Gender reversal in 46XX congenital virilizing adrenal hyperplasia

Sripathi, V.; Ahmed, Syed Fayaz; Sakati, Nadia; Al-Ashwal, Abdullah A.

doi:10.1046/j.1464-410x.1997.00127.x

Cited by 24 publications

(13 citation statements)

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“…We found 12 studies on gender identity in 46,XX individuals raised male. Most studies included only one or two individuals, except those of Rösler and Leiberman (1984), Sripathi, Ahmed, Sakati, and al-Ashwal (1997), and Wölfle et al (2002). At the time of study, 4 (12.1%) out of these 33 chromosomal females raised male identified themselves as female (1 patient) or were gender dysphoric (3 patients).…”

Section: Resultsmentioning

confidence: 99%

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Gender Dysphoria and Gender Change in Chromosomal Females with Congenital Adrenal Hyperplasia

2005

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This article reviews the literature on studies and case reports on gender identity and gender identity problems, gender dysphoria, and gender change in chromosomal females with congenital adrenal hyperplasia, raised male or female. The large majority (94.8%) of the patients raised female (N= 250) later developed a gender identity as girls and women and did not feel gender dysphoric. But 13 (5.2%) patients had serious problems with their gender identity. This percentage is higher than the prevalence of female-to-male transsexuals in the general population of chromosomal females. Among patients raised male, serious gender identity problems were reported in 4 (12.1%) out of 33 patients. From these observations, we conclude that the assignment to the female gender as a general policy for 46,XX patients with CAH appears justified, even in severely masculinized 46,XX newborns with CAH (Prader stage IV or V).

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Section: Resultsmentioning

confidence: 99%

“…In two brothers (Sripathi et al, 1997), the disorder was recognized at birth but the parents insisted on male upbringing. d…”

Section: Discussionmentioning

confidence: 99%

Gender Dysphoria and Gender Change in Chromosomal Females with Congenital Adrenal Hyperplasia

2005

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“…However, the problem becomes impossible to solve with the concurrent development of masculine identity in delayed cases. It is also accepted that attitudes towards sex of rearing in latediagnosed patients with intersexual disorders in Eastern countries are very different from those in Europe [8][9][10][11][12][13]. Thus the aim of this study was to determine the factors governing the decision-making process in 70 genotypic females with CAH.…”

Section: Introductionmentioning

confidence: 99%

Gender assignment in female congenital adrenal hyperplasia: a difficult experience

Özbey¹,

Darendeliler²,

Kayserili

et al. 2004

BJU International

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OBJECTIVETo report experience of gender (re)assignment in genotypic female (46XX) patients with congenital adrenal hyperplasia (f‐CAH), a difficult and stressful experience if complicated with delayed presentation and inadvertent assignment.PATIENTS AND METHODSBetween 1983 and 2002, 70 patients with f‐CAH were counselled for gender assignment. The age at diagnosis and operation, the degree of virilization, parental consanguinity, the gender preference of the families, and the factors governing the decision‐making process were determined.RESULTSForty‐one (59%) patients presented after the neonatal period. All parents had already assumed or were advised of a gender for their children, based on the suggestive appearance of the external genitalia. Consequently, 49 patients were reared as female and 21 as ‘male’. Only nine of these ‘males’ could be reassigned as females (mean age at presentation 7.87 months, sd 10.42). Twelve children had to be reared as ‘male’ (mean age at presentation 55.8 months, sd 32.42) in compliance with the parents’ and the study group's decision, and appropriate masculinizing reconstructive surgery was undertaken. The difference in the mean age of those reassigned as female and those who remained ‘male’ was significant (P < 0.001). The parental consanguinity rate among the families was especially high in the ‘male’ patients.CONCLUSIONSIt is extremely difficult to correct the gender of patients with f‐CAH when they present at >2.5 years old. Furthermore, the delay in diagnosis and the male bias in choice of gender in our population might be a result of strong social pressures on families, influenced by cultural, traditional and economic factors.

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“…Reproduction comprised two subdomains: studies which provided documentation of either the frequency of pregnancy or frequency with which CAH-affected women carried the pregnancy to term were classified under Conception; all other measured outcomes dealing with reproductive function were classified under Fertility Status. Studies examining psychological factors that could not reliably be classified within another domain/subdomain were listed as “Other.” Examples of outcomes falling into this category included parental attitudes toward gender assignment [33, 34] or reactions to genital anomalies [28]. …”

Section: Methodsmentioning

confidence: 99%

Congenital Adrenal Hyperplasia: Classification of Studies Employing Psychological Endpoints

Stout

Литвак

Robbins

et al. 2010

International Journal of Pediatric Endocrinology

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Psychological outcomes in persons with congenital adrenal hyperplasia (CAH) have received substantial attention. The objectives of this paper were to (1) catalog psychological endpoints assessed in CAH outcome studies and (2) classify the conceptual/theoretical model shaping the research design and interpretation of CAH-related psychological effects. A total of 98 original research studies, published between 1955 and 2009, were categorized based on psychological endpoints examined as well as the research design and conceptual model guiding analysis and interpretation of data. The majority of studies (68%) investigated endpoints related to psychosexual differentiation. The preponderance of studies (76%) examined a direct relationship (i.e., inferring causality) between prenatal androgen exposure and psychological outcomes. Findings are discussed in relation to the observed imbalance between theoretical interest in the role of prenatal androgens in shaping psychosexual differentiation and a broader conceptual model that examines the role of other potential factors in mediating or moderating the influence of CAH pathophysiology on psychological outcomes in both affected females and males. The latter approach offers to identify factors amenable to clinical intervention that enhance both health and quality of life outcomes in CAH as well as other disorders of sex development.

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Gender reversal in 46XX congenital virilizing adrenal hyperplasia

Cited by 24 publications

References 0 publications

Gender Dysphoria and Gender Change in Chromosomal Females with Congenital Adrenal Hyperplasia

Gender Dysphoria and Gender Change in Chromosomal Females with Congenital Adrenal Hyperplasia

Gender assignment in female congenital adrenal hyperplasia: a difficult experience

Congenital Adrenal Hyperplasia: Classification of Studies Employing Psychological Endpoints

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