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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 …

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.

Androgen Insensitivity Syndrome Support Group
"We are an international support group providing information and support to young people and adults with complete and partial Androgen Insensitivity Syndrome, and to parents of AIS youngsters. 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."

The UK Intersex Association
The United Kingdom Intersex Association (UKIA) is an education, advocacy, campaigning and support organisation which works on behalf of Intersex people. Intersex people are individuals whose anatomy or physiology differ from contemporary cultural stereotypes of what constitute typical male and female.

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 …

"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 …

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 …

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 …

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” …

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 …

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

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 ...


Last Updated: 2023/04/03


American Medical Association