see progestins - oral) or androgens
- testicular feminization syndrome
- XY gonadal dysgenesis
- XY gonadal agenesis
- chromosomal abnormalities
- cryptophthalmos
- Smith-Lemli-Opitz
- 4p syndrome
- 13q syndrome …

HUMAN RIGHTS FOR INTERSEXUALS INTERSEX INDIVIDUALS DISPUTE WISDOM OF SURGERY ON INFANTS
Althaea Yronwode
Cheryl Chase’s clitoris was surgically removed when she was 18 months old … Initially doctors thought Chase should be reared as a boy, and she was named Charlie. But further consultation with different doctors led to the decision to raise her as a girl … “Until I was 35, I was ashamed and terrified that people would find out that I was different than a woman. Like many, supposedly happy and successful patients, I was silenced.” … she took the revolutionary step of founding the Intersex Society of North America (ISNA), a San Francisco based peer support and advocacy group. Initially just a loose association on the internet, ISNA now has 1,400 members, holds retreats for intersex people, has produced a video, lobbies, holds demonstrations, maintains a website, and puts intersex people in touch with each other throughout North America … … when all causes of intersex are considered, as many as one in 2,000 babies are affected … Current medical treatment for intersex individuals is in a state of flux. Recently The Journal of Clinical Ethics devoted an entire issue to the subject, ultimately recommending major changes … Professor of Pediatric Endocrinology Melvin Grumbach … Speaking from an office cluttered with journals and papers on this subject in which he is a renowned expert, he cites the advantages of microsurgery, unavailable when Chase’s clitoris was removed. “When I was a fellow at Hopkins, they were doing clitorectomy on girls with congenital adrenal hyperplasia (CAH) … When I was at Columbia, clitoral recessions were developed … were all sexually functional and happy … … “We don’t see a difference between clitorectomy and clitoral recession [in which the bulk of the clitoris is reduced by removal of part of the erectile bodies of the clitoral shaft]. Kinsey showed that women masturbate by stimulating the shaft of the clitoris, not the glans. Recession removes the shaft.” Chase isn’t surprised by Grumbach’s and Baskin’s faith … “They are always saying these new techniques are better, but there are no long - term outcome studies … Surgeons are not trained to deal with parents who are upset. They are trained to “fix” things … … “There is a principle in medicine - do no harm. They [surgeons] don’t know what effect their drastic invasive, irreversible interventions are having … Endocrinologist Grumbach is skeptical of this viewpoint. “If someone is telling you, “you shouldn’t modify the external genitalia,” well, the parents wouldn’t stand for it. They want it repaired!” Baskin … acknowledges that it can be “very disturbing to parents” to give birth to an infant who is intersex. “It is very disturbing,” agrees Chase. “And when people are really disturbed, it’s not the time to make major, irreversible decisions.” Instead, ISNA proposes that the parents receive counseling, and are put in contact with other parents of intersex children … “The child will assert their gender identity between the ages of six and ten,” notes Devore … The ethical issue most prominent in this debate is that of patient autonomy - the right of patients to decide for themselves what treatments they receive … Baskin believes … “Parents make decisions for their kids. That’s what parents do.” … Chase was adamant that the surgery is being done for the psychological adjustment of the parents, which strikes her and other adult intersexes as horribly wrong. “The infant is the patient, not the parents! … ISNA also takes issue with the criteria through which sex is assigned. “In the case of congenital adrenal hyperplasia, the excuse for the surgery is that the girl who has a vagina created will be fertile as a woman. But if you had to choose between fertility and sexual function, which would you choose?” Baskin is horrified by this attitude. “The majority of these patients … don’t look like a girl … I am trying to help kids.” After all the debate and division … Everyone agrees that patients and parents need more psychosocial support … Grumbach believes that groups like ISNA have changed treatment in positive ways … Sensitivity has gone up a couple of logs … “A good psychological social worker would be a tremendous help … get long - term psychiatry … provide support. That is one of the real deficiencies in care.” … Psychologist Devore … scoffs at the idea that trained counselors aren’t available. “The parents need counseling. They need to be told that this [intersex] is a way a person can be, even though it is not what the parents expected … Genital mutilation in the name of “normality” is relatively new; older anatomical textbooks show many variations in genital structure among intersex individuals … If you personally enjoy sex at all … think about what it would be like to have had your genitals cut off when you were a baby for no other reason than that they were “too large” or “too small” …

