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- $Unique_ID{BRK04234}
- $Pretitle{}
- $Title{Stein-Leventhal Syndrome}
- $Subject{Stein-Leventhal Syndrome Polycystic Bilateral Ovarian Syndrome
- Bilateral Polycystic Ovarian Syndrome Ovarian Hyperthecosis Sclerocystic
- Ovarian Disease }
- $Volume{}
- $Log{}
-
- Copyright (C) 1986, 1989, 1991 National Organization for Rare Disorders,
- Inc.
-
- 111:
- Stein-Leventhal Syndrome
-
- ** IMPORTANT **
- It is possible that the main title of the article (Stein-Leventhal
- Syndrome) is not the name you expected. Please check the SYNONYM listing to
- find the alternate names and disorder subdivisions covered by this article.
-
- Synonyms
-
- Polycystic Bilateral Ovarian Syndrome
- Bilateral Polycystic Ovarian Syndrome
- Ovarian Hyperthecosis
- Sclerocystic Ovarian Disease
-
- General Discussion
-
- ** REMINDER **
- The information contained in the Rare Disease Database is provided for
- educational purposes only. It should not be used for diagnostic or treatment
- purposes. If you wish to obtain more information about this disorder, please
- contact your personal physician and/or the agencies listed in the "Resources"
- section.
-
-
- Stein-Leventhal Syndrome is a reproductive disorder affecting young
- women. It is characterized by absent or abnormal menstruation, sterility,
- mild signs of virilization, and sometimes obesity. The causes of the
- syndrome are not understood, but may involve faulty production of
- reproductive hormones. Therapy is often effective, but recurrences are
- common.
-
- Symptoms
-
- The first symptoms of Stein-Leventhal syndrome usually appear shortly after
- puberty and before age twenty, after menstruation has been normal or
- irregular for some time. Menstrual flow becomes irregular and gradually
- decreases over several months, until menstruation ceases. Sometimes, profuse
- menstrual flow interrupts longer intervals of amenorrhea. Menstrual pain
- occurs in about 20% of the patients.
-
- Many patients consult a physician because of infertility or the
- appearance of increased facial hair. Other signs of virilization include
- voice changes and increased body hair with a "male" distribution, such as on
- the chest. Obesity is a frequently associated problem.
-
- Cysts are found directly under the surface of the ovaries. Other
- pathological changes also occur. The uterus is often small and
- underdeveloped.
-
- Stein-Leventhal syndrome should be treated even when pregnancy is not
- desired because erratic, or breakthrough bleeding can be a problem, and
- because changes in the endometrium, the tissue lining the uterus that changes
- regularly in the normal menstrual cycle, can lead to precancerous or
- cancerous conditions.
-
- Causes
-
- The causes of Stein-Leventhal syndrome may vary from case to case, and are
- usually poorly understood. Generally, the abnormalities are thought to occur
- in hypothalamic, pituitary, or ovarian hormone production. Occasionally, a
- tumor of the adrenal gland produces excessive amounts of androgenic hormones.
- In all cases, the ovary fails to ovulate, and through a feed back mechanism,
- becomes enlarged and full of cysts.
-
- Affected Population
-
- Young women are affected by Stein-Leventhal Syndrome.
-
- Therapies: Standard
-
- If a patient afflicted with Stein-Leventhal Syndrome wants to become
- pregnant, ovulation and normal menstruation follows administration of
- clomiphene in many cases; pregnancy occurs in about half of these patients.
- If clomiphene is ineffective, gonadotropins may be tried. Should both fail,
- ovarian wedge resection, or removal of the cystic portions of the ovaries,
- can be performed. However, symptoms often recur.
-
- If pregnancy is not desired, the ovary can be fully suppressed with low
- dosage birth control pills or long acting progestins such as
- medroxyprogesterone.
-
- The investigational drug Flutamide is being tested in Stein-Leventhal
- Syndrome as a treatment for the control of hair growth. For further
- information physicians may contact:
-
- Dr. David Ehrmann
- Department of Medicine
- University of Chicago Medical Center
- 5841 Maryland Ave., Box 435
- (312) 702-9653
-
- Therapies: Investigational
-
- This disease entry is based upon medical information available through
- November 1991. 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.
-
- Resources
-
- For more information on Stein-Leventhal Syndrome, please contact:
-
- National Organization for Rare Disorders (NORD)
- P.O. Box 8923
- New Fairfield, CT 06812-1783
- (203) 746-6518
-
- NIH/National Institute of Child Health and Human Development (NICHHD)
- 9000 Rockville Pike
- Bethesda, MD 20892
- (301) 496-5133
-
- References
-
- THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme
- Research Laboratories, 1987. Pp. 1056, 1695.
-
- CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H.
- Smith, Jr., Eds.: W. B. Saunders Co., 1988. Pp. 1287.
-
-