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$Unique_ID{BRK03424}
$Pretitle{}
$Title{ACTH Deficiency}
$Subject{ACTH Deficiency Adrenocorticotropic Hormone Deficiency, Isolated
Addison's Disease Adrenal Hyperplasia, Congenital Adrenal Insufficiency,
Secondary}
$Volume{}
$Log{}
Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.
483:
ACTH Deficiency
** IMPORTANT **
It is possible the main title of the article (ACTH Deficiency) is not the
name you expected. Please check the SYNONYMS listing on the next page to
find alternate names, disorder subdivisions, and related disorders covered by
this article.
Synonyms
Adrenocorticotropic Hormone Deficiency, Isolated
Information on the following disorders may be found in the Related
Disorders section of this report:
Addison's Disease
Adrenal Hyperplasia, Congenital
Adrenal Insufficiency, Secondary
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.
ACTH Deficiency is characterized by adrenal insufficiency symptoms such
as weight loss, lack of appetite (anorexia), weakness, nausea, vomiting, and
low blood pressure (hypotension). The pituitary hormone called
"adrenocorticotropic hormone" (ACTH) is decreased or absent, and other
cortisol and other steroid hormone levels in the blood are abnormally low.
Symptoms
ACTH Deficiency is a very rare disorder that usually starts during adulthood,
although a few cases have begun during childhood. Symptoms include weight
loss, lack of appetite (anorexia), muscle weakness, nausea and vomiting, and
low blood pressure (hypotension). Low blood levels of sugar and sodium and
high potassium levels (hypoglycemia, hyponatremia and hyperkalemia) usually
occur. The pituitary hormone ACTH may be undetectable in blood tests, and
the level of the hormone cortisol is abnormally low. Concentration of the
adrenal cortex hormones 17-hydroxy- and 17-ketosteroid, are also abnormally
low in the urine. Some adrenal hormones which are decreased are precursors
of male sex hormones and are also known as "androgens".
Although males with this disorder usually have a normal hair pattern,
females have very little pubic and underarm (axillary) hair. Skin
pigmentation is decreased in most cases, but may be normal or increased in
others. Emotional symptoms may range from depression to psychosis.
Causes
The exact cause of ACTH Deficiency is unknown. A defect in the brain's
hypothalamus or in the pituitary gland may cause the deficiency.
Underproduction of ACTH causes the adrenal cortex to produce insufficient
levels of hormones, which thus causes the symptoms.
Affected Population
Symptoms of ACTH Deficiency usually only occur in adults, but the disorder
may be diagnosed biochemically in infancy. The disorder affects males and
females in equal numbers.
Related Disorders
Symptoms of the following disorders may be similar to those of ACTH
Deficiency. Comparisons may be useful for a differential diagnosis:
Congenital Adrenal Hyperplasia (CAH) is a group of disorders resulting
from defective synthesis of the corticosteroid hormones of the adrenal gland.
The adrenal gland becomes enlarged. The adrenal gland produces "male" sex
hormones (androgens) in both males and females because these are overproduced
in certain forms of CAH. The external genitals of some females with this
disorder become masculinized to various degrees. Lack of glucocorticoids,
especially cortisol, causes various metabolic problems. Lack of
mineralocorticoids, primarily aldosterone, causes salt and water imbalances
which may be life threatening. (For more information on this disorder,
choose "Adrenal Hyperplasia" as your search term in the Rare Disease
Database.)
Addison's Disease (primary adrenal insufficiency) usually develops later
in life. It is characterized by chronic diminished adrenocortical function.
The resulting deficiencies of glucocorticoids and mineralocorticoids cause
weakness, low resistance to physiological stress, metabolic abnormalities,
and circulatory insufficiency. Many Addison's patients have sufficient
levels of these corticosteroids to permit adequate functioning under normal
circumstances. Even mild physiologic stress, however, can precipitate an
Addisonian crisis consisting of circulatory collapse and, if untreated,
death. With replacement of the essential adrenal hormones, Addison's
patients can lead a normal life. (For more information on this disorder,
choose "Addison" as your search term in the Rare Disease Database.)
Secondary Adrenal Insufficiency results from insufficient production or
release of the pituitary hormone ACTH. It may be caused by prolonged
corticosteroid therapy. ACTH production doesn't return to normal for several
months after completion of the therapy. Cancers such as adenomas and
granulomas of the pituitary gland and subsequent tissue death (necrosis) of
this gland after a pregnancy also may cause adrenal insufficiency.
Therapies: Standard
Treatment of ACTH Deficiency consists of replacement of the hormone either
with cortisone or synthetic ACTH. With therapy, patients with ACTH
Deficiency can lead a normal life.
Therapies: Investigational
This disease entry is based upon medical information available through June
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 ACTH Deficiency, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
The National Adrenal Diseases Foundation
505 Northern Blvd., Suite 200
Great Neck, NY 11021
(516) 487-4992
National Digestive Diseases Information Clearinghouse
Box NDDIC
Bethesda, MD 20892
(301) 468-6344
The Endocrine Society
9650 Rockville Pike
Bethesda, MD 20205
(301) 530-9660
References
INTERNAL MEDICINE, 2nd ed.: Jay H. Stein, et al., eds.; Little, Brown, 1987.
P. 1899.