Intersexuality - A Plea for Honesty and Emotional Support
Bo Laurent, Association for Humanistic Psychology, 1997
The physicians and nursing staff in the delivery room grew suddenly silent, almost grim. “Is there something wrong with my baby?” queried the exhausted new mother … The baby was not sick; it had been born with “ambiguous genitals” … parents weren’t sure for a time whether you were a girl or a boy … “Diagnosis: true hermaphrodite. Operation: clitoridectomy.” “We advise you to fake an injury and leave quietly,” Olympic officials told Spanish hurdler Maria Patino … result of a laboratory test that indicated that her cells had only a single X chromosome. Patino was disqualified. Statistics are hard to come by, but it seems that as many as one in 500 women competitors are disqualified by the sex test. None are men masquerading as women; … Patino is a woman with “male” chromosomes; the medical label for her condition is androgen insensitivity syndrome. … About one in 400 men have two x chromosomes … Current medical thinking treats the birth of an intersexual infant as a “social emergency” … One surgeon, asked why intersex children are usually assigned female, explained, “It’s easier to dig a hole than to build a pole.” … Although physicians understand that they will actually impose, rather than determine, a sex, they tell parents that tests will reveal the child’s true sex, in a day or two at most, and assure them that surgery will let their child grow up normal, and heterosexual. They are careful to avoid words like “hermaphroditism” or intersexuality,” and speak only of “improperly formed gonads,” never of ovaries or testes … As a growing number of adult intersexuals have come forth to speak about their experiences …

Sex Reassignment at Birth: A Long Term Review and Clinical Implications
Milton Diamond, Ph.D. et al., Archives of Pediatrics and Adolescent Medicine March, 1997
Key Words: sex assignment, sex reassignment, gender, traumatized genitalia, penis ablation …
At puberty this individual switched to living as a male and has successfully lived as such from that time to the present … Subsequent cases should, however, be managed in light of this new evidence … These cases of successful gender change, as well as the present one, also challenge the belief that such a switch after the age of 2 years will be devastating. Indeed, in these cases it was salutary … In the interim, however, new guidelines are offered. We believe that any 46-chromosome, XY individual born with a normal nervous system, in keeping with the psychosexual bias thus prenataly imposed, should be raised up as a male. Surgery to repair any genital problem, while difficult, should be conducted in keeping with this paradigm. This decision is not simple 7, 13, 18, 63, 65-67 and analysis should continue. As parents will still want their children to be and look normal as soon after birth as possible, physicians will have to provide the best advice and care, consistent with present knowledge. We suggest this means referring the parents and child to appropriate and periodic long term counseling rather than immediate surgery and sex reassignment just because that seems a simpler immediate solution to a complicated problem. With this management, a male's predisposition to act as a boy and his actual behaviors will be reinforced in daily interactions on all sexual levels and his fertility preserved. Social difficulties may reveal themselves as puberty is experienced, however, there is no evidence that with proper counseling and surgical repair when best indicated, an adjustment will not be managed as teen-agers manage other severe handicaps. Future reports will determine if we are correct …

Three sisters with XY gonadal dysgenesis and sex reversal - a new variant?
A. Sinkus et al., Program Nr: 1702 ASHG 1999
We report on a family with three sisters, all XY females. Histological examination of their gonads showed only ovarian tissues. It consisted of hypoplastic ovarian stroma with … were apparent … Loss of heterozygosity was studied for markers in distal 9p, a region where deletions also contribute to gonadal dysgenesis. From the marker analyses it was apparent that all sisters had two alleles and all inherited the same allele from their father. This may indicate a mutation in a gene (DMT1?) from this chromosomal region … pedigree suggests an X-chromosomal mode of inheritance, we studied the dosage of genes from Xp21 - the DSS region … We conclude that mutations rather than dosage differences in a gene(s) from distal 9p or from a region around DSS may be responsible for the XY sex reversal.

Mullerian inhibiting substance in the diagnosis and management of intersex and gonadal abnormalities
ML Gustafson et al., Journal of Pediatric Surgery, VOL. 28, NO. 3: pp.439-44
Mullerian inhibiting substance (MIS), a gonadal hormone important in sexual differentiation, is high (10 to 70 ng/ml) in human male serum postnatally for several years before declining during the peripubertal period, but is undetectable in female serum until the onset of puberty. The sexually dimorphic secretion of MIS suggested possibilities for its use in several clinical settings … Serum MIS levels correlated with the presence of testicular tissue … MIS may be a more sensitive marker for the presence of testicular tissue than serum testosterone levels …

Transgender and Intersex Topics
Library of Congress Call Number
Looking queer …
Transsexual …
Hermaphrodites …

INTERSEX BIBLIOGRAPHY
James Park
John Money - Biographies of Gender and Hermaphroditism in Paired Comparisons: Clinical Supplement to the Handbook of Sexology

Intersex Related Books
Birth Disorder Information Directory
As Nature Made Him: The Boy Who Was Raised As a Girl
Transsexual Portraits
Gender Blending
Gender Shock: Exploding the Myths of Male and Female
Lessons from the Intersexed
Third Sex, Third Gender: Beyond Sexual Dysmorphism in Culture and History ...

