Treatment strategies for Zollinger-Ellison syndrome.
BACKGROUND
Zollinger-Ellison syndrome (ZES) is a rare disorder caused by tumor secretion of the hormone gastrin, which results in gastric acid hypersecretion and secondarily complicated peptic ulcer and diarrhea. Until the development of H(2)-receptor antagonists and later proton pump inhibitors (PPIs), the disease was virulent, often associated with ulcer-related mortality, and the mainstay of treatment was total gastrectomy.


OBJECTIVE
To evaluate current approaches to diagnosis and therapy, focusing on the role of PPIs.


METHODS
An extensive literature search through PubMed using the search term 'Zollinger-Ellison syndrome' from 1964 to the present was performed. Primary articles were identified, and pertinent articles obtained from the reference lists were also examined.


CONCLUSIONS
The clinical manifestations of ZES are well described, but overlaps with other more common disorders delay diagnosis. The use of abdominal imaging with somatostatin receptor scintigraphy and endoscopic ultrasound has improved tumor staging. PPI therapy is remarkably effective in controlling gastric acid hypersecretion, thereby reducing morbidity and potential mortality of this syndrome. The dose of drug necessary to control symptoms is highly variable but, even when used in high doses for prolonged periods of time, the disease remained controlled with very few drug-related side effects.
Division of Gastroenterology & Hepatology, Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama 35294-0007, USA. melw@uab.edu
C Mel
CM
Wilcox
Basil I
BI
Hirschowitz
D006635:Histamine H2 Antagonists / Q000627:therapeutic use*; D006801:Humans; D011379:Prognosis; D054328:Proton Pump Inhibitors / Q000627:therapeutic use*; D015043:Zollinger-Ellison Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology
D006635:Histamine H2 Antagonists; D054328:Proton Pump Inhibitors