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Pseudohermaphroditism

An intersexual or intersex person (or animal of any unisexual species) is one who is born with genitalia and/or secondary sex characteristics determined as neither exclusively male nor female, or which combine features of the male and female sexes. more...

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(The terms hermaphrodite and pseudohermaphrodite, which have been used in the past, are now considered pejorative and inaccurate and are no longer used to refer to an intersexual person.) Sometimes the phrase "ambiguous genitalia" is used.

Overview

According to the highest estimates (Fausto-Sterling et. al., 2000) perhaps 1 percent of live births exhibit some degree of sexual ambiguity , and that between 0.1% and 0.2% of live births are ambiguous enough to become the subject of specialist medical attention, including surgery to disguise their sexual ambiguity. Other sources (Leonard Sax, 2002) estimate the incidence of true intersexual conditions as far lower, at approximately 0.018%.

In typical fetal development, the presence of the SRY gene causes the fetal gonads to become testes; the absence of it allows the gonads to continue to develop into ovaries. Thereafter, the development of the internal reproductive organs and the external genitalia is determined by hormones produced by certain fetal gonads (ovaries or testes) and the cells' response to them. The initial appearance of the fetal genitalia (a few weeks after conception) is basically feminine: a pair of "urogenital folds" with a small protuberance in the middle, and the urethra behind the protuberance. If the fetus has testes, and if the testes produce testosterone, and if the cells of the genitals respond to the testosterone, the outer urogenital folds swell and fuse in the midline to produce the scrotum; the protuberance grows larger and straighter to form the penis; the inner urogenital swellings swell, wrap around the penis, and fuse in the midline to form the penile urethra.

Because there is variation in all of these processes, a child can be born with a sexual anatomy that is typically female, or feminine in appearance with a larger than average clitoris; or typically male, masculine in appearance with a smaller than average penis that is open along the underside. The appearance may be quite ambiguous, describable as female genitals with a very large clitoris and partially fused labia, or as male genitals with a very small penis, completely open along the midline ("hypospadic"), and empty scrotum.

There are dozens of named medical conditions that may lead to intersex anatomy. Fertility is variable. The distinctions "male pseudohermaphrodite", "female pseudohermaphrodite" and especially "true hermaphrodite" are vestiges of 19th century thinking that placed "true sex" in the histology (microscopic appearance) of the gonads.

The common habit in the 21st century of elevating the role of the sex chromosomes above all other factors when determining gender may be analogous to the older habit of finding "true" sex in the gonads. Though high school biology teaches that men have XY and women XX chromosomes, in fact there are quite a few other possible combinations such as Turner_syndrome XO, Triple-X syndrome XXX, Klinefelter's Syndrome XXY, XYY, XO/XY, XX male, Swyer syndrome XY female, and there are many individuals who do not follow the typical patterns (such as cases with four or even more sex chromosomes).

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A Lovemap of a Different Sort from John Money - A First Person History of Pediatric Psychoendocrinology - Book Review
From Journal of Sex Research, 2/1/04 by Tom Mazur

A First Person History of Pediatric Psychoendocrinology. By John Money. New York: Kluwer Academic/Plenum Publishers, 2002, 126 pages. Cloth, $55.00.

The autobiography is always the story of one's own journey. In A First Person History of Pediatric Psychoendocrinology, John Money shares with us his personal professional journey of over 50 years at The Johns Hopkins Hospital where, for 44 consecutive years, his work has been supported by federal government grants. After reading about his journey, one realizes that he has left us a map to follow--not surprising for John Money. Besides being a clinician, researcher, and writer, he is a teacher and a pioneer, most notably in the fields of psychoendocrinology and sexology.

All good pioneers who are also teachers leave maps ("leave tracks in the snow," he would often say) of where they have been, what they have explored, and what they have found. The maps are not always accurate; to be sure, there are errors and inaccuracies to be discovered by those who come after. Sucessors may also confirm and validate the accurate parts of the original map. Money's map will be revised and updated over time as it continues to serve as a guide for those who decide to pursue the domains of psychoendocrinology and sexology. Most of the material found here will not be new to those familiar with Money's work. The research studies and his thoughts have been previously published in various texts and journals. This is not to say that there is nothing new.

Undoubtedly, chapter 6 will be the first chapter read because it is here that Money speaks his mind publicly for the first time about the much-publicized case of David Reimer, also known as John/Joan. One of a set of identical genetic male twins, David's penis was completely burned off by electric cautery at 7 months of age. Because Money and his colleagues' previous work with cases of human hermaphroditism showed successful outcomes for individuals assigned and reared in a gender discordant with that of their gonads, David was brought to Hopkins at 17 months of age. In David's case, such work suggested the possibility--and the hope--of a successful outcome if he was reared female, which would involve removal of the testes, appropriate female hormonal therapy to induce a feminizing puberty, and eventual vaginal surgery. With this hope, David's parents assigned him a female status at 17 months of age, and the first stage of genital surgery occurred at 22 months of age. However, follow-up years later by Milton Diamond indicated that even though the child lived for years as a girl, he never identified as one, and eventually self-reassigned as a male.

David Reimer's history launched a dispute both inside and outside professional circles about psychosexual developmental theory, Money's work, the traditional medical management of infants born with ambiguous genitalia, and (although not entirely founded; see Money, 1991; Zucker, 1999) the lack of adequate long-term follow-up studies of individuals born with ambiguous genitalia. The dispute, both in scholarly print and in the mass media, inflamed the ongoing debate of "nature versus nurture." Furthermore, it became personal. In his account of the case, Colapinto (1997, 2000) pits Milton Diamond and John Money against one another. This might explain in part Money's title for chapter 6, "David and Goliath."

