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Androgen Insensitivity syndrome

How is it treated?

The most important part of the management of AIS is the explanation and counselling given to the parents as to what and how to tell the child. This should involve an expert psychologist. Unlike partial androgen insensitivity (see below) the problem is compounded by not being diagnosed at birth. Certainly, full revealment of the diagnosis, including the chromosome abnormality, should be given by the time the child has become an adult.

The testes are usually removed when the diagnosis is made. Puberty should be induced at the normal age and then an accurate assessment of the vaginal size can be made. The assessment of whether to perform a vaginal dilation or a vaginoplasty (an operation to create a vagina) should be made by a gynaecologist who is expert in this field. Certainly, the girl should be counselled not to attempt intercourse until the vagina is an adequate size. There may be longer-term problems such as Osteoporosis - see entry, Osteoporosis (Juvenile).

In Partial Androgen Insensitivity, the genetic abnormality in the androgen receptor produces an incomplete block of male hormone action. In this case, the child is usually born with ambiguous genitalia and appears a poorly virilised male. The child may be brought up as either a male or a female and the management very much depends on the severity of the condition in the individual

View How is it diagnosed? How is it diagnosed?  |  Inheritance and prenatal diagnosis View Inheritance and prenatal diagnosis

Medical text written May 1997 by Dr R Stanhope. Last reviewed October 2005 by Dr R Stanhope, Consultant Paediatric Endocrinologist, Great Ormond Street Hospital, London, UK.

 

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