46,XX Patients with Congenital Adrenal Hyperplasia: Initial Assignment as Male, Reassigned Female

Six 46,XX patients with congenital adrenal hyperplasia (CAH) presented with genital ambiguity, five so severe that initial gender assignment was male. Once diagnosis was realized, parents were involved in evaluation and chose sex re-assignment as female. To date, these girls and their parents all indicate satisfaction with their decision for a female sex of rearing. The girls have a female gender identity with behavior characteristics known for females with CAH. Thus, while outcome is satisfactory, it is realized that for most, expression of sexual orientation and adult life adjustments have not yet occurred.

[1]  M. Bullinger,et al.  Outcome of congenital adrenal hyperplasia , 1997, Pediatric Surgery International.

[2]  A. Wisniewski,et al.  Psychosexual outcome in women affected by congenital adrenal hyperplasia due to 21-hydroxylase deficiency. , 2004, The Journal of urology.

[3]  Peter A. Lee.  A Perspective on the Approach to the Intersex Child Born with Genital Ambiguity , 2004, Journal of pediatric endocrinology & metabolism : JPEM.

[4]  C. Woodhouse,et al.  The effect of clitoral surgery on sexual outcome in individuals who have intersex conditions with ambiguous genitalia: a cross-sectional study , 2003, The Lancet.

[5]  J. Bailey,et al.  Effects on gender identity of prenatal androgens and genital appearance: evidence from girls with congenital adrenal hyperplasia. , 2003, The Journal of clinical endocrinology and metabolism.

[6]  G. Bohlin,et al.  Sex-typed toy play behavior correlates with the degree of prenatal androgen exposure assessed by CYP21 genotype in girls with congenital adrenal hyperplasia. , 2002, The Journal of clinical endocrinology and metabolism.

[7]  J. Hutson,et al.  Androgen imprinting of the brain in animal models and humans with intersex disorders: review and recommendations. , 2002, The Journal of urology.

[8]  C. Minto,et al.  Objective cosmetic and anatomical outcomes at adolescence of feminising surgery for ambiguous genitalia done in childhood , 2001, The Lancet.

[9]  H. Meyer-Bahlburg.  Gender and sexuality in classic congenital adrenal hyperplasia. , 2001, Endocrinology and metabolism clinics of North America.

[10]  S. Berenbaum,et al.  Behavioral effects of prenatal versus postnatal androgen excess in children with 21-hydroxylase-deficient congenital adrenal hyperplasia. , 2000, The Journal of clinical endocrinology and metabolism.

[11]  I. McAleer,et al.  Sex assignment in cases of ambiguous genitalia and its outcome. , 2000, Urology.

[12]  K. Zucker,et al.  Psychosexual Development of Women with Congenital Adrenal Hyperplasia , 1996, Hormones and Behavior.

[13]  Maria I. New,et al.  Gender Change from Female to Male in Classical Congenital Adrenal Hyperplasia , 1996, Hormones and Behavior.