Zollinger-Ellison syndrome pathophysiology
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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
Development of Zollinger-Ellison syndrome is the result of increased levels of gastrin due to an existing gastrinoma in the duodenum or pancreas.[1]
Pathogenesis
- Zollinger-Ellison syndrome is a disorder where increased levels of gastrin are produced, causing the stomach to produce excess hydrochloric acid. Often, the cause is a tumor (gastrinoma) of the duodenum or pancreas producing the hormone gastrin. Gastrin then causes an excessive production of acid which can lead to peptic ulcers (in almost 95% of patients).[1]
- Gastrin works on stomach parietal cells causing them to secrete more hydrogen ions into the stomach lumen. In addition, gastrin acts as a trophic factor for parietal cells, causing parietal cell hyperplasia. Thus, there is an increase in the number of acid secreting cells and each of these cells produces acid at a higher rate. The increase in acidity contributes to the development of peptic ulcers in the stomach and duodenum. High acid levels lead to multiple ulcers in the stomach and small bowel.
- The pathophysiology of ZES is related to the trophic action of gastrin on parietal cells of the gastric antrum and the resulting hypersecretory acid milleu. [2]
- An overwhelming majority of patients with this disease consequently develop peptic ulcers, often large and multiple, frequently in distal duodenum and even proximal jejunum (an uncommon location for ulcers resulting from Helicobacter pylori or the use of nonsteroidal anti-inflammatory drugs). [2]
Genetics
- Approximately 80% of the time, the primary causative lesion is thought to arise sporadically; in the remainder of recorded cases, this entity exists as part of MEN-1, an autosomal dominant disorder characterized by tumors of the pituitary, the parathyroid, and the pancreas. [3]
Associated Conditions
- Multiple endocrine neoplasia type 1 (MEN 1)
- Gastrinoma
References
- ↑ 1.0 1.1 wikipedia.2015.https://en.wikipedia.org/wiki/Zollinger%E2%80%93Ellison_syndrome
- ↑ 2.0 2.1 Epelboym I, Mazeh H (2014). "Zollinger-Ellison syndrome: classical considerations and current controversies". Oncologist. 19 (1): 44–50. doi:10.1634/theoncologist.2013-0369. PMC 3903066. PMID 24319020.
- ↑ Thakker RV, Newey PJ, Walls GV, Bilezikian J, Dralle H, Ebeling PR; et al. (2012). "Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1)". J Clin Endocrinol Metab. 97 (9): 2990–3011. doi:10.1210/jc.2012-1230. PMID 22723327.