Androgen Insensitivity Syndrome is a cause of pseudohermaphroditism in males in which the child has male internal sex organs but have ambiguous external genitalia.
Children with Androgen Insensitivity Syndrome have:
- Normal male chromosomes
- Normal female external genitalia
This is called androgen insensitivity syndrome because male babies don’t respond to testosterone (androgens). This syndrome is inherited. It is caused by a problem with a gene on the X chromosome. This problem is called X-linked recessive. Mothers who carry the gene have a 1 in 2 chance of having a son with the syndrome. Daughters of mothers who carry the gene have a 1 in 2 chance of being carriers of the gene.