ZES
MCID: ZLL002
MIFTS: 65

Zollinger-Ellison Syndrome (ZES) malady

Rare diseases, Gastrointestinal diseases, Endocrine diseases, Cancer diseases categories

Summaries for Zollinger-Ellison Syndrome

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9Disease Ontology, 44NIH Rare Diseases, 66Wikipedia, 34MalaCards
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NIH Rare Diseases:44 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. last updated: 1/14/2014

MalaCards: Zollinger-Ellison Syndrome, also known as gastrinoma, is related to gastrinoma and multiple endocrine neoplasia. An important gene associated with Zollinger-Ellison Syndrome is MEN1 (multiple endocrine neoplasia I), and among its related pathways are Syndecan-3-mediated signaling events and Immune response MIF mediated glucocorticoid regulation. The drugs cimetidine and famotidine and the compounds pantoprazole and pepsinogen have been mentioned in the context of this disorder. Affiliated tissues include pancreas, liver and lymph node, and related mouse phenotypes are behavior/neurological and digestive/alimentary.

Disease Ontology:9 A syndrome that is haracterized by the development of a one or more tumors (gastrinoma) in the pancreas, duodenum, or both that secrete excessive levels of gastrin, a hormone that stimulates production of acid by the stomach.

Wikipedia:66 Zollinger?Ellison Syndrome (ZES) is caused by a non?beta islet cell, gastrin-secreting tumor of the... more...

Aliases & Classifications for Zollinger-Ellison Syndrome

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44NIH Rare Diseases, 50Orphanet, 63UMLS, 9Disease Ontology, 46Novoseek, 36MeSH, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet, 64UMLS via Orphanet, 37MESH via Orphanet
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Classifications:



Characteristics (Orphanet epidemiological data):

50
zollinger-ellison syndrome:
Inheritance: Sporadic; Prevalence: 1-9/100000; Age of onset: Adulthood; Age of death: Normal


Aliases & Descriptions:

zollinger-ellison syndrome 9 44 46 50 63
gastrinoma 44 50 63
pancreatic ulcerogenic tumor syndrome 44
z e syndrome 44
zes 44


External Ids:

Disease Ontology9 DOID:0050782
MeSH36 D015043
ICD10 via Orphanet27 E16.4
SNOMED-CT via Orphanet60 16189002, 302824004, 53132006
UMLS via Orphanet64 C0017150, C0043515
MESH via Orphanet37 D015043, D015408

