ZES
MCID: ZLL002
MIFTS: 58

Zollinger-Ellison Syndrome (ZES) malady

Gastrointestinal, Endocrine, Cancer categories

Summaries for Zollinger-Ellison Syndrome

Sources:
8Disease Ontology, 43NIH Rare Diseases, 64Wikipedia, 33MalaCards
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NIH Rare Diseases:43 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 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 lymph node, heart and small intestine, 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:64 Zollinger–Ellison Syndrome (ZES) is caused by a non–beta islet cell, gastrin-secreting tumor of the... more...

Aliases & Classifications for Zollinger-Ellison Syndrome

Sources:
8Disease Ontology, 43NIH Rare Diseases, 45Novoseek, 49Orphanet, 61UMLS, 35MeSH, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet, 36MESH via Orphanet
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

49
zollinger-ellison syndrome:
Inheritance: Sporadic; Prevalence: 1-9/100000; Age of onset: Adulthood; Age of death: Normal


Aliases & Descriptions:

zollinger-ellison syndrome 8 43 45 49 61
gastrinoma 43 49 61
pancreatic ulcerogenic tumor syndrome 43
z e syndrome 43
zes 43


External Ids:

Disease Ontology8 DOID:0050782
MeSH35 D015043
ICD10 via Orphanet26 E16.4
SNOMED-CT via Orphanet58 16189002, 302824004, 53132006
UMLS via Orphanet62 C0017150, C0043515
MESH via Orphanet36 D015043, D015408

Related Diseases for Zollinger-Ellison Syndrome

Sources:
17GeneCards, 18GeneDecks
See all sources

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

(show top 50)    (show all 215)
idRelated DiseaseScoreTop Affiliating Genes
1gastrinoma31.7SCT, GAST, MEN1, CHGA, GRP, SST
2multiple endocrine neoplasia31.5GAST, MEN1, CHGA
3carcinoid syndrome31.3NTS, SST, POMC, SLC18A2, GRP, CHGA
4duodenal ulcer31.0HRH2, GRP, GAST, SCT
5cushing's syndrome30.9POMC, SST
6steatorrhea30.8SST, SCT
7gastric ulcer30.7GAST, EGF, HRH2
8primary hyperparathyroidism30.7SCT, GAST, MEN1, CHGA
9pernicious anemia30.6GAST
10wermer syndrome30.6GAST, MEN1, CHGA, SST
11acromegaly30.6MEN1, GHRH, SST, POMC
12insulinoma30.5SCT, GAST, MEN1, GHRH, CHGA, GRP
13adenoma30.5DNAH8, CDH1, POMC, SST, SCT, GAST
14adenocarcinoma30.5SCT, GAST, MEN1, EGF, CHGA, GRP
15apudoma30.3SST
16cholelithiasis30.3SST
17tuberculosis30.3POMC
18atrophic gastritis30.3SCT, GAST, MEN1, CHGA, SST
19hypokalemia30.3POMC, DNAH8
20acute pancreatitis30.3SCT, SST, IL8, IL6, HRH2
21colorectal cancer30.3GAST, MEN1, EGF, GRP, NTS, CDH1
22gastroesophageal reflux disease30.3HRH2, SCT, GAST, MLN, NTS, DNAH8
23pheochromocytoma30.3SCT, MEN1, EGF, MLN, CHGA, NTS
24glucagonoma30.2SCT, GAST, MEN1, GHRH, CHGA, SST
25phaeochromocytoma29.9CHGA
26gastric outlet obstruction29.9GAST, MLN
27somatostatinoma29.9GAST, GHRH, CHGA, SST
28pancreatic islet cell tumors29.9GAST, MEN1, CHGA, SST
29pancreatitis, chronic29.9SST, NTS, GRP, SCT
30prolactinoma29.8POMC, SST, CHGA, MEN1
31n syndrome11.1
32gastric gastrinoma10.7
33cystadenoma10.6
34duodenal gastrinoma10.6
35micro syndrome10.6
36pancreatic gastrinoma10.5
37somatostatin analog10.5
38mucinous ovarian cystadenoma10.5
39mucinous cystadenocarcinoma10.5
40bod syndrome10.5
41h. pylori infection10.5
42crohn's disease10.4
43ovarian mucinous cystadenocarcinoma10.4
44barrett's esophagus10.4
45char syndrome10.4
46image syndrome10.4
47gastric endocrine tumor10.4
48neurofibromatosis10.2
49alcohol abuse10.2
50peptic esophagitis10.2

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



Diseases related to zollinger-ellison syndrome

Clinical Features for Zollinger-Ellison Syndrome

Drugs & Therapeutics for Zollinger-Ellison Syndrome

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
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Approved drugs:

Search CenterWatch for Zollinger-Ellison Syndrome

Drug clinical trials:

Search ClinicalTrials for Zollinger-Ellison Syndrome

Search NIH Clinical Center for Zollinger-Ellison Syndrome

Search CenterWatch for Zollinger-Ellison Syndrome

Inferred drug relations via UMLS61/NDF-RT41:

Genetic Tests for Zollinger-Ellison Syndrome

Anatomical Context for Zollinger-Ellison Syndrome

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Zollinger-Ellison Syndrome:

33
Lymph node, Heart, Small intestine, Colon, Kidney, Liver, Lung, Pancreas, Salivary gland, Ovary, B cells, Pancreatic islet

Animal Models for Zollinger-Ellison Syndrome or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Zollinger-Ellison Syndrome:

37
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

Sources:
51PubMed
See all sources

Articles related to Zollinger-Ellison Syndrome:

(show top 50)    (show all 517)
idTitleAuthorsYear
1
Diagnostic efficacy of the secretin stimulation test for the Zollinger-Ellison syndrome: an intra-individual comparison using different dosages in patients and controls. (20299818)
2010
2
Reoperative surgery for the Zollinger-Ellison syndrome. (20919530)
2010
3
Zollinger-Ellison syndrome: a case report.]. (20423792)
2010
4
Primary hepatic gastrinoma: an unusual case of zollinger-ellison syndrome. (20567542)
2010
5
Zollinger-Ellison syndrome: a personal perspective. (18646472)
2008
6
Intense F-18 FDG uptake in the stomach wall in follicular gastritis in Zollinger-Ellison syndrome. (17242575)
2007
7
An open-label study of rabeprazole in patients with Zollinger-Ellison syndrome or idiopathic gastric acid hypersecretion. (17081164)
2006
8
Three-year oral pantoprazole administration is effective for patients with Zollinger-Ellison syndrome and other hypersecretory conditions. (16423003)
2006
9
Zollinger-Ellison syndrome. Diagnosis and therapy. (16175161)
2005
10
Maintenance oral pantoprazole therapy is effective for patients with Zollinger-Ellison syndrome and idiopathic hypersecretion. (12591045)
2003
11
Comments on: case presentation of gastrinoma combined with gastric carcinoid with the longest survival record -Zollinger - Ellison syndrome: pathophysiology, diagnosis and therapy. (14598848)
2003
12
Comparison of surgical results in patients with advanced and limited disease with multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome. (11573043)
2001
13
Gastric secretion in Zollinger-Ellison syndrome. Correlation with clinical expression, tumor extent and role in diagnosis--a prospective NIH study of 235 patients and a review of 984 cases in the literature. (11388095)
2001
14
Intravenous pantoprazole rapidly controls gastric acid hypersecretion in patients with Zollinger-Ellison syndrome. (10734021)
2000
15
Fundic gland polyps: do they arise as a by-product of hypergastrinemia in patients with Zollinger-Ellison syndrome? (10923933)
2000
16
Surgery for the Zollinger-Ellison syndrome. (10460823)
1999
17
Zollinger-Ellison syndrome. Improved treatment options for this complex disorder. (9676569)
1998
18
Iron absorption in patients with Zollinger-Ellison syndrome treated with long-term gastric acid antisecretory therapy. (9692706)
1998
19
Current concepts in the surgical management of multiple endocrine neoplasia type 1 pancreatic-duodenal disease. Results in the treatment of 40 patients with Zollinger-Ellison syndrome, hypoglycaemia or both. (9681848)
1998
20
Gastric carcinoid tumors associated with chronic hypergastrinemia in a patient with Zollinger-Ellison syndrome. (8888225)
1996
21
Long-term treatment with lansoprazole for patients with Zollinger-Ellison syndrome. (8853754)
1996
22
Helicobacter pylori infection. A reversible cause of hypergastrinemia and hyperchlorhydria which may mimic Zollinger-Ellison syndrome. (7821103)
1995
23
Is there a relationship between hypergastrinemia and colorectal cancer risk? Rectal cell proliferation in Zollinger-Ellison syndrome. (7639262)
1995
24
Endocrine Cell Replacement of Oxyntic Glands in Zollinger-Ellison Syndrome: A Role for Female Sex Hormones? (12114816)
1995
25
Determinants of metastatic rate and survival in patients with Zollinger-Ellison syndrome: a prospective long-term study. (7768367)
1995
26
Duodenal gastrinomas, duodenotomy, and duodenal exploration in the surgical management of Zollinger-Ellison syndrome. (8103249)
1993
27
Use of omeprazole in Zollinger-Ellison syndrome: a prospective nine-year study of efficacy and safety. (8161665)
1993
28
Prospective study of the long-term efficacy and safety of lansoprazole in patients with the Zollinger-Ellison syndrome. (8490079)
1993
29
Stereologic investigations of human gastric mucosa. II. Oxyntic mucosa from patients with Zollinger-Ellison syndrome. (1439541)
1992
30
Curative resection in Zollinger-Ellison syndrome. Results of a 10-year prospective study. (1531004)
1992
31
An early case of the Zollinger-Ellison syndrome produced by a carcinoid tumor of the duodenum. (1517178)
1992
32
Zollinger-Ellison syndrome. Relation to Helicobacter pylori-associated chronic gastritis and gastric acid secretion. (1984995)
1991
33
Zollinger-Ellison syndrome. (2072795)
1991
34
The medical management of Zollinger-Ellison syndrome. (1675470)
1991
35
Gastrinomas in the duodenums of patients with multiple endocrine neoplasia type 1 and the Zollinger-Ellison syndrome. (1968616)
1990
36
Zollinger-Ellison syndrome, duodenal carcinoid (gastrinoma), and hyperthyroidism. (2508315)
1989
37
Zollinger-Ellison syndrome in a cat. (3176293)
1988
38
Medical management of patients with Zollinger-Ellison syndrome who have had previous gastric surgery: a prospective study. (3335308)
1988
39
Thymic carcinoid in a case of Zollinger Ellison syndrome. A case report. (3202759)
1988
40
Omeprazole in the Zollinger-Ellison syndrome. (3715402)
1986
41
The nature of gastric secretions in a patient with Zollinger-Ellison syndrome without peptic ulceration. (3757267)
1986
42
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
43
Cimetidine treatment for Zollinger-Ellison syndrome. A case report. (6565644)
1984
44
Boots secretin and the Zollinger-Ellison syndrome. (6715857)
1984
45
Failure of cimetidine in Zollinger-Ellison syndrome. (6628158)
1983
46
The Zollinger-Ellison syndrome and ectopic gastric mucosa. (6815626)
1982
47
Studies with bombesin in the Zollinger--Ellison syndrome. (7459630)
1981
48
Prediction of progressive disease in Zollinger-Ellison Syndrome-comparison of available preoperative tests. (7265952)
1981
49
Elevated serum prolactin level in the Zollinger-Ellison syndrome. (6260061)
1981
50
Zollinger-Ellison syndrome: a clinical update. (7393784)
1980

