Review of Outcome Information in 46,XX Patients with Congenital Adrenal Hyperplasia Assigned/Reared Male: What Does It Say about Gender Assignment?

Hdl Handle:
http://hdl.handle.net/10675.2/619
Title:
Review of Outcome Information in 46,XX Patients with Congenital Adrenal Hyperplasia Assigned/Reared Male: What Does It Say about Gender Assignment?
Authors:
Lee, Peter A.; Houk, Christopher Phil
Abstract:
There is ample historical verification of 46,XX congenital adrenal hyperplasia (CAH) patients being born with essentially male genitaliawhile outcome information is scant. Prior to glucocorticoid therapy, most patients died very young from adrenal insufficiency. Most available reports from laterchildhood, contain little information concerning sexual identity. Reports on older individuals lack adequate information about sexual identity and quality of life. The difficulty in assessing the relative impact of multiple dynamic environmental factors on the development of sexual identity, self- and body esteem and overall adjustment to life is clear. Nevertheless, it remains unclear whether those infants whose masculine genitalia at birth resulted in an initial male assignment would have enjoyed a better adult outcome had they been allowed to remain male rather than the female reassignment that most received. Further, one could ask whether a male sex of rearing should be considered in 46,XX CAH infants with male external genitalia. After reviewing available literature, we conclude that because those extremely virlized 46,XX CAH patients who were reared male with healthy social support demonstrated satisfactory levels of social and sexual function as adults a male sex assignment should be considered in these types of infants when social and cultural environment are supportive.
Citation:
Int J Pediatr Endocrinol. 2010 Dec 21; 2010:982025
Issue Date:
21-Dec-2010
URI:
http://hdl.handle.net/10675.2/619
DOI:
10.1155/2010/982025
PubMed ID:
21197442
PubMed Central ID:
PMC3010645
Type:
Article
ISSN:
1687-9856
Appears in Collections:
Department of Pediatrics: Faculty Research and Presentations

Full metadata record

DC FieldValue Language
dc.contributor.authorLee, Peter A.en_US
dc.contributor.authorHouk, Christopher Philen_US
dc.date.accessioned2012-10-26T16:26:54Z-
dc.date.available2012-10-26T16:26:54Z-
dc.date.issued2010-12-21en_US
dc.identifier.citationInt J Pediatr Endocrinol. 2010 Dec 21; 2010:982025en_US
dc.identifier.issn1687-9856en_US
dc.identifier.pmid21197442en_US
dc.identifier.doi10.1155/2010/982025en_US
dc.identifier.urihttp://hdl.handle.net/10675.2/619-
dc.description.abstractThere is ample historical verification of 46,XX congenital adrenal hyperplasia (CAH) patients being born with essentially male genitaliawhile outcome information is scant. Prior to glucocorticoid therapy, most patients died very young from adrenal insufficiency. Most available reports from laterchildhood, contain little information concerning sexual identity. Reports on older individuals lack adequate information about sexual identity and quality of life. The difficulty in assessing the relative impact of multiple dynamic environmental factors on the development of sexual identity, self- and body esteem and overall adjustment to life is clear. Nevertheless, it remains unclear whether those infants whose masculine genitalia at birth resulted in an initial male assignment would have enjoyed a better adult outcome had they been allowed to remain male rather than the female reassignment that most received. Further, one could ask whether a male sex of rearing should be considered in 46,XX CAH infants with male external genitalia. After reviewing available literature, we conclude that because those extremely virlized 46,XX CAH patients who were reared male with healthy social support demonstrated satisfactory levels of social and sexual function as adults a male sex assignment should be considered in these types of infants when social and cultural environment are supportive.en_US
dc.rightsCopyright © 2010 P. A. Lee and C. P. Houk.en_US
dc.subjectReview Articleen_US
dc.titleReview of Outcome Information in 46,XX Patients with Congenital Adrenal Hyperplasia Assigned/Reared Male: What Does It Say about Gender Assignment?en_US
dc.typeArticleen_US
dc.identifier.pmcidPMC3010645en_US
dc.contributor.corporatenameDepartment of Pediatrics-

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