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{{Congenital adrenal hyperplasia due to 21-hydroxylase deficiency }} | {{Congenital adrenal hyperplasia due to 21-hydroxylase deficiency }} | ||
{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}} | {{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}} {{AAM}} | ||
==Overview== | ==Overview== | ||
Congenital adrenal hyperplasia was first discovered by Luigi De Crecchio, an Italian anatomist. | Congenital adrenal hyperplasia was first discovered by Luigi De Crecchio, an Italian anatomist. | ||
==Historical Perspective== | ==Historical Perspective== | ||
* Until the 1950s, some virilized XX infants were assigned and raised as girls, and some as boys. Most developed gender identities congruent with their sex of rearing. In a few cases of male rearing, a [[sex reassignment]] was attempted in mid-childhood when newly discovered karyotyping revealed "female" chromosomes. These reassignments have been rarely successful, leading John Money and other influential psychologists and physicians to conclude that gender identity was | * Until the 1950s, some virilized XX infants were assigned and raised as girls, and some as boys. Most developed gender identities congruent with their sex of rearing. In a few cases of male rearing, a [[sex reassignment]] was attempted in mid-childhood when newly discovered karyotyping revealed "female" chromosomes. These reassignments have been rarely successful, leading John Money and other influential psychologists and physicians to conclude that gender identity was: | ||
:*Unrelated to chromosomes | |||
:*Primarily a result of social learning | |||
:*Could not be easily changed after infancy | |||
* In the 1950s and 1960s, surgery often involved clitorectomy (removal of most of the clitoris), an operation that also reduced genital sensation. In the 1970s, new operating methods were developed to preserve innervation and clitoral function. However, a number of retrospective surveys in the last decade suggest that | * In the 1950s and 1960s, surgery often involved clitorectomy (removal of most of the clitoris), an operation that also reduced genital sensation. In the 1970s, new operating methods were developed to preserve innervation and clitoral function. However, a number of retrospective surveys in the last decade suggest that: | ||
:*Sexual enjoyment is reduced in many women even after nerve-sparing procedures | |||
:*Women with congenital adrenal hyperplasia who have not had surgery also have a substantial rate of sexual dysfunction | |||
Many patient advocates and surgeons argue for deferring surgery until adolescence or later, while some surgeons continue to argue that infant surgery has advantages. | |||
* By the 1960s, congenital adrenal hyperplasia was well understood, [[karyotype|karyotyping]] was routine, and standard management was to assign and raise all children with congenital adrenal hyperplasia according to their [[gonad]]s and karyotypes, no matter how virilized. Markedly virilized girls were usually referred to a [[pediatric surgery|pediatric surgeon]], often a [[pediatric urology|pediatric urologist]] for a [[intersex surgery|reconstructive Vaginoplasty and clitoral reduction]] or recession—surgery to create or enlarge a vaginal opening and reduce the size or protrusion of the clitoris. This approach was designed to preserve fertility for both sexes and remains the standard management, but two aspects of this management have been challenged: assignment of completely virilized genetic females and the value and age of corrective surgery. | * By the 1960s, congenital adrenal hyperplasia was well understood, [[karyotype|karyotyping]] was routine, and standard management was to assign and raise all children with congenital adrenal hyperplasia according to their [[gonad]]s and karyotypes, no matter how virilized. Markedly virilized girls were usually referred to a [[pediatric surgery|pediatric surgeon]], often a [[pediatric urology|pediatric urologist]] for a [[intersex surgery|reconstructive Vaginoplasty and clitoral reduction]] or recession—surgery to create or enlarge a vaginal opening and reduce the size or protrusion of the clitoris. This approach was designed to preserve fertility for both sexes and remains the standard management, but two aspects of this management have been challenged: assignment of completely virilized genetic females and the value and age of corrective surgery. | ||
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* The first questions about assignment were raised in the early 1980s, when Money and others reported an unexpectedly high rate of failure to achieve normal adult sexual relationships (i.e., heterosexual orientation, marriage, and children) in grown women with congenital adrenal hyperplasia (though all had female gender identities). However, the sample was small, and the results seemed interpretable in many ways: selection bias, early hormone effects on orientation, sexual dysfunction created by residual body abnormalities, or by the genital surgery itself. From a perspective two decades later, the report was one of the first pieces of evidence that the standard management paradigm was not always producing hoped-for outcomes. | * The first questions about assignment were raised in the early 1980s, when Money and others reported an unexpectedly high rate of failure to achieve normal adult sexual relationships (i.e., heterosexual orientation, marriage, and children) in grown women with congenital adrenal hyperplasia (though all had female gender identities). However, the sample was small, and the results seemed interpretable in many ways: selection bias, early hormone effects on orientation, sexual dysfunction created by residual body abnormalities, or by the genital surgery itself. From a perspective two decades later, the report was one of the first pieces of evidence that the standard management paradigm was not always producing hoped-for outcomes. | ||
