zollinger-ellison%20syndrome
ZOLLINGER-ELLISON SYNDROME
Zollinger-Ellison syndrome (ZES) is a disease entity which refers to the triad of severe peptic ulcer disease (PUD), gastric acid hypersecretion and non-beta cell gastrin-secreting tumor primarily of the pancreas and duodenum (gastrinoma).
Approximately two-thirds of patients have sporadic ZES while the rest is part of multiple endocrine neoplasia type 1.
ZES should be considered in the differential diagnoses of patients who present with abdominal pain, malabsorption and chronic watery diarrhea.
A high index of clinical awareness is necessary to correctly diagnose ZES.
  1. Gibril F, Jensen RT. Advances in evaluation and management of gastrinoma in patients with Zollinger-Ellison syndrome. Curr Gastroenterol Rep. 2005 May;7(2):114-121. PMID: 15802099
  2. Gibril F, Jensen RT. Zollinger-Ellison syndrome revisited: diagnosis, biologic markers, associated inherited disorders, and acid hypersecretion. Curr Gastroenterol Rep. 2004 Dec;6(6):454-463. PMID: 15527675
  3. Morrow EH, Norton JA. Surgical management of Zollinger-Ellison syndrome; state of the art. Surg Clin North Am. 2009 Oct;89(5):1091-1103. doi: 10.1016/j.suc.2009.06.018. PMID: 19836486
  4. Pritchard DM. Zollinger-Ellison syndrome: still a diagnostic challenge in the 21st century?. Gastroenterology. 2011 May;140(5):1380-1383. doi: 10.1053/j.gastro.2011.03.026. PMID: 21443889
  5. Norton JA, Jensen RT. Role of surgery in Zollinger-Ellison syndrome. J Am Coll Surg. 2007 Oct;205(Suppl 4):S34-S37. PMID: 17916516
  6. Zimmer V, Schilling MK, Buecker A, et al. Chronic diarrhea responding to proton pump inhibitors: a clinical sign of Zollinger-Ellison syndrome. Am J Med. 2009 Nov;122(11):e9-e10. doi: 10.1016/j.amjmed.2009.06.018. PMID: 19854317
  7. Jensen RT. Pancreatic endocrine tumors. In: Goldman L, Ausiello D, eds. Goldman's Cecil medicine. 24th ed. Philadelphia: Saunders, Elsevier; 2011
  8. Ferri FF. Zollinger-Ellison syndrome. In: Alvero R, Borkan JM, Dobbs MR, et al, eds. Ferri’s clinical advisor. 1st ed. Philadelphia: Mosby Inc, an affiliate of Elsevier; 2012
  9. Jensen RT, Norton JA. Gastrinomas. In: Feldman M, Friedman LS, Sleisenger MH, eds. Sleisenger and Fordtran’s gastrointestinal and liver disease: pathophysiology/diagnosis/management. 9th ed. Philadelphia: Elsevier; 2011
  10. Thompson JC, Townsend CM Jr. Zollinger-Ellison syndrome. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 18th ed. Philadelphia: Saunders, Elsevier; 2007
  11. MedWormhttp://www.medworm.com/rss/index.php/Gastroenterology/17/http://www.medworm.com/rss/medicalfeeds/specialities/Gastroenterology.xml
  12. MedWormhttp://www.medworm.com/rss/index.php/Pediatrics/33/http://www.medworm.com/rss/medicalfeeds/specialities/Pediatrics.xml
  13. Arnold A. Approach to therapy in multiple endocrine neoplasia type 1. UpToDate. http://www.uptodate.com. 20 Apr 2011. Accessed 08 Jul 2011.
  14. Goldfinger SE. Clinical manifestations and diagnosis of Zollinger-Ellison syndrome (gastrinoma). UpToDate. http://www.uptodate.com. 26 Oct 2010. Accessed 04 Jul 2011.
  15. Chan JA, Kulke M. Metastatic gastroenteropancreatic neuroendocrine tumors: treatment options. UpToDate. http://www.uptodate.com. 13 Jun 2011. Accessed 08 Jul 2011.
  16. Goldfinger SE. Management and prognosis of the Zollinger-Ellison syndrome (gastrinoma). UpToDate. http://www.uptodate.com. 10 Oct 2013. Accessed 13 Mar 2014.
  17. Zollinger-Ellison Syndrome. National Digestive Diseases Information Clearinghouse. http://digestive.niddk.nih.gov. 24 Dec 2013. Accessed 13 Mar 2014.
  18. Jensen RT. Pancreatic endocrine tumors. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016. https://www.clinicalkey.com. Accessed 18 Jan 2016:1335-1337.
  19. National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine tumors version 1.2016. NCCN. https://www.nccn.org. 15 Apr 2016. Accessed 13 May 2016.
  20. Bergsland E. Management and prognosis of the Zollinger-Ellison syndrome (gastrinoma). UpToDate. https://www.uptodate.com. 09 Jul 2018.
Digital Edition
Asia's trusted medical magazine for healthcare professionals. Get your MIMS Gastroenterology - Malaysia digital copy today!
Sign In To Download
Editor's Recommendations
Most Read Articles
15 Jul 2019
Fluticasone, swallowed from a multidose inhaler, and oral viscous budesonide slurry have comparable efficacies as initial treatment for eosinophilic oesophagitis, a recent study has found.
31 May 2019
Co-therapy with proton pump inhibitors (PPIs) in patients receiving low-dose anticoagulation and/or aspirin for stable cardiovascular disease confers no benefit for upper gastrointestinal events but may reduce bleeding due to gastroduodenal lesions, a study has shown.
29 Jun 2019
Monotherapy with a proton pump inhibitor appears to be effective in the treatment of functional dyspepsia symptoms associated with gastro-oesophageal reflux disease, according to a study.
07 Aug 2019
The combination of metformin, nystatin, linagliptin, rivaroxaban and a high-protein diet is the main cause of nasoenteral tube obstruction, with corresponding odds ratios of 2.0, 3.1, 4.3, 2.4 and 1.9, according to a recent study.