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Amenorrhea-Galactorrhea Syndrome


National Organization for Rare Disorders, Inc.

Synonyms

  • Amenorrhea-Galactorrhea, Nonpuerperal
  • Galactorrhea-Amenorrhea Syndrome
  • Nonpuerperal Galactorrhea
  • Forbes-Albright Syndrome

Disorder Subdivisions

  • None

Related Disorders List

Information on the following diseases can be found in the Related Disorders section of this report:

  • Chiari-Frommel Syndrome
  • Ahumada-del Castillo Syndrome

General Discussion

Forbes-Albright Syndrome is a rare endocrine disorder characterized by the abnormal production of breast milk (lactation), lack of ovulation (anovulation), and the absence of regular menstrual periods (amenorrhea). This disorder is usually caused by a hormone secreting tumor of the pituitary or hypothalamus gland (adenoma) which produces excessive amounts of prolactin.

Symptoms

The symptoms of Forbes-Albright Syndrome typically begin between the ages of 20 to 40 years. Symptoms include the absence of regular menstrual periods (amenorrhea) in females and abnormal milk production (galactorrhea) in affected males and females. Women with this disorder typically have breasts and nipples of normal size and appearance, but the pattern of body hair may change and sexual desire may be diminished. Some people with Forbes-Albright Syndrome have exceptionally oily skin and have an excessive amount of body fat (obese).

Women with Forbes-Albright Syndrome usually have abnormally enlarged breasts and they may also begin to secrete milk (lactation). Some men may experience a diminished sexual desire, loss of sexual function, and/or impaired fertility due to a decrease in sperm production.

Causes

Forbes-Albright Syndrome is caused by a tumor in the pituitary gland which secretes the hormone prolactin. A tumor in the hypothalamus gland may also cause this disorder. Thyroid disease (i.e., hypothyroidism), the long-term use of certain drugs (i.e., chlorpromazine), and oral birth control pills (contraceptives) have also been implicated as possible causes of Forbes- Albright Syndrome. (For more information, choose "Hypothyroidism" as your search term in the Rare Disease Database.)

Affected Populations

Forbes-Albright Syndrome is a rare disorder that affects males and females in equal numbers.

Related Disorders

Symptoms of the following disorders can be similar to those of Forbes- Albright Syndrome. Comparisons may be useful for a differential diagnosis:

Chiari-Frommel Syndrome is a rare endocrine disorder that affects women who have recently had a child (postpartum) and is characterized by the production of breast milk (lactation), lack of ovulation (anovulation), and the absence of regular menstrual periods (amenorrhea). These symptoms persist long after childbirth. The absence of normal hormonal cycles may result in a reduction in the size of the uterus (atrophy). Other symptoms may include emotional distress, anxiety, headaches, backaches, abdominal pain, and occasionally obesity. (For more information on this disorder, choose "Chiari-Frommel" as your search term in the Rare Disease Database.)

Ahumada-del Castillo Syndrome is a rare endocrine disorder characterized by the abnormal function of the hypothalamus and pituitary glands affecting the secretion of hormones. This disorder affects only women and is not related to pregnancy. The two major symptoms of this disorder include the production and expression of milk from the breasts not associated with childbirth or nursing, and the absence of regular menstruation. There is normal development of secondary sexual characteristics. (For more information on this disorder, choose "Ahumada-del Castillo" as your search term in the Rare Disease Database.)

Standard Therapies

Individuals with Forbes-Albright Syndrome usually have abnormally high levels of prolactin in the blood. Other laboratory findings may include abnormally low levels of other hormones such as follicle-stimulating hormone (FSH) and leutinizing hormone (LH).

The treatment of Forbes-Albright Syndrome usually involves surgical removal of the pituitary tumor causing the excessive prolactin levels. Smaller or inoperable tumors often respond to irradiation or drug therapy with bromocriptine or lergotrile mesilate. These drugs, which lower prolactin levels, stop abnormal milk secretion and often restore normal menstrual functions.

References

TEXTBOOK OF ENDOCRINOLOGY, 8TH ED.: Jean D. Wilson and Daniel W. Foster, Editors; W.B. Saunders Co., 1992. Pp. 958-59.

CECIL TEXTBOOK OF MEDICINE, 19th Ed.: James B. Wyngaarden and Lloyd H. Smith, Jr., Editors; W.B. Saunders Co., 1992. Pp. 1365-68.

THE MERCK MANUAL, 16th Ed.: Robert Berkow, Editor.; Merck Research Laboratories, 1992. P. 1066.

NOVAK'S TEXTBOOK OF GYNECOLOGY, 11th Ed.: Howard Jones III, Anne Colston Wentz, and Lonnie S. Burnett, Editors; Williams & Wilkins, 1988. Pp. 351-57.

DICTIONARY OF MEDICAL SYNDROMES, 3rd Ed.: Sergio I. Magalini, Sabina C. Magalini, and Giovanni de Francisci, Editors; J.B. Lippincott Company, 1990. P. 44.

ASSESSMENT AND MANAGEMENT OF GALACTORRHEA. D.S. Edge, et al.; Nurse Pract (Jun 1993; 18(6)). Pp. 35-6, 38, 43-4.

Resources

National Women's Health Resource Center
157 Broad Street
Suite 315
Red Bank, NJ 07701
Tel: (732)530-3425
Fax: (732)530-3347
Tel: (877)986-9472
Email: mchin@healthywomen.org
Internet: http://www.healthywomen.org

National Women's Health Network
514 10th Street NW
Suite 400
Washington, D.C. 20004
USA
Tel: 2026287814
Fax: 2023471168
Email: nwhn@nwhn.org
Internet: http://www.womenshealthnetwork.org

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  4/15/2003
Copyright  1986, 1989, 1994, 2003 National Organization for Rare Disorders, Inc.



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