KEGG   DISEASE: Zollinger-Ellison syndromeHelp
Entry
H01522                      Disease                                

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 (see 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
Brite
Human diseases [BR:br08402]
 Endocrine and metabolic diseases
  Other endocrine and metabolic diseases
   H01522  Zollinger-Ellison syndrome
Human diseases in ICD-11 classification [BR:br08403]
 05 Endocrine, nutritional or metabolic diseases
  Endocrine diseases
   Other disorders of glucose regulation or pancreatic internal secretion
    5A43  Abnormal secretion of gastrin
     H01522  Zollinger-Ellison syndrome
BRITE hierarchy
Pathway
hsa04971  Gastric acid secretion
Network
N00290  Mutation-inactivated MEN1 to transcription
Gene
MEN1 [HSA:4221] [KO:K14970]
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]
Other DBs
ICD-11: 5A43.1
ICD-10: E16.4
MeSH: D015043
OMIM: 131100
Reference
  Authors
Ito T, Igarashi H, Uehara H, Jensen RT
  Title
Pharmacotherapy of Zollinger-Ellison syndrome.
  Journal
Expert Opin Pharmacother 14:307-21 (2013)
DOI:10.1517/14656566.2013.767332
Reference
  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
Reference
PMID:24319020 (description and diagnosis)
  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
Reference
PMID:19351273 (drug)
  Authors
Wilcox CM, Hirschowitz BI
  Title
Treatment strategies for Zollinger-Ellison syndrome.
  Journal
Expert Opin Pharmacother 10:1145-57 (2009)
DOI:10.1517/14656560902887035
Reference
PMID:16433582 (drug)
  Authors
Nieto JM, Pisegna JR
  Title
The role of proton pump inhibitors in the treatment of Zollinger-Ellison syndrome.
  Journal
Expert Opin Pharmacother 7:169-75 (2006)
DOI:10.1517/14656566.7.2.169

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