title: Zollinger-Ellison syndrome: presentation, response to therapy, and outcome.
abstract: BACKGROUND
  Recent series describing the clinical presentation, response to therapy, and long-term outcome of Zollinger-Ellison syndrome are limited.
  
  
  OBJECTIVE
  To assess the clinical characteristics and long-term outcome of patients with Zollinger-Ellison syndrome.
  
  
  METHODS
  Over a 20-year period, patients with Zollinger-Ellison syndrome were enrolled in a prospective trial evaluating the efficacy of lansoprazole. Following dose stabilization, patients were followed on a 6-monthly basis with interval history, physical examination, endoscopy with gastric biopsies, gastric acid analysis and laboratory studies.
  
  
  RESULTS
  72 patients (mean age 54±12 years, % male 58%, % Caucasian 69%) were prospectively enrolled. The clinical presentation was stereotypical for Zollinger-Ellison syndrome. Symptoms had been reported for a median of 9 years prior to diagnosis. Cross-sectional abdominal imaging was often negative for demonstrable tumour. All patients had gastric acid hypersecretion controlled with variable doses of lansoprazole (median dose 60 mg/day, range 15-480 mg/day). The median survival from the time of diagnosis was 6.6 years; only two of 19 deaths were due to metastatic gastrinoma.
  
  
  CONCLUSIONS
  The clinical presentation of Zollinger-Ellison syndrome was similar to prior reports. Acid hypersecretion was controlled in all patients with variable doses of lansoprazole. Long-term survival was principally related to underlying co-morbidity.
authors.affiliation: University of Alabama at Birmingham, Division of Gastroenterology and Hepatology, Birmingham, AL, United States. melw@uab.edu
authors.forename: C Mel
authors.initials: CM
authors.lastname: Wilcox
authors.forename: Toni
authors.initials: T
authors.lastname: Seay
authors.forename: Justin T
authors.initials: JT
authors.lastname: Arcury
authors.forename: Jean
authors.initials: J
authors.lastname: Mohnen
authors.forename: Basil I
authors.initials: BI
authors.lastname: Hirschowitz
mesh_terms: D053799:2-Pyridinylmethylsulfinylbenzimidazoles / Q000008:administration & dosage / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000897:Anti-Ulcer Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D004334:Drug Administration Schedule; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D064747:Lansoprazole; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D016896:Treatment Outcome; D015043:Zollinger-Ellison Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality
chemical_list: D053799:2-Pyridinylmethylsulfinylbenzimidazoles; D000897:Anti-Ulcer Agents; D064747:Lansoprazole