Gender and Identity: Process, Roles, and Culture Turner syndrome can be diagnosed at any stage of life. It may be diagnosed before birth if a chromosome analysis is done during prenatal testing. The doctor will perform a physical exam and look for signs of poor development, as infants with Turner syndrome often have more serious physical symptoms (compared to infants with Klinefelter syn-drome) though a wide range of symptoms exists. Infants with Turner syndrome often have swollen hands and feet. Other common symptoms or developmental indicators are having absent or incomplete pubertal development, a broad flat chest, a short height, lack of menstrual periods, or a wide webbed neck. Estrogen replacement therapy starting at around age 12 may greatly help the child's development and puberry. 5-Alpha-Reductase Deficiency The rare condition 5-alpha-reductase deficiency occurs in genetic males. In developed societies such as the United States, this biological con-dition occurs at the rate of 1 in 200,000 individuals, but in communities that experi-ence inbreeding, it can be as high as 1 in 200 (Imperato-McGinley et al., 1974). This condition does not allow a fetus to convert testosterone to dihydrotestosterone (DHT), which is necessary for the development of male genitalia. The absence of DHT tends to result in ambiguous genitalia at birth; there may be a small or microscopic penis with testes and a vagina and labia. The small penis is capable of ejaculation but it looks like a clitoris at birch. This results in many of these boys being raised as girls. When addi-tional sex hormones are released during puberty around age 11 or 12, these boys' genitals develop to look more like an adulc male penis and testes, though smaller. To read about chis condition in two areas of the world, see "Research and Sexual Well-Being: Studies of 5-Alpha-Reductase Deficiency." Additionally, see Table 9.2 for a summary of the intersex conditions just discussed. Treatment of People Who Are lntersex It is interesting to note how different culcures react to people who are intersex. Navaho Indians, for example, regard intersex people as powerful or as having special healing or shamanic qualities (Edgerton, 1964). In l 9th-century Western society, we see how one person was treated in the moving memoirs of Herculine Barbin, a French girl who later developed male genitals and sexual interest in Ta ble 9.2 Summary of biological intersex variations CHROMOSOMES xx xx XY XXY XO XY BIOLOGICAL INTERSEX VARIATION Congenital adrenal hyperplasia (CAH) Progestin-induced virilization Androgen insensitivity syndrome (AIS) Klinefelter syndrome Turner syndrome 5-alpha-reductase deficiency DESCRIPTION The most common cause of sexual ambiguity in individuals with XX sex chromosomes.