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Medicina Legal de Costa Rica

On-line version ISSN 2215-5287Print version ISSN 1409-0015


FERRANDINO CARBALLO, Fiorella  and  UMANA BRENES, Alberto Alonso. Síndrome de zolinger - Elison. Med. leg. Costa Rica [online]. 2016, vol.33, n.1, pp.275-281. ISSN 2215-5287.

Zollinger - Ellison syndrome is an endocrinopathy that was first described in 1955 by doctors Robert Zollinger and Edwin Ellison, who proposed the diagnostic triad that includes gastric acid hypersecretion, peptic ulcer and gastrinoma. This disease predominates in women between 50 and 60 years old. Based on the etiology, the syndrome is classified in sporadic or associated with multiple endocrine neoplasia type 1 (NEM - 1). Over half of gastrinomas are located in the duodenal wall, the pancreas is the second frequency location. There are ectopic locations, such as ovary, mesentery, liver and bile duct. Round cells, small nuclei and prominent nucleoli, are the main hispathologycal characteristics. Gastric acid hypersecretion is associated with a defect in the negative feedback inhibition of somatostatin on G antral gastrin-producing cells. Clinically, patients present abdominal pain, diarrhea, heartburn, nausea and vomiting; primarily related to the development of peptic ulcers. Diagnosis includes a measurement in serum gastrin levels and gastric pH values. The first line treatment is the antisecretory therapy, primarily proton-pump inhibitor. Imaging studies are useful to detect metastases and evaluate the surgically resectable disease. Neuroendocrine tumors and hypergastrinemia causes are the main differential diagnoses, the clinician should consider.

Keywords : Zollinger; Ellison Syndrome; gastrins; gastrinoma; multiple endocrine neoplasia type 1; hypergastrinemia; gastric Acid; neuroendocrine tumo.

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