Throughout the period of intense media coverage, Dr. Money remained silent, preferring "... to allow the fire to burn itself out rather than add fuel to the flames by engaging in adversarial argument in print or television" (p. 76). He finally breaks this silence in chapter 6, in which he chronicles his involvement in the Reimer case and calls into question the accuracy of Colapinto's reporting of events. He also expresses his belief not only that Colapinto's reporting was inaccurate, but also that he resurrected and promoted the David and Goliath myth for "financial gain" (p. 75). Money goes on to defend himself as being no "Goliath to anyone's Dr. Frankenstein in search of a monster to construct" (p. 75).

Money's very personal response ("...having been sprayed by the blinding venom of a spitting cobra" [p. 76]) is understandable. He has been made to appear the villain. Paradoxically, this portrayal pays tribute to his stature and his pioneering efforts in the field. One does not make a villain of a nobody! This not withstanding, I would have liked to see Money bring to this issue the same analytical skill he demonstrates in his search for the origins of sexual attraction (amative orientation) in human beings (chapter 4).

It can appear to many that the John/Joan case is testimony that the traditional way to manage intersex infants is completely wrong and that in the end the explanation is straightforward and simple: We should remain "as nature made us." These conclusions are not only overly simplistic but are also based on a single case that does not even involve a child born with genital ambiguity, thus limiting their generalizability. These limitations must be taken into account when using the John/Joan case as a model in the debate about sex assignment in intersex individuals, as has been aptly described by Meyer-Bahlburg (1999).

Furthermore, I wonder how many people are aware that there is another case of ablatio penis where the outcome is different from that of John/Joan? Bradley, Oliver, Chernick, and Zucker (1998) interviewed a patient at 16 years and 26 years who, as an infant, had the penis burned off by electrocautry during a circumcision procedure. Although tomboyish as a child and bisexual as an adult, this person has maintained a female gender identity.

In the end, this debate is not about David or Goliath but about how, in the face of imperfect knowledge, we can provide the best possible treatment, and how best to deliver that treatment to infants born with genital ambiguity and to their families. The lesson is humility in the face of a complex and profound dimension of human existence--that of the formation of one's psychosexual status (gender identity, gender role, and sexual orientation).

It would be unfortunate if chapter 6 is the only chapter read, or if this controversy overshadows the larger legacy left to us by this man who accomplished so much through persistence and singleness of purpose. Money, always the teacher, tells us that he selected the material for the 11 chapters of his book to illustrate certain themes that he deemed "conceptually significant" in his development as a pediatric psychoendocrinologist. It is this selective aspect of the book, along with such interesting information as how he met Dr. Lawson Wilkins and came to work at The Johns Hopkins Hospital and why his dream of establishing an institute of pediatric psychoendocrinology and sexology never materialized, that gives us what series editor Richard Green describes as an "autobiographical dimension that delivers a document not elsewhere available."

Money's pioneering efforts are far-ranging because the themes important to his career development emerged from what basic science provided him with at the time. As he states, "Early on, one proviso that I made was to pick only plums that were ripe for the proverbial picking, by which I meant that the basic science of a syndrome should be well enough advanced to yield an answer, positive or negative, to a conceptually significant psychoendocrine question" (p. 2). In current athletic coinage, he let the game come to him. Then he observed, systematically collected data, and recorded and classified it. Such an approach was not as common in clinical psychology 50 years ago as it is today. The results were new concepts, new language, and information of practical value to both clinicians and patients.

Examples of a few of the research "plums" he picked include the various syndromes of hermaphroditism, which taught him that the concept of sex is multidimensional and from which came the terms gender identity and gender role (or as he originally coined it, gender identity/role); his work in behavioral cytogenetics with individuals with chromosomal variants (i.e., XXY, XYY, and X0); and the concept of paraphilia and how lovemaps (another term coined by him) become normative or vandalized. One comes away from Money's book with the impression that, regardless of the puzzle before him, he worked with a driving tension created by a desire to fit the pieces into a whole while not falling into the traditional nature-nurture debate. Chapter 4, on the search for the origins of sexual attraction between individuals using human psychoendocrine syndromes, is a good example.

During his productive career, John Money received many accolades, including being recognized as one of the pioneers of pediatric psychology. After reading this book, one can better appreciate the praise and understand why those working in psychoendocrinology and sexology need to become aware of the map he has left us.

REFERENCES

Bradley, S. J., Oliver, G. D., Chernick, A. B., & Zucker, K. J. (1998). Experiment of nurture: Ablatio penis at 2 months, sex reassignment at 7 months, and a psychosexual follow-up in young adulthood. Pediatrics, 102, E91-E95.

Colapinto, J. (1997, December 11). The true story of John/Joan. Rolling Stone, p. 55ff.

Colapinto, J. (2000). As nature made him: The boy who was raised as a girl. New York: Harper Collins.

Meyer-Bahlburg, H. E L. (1999). Gender assignment and reassignment in 46,XY pseudohermaphroditism and related conditions. Journal of Clinical Endocrinology & Metabolism, 84, 3455-3458.

Money, J. (1991). Biographies of gender and hermaphroditism in paired comparisons. Amsterdam: Elsevier.

Zucker, K. J. (1999). Intersexuality and gender identity differentiation. Annual Review of Sex Research, 10, 1-69.

Reviewed by Tom Mazur, Psy.D., The Women and Children's Hospital of Buffalo, 219 Bryant Street, Buffalo, NY 14222; e-mail: tamazur@acsu.buffalo.edu.

COPYRIGHT 2004 Society for the Scientific Study of Sexuality, Inc.
COPYRIGHT 2004 Gale Group

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