Related Diseases for Zollinger-Ellison Syndrome

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18GeneCards, 19GeneDecks
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Diseases related to Zollinger-Ellison Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 180)
idRelated DiseaseScoreTop Affiliating Genes
1gastrinoma31.7SCT, CHGA, SST, MEN1, GRP, GAST
2multiple endocrine neoplasia31.6GAST, MEN1, CHGA
3pancreatitis31.4SCT, SST, IL6
4esophagitis31.3NTS, GAST, HRH2
5duodenal ulcer31.1SCT, GRP, GAST, HRH2
6gastritis31.1GAST, IL8, SCT
7islet cell tumor31.1SST, GAST
8hyperparathyroidism31.1SCT, CHGA, MEN1, GAST
9steatorrhea31.0SST, SCT
10cushing's syndrome31.0POMC, SST
11primary hyperparathyroidism30.8SCT, CHGA, MEN1, GAST
12pernicious anemia30.8GAST
13diarrhea30.8MLN, CHGA, SST, GAST
14acromegaly30.7GHRH, MEN1, SST, POMC
15peptic ulcer disease30.6GAST, DNAH8, IL8, SST, SCT, HRH2
16apudoma30.6SST
17neuroendocrine tumor30.5GHRH, SST, CHGA, POMC
18carcinoid syndrome30.5SST, CHGA, POMC, SCT, MEN1, SLC18A2
19wermer syndrome30.5GAST, MEN1, SST, CHGA
20insulinoma30.5SCT, SLC18A2, GAST, GRP, GHRH, NTS
21hypokalemia30.4POMC, DNAH8
22hyperthyroidism30.4SST, NTS, DNAH8, GHRH
23adenocarcinoma30.4SCT, CHGA, SST, MEN1, NTS, DNAH8
24colorectal cancer30.4MEN1, NTS, GRP, GAST, EGF
25pneumonia30.4IL8, IL6, GRP, HRH2
26gastroesophageal reflux disease30.4MLN, SCT, NTS, DNAH8, GAST, HRH2
27lung cancer30.3CHGA, NTS, DNAH8, GRP, EGF
28obesity30.3MLN, POMC, NTS, IL6, GHRH, GRP
29glucagonoma30.3GAST, GHRH, MEN1, SST, CHGA, SCT
30atrophic gastritis30.2SCT, CHGA, SST, MEN1, GAST
31pheochromocytoma30.1MLN, SLC18A2, EGF, NTS, MEN1, SST
32gastric outlet obstruction30.1GAST, MLN
33somatostatinoma30.0GAST, GHRH, SST, CHGA
34pancreatic islet cell tumors30.0CHGA, SST, MEN1, GAST
35prolactinoma30.0POMC, CHGA, SST, MEN1
36duodenitis10.9
37cystadenocarcinoma10.7
38cystadenoma10.6
39hepatitis10.6
40duodenal gastrinoma10.5
41mucinous cystadenocarcinoma10.5
42somatostatin analog10.5
43crohn's disease10.4
44barrett's esophagus10.4
45gastroduodenitis10.4
46ovarian mucinous cystadenocarcinoma10.4
47ovarian cystadenocarcinoma10.4
48pancreatic endocrine tumors10.4
49neurofibromatosis10.3
50alcohol abuse10.3

Graphical network of the top 20 diseases related to Zollinger-Ellison Syndrome:



Diseases related to zollinger-ellison syndrome

Symptoms for Zollinger-Ellison Syndrome

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Drugs & Therapeutics for Zollinger-Ellison Syndrome

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6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

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Drug clinical trials:

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Inferred drug relations via UMLS63/NDF-RT42:

Genetic Tests for Zollinger-Ellison Syndrome

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Anatomical Context for Zollinger-Ellison Syndrome

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34MalaCards
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MalaCards organs/tissues related to Zollinger-Ellison Syndrome:

34
Pancreas, Liver, Lymph node, Ovary, Kidney, Small intestine, Heart, Testes, Colon, Pancreatic islet, Lung

Animal Models for Zollinger-Ellison Syndrome or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Zollinger-Ellison Syndrome:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053868.3POMC, SST, NTS, IL6, HRH2, SLC18A2
2MP:00053818.1SST, MEN1, IL6, GAST, EGF, HRH2
3MP:00053798.0POMC, CHGA, MEN1, IL6, GHRH, GAST
4MP:00036317.9SCT, POMC, SST, MEN1, NTS, IL6
5MP:00053787.7POMC, CHGA, SST, MEN1, NTS, IL6
6MP:00053877.3POMC, SST, MEN1, IL8, IL6, GHRH
7MP:00053767.2SLC18A2, SCT, POMC, CHGA, SST, MEN1

Publications for Zollinger-Ellison Syndrome

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53PubMed
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Articles related to Zollinger-Ellison Syndrome:

