ZES
MCID: ZLL002
MIFTS: 64

Zollinger-Ellison Syndrome (ZES) malady

Gastrointestinal diseases, Endocrine diseases, Cancer diseases categories

Summaries for Zollinger-Ellison Syndrome

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8Disease Ontology, 42NIH Rare Diseases, 63Wikipedia, 32MalaCards
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NIH Rare Diseases:42 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 duodenitis. An important gene associated with Zollinger-Ellison Syndrome is MEN1 (multiple endocrine neoplasia I), and among its related pathways are PAK Pathway and Prostacyclin signalling through prostacyclin receptor. The drugs cimetidine and famotidine and the compounds cycloheximide and forskolin have been mentioned in the context of this disorder. Affiliated tissues include pancreas, liver and lymph node, and related mouse phenotypes are endocrine/exocrine gland and nervous system.

Disease Ontology:8 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:63 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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42NIH Rare Diseases, 48Orphanet, 60UMLS, 8Disease Ontology, 44Novoseek, 34MeSH, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet, 35MESH via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases
Anatomical: Gastrointestinal diseases, Endocrine diseases


Characteristics (Orphanet epidemiological data):

48
zollinger-ellison syndrome:
Inheritance: Sporadic; Prevalence: 1-9/100000; Age of onset: Adulthood; Age of death: Normal


Aliases & Descriptions:

zollinger-ellison syndrome 8 42 44 48 60
gastrinoma 42 48 60
pancreatic ulcerogenic tumor syndrome 42
z e syndrome 42
zes 42


External Ids:

Disease Ontology8 DOID:0050782
MeSH34 D015043
ICD10 via Orphanet26 E16.4
SNOMED-CT via Orphanet57 16189002, 302824004, 53132006
UMLS via Orphanet61 C0017150, C0043515
MESH via Orphanet35 D015043, D015408

Related Diseases for Zollinger-Ellison Syndrome

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17GeneCards, 18GeneDecks
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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 217)
idRelated DiseaseScoreTop Affiliating Genes
1gastrinoma31.6CHGA, GRP, MEN1, SCT, SST, GAST
2duodenitis31.2GAST
3pancreatitis31.2IL6, SCT, SST
4esophagitis31.0GAST, NTS, HRH2
5peptic ulcer31.0GAST, SCT, SST, DNAH8, HRH2, IL8
6duodenal ulcer30.9HRH2, GAST, GRP, SCT
7gastritis30.9IL8, SCT, GAST
8hepatitis30.9HRH2
9hyperparathyroidism30.9CHGA, MEN1, GAST, SCT
10islet cell tumor30.8GAST, SST
11cushing's syndrome30.8SST, POMC
12steatorrhea30.8SCT, SST
13gastric ulcer30.7GAST, EGF, HRH2
14primary hyperparathyroidism30.7GAST, SCT, CHGA, MEN1
15diarrhea30.7GAST, MLN, SST, CHGA
16gastroduodenitis30.5GAST
17pernicious anemia30.5GAST
18acromegaly30.5POMC, SST, GHRH, MEN1
19insulinoma30.5GRP, NTS, SST, SLC18A2, SCT, GAST
20adenocarcinoma30.5DNAH8, SCT, GRP, GAST, MEN1, EGF
21neuroendocrine tumor30.4CHGA, GHRH, SST, POMC
22cholelithiasis30.3SST
23tuberculosis30.3POMC
24wermer syndrome30.3SST, CHGA, GAST, MEN1
25atrophic gastritis30.3SST, CHGA, GAST, SCT, MEN1
26hypokalemia30.3DNAH8, POMC
27gastric adenocarcinoma30.3HRH2, GRP, CDH1, GAST, CHGA
28hyperthyroidism30.3DNAH8, SST, NTS, GHRH
29pneumonia30.3IL8, IL6, HRH2, GRP
30acute pancreatitis30.3HRH2, SCT, SST, IL8, IL6
31obesity30.3NTS, GRP, POMC, GHRH, IL6, MLN
32colorectal cancer30.3GRP, CDH1, NTS, MEN1, GAST, EGF
33gastroesophageal reflux disease30.3HRH2, DNAH8, NTS, MLN, GAST, SCT
34lung cancer30.3GRP, NTS, EGF, CDH1, CHGA, DNAH8
35pheochromocytoma30.3SLC18A2, POMC, SST, NTS, CHGA, MLN
36phaeochromocytoma29.8CHGA
37hemangioma29.8MEN1
38somatostatinoma29.8CHGA, GAST, GHRH, SST
39prolactinoma29.8SST, MEN1, CHGA, POMC
40colon cancer29.8NTS, GRP, CDH1, EGF, GAST
41gastric gastrinoma10.6
42cystadenocarcinoma10.6
43image syndrome10.6
44cystadenoma10.6
45duodenal gastrinoma10.6
46pancreatic gastrinoma10.5
47ovarian cystadenocarcinoma10.5
48ovarian cystadenoma10.5
49syndromic diarrhea10.5
50mucinous ovarian cystadenoma10.5

