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$Unique_ID{BRK04348}
$Pretitle{}
$Title{Zollinger-Ellison Syndrome}
$Subject{Zollinger-Ellison Syndrome Pancreatic Ulcerogenic Tumor Syndrome
Multiple Endocrine Neoplasia Type I Partial Multiple Endocrine Adenomatosis
Z-E Syndrome Gastrinoma Cushing Syndrome Duodenal Ulcers }
$Volume{}
$Log{}
Copyright (C) 1987, 1988, 1990 National Organization for Rare Disorders, Inc.
360:
Zollinger-Ellison Syndrome
** IMPORTANT **
It is possible the main title of the article (Zollinger-Ellison Syndrome)
is not the name you expected. Please check the SYNONYMS listing to find the
alternate names, disorder subdivisions, and related disorders covered by this
article.
Synonyms
Pancreatic Ulcerogenic Tumor Syndrome
Multiple Endocrine Neoplasia, Type I
Partial Multiple Endocrine Adenomatosis
Z-E Syndrome
Gastrinoma
Information on the following diseases can be found in the Related
Disorders section of this report:
Cushing Syndrome
Duodenal Ulcers
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.
Zollinger-Ellison Syndrome is an unusual ulcerative condition
characterized by small tumors (usually of the pancreas) which secrete a
hormone that produces excess amounts of stomach (gastric) juices. These
tumors can also appear in the lower stomach wall, spleen or lymph nodes close
to the stomach. Large amounts of gastric acid can be found in lower stomach
areas where ulcers can form. Ulcers can appear suddenly even in areas where
they are rarely found, may persist following treatment, and can be
accompanied by diarrhea. Prompt medical treatment of these ulcers is
necessary to prevent complications such as bleeding and perforation.
Symptoms
Tumors which characterize Zollinger-Ellison Syndrome secrete a hormone which
produces excess amounts of stomach acid which can cause ulcers in the
stomach, duodenum and jejunum, and in the esophagus due to a backward flow of
gastric acid from the stomach. Pain from these persistent ulcers may be
severe. Diarrhea and excretion of fat in the feces (steatorrhea) commonly
occurs. Ulcers may persist for years despite medical or surgical treatment.
This can result in a decrease of potassium levels in the blood.
Complications, including holes in the organ walls where ulcers occur
(perforations), bleeding or obstruction of the stomach where it empties into
the intestines, may be serious and require prompt treatment.
Causes
Two common forms of Zollinger-Ellison Syndrome were identified in 1982. One
form begins sporadically, usually during late adulthood, and is usually
malignant. The other form is inherited as an autosomal dominant trait and
occurs as a symptom of Multiple Endocrine Adenomatosis. (Human traits
including the classic genetic diseases, are the product of the interaction of
two genes for that condition, one received from the father and one from the
mother. In dominant disorders, a single copy of the disease gene (received
from either the mother or father) will be expressed "dominating" the normal
gene and resulting in appearance of the disease. The risk of transmitting
the disorder from affected parent to offspring is 50% for each pregnancy
regardless of the sex of the resulting child.) Other forms of Zollinger-
Ellison Syndrome may also exist. The mechanism that causes the tumors to
grow is unknown.
Affected Population
Zollinger-Ellison Syndrome can begin in childhood, but it is usually
diagnosed between twenty to seventy years of age. This disorder seems to
affect males and females in equal numbers.
Related Disorders
Zollinger-Ellison Syndrome may be associated with endocrine disorders such as
increased activity of the parathyroid glands (hyperparathyroidism) and benign
tissue cell growth of the adrenal or pituitary glands (adenomas).
Cushing Syndrome consists of a group of symptoms attributable to an
excess of cortisol and other hormones from the cortex of the adrenal gland.
It is usually caused by hormone secreting tumors of the adrenal or pituitary
glands; sometimes a hyperfunctioning pituitary gland can be the cause.
Hormone secreting tumors may also develop in other organs. Cushing syndrome
occurs more frequently in females than in males, particularly women in their
thirties following a pregnancy. The prognosis is good if the causative
tumors can be removed and/or drug therapy suppresses production of the
hormone. (For more information, choose "Cushing" as your search term in the
Rare Disease Database.)
Duodenal ulcers are very common. A possible genetic predisposition to
the disorder is possible. Steroid drugs can be one cause of these ulcers.
Other possible causes include emotional stress, complications of cirrhosis of
the liver, chronic pancreatitis, cystic fibrosis, or pulmonary emphysema.
Approximately ten percent of the United States population may be affected
by duodenal ulcers at some time. The highest incidence occurs in Americans
between twenty and fifty years of age, although duodenal ulcers can occur in
children. The prognosis is generally favorable with standard treatments.
However, without treatment duodenal ulcers can be serious due to possible
bleeding (hemorrhage), low red blood cell count (anemia), or formation of
small holes in the wall of organs where the ulcers occur (perforations).
Therapies: Standard
Patients with Zollinger-Ellison Syndrome are treated to control or reduce
gastric acid production. In the past the most serious cases were treated by
removal of the stomach (gastrectomy). This has been replaced (excluding the
most serious cases) by use of currently available antacids and drugs such as
cimetidine or ranitidine. These drugs (with or without an anticholinergic
agent) control excess gastric acid in most patients. Many patients with Z-E
Syndrome require higher doses than those used routinely to treat ulcers.
Imaging procedures now in use to locate the tumors are improving the
success rate of treatment. These procedures include ultrasound, CT scan, and
selective angiograms. Abdominal surgery to remove tumors may be indicated
and anti-tumor chemotherapy may be of benefit in some cases. Genetic
counseling can be useful to families of patients with Z-E Syndrome.
In March 1989 the FDA approved a new drug, Losec, for the treatment of
Zollinger-Ellison Syndrome and other serious gastric disorders. Losec is
manufactured by Merck & Company.
Therapies: Investigational
New tests are being developed for the diagnosis of Zollinger-Ellison Syndrome
such as transhepatic pancreatic vein catheterization supplemented by
determination of local hormone gradients. This may permit preoperative
location of even the smallest tumors. At present, the success rate of tumor
surgery is approximately twenty percent.
Surgery on the tenth cranial (vagus) nerve is being tried experimentally.
Severing this nerve interrupts impulses to the acid-secreting glands of the
stomach. Those who require very high doses of drugs such as cimetidine or
ranitidine may be candidates for this surgery which is recommended only for
the most serious cases.
Newer more potent drugs being developed for treatment of Z-E Syndrome
include omeprazole. This drug is still experimental in the United States.
This disease entry is based upon medical information available through
January 1990. 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 Zollinger-Ellison 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 NDIC
Bethesda, MD 20892
(301) 468-2162
For information on genetics and genetic counseling referrals, please
contact:
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
(914) 428-7100
Alliance of Genetic Support Groups
35 Wisconsin Circle, Suite 440
Chevy Chase, MD 20815
(800) 336-GENE
(301) 652-5553
References
CURRENT MANAGEMENT OF ZOLLINGER-ELLISON SYNDROME: R.T. Jensen, et. al.;
Drugs (August 1986, issue 32(2)). Pp. 188-196.
DIAGNOSIS AND CURATIVE THERAPY IN ZOLLINGER-ELLISON SYNDROME: W.H.
Hacki; Schweiz Med Wochenschr (April 27, 1985, issue 115(17). Pp. 575-581.
ZOLLINGER-ELLISON SYNDROME: H.D. Becker; Wien Klin Wochenschr (February
17, 1984, issue 96(4)). Pp. 138-144.