(show top 50)    (show all 506)
idTitleAuthorsYear
1
Case of Zollinger-Ellison syndrome diagnosed three years after ulcer perforation in the third portion of the duodenum]. (18460860)
2008
2
Gastric carcinoid tumors in multiple endocrine neoplasia-1 patients with Zollinger-Ellison syndrome can be symptomatic, demonstrate aggressive growth, and require surgical treatment. (15657586)
2004
3
Gastric collision between a papillotubular adenocarcinoma and a gastrinoma in a patient with Zollinger-Ellison syndrome. (12447676)
2002
4
Bleeding from alimentary tract in pseudo Zollinger-Ellison syndrome. (11208377)
2000
5
Zollinger-Ellison syndrome. Improved treatment options for this complex disorder. (9676569)
1998
6
Zollinger-Ellison syndrome with fasting hypoglycaemia. (9602752)
1998
7
Should the 1996 citation for Zollinger-Ellison syndrome read: "Acid-reducing surgery in, aggressive resection out"? (8651148)
1996
8
Pregnancy does not offer protection against Zollinger-Ellison syndrome. (8607350)
1996
9
Severe, recurrent peptic ulcer disease: a case of the Zollinger-Ellison syndrome. (7674667)
1995
10
Three months of octreotide treatment decreases gastric acid secretion and argyrophil cell density in patients with Zollinger-Ellison syndrome and antral G-cell hyperfunction. (7910489)
1994
11
Surgery in Zollinger-Ellison syndrome alters the natural history of gastrinoma. (7916560)
1994
12
Duodenal gastrinomas, duodenotomy, and duodenal exploration in the surgical management of Zollinger-Ellison syndrome. (8103249)
1993
13
Influence of multiple endocrine neoplasia type 1 on gastric endocrine cells in patients with the Zollinger-Ellison syndrome. (1358767)
1992
14
Stereologic investigations of human gastric mucosa. II. Oxyntic mucosa from patients with Zollinger-Ellison syndrome. (1439541)
1992
15
Effects of curative gastrinoma resection on gastric secretory function and antisecretory drug requirement in the Zollinger-Ellison syndrome. (1537514)
1992
16
Pneumocystis carinii pneumonia complicating somatostatin therapy of Cushing's syndrome in a patient with metastatic pancreatic islet cell carcinoma and Zollinger-Ellison syndrome. (1672788)
1991
17
Changing patterns in presentation and management of the Zollinger-Ellison syndrome in Northern Ireland, 1970-1988. (1675471)
1991
18
Zollinger-Ellison syndrome. Relation to Helicobacter pylori-associated chronic gastritis and gastric acid secretion. (1984995)
1991
19
Progastrin in pancreas and the Zollinger-Ellison syndrome. (2274740)
1990
20
Secretin and calcium provocative tests in the Zollinger-Ellison syndrome. A prospective study. (2572194)
1989
21
Zollinger-Ellison syndrome in a cat. (3176293)
1988
22
Gastrinoma (Zollinger-Ellison Syndrome) in the Dog and cat. (17423047)
1988
23
Gastrin-producing ovarian mucinous cystadenomas: a cause of the Zollinger-Ellison syndrome. (3187898)
1988
24
Zollinger-Ellison syndrome. Current concepts in diagnosis and management. (3309661)
1987
25
Prospective study of the ability of computed axial tomography to localize gastrinomas in patients with Zollinger-Ellison syndrome. (3556996)
1987
26
Pseudo-Zollinger--Ellison syndrome in a child presenting with anemia. (3792786)
1987
27
Ectopic gastrinoma and Zollinger-Ellison syndrome. (3710471)
1986
28
Oxyntic mucosa histology in omeprazole-treated patients suffering from duodenal ulcer or Zollinger-Ellison syndrome. (3792669)
1986
29
Controversies in the management of Zollinger-Ellison syndrome. (2877606)
1986
30
Finger clubbing in the Zollinger-Ellison syndrome. (3098343)
1986
31
Cushing's syndrome in patients with the Zollinger-Ellison syndrome. (2872593)
1986
32
Case 3. Renal gastrinoma with Zollinger-Ellison syndrome. (3588443)
1986
33
Synchronous perforation of the jejunum as presenting feature of Zollinger-Ellison syndrome. (4013608)
1985
34
Vagotomy in the management of patients with Zollinger-Ellison syndrome: pros and cons. (2861141)
1985
35
Zollinger-Ellison syndrome due to a gastrin secreting ovarian mucinous cystadenoma. Case report. (4027214)
1985
36
Omeprazole in Zollinger-Ellison syndrome. Effects of a single dose and of long-term treatment in patients resistant to histamine H2-receptor antagonists. (6142418)
1984
37
Zollinger-Ellison syndrome. A report of eight cases. (6510941)
1984
38
Control of the Zollinger-Ellison syndrome by excision of primary and metastatic tumor. (6703215)
1984
39
Secretin-induced gastrin response in the Zollinger-Ellison syndrome and chronic duodenal ulcer patients before and after cimetidine treatment. (6720654)
1984
40
Zollinger-Ellison syndrome--report of four cases and review of literature. (6146289)
1983
41
Reliability of symptoms in assessing control of gastric acid secretion in patients with Zollinger-Ellison syndrome. (6128284)
1983
42
Zollinger-Ellison Syndrome. (7142852)
1982
43
Diagnosis and management of gastrinoma (Zollinger-Ellison Syndrome) (7070121)
1982
44
The Zollinger-Ellison syndrome and ectopic gastric mucosa. (6815626)
1982
45
Zollinger-Ellison syndrome: intermittent diarrhea, relentless hypokalemia, and hypergastrinemia. (7328691)
1981
46
Pathophysiological responses to meals in the Zollinger-Ellison syndrome: 2. Gastric emptying and its effect on duodenal function. (7380344)
1980
47
Zollinger-Ellison syndrome. (7421193)
1980
48
Conservative management of the Zollinger-Ellison syndrome. Ectopic gastrin production by an ovarian cystadenoma. (7365959)
1980
49
Perforation of a gastrojejunal ulcer into the pericardium. Complication of Wermer's disease (Zollinger-Ellison syndrome). (4380997)
1967
50
Islet cell tumor. Diarrhea, and hypopotassemia (the Zollinger-Ellison syndrome?). (13903135)
1962

