Simple virilizing congenital adrenal hyperplasia (CAH) is primarily caused by 21-hydroxylase deficiency, leading to prenatal virilization and postnatal masculinization without mineralocorticoid deficiency. Affected females may exhibit genital ambiguity at birth due to androgenic stimulation. The condition can influence psychosexual development, where females with CAH showed altered childhood play behaviors, potentially correlating with sexual orientation in adulthood.

1. Simple Virilizing Congenital Adrenal Hyperplasia The salient features of classical simple virilizing 21-OHD are prenatal virilization and progressive postnatal masculinization with accelerated growth and advanced bone ages but no evidence of mineralocorticoid deficiency 21-hydroxylase deficiency is most common form Causes build-up of certain intermediate hormones along the pathway toward cortisol Causes accumulation of 17-hydroxy progesterone and other hormones with androgenic properties

2. Two Types of Adrenogenital Syndrome 21  -hydroxylase deficiency 11  -hydroxylase deficiency Ganong, William F. REVIEW OF MEDICAL PHYSIOLOGY 22 nd ed. Lange Medical Books/McGraw-Hill, 2005

3. Simple Virilizing CAH Diagnosis at birth of a female with simple virilizing CAH usually is made immediately because of the apparent genital ambiguity In female embryos causes androgenic stimulation that results in enlargement of the genital tubercle, fusion of the internal and external urethral folds and fusion of the labio-scrotal folds just as normal production of testosterone does in male embryos Can result in a female infant having marked enlargement of the clitoris and fusion of the labioscrotal folds This 'virilization' can cause an infant with a 46-XX karyotype to have the appearance of having hypospadias and cryptorchidism

4. Five Degrees of Virilization Affecting the Urogenital Sinus and External Genitalia in Females Hines M, Brook C., Conway, G.S. Androgen And Psychosexual Development Core Gender Identity, Sexual Orientation And Recalled Childhood Gender Role Behavior In Women And Men With Congenital Adrenal Hyperplasiz (CAH). J SEX RES, 41: 75-81 (2004)

5. Congenital Adrenal Hyperplasia (CAH) Luria, Z., Friedman, S., and Rose, M.D. HUMAN SEXUALITY. New York: John Wiley & Sons (1987) Forbes, C.D. and Jackson, W.F. A COLOUR ATLAS AND TEXT OF CLINICAL MEDICINE. England: Mosby-Wolfe (1993)

6. Frequency of CAH Figure 4. Frequency of nonclassical and classical 21-hydroxylase deficiency in comparison with other autosomal recessive disease incidences Hines M, Brook C., Conway, G.S. Androgen And Psychosexual Development Core Gender Identity, Sexual Orientation And Recalled Childhood Gender Role Behavior In Women And Men With Congenital Adrenal Hyperplasiz (CAH). J SEX RES, 41: 75-81 (2004)

7. Adrenogenital Syndrome Luria, Z., Friedman, S., and Rose, M.D. HUMAN SEXUALITY. New York: John Wiley & Sons (1987)

8. Sexuality of CAH Women Recalled significantly more male-typical play behavior as children Reported significantly less satisfaction with the female sex of assignment and less heterosexual interest Results suggest those girls with CAH who show the greatest alterations in childhood play behavior may be the most likely to develop a bisexual or homosexual orientation as adults and to be dissatisfied with the female sex of assignment Hines M, Brook C., Conway, G.S. Androgen And Psychosexual Development Core Gender Identity, Sexual Orientation And Recalled Childhood Gender Role Behavior In Women And Men With Congenital Adrenal Hyperplasia (CAH). J SEX RES, 41: 75-81 (2004)

9. Sexuality of CAH Women The finding of greater same-sex fantasy among CAH women than among comparison women is consistent with the prenatal hormonal hypothesis* However, most CAH women were categorized as exclusively heterosexual in fantasy and behavior* "These results suggest that excess prenatal androgens predispose some women to the development of same-gender sexual orientation”** “ However, because CAH is a relatively rare condition, and a majority develop a heterosexual orientation, CAH plays a minor role in the overall variability of women's sexual orientation within the general population”** *Veniegas, Rosemary C. Biological Research on Women's Sexual Orientations: Evaluating the Scientific Evidence. J SOCIAL ISSUES, (2000) **Pattatucci, A. M. L., & Hamer, D. H. Development And Familiality Of Sexual Orientation In Females. BEHAVIOR GENETICS, 25: 407-420 (1995)