Zollinger-Ellison Syndrome (ZES)
Overview
Plain-Language Overview
Zollinger-Ellison Syndrome (ZES) is a rare condition that affects the digestive system, specifically the stomach and pancreas. It occurs when tumors called gastrinomas produce too much of the hormone gastrin, which causes the stomach to make excessive acid. This excess acid can lead to painful stomach ulcers, diarrhea, and damage to the intestines. The condition mainly affects the gastrointestinal tract and can cause symptoms like abdominal pain and heartburn. Because of the high acid levels, people with this syndrome often have trouble digesting food properly and may experience complications like bleeding ulcers.
Clinical Definition
Zollinger-Ellison Syndrome (ZES) is characterized by the presence of one or more gastrin-secreting neuroendocrine tumors (gastrinomas), typically located in the pancreas or duodenum. These tumors cause hypergastrinemia, leading to excessive gastric acid secretion and resulting in severe peptic ulcer disease, often refractory to standard treatment. The syndrome is associated with multiple endocrine neoplasia type 1 (MEN1) in some cases, involving mutations in the MEN1 gene. Clinically, patients present with recurrent or multiple ulcers, diarrhea, and abdominal pain. The excessive acid secretion can cause complications such as esophagitis, gastric mucosal hypertrophy, and malabsorption. Diagnosis and management require understanding the tumor's secretory activity and its systemic effects.
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