Reifenstein syndrome

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

Male reproductive system

Reifenstein syndrome

Alternative Names:

Partial androgen insensitivity syndrome; Incomplete male pseudohermaphroditism
Treatment:

Early and continuous testosterone treatment may improve the chance that a boy will grow up to be fertile.

Support Groups:

Support can be obtained from the Androgen Insensitivity Syndrome Support Group (AISSG).

Intersex Society of North America (ISNA)

4500 9th Avenue NE,

Suite 300

Seattle, WA 98105

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phone: 206-633-6077

fax: 206-633-6049

Expectations (prognosis):

Androgens are most important during early development in the womb. A male with Reifenstein syndrome can have a normal lifespan and be totally healthy, with the exception of infertility and possible psychological problems related to gender identity.

In the most severe cases, feminized external genitalia or an extremely small penis tend to cause psychological and emotional problems for these boys. In these cases, some parents have tried to raise people with Reifenstein syndrome as girls; however, this is rarely effective.

Genital surgery should be postponed until the affected person can make an informed choice. There are numerous accounts of people with ambiguous genitalia who had surgery during infancy to feminize their organs (since it is easier to do this than to create male organs) who later feel maimed.

Complications:

Infertility and failure to develop normal male gonads/genitals.
Breast development in men can be a cosmetic concern.
Psychological problems like depression may occur.

Calling your health care provider:

Call your health care provider if you, your son, or a male family member has infertility or incomplete development of male external organs, despite high blood testosterone. Genetic testing and counseling is available if there is a strong suspicion that this disease is present.