The adrenogenital syndrome is the most common childhood adrenal disorder, occurring in approximately 1 out of 50,000 births. Congenital adrenogenital syndrome is familial and tends to occur in sisters and brothers.
When the disorder occurs in males, it is called macrogenitosomia praecox. After the child’s first birthday, there is enlargement of the penis as well as the appearance of frequent erections. The body contour matures early and the child develops hair in the pubic area and under the arms as early as age three. However, growth is affected and, if the condition is left untreated, the child is short and the testicles remain small when the child reaches puberty. Furthermore, he does not produce sperm, and therefore is sterile.
In females, the condition is called pseudohermaphroditism, ‘The child has ovaries and a uterus, but also external masculine characteristics, including an enlarged clitoris. However, the female sex is positively identified by a “buccal smear (an examination of chromosomes in tissue scratched from the inside of the cheek).