Intersex Society of North America
Mission
To end shame, secrecy, and unwanted genital surgeries for people born with atypical sex anatomy …

The UK Intersex Association
The United Kingdom Intersex Association (UKIA) is an education, advocacy, campaigning and support organization which works on behalf of intersexed people. Intersex people are individuals born with anatomy or physiology which differs from contemporary ideals of what constitutes “normal” male and female …

The International Foundation for Androgynous Studies, Inc.
Promotes medical, social and educational research …
Actively disseminates information …
Advocates legislative change for …
Recognizes the legal status of androgynous persons …
Encourages tolerance and …

Pediatric Ethics and the Surgical Assignment of Sex
Kenneth Kipnis, Ph.D. Department of Philosophy University of Hawaii, The UK Intersex Association
Standard pediatric practice to recommend surgery for infants with ambiguous genitalia or loss of the penis … It is argued that there should be a moratorium on such surgery; that the medical profession should complete comprehensive lookback …

Le Groupe de Soutien a l’AIS
Androgen Insensitivity Syndrome Support Group
Visitor Comments [Support Groups] [French]
L’insensibilite aux hormones androgenes (Androgen Insensitivity syndrome) entraine une interruption du developpement foetal de l’appareil de reproduction. Un enfant atteint d’AIS a des chromosomes sexuels male (XY). Des testicules embryonnaires se developpent dans son corps et commencent a produire des hormones androgenes. Mais ces hormones males ne peuvent pas achever un developpement masculin a cause d’une rare insensibilite des tissus du foetus aux hormones androgenes. De ce fait, le developpement genital externe se poursuit selon le modele feminin ( la voie de secours) mais le developpement des organes femelles internes a deja ete stoppe par une hormone (MIF – Mullerian Inhibiteur Factor) provenant des testicules foetaux …

?Que es el SIA?
Website del Grupo de Apoyo del Sindrome de Insensibilidad a los Androgenos, 2007
Visitor Comments [Support Groups] [Spanish]
Cada feto, tanto si es geneticamente masculino (XY) o femenino (XX), comienza su vida con la capacidad de desarrollar tanto un sistema reproductivo masculino como femenino. Todos los fetos tienen genitales no diferenciados durante aproximadamente las 8 primeras semanas desde su concepcion. Despues de unas cuantas semanas, en un feto XY (sin SIA), los genitales no diferenciados se desarrollan como genitales masculinos debido a la presencia de hormonas masculinas (androgenos) (med term) …

Grupo de Apoyo del Sindrome de Insensibilidad a los Androgenos
Visitor Comments [Support Groups] [Spanish]
El SIA es una condicion que afecta al desarrollo de los organos reproductivos y genitales. Tanto hombres como mujeres tienen, normalmente, al menos un cromosoma X que contiene un gen que da a sus cuerpos la capacidad de reconocer y reaccionar a las hormonas que masculinizan el organismo (androgenos). Los hombres tienen, usualmente, tambien un cromosoma Y que se ocupa de la produccion de androgenos y de inhibir el desarrollo de los organos internos femeninos.Las mujeres con SIA tienen un cromosoma Y que funciona ( y consecuentemente no hay organos internos femeninos), pero el cromosoma X tiene una anormalidad que hace al cuerpo parcial o completamente incapaz de reconocer los androgenos que se producen, y por lo tanto permite el desarrollo de los genitales externos en la forma ocurre por defecto, es decir, siguiendo lineas femeninas. Otras condiciones relacionadas, causadas por distintos cromosomas, tambien interrumpen el camino normal de la accion de los androgenos, dando d4e nuevo como resultado un fenotipo femenino …

Intersex - Hermaphroditism
I.B.I.S. Birth Defects, September 2, 2003
Visitor Comments [Ukrainian]
A fact sheet for specialists in Ukrainian
Definition ... Clinical symptoms ... Classification ... Role of hormones ... Consection of sexual development ... Diagnosis and differential diagnosis ... Sexual development charts ... Diagnostic algorithm ... Determination of child's sex ... Treatment ... Care: psychological peculiarities ... Prenatal diagnosis and prevention ...

Intersex - Hermaphroditism
I.B.I.S. Birth Defects, August 27, 2003
Visitor Comments [Ukrainian]
A fact sheet for parents in Ukrainian
What is intersex ... Why does it happen ... How can sex of a child be determined ... Treatment possibilities ... Care for children with intersex ...

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Last Updated: 2008/1/10

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American Medical Association


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