Zollinger-Ellison syndrome

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Zollinger-Ellison syndrome, abbreviated ZES, is the triad of pancreatic gastrinoma, peptic ulcers of the small bowel and gastric hypersecretion.[1]

General

Clinical:

  • Abdominal pain.
  • Diarrhea.

Laboratory:

  • Serum gastrin often elevated - though not usually not very high.[2]
    • Gastrin may be elevated due to chronic proton pump inhibitor (PPI) use - 35+/-20 pg/ml (normal) versus 170+/-135 pg/ml (chronic PPI use).[3]

Microscopic

Pancreas

See: Pancreatic neuroendocrine tumour.

Stomach

  • G-cell hyperplasia.

DDx:

  • Pseudo-Zollinger-Ellison syndrome.[4]

See also

References

  1. Zollinger RM, Ellison EH (1955). "Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas". Ann. Surg. 142 (4): 709–23; discussion, 724–8. doi:10.1097/00000658-195510000-00015. PMC 1465210. PMID 13259432. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1465210/.
  2. Berna, MJ.; Hoffmann, KM.; Serrano, J.; Gibril, F.; Jensen, RT. (Nov 2006). "Serum gastrin in Zollinger-Ellison syndrome: I. Prospective study of fasting serum gastrin in 309 patients from the National Institutes of Health and comparison with 2229 cases from the literature.". Medicine (Baltimore) 85 (6): 295-330. doi:10.1097/01.md.0000236956.74128.76. PMID 17108778.
  3. Raines, D.; Chester, M.; Diebold, AE.; Mamikunian, P.; Anthony, CT.; Mamikunian, G.; Woltering, EA. (May 2012). "A prospective evaluation of the effect of chronic proton pump inhibitor use on plasma biomarker levels in humans.". Pancreas 41 (4): 508-11. doi:10.1097/MPA.0b013e318243a0b6. PMID 22460728.
  4. Tomaszewska, R.; Kedra, B.; Stachura, J. (2000). "Hypertrophic gastritis, primary diffuse G-cell hyperplasia and pancreatic metaplasia of the gastric mucosa (pseudo-Zollinger-Ellison syndrome)--case report.". Pol J Pathol 51 (1): 51-4. PMID 10833904.