Variations for Zollinger-Ellison Syndrome

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Expression for genes affiliated with Zollinger-Ellison Syndrome

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Zollinger-Ellison Syndrome

Search GEO for disease gene expression data for Zollinger-Ellison Syndrome.

Pathways for genes affiliated with Zollinger-Ellison Syndrome

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51PathCards, 39NCBI BioSystems Database, 61Thomson Reuters, 31KEGG, 56Reactome, 54QIAGEN
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Compounds for genes affiliated with Zollinger-Ellison Syndrome

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46Novoseek, 52PharmGKB, 25HMDB, 12DrugBank, 30IUPHAR, 62Tocris Bioscience, 3BitterDB
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Compounds related to Zollinger-Ellison Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 210)
idCompoundScoreTop Affiliating Genes
1pantoprazole46 52 25 1212.0SST, DNAH8, GAST, HRH2
2pepsinogen469.0HRH2, GAST, SCT, CHGA, GRP
3loxiglumide468.9SCT, GAST, GRP, NTS, SST
4pirenzepine46 30 1210.9SST, GAST, HRH2, GRP, GHRH
5amine468.9HRH2, SLC18A2, SCT, CHGA, NTS, GAST
6c-peptide468.9GAST, CHGA, SST, NTS, SCT
7peptidylglycine468.8GRP, CHGA, POMC, GAST
8lafutidine468.8HRH2, GAST, SST, SCT, MLN
9amphetamine46 52 1210.8POMC, GRP, NTS, SST, SLC18A2
10ranitidine46 30 25 1211.7MLN, CHGA, GAST, SCT, HRH2
11clonidine46 52 30 1211.7SCT, POMC, SST, NTS, DNAH8, GHRH
12propranolol46 30 52 25 1212.6SCT, CHGA, SST, GHRH, GRP, GAST
13opiate468.6POMC, NTS, DNAH8, GRP, GHRH, HRH2
14bethanechol46 30 1210.4SST, GAST, GRP, MLN
15isoproterenol46 129.4GAST, NTS, CHGA, HRH2, SCT, GRP
16domperidone46 30 1210.3SST, MLN, HRH2, GAST
17carbachol46 30 1210.2SST, GRP, CHGA, NTS, GAST, EGF
18forskolin46 52 1210.2NTS, SCT, GHRH, HRH2, EGF, POMC
19tetrodotoxin46 62 3010.2MLN, HRH2, NTS, SCT, GRP
20omeprazole46 52 30 25 1212.0MLN, SCT, SST, DNAH8, GAST, HRH2
21gaba468.0POMC, CHGA, SCT, SST, NTS, GRP
22gnrh467.9GRP, NTS, EGF, GAST, GHRH, SST
23indomethacin46 30 62 1210.9IL6, GRP, EGF, DNAH8, SCT, POMC
24hydrocortisone46 3 62 1210.9POMC, SST, IL8, IL6, GAST, EGF
25oxyntomodulin46 628.8MLN, SCT, CHGA, SST, NTS, GRP
26thymidine46 258.8MEN1, IL6, GRP, SCT, GAST, NTS
27nifedipine46 30 52 1210.8MLN, NTS, SST, GHRH, GRP, GAST
28cycloheximide467.7IL8, IL6, GRP, GHRH, POMC, EGF
