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- $Unique_ID{BRK04215}
- $Pretitle{}
- $Title{Sheehan Syndrome}
- $Subject{Sheehan Syndrome Simmond's Disease Postpartum Panhypopituitarism
- Postpartum Pituitary Necrosis Postpartum Hypopituitarism Postpartum
- Panhypopituitary Syndrome }
- $Volume{}
- $Log{}
-
- Copyright (C) 1986, 1989 National Organization for Rare Disorders, Inc.
-
- 228:
- Sheehan Syndrome
-
- ** IMPORTANT **
- It is possible the main title of the article (Sheehan Syndrome) is not
- the name you expected. Please check the SYNONYMS listing the find the
- alternate names and disorder subdivisions covered by this article.
-
- Synonyms
-
- Simmond's Disease
- Postpartum Panhypopituitarism
- Postpartum Pituitary Necrosis
- Postpartum Hypopituitarism
- Postpartum Panhypopituitary Syndrome
-
- 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.
-
-
- Sheehan Syndrome is caused by the subnormal pituitary gland functioning
- (hypopituitarism). The disorder affects females and is caused by necrosis
- (death of cells) of the anterior pituitary gland secondary to profound blood
- loss during and after childbirth, with vascular collapse and shock
- (postpartum collapse).
-
- Symptoms
-
- The clinical features of Sheehan Syndrome are highly variable and depend on
- the degree of failure of secretion of pituitary hormones including:
-
- 1. prolactin (the hormone which stimulates lactation)
- 2. gonadotrophin (which regulates the function of the gonads)
- 3. TSH (which stimulates the thyroid gland)
- 4. ACTH (adrenocorticotropin) (which stimulates the adrenal cortex)
- 5. growth hormone (GH).
-
- Fully expressed, the condition is associated with failure of lactation
- after a woman has a baby. Menstruation does not begin again, pubic hair not
- grow back, and axillary hair slowly disappears. Breasts and genitalia atrophy
- (diminish in size).
-
- The characteristics of hypothyroidism usually develop gradually. A dry,
- waxy type of swelling (myxedema) may take years or decades to become
- apparent. Decreased blood sugar level (hypoglycemia), chronic hypotension
- with fainting and impaired resistance to infection such as of cuts and
- abrasions are the main problems associated with severe ACTH deficiency. If
- these symptoms occur, they usually appear within weeks or months after the
- baby is born. Since Sheehan Syndrome is a disorder affecting adults, the
- effects of growth hormone deficiency are limited to some loss of muscle
- strength, mild anemia and increased insulin sensitivity.
-
- Causes
-
- Sheehan Syndrome is thought to be caused by severe arteriolar spasm
- (associated with shock) in the vessels supplying the hypothalamic area of the
- brain from which the stalk of the anterior lobe of the pituitary gland
- (adenohypophysis) arises. Spasm leads to lack of oxygen in the pituitary
- (pituitary ischemia) and various degrees of cellular damage depending on the
- severity and duration of arteriolar spasm.
-
- Affected Population
-
- Sheehan Syndrome affects women with excessive blood loss and circulatory
- collapse following childbirth.
-
- Therapies: Standard
-
- Treatment of Sheehan Syndrome consists of hormone replacement; i.e., ovarian,
- thyroid, and adrenocortical hormones (ACTH). Since in most cases ACTH
- deficiency is only partial, continuing cortisol replacement therapy may not
- be required.
-
- Therapies: Investigational
-
- This disease entry is based upon medical information available through
- September 1989. 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 Sheehan Syndrome, please contact:
-
- National Organization for Rare Disorders (NORD)
- P.O. Box 8923
- New Fairfield, CT 06812-1783
- (203) 746-6518
-
- National Digestive Diseases Information Clearinghouse
- Box NDDIC
- Bethesda, MD 20892
- (301) 468-6344
-
- References
-
- THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme
- Research Laboratories, 1987. P. 1022.
-
-