Androgen Insensitivity Syndrome (AIS) Partial, also known as Partial Androgen Insensitivity Syndrome (PAIS), is a rare genetic disorder that affects sexual development in individuals with XY chromosomes. It is classified as an intersex condition due to the incongruence between the chromosomal sex and the external genitalia.
In PAIS, individuals have a partial resistance or insensitivity to the effects of androgens, which are male sex hormones such as testosterone. This insensitivity prevents the normal development of male reproductive organs and produces a wide range of phenotypic variations. The severity of the condition can vary greatly among affected individuals.
Typically, individuals with PAIS are born with ambiguous genitalia, meaning that the external genitalia do not clearly appear male or female. The severity of ambiguity can be mild or severe, with different degrees of virilization or feminization. Some individuals may have external genitalia that appear predominantly female, while others may exhibit a mix of male and female characteristics.
Internally, individuals with PAIS usually have testes instead of ovaries. However, due to the reduced response to androgens, the development of secondary male sexual characteristics may be impaired, leading to incomplete masculinization. Hormone levels and fertility can also be affected, with some individuals experiencing hormonal imbalances and reduced fertility potential.
Treatment for individuals with PAIS is highly individualized and may involve a multidisciplinary approach, including input from endocrinologists, geneticists, and psychologists. Management options may include hormonal therapy, surgical interventions, and psychological support to address the physical, emotional, and psychosexual aspects related to this condition.
In summary, PAIS is a rare genetic condition characterized by partial insensitivity to androgens, leading to an incomplete development of male genitalia and
