Zollinger-Ellison syndrome

Common Name(s)

Zollinger-Ellison syndrome

Zollinger-Ellison syndrome (ZES) is a condition in which tumors called gastrinomas in the pancreas and duodenum (part of the small intestine) cause high levels of the hormone gastrin in the blood. High levels of gastrin then cause production of too much stomach acid. Signs and symptoms may include abdominal pain, peptic ulcers, vomiting blood, and diarrhea. The tumors are sometimes cancerous and may spread to other areas of the body. In most cases, the cause of ZES is unknown. However, about 25-30% of gastrinomas are caused by an inherited condition called multiple endocrine neoplasia type 1 (MEN1). Treatment for ZES may include medication to reduce the production of stomach acid, and surgery for peptic ulcers or to remove tumors.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Zollinger-Ellison syndrome" for support, advocacy or research.

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Zollinger-Ellison syndrome" returned 98 free, full-text research articles on human participants. First 3 results:

A case of Zollinger-Ellison syndrome in multiple endocrine neoplasia type 1 with urolithiasis as the initial presentation.
 

Author(s): Na Eun Lee, Young Jae Lee, So Hee Yun, Jae Un Lee, Moon Sik Park, Joong Keun Kim, Ji Woong Kim, Jin Woong Cho

Journal: Korean J Gastroenterol. 2013 Jun;61(6):333-7.

 

Zollinger-Ellison syndrome (ZES) is characterized by gastrinoma and resultant hypergastrinemia, which leads to recurrent peptic ulcers. Because gastrinoma is the most common pancreatic endocrine tumor seen in multiple endocrine neoplasia type I (MEN 1), the possibility of gastrinoma ...

Last Updated: 23 Jul 2013

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Hypochlorhydria and achlorhydria are associated with false-positive secretin stimulation testing for Zollinger-Ellison syndrome.
 

Author(s): Pari Shah, Maneesh H Singh, Yu-Xiao Yang, David C Metz

Journal: Pancreas. 2013 Aug;42(6):932-6.

 

Secretin stimulation testing (SST) is used to evaluate patients with hypergastrinemia in the diagnosis of Zollinger-Ellison syndrome. Case series have documented false-positive SST in patients with achlorhydria. This study reviews our experience with SST in hypochlorhydric and achlorhydric patients.

Last Updated: 15 Jul 2013

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Causes of death and prognostic factors in multiple endocrine neoplasia type 1: a prospective study: comparison of 106 MEN1/Zollinger-Ellison syndrome patients with 1613 literature MEN1 patients with or without pancreatic endocrine tumors.
 

Author(s): Tetsuhide Ito, Hisato Igarashi, Hirotsugu Uehara, Marc J Berna, Robert T Jensen

Journal: Medicine (Baltimore). 2013 May;92(3):135-81.

 

Multiple endocrine neoplasia type 1 (MEN1) is classically characterized by the development of functional or nonfunctional hyperplasia or tumors in endocrine tissues (parathyroid, pancreas, pituitary, adrenal). Because effective treatments have been developed for the hormone excess ...

Last Updated: 9 May 2013

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Zollinger-Ellison syndrome" returned 8 free, full-text review articles on human participants. First 3 results:

Zollinger-Ellison syndrome: classical considerations and current controversies.
 

Author(s): Irene Epelboym, Haggi Mazeh

Journal: Oncologist. 2014 Jan;19(1):44-50.

 

Zollinger-Ellison syndrome (ZES) is an endocrinopathy characterized by gastrin-secreting tumors, responsible for causing the formation of multiple, refractory, and recurrent peptic ulcers in the distal duodenum and proximal jejunum. Two main variants have been described, sporadic ...

Last Updated: 20 Jan 2014

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Pharmacotherapy of Zollinger-Ellison syndrome.
 

Author(s): Tetsuhide Ito, Hisato Igarashi, Hirotsugu Uehara, Robert T Jensen

Journal: Expert Opin Pharmacother. 2013 Feb;14(3):307-21.

 

The role of pharmacotherapy in the management of patients with Zollinger-Ellison syndrome (ZES) is often equated with the medical management of acid hypersecretion. However, pharmacotherapy is also increasingly involved in the other management areas of these patients.

Last Updated: 4 Feb 2013

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Diagnosis of Zollinger-Ellison syndrome: increasingly difficult.
 

Author(s): Tetsuhide Ito, Guillaume Cadiot, Robert T Jensen

Journal: World J. Gastroenterol.. 2012 Oct;18(39):5495-503.

 

In the present paper the increasing difficulty of diagnosis of Zollinger-Ellison syndrome (ZES) due to issues raised in two recent papers is discussed. These issues involve the difficulty and need to withdraw patients suspected of ZES from treatment with Proton Pump Inhibitors (omeprazole, ...

Last Updated: 31 Oct 2012

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

A Phase I/III Study of D961H 10 mg and 20 mg in Japanese Paediatric Patients With Gastrointestinal Acid Related Diseases
 

Status: Recruiting

Condition Summary: Gastric Ulcer (GU); Duodenal Ulcer (DU); Anastomotic Ulcer (AU); Non-erosive Reflux Esophagitis Disease (NERD); Reflux Esophagitis (RE); Zollinger-Ellison Syndrome

 

Last Updated: 4 Aug 2015

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Last Updated: 27 Mar 2015

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Pharmacokinetically Guided Everolimus in Patients With Breast Cancer, Pancreatic Neuroendocrine Tumors, or Kidney Cancer
 

Status: Recruiting

Condition Summary: Estrogen Receptor-positive Breast Cancer; Gastrinoma; Glucagonoma; HER2-negative Breast Cancer; Insulinoma; Mucositis; Oral Complications; Pancreatic Polypeptide Tumor; Progesterone Receptor-positive Breast Cancer; Recurrent Breast Cancer; Recurrent Islet Cell Carcinoma; Recurrent Renal Cell Cancer; Somatostatinoma; Stage III Renal Cell Cancer; Stage IIIA Breast Cancer; Stage IIIB Breast Cancer; Stage IIIC Breast Cancer; Stage IV Breast Cancer; Stage IV Renal Cell Cancer

 

Last Updated: 14 Jun 2015

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