29atropine46 30 129.6GRP, GAST, HRH2, GHRH, NTS, MLN
30thyroxine46 258.6GAST, DNAH8, GHRH, SCT, POMC, SST
31hydrogen46 258.5SCT, MLN, DNAH8, GAST, CHGA, SLC18A2
32glutamate467.4GRP, SST, CHGA, SCT, GHRH, DNAH8
33retinoic acid46 258.3HRH2, CHGA, SCT, IL6, DNAH8, SST
34vegf467.3GRP, IL6, IL8, MEN1, EGF, POMC
35pge2467.2HRH2, EGF, GRP, IL6, IL8, NTS
36arginine467.1SST, SCT, MLN, POMC, CHGA, DNAH8
37dexamethasone46 52 30 1210.0CHGA, IL8, EGF, IL6, GHRH, SST
38glucose467.0SLC18A2, MLN, SCT, POMC, SST, NTS
39octreotide46 62 30 129.9EGF, NTS, SST, GHRH, CHGA, POMC
40dopamine46 30 25 129.9SLC18A2, GAST, MLN, SCT, POMC, CHGA
41acth466.8POMC, MLN, SCT, CHGA, SST, NTS
42epinephrine46 25 128.8SCT, POMC, CHGA, SST, NTS, IL6
43norepinephrine46 25 128.8GHRH, GRP, GAST, SLC18A2, DNAH8, IL6
44testosterone46 62 25 129.7GAST, GRP, GHRH, DNAH8, IL6, EGF
455-hydroxytryptamine466.7SLC18A2, HRH2, GAST, GRP, DNAH8, NTS
46acetylcholine46 52 30 25 1210.4SLC18A2, HRH2, GAST, GRP, DNAH8, NTS
47histamine46 30 258.4NTS, IL8, IL6, GRP, GAST, SST
48adenylate466.2MLN, SCT, POMC, CHGA, SST, NTS
49calcium46 52 25 129.1SCT, MLN, SLC18A2, CHGA, MEN1, NTS
50serine466.0MLN, SCT, NTS, SLC18A2, HRH2, EGF

GO Terms for genes affiliated with Zollinger-Ellison Syndrome

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17Gene Ontology
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Cellular components related to Zollinger-Ellison Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:0056157.8SST, IL8, IL6, GHRH, GRP, EGF
2extracellular regionGO:0055765.3EGF, MLN, SCT, POMC, CHGA, SST

Biological processes related to Zollinger-Ellison Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1response to amino acidGO:0432009.7IL6, SST
2negative regulation of cell proliferationGO:0082858.8IL6, IL8, MEN1, SST
3cell-cell signalingGO:0072678.8MLN, POMC, SST, GHRH
4G-protein coupled receptor signaling pathwayGO:0071868.3MLN, SST, IL8, GAST
5signal transductionGO:0071658.0EGF, GAST, GRP, IL8, NTS, POMC

Molecular functions related to Zollinger-Ellison Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1neuropeptide hormone activityGO:0051849.6GRP, NTS
2hormone activityGO:0051798.0GAST, SST, POMC, SCT, MLN

Products for genes affiliated with Zollinger-Ellison Syndrome

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Sources for Zollinger-Ellison Syndrome

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet