Androgen Insensitivity Syndrome Sexual Disorder
Androgen Insensitivity Syndrome Information
Androgen Insensitivity Syndrome is a condition that affects the development of the reproductive and genital organs.
In an individual with complete AIS and karyotype 46 XY, testes develop during gestation.
Androgen insensitivity syndrome (AIS), formerly known as testicular feminization, is an X-linked recessive condition resulting in a failure of normal masculinization of the external genitalia in chromosomally male individuals.
There are two forms of the Androgen Insensitivity Syndrome (AIS):
Complete AIS (CAIS) - where the tissues are completely insensitive to androgens, and
Partial AIS (PAIS) - where the tissues are partially sensitive to varying degrees. The condition is actually represented by a spectrum, with CAIS being a single entity at one end of a range of various PAIS manifestations.
Causes of Androgen Insensitivity Syndrome
Most cases of AIS are identified in the
- newborn period by the presence of inguinal masses,
- evaluation of primary amenorrhea.
- Because AIS is a genetic defect located on the X chromosome, it runs in families.
- history of surgery for hernias and/or the presence of gonads in the inguinal canals
Symptoms of Androgen Insensitivity Syndrome
When an AIS girl is diagnosed during infancy, physicians often perform surgery to remove her undescended testes.
Variants of Reifenstein syndrome occur with greater or less androgen sensitivity and correspondingly more or less genital masculinization.
Androgen Insensitivity Syndrome Treatment and Medication
For patients with CAIS, hormone therapy almost always consists of estrogen replacement.
For individuals with AIS, the standard of care is an orchidectomy to prevent possible malignant degeneration of the testes.
Many women with AIS require vaginal lengthening procedures.
Women's Health
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