Zollinger-Ellison syndrome
Pronunciation: ZAHL-in-jer EL-ih-son SIN-drome
A condition caused by gastrin-secreting tumors leading to excessive gastric acid production and peptic ulceration.
Full Definition
Zollinger-Ellison syndrome (ZES) is a rare disorder characterized by gastrin-secreting neuroendocrine tumors (gastrinomas) that cause hypergastrinemia and subsequent gastric acid hypersecretion. The majority of gastrinomas occur in the pancreas or duodenum, forming part of the 'gastrinoma triangle.' Patients typically present with refractory peptic ulcer disease, diarrhea, and abdominal pain. Diagnosis requires demonstration of elevated fasting gastrin levels with concurrent gastric acid hypersecretion. About 25% of cases are associated with Multiple Endocrine Neoplasia Type 1 (MEN1) syndrome. Treatment involves proton pump inhibitors for acid suppression and surgical resection when feasible.
Usage
Usage note: Hyphenate as 'Zollinger-Ellison syndrome.' Capitalize both surnames as eponyms. Abbreviate as ZES.
In Context
- "The patient's refractory ulcer disease and markedly elevated gastrin levels were consistent with Zollinger-Ellison syndrome." — Discharge summary
- "Genetic testing revealed MEN1 mutation, explaining the familial occurrence of Zollinger-Ellison syndrome." — Genetics consultation report