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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8Disease Ontology, 42NIH Rare Diseases, 44Novoseek, 48Orphanet, 60UMLS, 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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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

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Drug clinical trials:

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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, Heart, Small intestine, 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
Long-term follow-up of a child with primary lymph node gastrinoma and Zollinger-Ellison syndrome. (21616263)
2011
2
Zollinger-Ellison syndrome due to primary gastrinoma of the extrahepatic biliary tree: three case reports and review of literature. (19491075)
2009
3
Case of Zollinger-Ellison syndrome diagnosed three years after ulcer perforation in the third portion of the duodenum]. (18460860)
2008
4
Serum gastrin in Zollinger-Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National Institutes of Health and comparison with 537 cases from the literature. evaluation of diagnostic criteria, proposal of new criteria, and correlations with clinical and tumoral features. (17108779)
2006
5
Zollinger-Ellison syndrome caused by solitary gastrinoma blocking the pancreatic duct: enucleation and pancreatic duct repair. (16003327)
2005
6
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
7
Zollinger-Ellison syndrome revisited: diagnosis, biologic markers, associated inherited disorders, and acid hypersecretion. (15527675)
2004
8
Prognostic value of initial fasting serum gastrin levels in patients with Zollinger-Ellison syndrome. (11408501)
2001
9
Association of Zollinger-Ellison syndrome with pancreatitis: report of five cases. (11007101)
2000
10
The gastrinoma/Zollinger-Ellison syndrome: statistical evaluation of a Japanese series of 359 cases. (9683758)
1998
11
Processing-independent assay of serum gastrin for diagnosis of liver metastases in the Zollinger-Ellison syndrome. GRESZE (Groupe de Recherche et d'Etude du syndrome de Zollinger-Ellison) (9139858)
1997
12
Management of the Zollinger-Ellison syndrome in pregnancy. (9024119)
1997
13
Ultrastructural aspects of the neuroendocrine complex in the stomach in patients with either pernicious anemia or the Zollinger-Ellison syndrome. (8776836)
1996
14
Esophageal function and occurrence of Barrett's esophagus in Zollinger-Ellison syndrome. (8549876)
1995
15
Zollinger-Ellison syndrome and antral G-cell hyperfunction in patients with resistant duodenal ulcer disease. (8186351)
1994
16
Assessing the cure of the Zollinger-Ellison syndrome after gastrinoma resection. (8256978)
1994
17
Clinical, anatomical, and evolutive features of patients with the Zollinger-Ellison syndrome combined with type I multiple endocrine neoplasia. (8097874)
1993
18
Long-term prognosis of Zollinger-Ellison syndrome in multiple endocrine neoplasia. (7903006)
1993
19
Gastric endocrine cell proliferation and fundic argyrophil carcinoid tumors in patients with the Zollinger-Ellison syndrome. (8323754)
1993
20
Flow cytometry and Zollinger-Ellison syndrome: relationship to clinical course. (8359651)
1993
21
Current issues in the management of Zollinger-Ellison syndrome. (1347431)
1992
22
Changing patterns in presentation and management of the Zollinger-Ellison syndrome in Northern Ireland, 1970-1988. (1675471)
1991
23
Effect of propranolol on secretin-induced gastrin release and secretin-induced tachycardia in patients with the Zollinger-Ellison syndrome. (2103752)
1990
24
Misdiagnosis of the Zollinger-Ellison syndrome due to hyperlipidemia. (2210261)
1990
25
Effects of omeprazole on acid secretion and acid-related symptoms in patients with Zollinger-Ellison syndrome. (2574912)
1989
26
Gastrinoma (Zollinger-Ellison Syndrome) in the Dog and cat. (17423047)
1988
27
Omeprazole in the treatment of Zollinger-Ellison syndrome: a 4-year international study. (2979229)
1988
28
The progressive Zollinger-Ellison syndrome in multiple endocrine neoplasia. (2902696)
1988
29
Zollinger-Ellison syndrome: a surgical perspective. (3181695)
1988
30
Prospective study of the ability of computed axial tomography to localize gastrinomas in patients with Zollinger-Ellison syndrome. (3556996)
1987
31
Development of gastric argyrophil carcinoid tumors in a case of Zollinger-Ellison syndrome with primary hyperparathyroidism during long-term antisecretory treatment. (2436742)
1987
32
Lack of effect of parathyroidectomy or calcium channel blockade on serum gastrin concentration and gastric acid secretion in a patient with hyperparathyroidism and Zollinger-Ellison syndrome. (3798319)
1987
33
Effect of parathyroidectomy in patients with hyperparathyroidism, Zollinger-Ellison syndrome, and multiple endocrine neoplasia type I: a prospective study. (2891201)
1987
34
Gastrinoma: the Zollinger-Ellison syndrome. (2888193)
1987
35
Famotidine. Pharmacodynamic and pharmacokinetic properties and a preliminary review of its therapeutic use in peptic ulcer disease and Zollinger-Ellison syndrome. (2875864)
1986
36
Present experiences with omeprazole in the Zollinger-Ellison syndrome. (3460167)
1986
37
Synchronous perforation of the jejunum as presenting feature of Zollinger-Ellison syndrome. (4013608)
1985
38
The Zollinger-Ellison syndrome--diagnosis and management. (2864745)
1985
39
Removal of gastrinomas for control of Zollinger-Ellison syndrome. (6148919)
1984
40
Zollinger Ellison syndrome: a case report. (6654825)
1983
41
Zollinger-Ellison syndrome with esophagitis and Barrett mucosa. (6624733)
1983
42
Cimetidine-resistant Zollinger-Ellison syndrome: successful management with ranitidine. (6305470)
1983
43
Reduction of gastric hypersecretion in Zollinger-Ellison syndrome with omeprazole. (6129395)
1983
44
Resection of gastrinoma in the Zollinger-Ellison syndrome. (7060915)
1982
45
Testing antisecretory drugs in Zollinger-Ellison syndrome: problems and limits in pharmacological studies. (6121691)
1982
46
Zollinger-Ellison syndrome. Streptozotocin therapy for metastatic gastrinomas. (6453914)
1981
47
Pseudo-Zollinger-Ellison syndrome: hypergastrinemia, hyperchlorhydria without tumor. (7283508)
1981
48
Adenocarcinoma arising in Barrett's esophagus with Zollinger-Ellison syndrome. (7395808)
1980
49
Zollinger-Ellison syndrome unresponsive to cimetidine. (474557)
1979
50
The Zollinger-Ellison syndrome with steatorrhea. I. Anticholinergic treatment followed by total gastrectomy and colonic interposition. (5727784)
1968

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

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Pathways for genes affiliated with Zollinger-Ellison Syndrome

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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:20000089.8EGF, CDH1
2G-protein coupled receptor signaling pathwayGO:0071868.8GAST, SST, IL8, MLN
3cell-cell signalingGO:0072678.7MLN, GHRH, SST, POMC
4signal transductionGO:0071658.1IL8, GAST, EGF, GRP, POMC, NTS

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