Childhood Sex-Typed Behavior and Gender Change in Individuals with 46,XY and 46,XX Disorders of Sex Development: An Iranian Multicenter Study

• Published in: Archives of sexual behavior 2018-11, Vol.47 (8), p.2287-2298
• Main Authors: Khorashad, Behzad S., Roshan, Ghasem M., Reid, Alistair G., Aghili, Zahra, Moghadam, Maliheh Dadgar, Khazai, Behnaz, Hiradfar, Mehran, Afkhamizadeh, Mozhgan, Ghaemi, Nosrat, Talaei, Ali, Abbaszadegan, Mohammad Reza, Aarabi, Azadeh, Dastmalchi, Samira, Van de Grift, Tim C.
• Format: Article
• Language: English
• Subjects: 3-Oxo-5-alpha-Steroid 4-Dehydrogenase - deficiency; 3-Oxo-5-alpha-Steroid 4-Dehydrogenase - genetics; 3-Oxo-5-alpha-Steroid 4-Dehydrogenase - metabolism; Adolescent; Adrenal Hyperplasia, Congenital - genetics; Adrenal Hyperplasia, Congenital - metabolism; Adrenal Hyperplasia, Congenital - physiopathology; Adult; Age; Androgen-Insensitivity Syndrome - genetics; Androgen-Insensitivity Syndrome - metabolism; Androgen-Insensitivity Syndrome - physiopathology; Androgens; Androgens - metabolism; Behavioral Science and Psychology; Child; Child Behavior; Child, Preschool; Childhood; Congenital diseases; Differences of sex development; Disorder of Sex Development, 46,XY - genetics; Disorder of Sex Development, 46,XY - metabolism; Disorder of Sex Development, 46,XY - physiopathology; Female; Females; Gender Identity; Humans; Hyperplasia; Hypospadias - genetics; Hypospadias - metabolism; Hypospadias - physiopathology; Iran; Male; Original Paper; Psychology; Public Health; Retrospective Studies; Self Report; Sex Characteristics; Sex Differentiation; Sexual Behavior; Sexual Development; Social Sciences; Steroid Metabolism, Inborn Errors - genetics; Steroid Metabolism, Inborn Errors - metabolism; Steroid Metabolism, Inborn Errors - physiopathology
• ISSN: 0004-0002; 1573-2800
• DOI: 10.1007/s10508-018-1281-9

Disorders of sex development (DSD) are congenital conditions in which the typical genetic and hormonal profiles are affected and thereby the usual process of sexual differentiation. Most of these studies, however, have been conducted in Western countries. In the present study, preschool sex-typed activities of Iranian individuals with DSD and their age-matched non-affected male and female relatives were assessed using the Pre-School Activities Inventory (PSAI) modified for retrospective self-report. A total of 192 individuals participated in our study, including 33 46,XX individuals with congenital adrenal hyperplasia (CAH; M age = 10.36, SD = 5.52), 15 46,XY individuals with complete androgen insensitivity syndrome (CAIS; M age = 19.8, SD = 7.14), and 16 46,XY individuals with 5-alpha reductase deficiency type-2 (5α-RD-2; M age = 17.31, SD = 7.28), as well as one age-matched non-affected male and female relative for each patient. With regard to PSAI scores, male-identifying participants with 5α-RD-2 and male controls reported similar levels of male-typical childhood play. Female-identifying participants with 5α-RD-2 and CAH showed comparable scores: significantly less masculine and more feminine than male controls, but significantly more masculine and less feminine than females with CAIS and female controls. These findings support the role of androgens in the development of sex-typical childhood play behavior, with those being exposed to higher levels of fetal functional androgens expressing more masculine behavior at preschool ages.