Zollinger-Ellison syndrome natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Aravind Reddy Kothagadi M.B.B.S[2] Mohamad Alkateb, MBBCh [3]
Overview
In mild cases if left untreated, patients with Zollinger-Ellison syndrome may further develop abdominal pain, diarrhea, heartburn. Common complications of Zollinger-Ellison syndrome include upper gastrointestinal bleeding, anemia, and duodenal ulcer perforation. Prognosis is generally good when treated and the 5 and 10-year survival rate of patients with Zollinger-Ellison syndrome is around 94% and 75%, respectively. In severe cases if left untreated, ZES results in refractory peptic ulcer disease, severe gastroesophageal reflux disease, diarrhea, weight loss, anemia and finally death, mainly due to the complications of the refractory peptic ulcer disease.
Natural History, Complications, and Prognosis
Natural History
- ZES is a condition wherein the ectopic secretion of gastrin by a gastrinoma, primarily in the duodenum (60%-80%) or pancreas (10%-40%), results in the gastric acid hypersecretion. If left untreated, it results in refractory peptic ulcer disease, severe gastroesophageal reflux disease (GERD), diarrhea and finally death, mainly due to complications of the refractory peptic ulcer disease. [1]
- In mild cases if left untreated, patients may further develop abdominal pain, diarrhea, and heartburn.[2]
- The symptoms usually develop in the third decade of life and when left untreated, the patient may develop symptoms of gastrinoma, which may eventually lead to anemia and death.[3]
Complications
Common complications associated with Zollinger-Ellison syndrome include:
Prognosis
- The prognosis of Zollinger-Ellison syndrome is generally good when treated.
- The 5 and 10-year survival rates of patients with ZES is around 94% and 75%, respectively.
- In most of the cases when left untreated, Zollinger-Ellison syndrome may result in weight loss, anemia, and death.[4]
References
- ↑ Ito T, Cadiot G, Jensen RT (2012). "Diagnosis of Zollinger-Ellison syndrome: increasingly difficult". World J Gastroenterol. 18 (39): 5495–503. doi:10.3748/wjg.v18.i39.5495. PMC 3482635. PMID 23112541.
- ↑ Roy PK, Venzon DJ, Shojamanesh H, Abou-Saif A, Peghini P, Doppman JL; et al. (2000). "Zollinger-Ellison syndrome. Clinical presentation in 261 patients". Medicine (Baltimore). 79 (6): 379–411. PMID 11144036.
- ↑ Riegert-Johnson D, Gleeson FC, Westra W, et al. Peutz-Jeghers Syndrome. 2008 Jul 18 [Updated 2008 Aug 9]. In: Riegert-Johnson DL, Boardman LA, Hefferon T, et al., editors. Cancer Syndromes [Internet]. Bethesda (MD): National Center for Biotechnology Information (US); 2009-. Available from: http://www.ncbi.nlm.nih.gov/books/NBK1826/
- ↑ Melvin WS, Johnson JA, Sparks J, Innes JT, Ellison EC (1993). "Long-term prognosis of Zollinger-Ellison syndrome in multiple endocrine neoplasia". Surgery. 114 (6): 1183–8. PMID 7903006.