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



Diseases related to zollinger-ellison syndrome

Clinical Features for Zollinger-Ellison Syndrome

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Drugs & Therapeutics for Zollinger-Ellison Syndrome

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

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Drug clinical trials:

Search ClinicalTrials for Zollinger-Ellison Syndrome

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Search CenterWatch for Zollinger-Ellison Syndrome

Inferred drug relations via UMLS60/NDF-RT40:

Genetic Tests for Zollinger-Ellison Syndrome

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Anatomical Context for Zollinger-Ellison Syndrome

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32MalaCards
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MalaCards organs/tissues related to Zollinger-Ellison Syndrome:

32
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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36MGI
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MGI Mouse Phenotypes related to Zollinger-Ellison Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053798.8GAST, MEN1, EGF, GHRH, IL6
2MP:00036318.6SLC18A2, POMC, NTS, GHRH, SCT
3MP:00053767.8SLC18A2, SCT, GAST, MEN1, GHRH, NTS
4MP:00053817.5GAST, MEN1, EGF, SST, CDH1, IL6
5MP:00053877.2GAST, MEN1, EGF, GHRH, SST, POMC
6MP:00053786.9MEN1, EGF, GHRH, CHGA, NTS, SST

Publications for Zollinger-Ellison Syndrome

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50PubMed
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Articles related to Zollinger-Ellison Syndrome:

