| H01522 | |
| H number | H01522 |
| Name | Zollinger-Ellison syndrome |
| Description | Zollinger-Ellison syndrome (ZES) is a rare endocrinopathy caused by tumors of the pancreas and duodenum. These tumors, called gastrinomas, release gastrin to produce large amounts of acid that result in severe gastroesophageal peptic ulcer disease and diarrhea. Most ZES cases are sporadic, but about over 20 percent are caused by an inherited genetic disorder called multiple endocrine neoplasia type 1 (MEN1) [DS:H00247]. The clinical presentation is not specific for this disease and there is overlap of symptoms similar to those of a peptic ulcer. The most common symptoms include abdominal pain and diarrhea, sometimes accompanied by heartburn, nausea, and weight loss. Peptic ulceration complicated by bleeding is present in 25% of patients, and is more frequently in patients with sporadic ZES than in those with MEN1. In addition, the gastrinomas may be cancerous. The cancer can be spread to other parts of the body, most commonly to regional lymph nodes and the liver. The treatment of the ZES includes surgical removal and medical management of gastric acid hypersecretion for the prevention of malignant transformation and the genesis of complications. |
| Category | Endocrine and metabolic disease |
| Network | - |
| Gene | MEN1 [HSA:4221] [KO:K14970] |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | Cimetidine [DR:D00295] Cimetidine hydrochloride [DR:D03503] Ranitidine hydrochloride [DR:D00673] Famotidine [DR:D00318] Omeprazole [DR:D00455] Omeprazole magnesium [DR:D05259] Pantoprazole sodium [DR:D02593] Lansoprazole [DR:D00355] Rabeprazole sodium [DR:D00724] Esomeprazole magnesium [DR:D01984] Esomeprazole strontium [DR:D10120] Esomeprazole magnesium dihydrate [DR:D11114] |
| Comment | - |
| Other DBs | ICD-11: 5A43.1 ICD-10: E16.4 MeSH: D015043 OMIM: 131100 |
| Reference | PMID:24319020 AUTHORS Epelboym I, Mazeh H TITLE Zollinger-Ellison syndrome: classical considerations and current controversies. JOURNAL Oncologist 19:44-50 (2014) DOI:10.1634/theoncologist.2013-0369 PMID:9215689 (MEN1) AUTHORS Agarwal SK, Kester MB, Debelenko LV, Heppner C, Emmert-Buck MR, Skarulis MC, Doppman JL, Kim YS, Lubensky IA, Zhuang Z, Green JS, Guru SC, Manickam P, Olufemi SE, Liotta LA, Chandrasekharappa SC, Collins FS, Spiegel AM, Burns AL, Marx SJ TITLE Germline mutations of the MEN1 gene in familial multiple endocrine neoplasia type 1 and related states. JOURNAL Hum Mol Genet 6:1169-75 (1997) DOI:10.1093/hmg/6.7.1169 PMID:14747767 AUTHORS Gibril F, Schumann M, Pace A, Jensen RT TITLE Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective study of 107 cases and comparison with 1009 cases from the literature. JOURNAL Medicine (Baltimore) 83:43-83 (2004) DOI:10.1097/01.md.0000112297.72510.32 |