On the word "hermaphrodite"
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
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
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
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
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.
Androgen Insensitivity Syndrome Support Group, June 9, 2006
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
there is no other commonly used term
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
pure XY gonadal dysgenesis, is the name given to a condition in which individuals possess a 46XY karyotype and female external genitalia. Women with Swyers 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
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
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 Swyers 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
[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 todays 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
assessment of immediate and extended family
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 childs 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
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
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
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
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
[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 patients self - described sexual identity was bisexual
Intersex Support Group International
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 Gods will
"Intersex" - Frequently Asked Questions
Intersex Society of North America
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 50s and 60s 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
dont 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 Support Group International, 2002
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
46,XY complete gonadal dysgenesis ???
46,XY partial gonadal dysgenesis???
Congenital adrenal hyperplasia 0.05/1000
Hypospadias 3.33/1000 males ...
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
David A. Hatch, M.D., 2003
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
MEDLINE plus, Health Information , October 11, 2007
Alternative names: hermaphrodite; intersexual; pseudohermaphroditism
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
- 4p syndrome
- 13q syndrome
HUMAN RIGHTS FOR INTERSEXUALS INTERSEX INDIVIDUALS DISPUTE WISDOM OF SURGERY ON INFANTS
Cheryl Chases 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 Chases 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 dont 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 isnt surprised by Grumbachs and Baskins 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] dont know what effect their drastic invasive, irreversible interventions are having
Endocrinologist Grumbach is skeptical of this viewpoint. If someone is telling you, you shouldnt modify the external genitalia, well, the parents wouldnt stand for it. They want it repaired!
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, its 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
Parents make decisions for their kids. Thats 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
dont 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.
scoffs at the idea that trained counselors arent 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 werent 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, Its 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 childs 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
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
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
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
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
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 lAIS
Androgen Insensitivity Syndrome Support Group
[Support Groups] [French]
Linsensibilite aux hormones androgenes (Androgen Insensitivity syndrome) entraine une interruption du developpement foetal de lappareil de reproduction. Un enfant atteint dAIS 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 dune 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
[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
[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
Intersex - Hermaphroditism
I.B.I.S. Birth Defects, September 2, 2003
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
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 ...
Last Updated: 2008/1/10