(show top 50)    (show all 515)
idTitleAuthorsYear
1
Pharmacotherapy of Zollinger-Ellison syndrome. (23363383)
2013
2
A case of Zollinger-Ellison syndrome in multiple endocrine neoplasia type 1 with urolithiasis as the initial presentation. (23877214)
2013
3
Image of the month. Wermer syndrome and Zollinger-Ellison syndrome. (19380653)
2009
4
Duodenal bulb mucosa with hypertrophic gastric oxyntic heterotopia in patients with zollinger ellison syndrome. (19587828)
2009
5
Zollinger-Ellison syndrome in 2006: concepts from a clinical point of view. (16633330)
2006
6
Advances in evaluation and management of gastrinoma in patients with Zollinger-Ellison syndrome. (15802099)
2005
7
A follow-up study of patients with Zollinger-Ellison syndrome in the period 1966-2002: effects of surgical and medical treatments on long-term survival. (15815204)
2005
8
Surgical treatment of Zollinger-Ellison syndrome in multiple endocrine neoplasia, type 1. (16295034)
2005
9
Risk factors for esophagitis in extreme acid hypersecretors with and without Zollinger-Ellison syndrome. (15017606)
2004
10
Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective study of 107 cases and comparison with 1009 cases from the literature. (14747767)
2004
11
Sporadic, syndromic, and Zollinger-Ellison syndrome associated fundic gland polyps consistently express cytokeratin 7. (12111207)
2002
12
A randomized controlled crossover study comparing synthetic porcine and human secretins with biologically derived porcine secretin to diagnose Zollinger-Ellison Syndrome. (11328261)
2001
13
The "serendipitous" surgical cure of the Zollinger-Ellison syndrome in a patient with multiple endocrine neoplasia type 1 despite an unsuspected diagnosis of either disease. (11246362)
2001
14
Hypertrophic gastritis, primary diffuse G-cell hyperplasia and pancreatic metaplasia of the gastric mucosa (pseudo-Zollinger-Ellison syndrome)--case report. (10833904)
2000
15
Duodenal microgastrinomas associated with Zollinger-Ellison syndrome. (8975949)
1996
16
Surgery in Zollinger-Ellison syndrome alters the natural history of gastrinoma. (7916560)
1994
17
The role of surgical treatment in Zollinger-Ellison syndrome. (7905671)
1994
18
Gastric endocrine cell evolution in patients with Zollinger-Ellison syndrome. Influence of gastrinoma growth and long-term omeprazole treatment. (8100759)
1993
19
Assessment and prediction of long-term cure in patients with the Zollinger-Ellison syndrome: the best approach. (8323088)
1993
20
Use of omeprazole in patients with Zollinger-Ellison syndrome. (2007355)
1991
21
Which diagnostic and therapeutic approach to the Zollinger-Ellison syndrome should be adopted in 1990?]. (1676864)
1991
22
Zollinger-Ellison syndrome. A new look at regression of gastrinomas. (2021333)
1991
23
Prospective study of the standard meal provocative test in Zollinger-Ellison syndrome. (2573280)
1989
24
Linear erosions on Kerckring's folds may be diagnostic of Zollinger-Ellison syndrome. (2754213)
1989
25
Microgastrinomas of the duodenum. A cause of failed operations for the Zollinger-Ellison syndrome. (2930285)
1989
26
Mechanism for increase of gastrin release by secretin in Zollinger-Ellison syndrome. (2565843)
1989
27
Recent advances in the management of gastric acid hypersecretion in patients with Zollinger-Ellison syndrome. (2575602)
1989
28
Long-acting somatostatin analog controls acid and gastrin secretion in benign, not in malignant, Zollinger-Ellison syndrome. (2754214)
1989
29
Management of Zollinger-Ellison syndrome with gastric antisecretory drugs. (3067333)
1988
30
Zollinger-Ellison syndrome and myelofibrosis in a dog. (3391836)
1988
31
Vagotomy in the management of patients with Zollinger-Ellison syndrome: pros and cons. (2861141)
1985
32
Pancreatic polypeptide and Zollinger-Ellison syndrome. (4053921)
1985
33
Omeprazole: effective, convenient therapy for Zollinger-Ellison syndrome. (3972233)
1985
34
Frequency of endocrine disorders in patients with the Zollinger-Ellison syndrome. (3887554)
1985
35
Control of the Zollinger-Ellison syndrome by excision of primary and metastatic tumor. (6703215)
1984
36
Gastric acid secretion in Zollinger-Ellison syndrome. (6483461)
1984
37
Reliability of symptoms in assessing control of gastric acid secretion in patients with Zollinger-Ellison syndrome. (6128284)
1983
38
Uncertainties in the management of the Zollinger-Ellison Syndrome. (6128287)
1983
39
Medical treatment of Zollinger-Ellison syndrome with ranitidine. (6323334)
1983
40
Effect of changes in serum calcium on secretin-stimulated serum gastrin in patients with Zollinger-Ellison syndrome. (6806138)
1982
41
Effect of somatostatin on gastrin, insulin and glucagon secretion in two patients with Zollinger-Ellison syndrome. (6120969)
1981
42
Results of surgical management in 92 consecutive patients with Zollinger-Ellison syndrome. (7030236)
1981
43
Diarrhoea and reversible enteropathy in Zollinger-Ellison syndrome. (6116088)
1981
44
Interest of a combined antisecretory treatment, cimetidine and pirenzepin, in the management of severe forms of Zollinger-Ellison syndrome. (7353734)
1980
45
CT scanning in patients with Zollinger-Ellison syndrome and carcinoid syndrome. (290030)
1979
46
The Zollinger--Ellison syndrome. (383324)
1979
47
Zollinger-Ellison syndrome with pneumopericardium. (456233)
1979
48
Non-beta islet cell tumor calcification assoicated with Zollinger-Ellison syndrome and multiple endocrine adenomatosis. (28264)
1977
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

Genetic Variations for Zollinger-Ellison Syndrome

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Expression for genes affiliated with Zollinger-Ellison Syndrome

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1BioGPS, 15Gene 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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Sources:
51QIAGEN, 53Reactome, 37NCBI BioSystems Database, 29KEGG
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Compounds for genes affiliated with Zollinger-Ellison Syndrome

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44Novoseek, 49PharmGKB, 11DrugBank, 28IUPHAR, 24HMDB, 59Tocris Bioscience, 2BitterDB
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Compounds related to Zollinger-Ellison Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 186)
idCompoundScoreTop Affiliating Genes
1cycloheximide4410.1GHRH
2forskolin44 49 1111.8GHRH, POMC, HRH2, SCT
3ranitidine44 28 11 2412.7SCT, HRH2, MLN
4pge2449.5CDH1, POMC, SST, GRP
5pepsinogen449.2SCT, GAST, CHGA, GRP, HRH2
6loxiglumide449.1NTS, SCT, GAST, GRP, SST
7pirenzepine44 28 1111.1SST, HRH2, GAST, GHRH, GRP
8gnrh449.1GHRH, NTS, GAST, CHGA, GRP
9lafutidine449.1SCT, HRH2, SST, MLN, GAST
10pantoprazole44 49 11 2412.1GAST, SST, DNAH8, HRH2
11peptidylglycine449.1CHGA, POMC, GRP, GAST
12domperidone44 28 1110.9HRH2, SST, MLN, GAST
13bethanechol44 28 1110.8MLN, GRP, SST, GAST
14propranolol44 28 49 11 2412.8GHRH, SCT, GAST, SST, GRP, CHGA
15clonidine44 28 1110.8SCT, SST, CHGA, NTS, GHRH, DNAH8
16dexamethasone44 49 28 1111.8GHRH, NTS, POMC, CDH1, GRP, IL6
17opiate448.7HRH2, DNAH8, POMC, NTS, GRP, GHRH
18isoproterenol44 119.6HRH2, SST, NTS, GRP, CHGA, GAST
19amine448.5NTS, SLC18A2, SCT, GAST, CHGA, HRH2
20amphetamine44 119.4GRP, NTS, SLC18A2, POMC, SST
21atropine44 28 1110.3HRH2, NTS, GRP, GHRH, MLN, GAST
22omeprazole44 28 49 11 2412.3GAST, CHGA, HRH2, DNAH8, SST, MLN
23oxyntomodulin44 599.3SCT, GAST, MLN, SST, NTS, GRP
24dopamine44 28 11 2411.3GHRH, CHGA, NTS, GRP, MLN, SCT
25glutamate448.3SLC18A2, SCT, GAST, GHRH, NTS, DNAH8
26carbachol44 28 1110.2SCT, EGF, GAST, GRP, CHGA, NTS
27nifedipine44 49 28 1111.2SST, GHRH, GRP, CHGA, GAST, NTS
28hydrocortisone44 2 59 1111.2IL6, IL8, POMC, SST, GRP, EGF
29cyclic amp44 249.1NTS, HRH2, EGF, GHRH, CHGA, GRP
30thymidine44 249.0SCT, IL6, GAST, MEN1, EGF, GRP
31thyroxine44 249.0SST, NTS, GHRH, GAST, SCT, POMC
32indomethacin44 59 28 1110.9POMC, EGF, NTS, GRP, IL6, CDH1
33glucose447.8MLN, GHRH, NTS, SLC18A2, POMC, GAST
34gaba447.7SLC18A2, GAST, NTS, GRP, SCT, CHGA
35creatinine447.6GRP, CDH1, DNAH8, HRH2, CHGA, POMC
36norepinephrine44 11 249.5SLC18A2, DNAH8, POMC, NTS, GRP, CHGA
37octreotide44 59 28 1110.4EGF, MLN, GHRH, CHGA, GRP, NTS
38vegf447.4IL6, MEN1, GAST, CHGA, GRP, SST
39acth447.3POMC, MLN, GAST, SCT, GHRH, CHGA
40serine447.3HRH2, SLC18A2, IL8, POMC, NTS, GHRH
41arginine447.3CDH1, SCT, MLN, GHRH, CHGA, GRP
42retinoic acid44 247.8EGF, DNAH8, GRP, IL6, CDH1, CHGA
43testosterone44 59 11 249.8IL6, CDH1, DNAH8, POMC, SCT, GAST
445-hydroxytryptamine446.6SLC18A2, HRH2, DNAH8, POMC, SST, NTS
45epinephrine44 11 248.6POMC, SST, NTS, GRP, CHGA, GAST
46acetylcholine44 49 28 11 2410.4SLC18A2, GAST, MLN, CHGA, GRP, NTS
47adenylate446.3SCT, GAST, EGF, MLN, GHRH, CHGA
48histamine44 28 248.1SLC18A2, GAST, MLN, CHGA, GRP, NTS
49calcium44 49 11 248.7GHRH, SLC18A2, MLN, NTS, DNAH8, CDH1
50tyrosine445.7GRP, MLN, EGF, CDH1, NTS, IL6

GO Terms for genes affiliated with Zollinger-Ellison Syndrome

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16Gene Ontology
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Cellular components related to Zollinger-Ellison Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:0056158.0GHRH, GRP, SST, POMC, IL8, IL6
2extracellular regionGO:0055765.9IL6, SCT, GAST, EGF, MLN, GHRH

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

idNameGO IDScoreTop Affiliating Genes
1regulation of protein localization to cell surfaceGO:20000089.8CDH1, EGF
2G-protein coupled receptor signaling pathwayGO:0071868.8IL8, SST, MLN, GAST
3cell-cell signalingGO:0072678.7MLN, GHRH, SST, POMC
4signal transductionGO:0071658.1GAST, EGF, GRP, NTS, POMC, IL8

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

idNameGO IDScoreTop Affiliating Genes
1hormone activityGO:0051798.6POMC, SST, MLN, GAST, SCT

Products for genes affiliated with Zollinger-Ellison Syndrome

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Sources for Zollinger-Ellison Syndrome

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet