Reifenstein syndrome
Alternative Names
Partial androgen insensitivity syndrome; Incomplete male pseudohermaphroditismTreatment
Early and continuous testosterone treatment may improve the chance that a boy will grow up to be fertile.
Support Groups
Androgen Insensitivity Syndrome Support Group (AISSG) -- www.medhelp.org/www/ais/
Intersex Society of North America (ISNA) -- www.isna.org
Outlook (Prognosis)
Androgens are most important during early development in the womb. Those 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, psychological and emotional problems may occur in boys with outer female genitalia or an extremely small penis. Management of these patients is very complex.
Genital surgery should be postponed until the patient can make an informed choice.
Possible 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.
When to Contact a Medical Professional
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.
A.D.A.M., Inc. is accredited by
URAC, also known as the American
Accreditation HealthCare Commission. URAC's accreditation program is
the first of its kind, requiring compliance with 53 standards of quality and
accountability, verified by independent audit.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2005 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.