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Zollinger-Ellison
syndrome is classified as a hypersecretory
condition associated with gastrinomas, i.e., gastrin-secreting tumors.
This unregulated gastrin secretion stimulates parietal cells to produce
large amounts of hydrochloric acid. Increased hydrochloric acid release
results in ulceration of the stomach and duodenum. These
tumors secrete a substance called gastrin which in turn causes
the stomach to produce excessive amounts of acid. The ulcers produced by
this acid are usually more painful and less responsive to treatment than
routine ulcers. Named
for the two surgeons who first described it, Zollinger-Ellison
syndrome is an uncommon condition that produces tumors in the pancreas
and duodenum, and ulcers in the stomach and duodenum. In approximately
one-half of reported cases, the tumors caused by Zollinger-Ellison
syndrome are cancerous.59 How
is Zollinger-Ellison Syndrome Diagnosed? For a variety of
reasons, the syndrome is difficult to diagnose. First, the gastrin-secreting
tumors that define Zollinger-Ellison syndrome are very small and
difficult to detect. Additionally, Zollinger-Ellison syndrome can easily
be mistaken for the far more common duodenal ulcer. The symptoms of each
are similar, and even some powerful diagnostic techniques such as an
upper-GI series can fail to distinguish Zollinger-Ellison syndrome from
duodenal ulcers. High gastrin
levels in the blood are one indicator of Zollinger-Ellison symptoms, as
are duodenal ulcers that fail to respond to conventional therapy. Other
techniques such as ultrasound and CAT scans can also be useful in
detecting gastrinomas, the tumors associated with Zollinger-Ellison
syndrome. How
is Zollinger-Ellison Syndrome Treated? The most common
therapeutic goal in treating Zollinger-Ellison syndrome is the reduction
of stomach acid. For this purpose, antisecretory agents such as proton
pump inhibitors have been proven effective. Surgery to remove tumors or
to treat duodenal ulcers are also treatment options. In the most serious
cases, Zollinger-Ellison syndrome may require surgery to remove the
entire stomach. Demographics and
Statistics Because even
advanced diagnostic techniques can sometimes fail to detect gastrinomas
and to differentiate cases of Zollinger-Ellison syndrome from duodenal
ulcers, prevalence statistics are approximate at best. One estimate
suggests that this very rare condition occurs in 0.1% of the people who
suffer from peptic ulcer disease.69
Zollinger-Ellison syndrome usually affects people between the ages of 30
and 50 and is more common in men than in women. Among the younger age
group affected, the majority have inherited the genetic defect MEN I.
Many Zollinger-Ellison syndrome patients also have MEN I.69 Adverse events
reported most frequently were diarrhea, abdominal pain and nausea.
Symptomatic response to therapy does not preclude the presence of
gastric malignancy. PREVACID is contraindicated in patients with known
hypersensitivity to any component of the formulation. For further
information, please see the complete
prescribing information for PREVACID. Adverse events
reported most frequently with PREVACID were diarrhea, abdominal pain and
nausea. Symptomatic response to therapy does not preclude the presence
of gastric malignancy. PREVACID is contraindicated in patients with
known hypersensitivity to any component of the formulation. For
further information, please see the safety
section and accompanying complete
prescribing information for PREVACID and PREVPAC (lansoprazole,
amoxicillin, clarithromycin). |
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