Reifenstein syndrome

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Male reproductive system

Partial androgen insensitivity syndrome; Incomplete male pseudohermaphroditism

Treatment with testosterone may improve the chance that a boy will be able to have children when he grows up.

Androgen Insensitivity Syndrome Support Group (AISSG) -- www.medhelp.org/www/ais/

Intersex Society of North America (ISNA) -- www.isna.org

Androgens are most important during early development in the womb. Those with Reifenstein syndrome can have a normal lifespan and be totally healthy, but they may be infertile and have psychological problems related to gender identity.

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In the most severe cases, boys with outer female genitals or an extremely small penis may have psychological and emotional problems.

Genital surgery should be postponed until the patient is old enough to make the decision.

• Breast development in men
• Infertility and failure to develop normal male genitals
• Psychological problems, such as depression

Call your health care provider if you, your son, or a male family member has infertility or incomplete development of male genitals. Genetic testing and counseling are always recommended if this disease is suspected.

Review Date: 11/01/2010
Reviewed By: Chad Haldeman-Englert, MD, Wake Forest University School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org).