* Despite these concerns, no significant opposition to standard management arose until the mid-1990s, when a confluence of evidence and opinion from several sources led to a re-examination of outcomes. Several intersex support and advocacy groups (e.g., Intersex Society of North America) began to publicly criticize infant genital surgery based on unsatisfactory outcomes of some adults who had been operated on as infants. Their complaints were that they had reduced ability to enjoy sexual relations or that they resented not having had the choice of gender assignment or surgical reconstruction left until they were old enough to participate. | * Despite these concerns, no significant opposition to standard management arose until the mid-1990s, when a confluence of evidence and opinion from several sources led to a re-examination of outcomes. Several intersex support and advocacy groups (e.g., Intersex Society of North America) began to publicly criticize infant genital surgery based on unsatisfactory outcomes of some adults who had been operated on as infants. Their complaints were that they had reduced ability to enjoy sexual relations or that they resented not having had the choice of gender assignment or surgical reconstruction left until they were old enough to participate. | ||
* In 1997, influential articles by Reiner, Diamond, and Sigmundson advocated ''consideration'' of | * In 1997, influential articles by Reiner, Diamond, and Sigmundson advocated ''consideration'' of: | ||
:*Male sex assignment in the unambiguously male XX infants (most of whom are considered male until the CAH is recognized at 1-2 weeks of age) | |||
:*Delaying reconstructive surgery until the patient is old enough to participate in the decision | |||
==References== | ==References== | ||
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[[Category:Genetic disorders]] | [[Category:Genetic disorders]] | ||
[[Category:Intersexuality]] | [[Category:Intersexuality]] | ||
{{WikiDoc Help Menu}} | {{WikiDoc Help Menu}} | ||
{{WikiDoc Sources}} | {{WikiDoc Sources}} | ||
Revision as of 18:27, 14 September 2015
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Congenital adrenal hyperplasia due to 21-hydroxylase deficiency Microchapters |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2] Ahmad Al Maradni, M.D. [3]
Overview
Congenital adrenal hyperplasia was first discovered by Luigi De Crecchio, an Italian anatomist.
Historical Perspective
- Until the 1950s, some virilized XX infants were assigned and raised as girls, and some as boys. Most developed gender identities congruent with their sex of rearing. In a few cases of male rearing, a sex reassignment was attempted in mid-childhood when newly discovered karyotyping revealed "female" chromosomes. These reassignments have been rarely successful, leading John Money and other influential psychologists and physicians to conclude that gender identity was:
- Unrelated to chromosomes
- Primarily a result of social learning
- Could not be easily changed after infancy
- In the 1950s and 1960s, surgery often involved clitorectomy (removal of most of the clitoris), an operation that also reduced genital sensation. In the 1970s, new operating methods were developed to preserve innervation and clitoral function. However, a number of retrospective surveys in the last decade suggest that:
- Sexual enjoyment is reduced in many women even after nerve-sparing procedures
- Women with congenital adrenal hyperplasia who have not had surgery also have a substantial rate of sexual dysfunction
Many patient advocates and surgeons argue for deferring surgery until adolescence or later, while some surgeons continue to argue that infant surgery has advantages.
- By the 1960s, congenital adrenal hyperplasia was well understood, karyotyping was routine, and standard management was to assign and raise all children with congenital adrenal hyperplasia according to their gonads and karyotypes, no matter how virilized. Markedly virilized girls were usually referred to a pediatric surgeon, often a pediatric urologist for a reconstructive Vaginoplasty and clitoral reduction or recession—surgery to create or enlarge a vaginal opening and reduce the size or protrusion of the clitoris. This approach was designed to preserve fertility for both sexes and remains the standard management, but two aspects of this management have been challenged: assignment of completely virilized genetic females and the value and age of corrective surgery.
- The first questions about assignment were raised in the early 1980s, when Money and others reported an unexpectedly high rate of failure to achieve normal adult sexual relationships (i.e., heterosexual orientation, marriage, and children) in grown women with congenital adrenal hyperplasia (though all had female gender identities). However, the sample was small, and the results seemed interpretable in many ways: selection bias, early hormone effects on orientation, sexual dysfunction created by residual body abnormalities, or by the genital surgery itself. From a perspective two decades later, the report was one of the first pieces of evidence that the standard management paradigm was not always producing hoped-for outcomes.
- Despite these concerns, no significant opposition to standard management arose until the mid-1990s, when a confluence of evidence and opinion from several sources led to a re-examination of outcomes. Several intersex support and advocacy groups (e.g., Intersex Society of North America) began to publicly criticize infant genital surgery based on unsatisfactory outcomes of some adults who had been operated on as infants. Their complaints were that they had reduced ability to enjoy sexual relations or that they resented not having had the choice of gender assignment or surgical reconstruction left until they were old enough to participate.
- In 1997, influential articles by Reiner, Diamond, and Sigmundson advocated consideration of:
- Male sex assignment in the unambiguously male XX infants (most of whom are considered male until the CAH is recognized at 1-2 weeks of age)
- Delaying reconstructive surgery until the patient is old enough to participate in the decision