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$Unique_ID{BRK03451}
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$Title{Amenorrhea, Primary}
$Subject{Amenorrhea, Primary Absence of Menstruation PA}
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Copyright (C) 1986, 1989, 1993 National Organization for Rare Disorders,
Inc.

70:
Amenorrhea, Primary

** IMPORTANT **
It is possible that the main title of the article (Primary Amenorrhea) is
not the name you expected.  Please check the SYNONYMS listing to find the
alternate name and disorder subdivisions covered by this article.

Synonyms

     Absence of Menstruation
     PA

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 of this report.


Primary Amenorrhea is a rare gynecological disorder characterized by the
absence of menstrual flow (menses) in a female age 18 or older.  The
beginning of regular menstruation (menarche) usually begins within two years
after the onset of puberty.  Absence of menses by age 18 or older constitutes
Primary Amenorrhea.

Symptoms

The sole symptom of Primary Amenorrhea is absence of the first menstruation
and the lack of a regular monthly cycle of menstruation.  Other features may
include lack of secondary sexual characteristics (i.e., the development of
axillary hair and pubic hair), and incomplete or underdeveloped sexual organs
(external genitalia) and breasts.  Laboratory tests may reveal a deficiency
in the functioning of the ovaries or an underactive pituitary gland.  The
hymen may completely cover the opening of the vagina (imperforate hymen).

Causes

Primary Amenorrhea is usually caused by an excess or a deficiency of a
hormone called gonadotropic-releasing hormone (GnRH), which is produced in
the hypothalamus.  Severe eating disorders (i.e., Anorexia Nervosa), crash
dieting, emotional stress (i.e., depression), and obesity can cause
Amenorrhea.  Tuberculosis or Lymphoma can alter the function of the
hypothalamus gland resulting in Primary Amenorrhea.  (For more information,
choose "Anorexia Nervosa," "Tuberculosis," and "Lymphoma" as your search
terms in the Rare Disease Database.)

Some chromosomal disorders (e.g., Turner Syndrome) may cause primary
ovarian failure.  It is possible that autoimmune disease or menopause before
the first menstrual flow (premenarchal menopause) may also cause Primary
Amenorrhea.

Primary Amenorrhea may also be caused by abnormalities of the anatomy
including the absence at birth of the vagina, uterus, and/or ovaries.  Other
abnormalities include the underdevelopment of the lining of the uterus
(atrophic endometrium) and a rare condition in which both ovaries and testes
are present in one individual (hermaphroditism).  Menstrual flow may also be
obstructed by the complete closure of the vaginal opening by the hymen
(imperforate hymen) or the presence of a membranous partition across the
vaginal canal (transverse vaginal septum).  (For more information, choose
"Hermaphroditism" as your search term in the Rare Disease Database.)

A variety of drugs can cause secondary Amenorrhea including barbiturates,
opiates, corticosteroids, chlordiazepoxide, phenothiazines, and progesterone.

For some women with Primary Amenorrhea, functioning that is normal for
that person (simple physiologic delay) may explain why a female as old as 18
years of age has not menstruated.  In these cases, secondary sexual
characteristics are usually present and the external sexual organs appear
normal.

Affected Population

Disorders of menstruation are among the most common form of diseases
affecting females that result from the abnormal function of glands and
tissues that secrete hormones (endocrinopathy).

Related Disorders

Symptoms of the following disorders can be similar to those of Primary
Amenorrhea.  Comparisons may be useful for a differential diagnosis:

Amenorrhea can be a symptom of many disorders and the absence of
menstruation may occur for many different reasons.  These disorders include
Acromegaly, Stein-Leventhal Syndrome (Polycystic Ovary Disease), Congenital
Adrenal Hyperplasia, Hypogonadotropic Hypogonadism, Cushing Disease,
Hyperthyroidism, Hypothyroidism, and tumors of the pituitary gland.  (For
more information on these disorders, choose "Acromegaly," "Stein-Leventhal,"
"Adrenal Hyperplasia, Congenital," "Cushing," and "Hypothyroidism" as your
search terms in the Rare Disease Database.)

Therapies:  Standard

Women with Primary Amenorrhea should be examined by a physician who
specializes in treating disorders related to women's health (gynecologist) or
improper functioning of glands (endocrinologist).  If the Amenorrhea is a
result of a normal delay (physiologic delay), generally no therapy is
indicated before age 18.  If secondary sexual development is lacking by age
14, then a thorough investigation is warranted.

The treatment of Primary Amenorrhea depends on the cause.  The
administration of hormones such as progesterone and estrogen and/or
corticosteroid drugs may be effective in the treatment of some types of
Amenorrhea.  The patient may also benefit from emotional support and
counseling.  Imperforate hymen, other anatomical malformations, and tumors may
require surgery.

Therapies:  Investigational

Scientists are investigating the use of various combinations of hormones in
the treatment of Primary Amenorrhea.  In a recent study, women with Primary
Amenorrhea due to hypothalamic dysfunction were treated with a combination of
human menopausal gonadotrophin (HMG) and gonadotrophin releasing hormone
analoque (GnRHa).  This treatment successfully stimulated ovarian function in
some patients.  More study is needed to determine the long-term safety and
effectiveness of combination hormone therapy in the treatment of this form of
Primary Amenorrhea.

This disease entry is based upon medical information available through
April 1993.  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 Primary Amenorrhea, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     National Women's Health Network
     1325 G St., NW, Lower Level B
     Washington, DC  20005
     [phone]

     The National Adrenal Diseases Foundation, Inc.
     505 Northern Blvd., Suite 200
     Great Neck, NY 11021
     [phone]

     NIH/Niational Institute of Child Health and Human Development (NICHD)
     9000 Rockville Pike
     Bethesda, MD  20892
     [phone]

References

TEXTBOOK OF ENDOCRINOLOGY, 8TH ED.:  Jean D. Wilson and Daniel W. Foster,
Editors; W.B. Saunders Co., 1992.  Pp. 764-768.

CECIL TEXTBOOK OF MEDICINE, 19th Ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1990.  Pp. 1365-1370.

THE MERCK MANUAL, 16th Ed.:  Robert Berkow Ed.; Merck Research
Laboratories, 1992.  Pp. 1795

SUCCESSFUL TREATMENT OF INFERTILE WOMEN WITH HYPOTHALAMIC PRIMARY AND
SECONDARY PROTRACTED AMENORRHEA USING GONADOTROPHIN RELEASING HORMONE
ANALOQUE AND HUMAN MENOPAUSAL HONADOTROPHIN.  M.A. Aboulghar; Hum Reprod
(July 1990; 5(5)).  Pp. 557-560.