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Zollinger-Ellison syndrome. Clinical presentation in 261 patients.
P K Roy1, D J Venzon, H Shojamanesh
1Digestive Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA.
Medicine
|January 6, 2001
Summary
Zollinger-Ellison syndrome (ZES) commonly presents with abdominal pain and diarrhea. Prominent gastric folds on endoscopy are a key diagnostic sign, yet diagnosis is often delayed, even with advanced treatments.
Area of Science:
- Gastroenterology
- Endocrinology
- Oncology
Background:
- Prospective evaluation of 261 Zollinger-Ellison syndrome (ZES) patients over 25 years.
- Investigated initial presenting symptoms, diagnostic delays, and impact of Multiple Endocrine Neoplasia type 1 (MEN-1).
- Assessed influence of antisecretory therapies on clinical presentation.
Observation:
- Abdominal pain (75%) and diarrhea (73%) were most common ZES symptoms; heartburn (44%) and weight loss (17%) also noted.
- Prominent gastric body folds observed in 94% of patients on endoscopy, a crucial but often overlooked diagnostic sign.
- Mean diagnostic delay of 5.2 years; only 3% of patients initially diagnosed correctly by referring physicians.
- Patients with MEN-1 presented younger and more frequently with nephrolithiasis, less often with pain and bleeding.
- Antisecretory therapies (H2RAs, PPIs) did not shorten diagnostic delay or reduce peptic complication rates but decreased surgery rates.
Findings:
- Key presenting symptoms for ZES include abdominal pain, diarrhea, and heartburn, with weight loss being a significant indicator.
- Prominent gastric folds on endoscopy are a highly prevalent sign, suggesting ZES.
- Despite advances in treatment and awareness, diagnostic delays persist, with common misdiagnoses including peptic ulcer disease and GERD.
Implications:
- Increased clinical awareness of prominent gastric folds and symptom patterns (pain, diarrhea, weight loss) can improve early ZES diagnosis.
- The findings underscore the need for improved diagnostic strategies to reduce delays and associated complications.
- While antisecretory therapies manage acid hypersecretion, they do not resolve the underlying diagnostic challenges of ZES.
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