Genetic Variations for Zollinger-Ellison Syndrome

Expression for genes affiliated with Zollinger-Ellison Syndrome

Sources:
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

Sources:
52QIAGEN, 54Reactome, 38NCBI BioSystems Database, 30KEGG
See all sources

Compounds for genes affiliated with Zollinger-Ellison Syndrome

Sources:
45Novoseek, 50PharmGKB, 11DrugBank, 29IUPHAR, 24HMDB, 60Tocris Bioscience, 2BitterDB
See all sources

Compounds related to Zollinger-Ellison Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 186)
idCompoundScoreTop Affiliating Genes
1cycloheximide4510.1GHRH
2forskolin45 50 1111.8GHRH, POMC, HRH2, SCT
3ranitidine45 29 11 2412.7SCT, HRH2, MLN
4pge2459.5CDH1, POMC, SST, GRP
5pepsinogen459.2SCT, GAST, CHGA, GRP, HRH2
6loxiglumide459.1NTS, SCT, GAST, GRP, SST
7pirenzepine45 29 1111.1SST, HRH2, GAST, GHRH, GRP
8gnrh459.1GHRH, NTS, GAST, CHGA, GRP
9lafutidine459.1SCT, HRH2, SST, MLN, GAST
10pantoprazole45 50 11 2412.1GAST, SST, DNAH8, HRH2
11peptidylglycine459.1CHGA, POMC, GRP, GAST
12domperidone45 29 1110.9HRH2, SST, MLN, GAST
13bethanechol45 29 1110.8MLN, GRP, SST, GAST
14propranolol45 29 50 11 2412.8GHRH, SCT, GAST, SST, GRP, CHGA
15clonidine45 29 1110.8SCT, SST, CHGA, NTS, GHRH, DNAH8
16dexamethasone45 50 29 1111.8GHRH, NTS, POMC, CDH1, GRP, IL6
17opiate458.7HRH2, DNAH8, POMC, NTS, GRP, GHRH
18isoproterenol45 119.6HRH2, SST, NTS, GRP, CHGA, GAST
19amine458.5NTS, SLC18A2, SCT, GAST, CHGA, HRH2
20amphetamine45 119.4GRP, NTS, SLC18A2, POMC, SST
21atropine45 29 1110.3HRH2, NTS, GRP, GHRH, MLN, GAST
22omeprazole45 29 50 11 2412.3GAST, CHGA, HRH2, DNAH8, SST, MLN
23oxyntomodulin45 609.3SCT, GAST, MLN, SST, NTS, GRP
24dopamine45 29 11 2411.3GHRH, CHGA, NTS, GRP, MLN, SCT
25glutamate458.3SLC18A2, SCT, GAST, GHRH, NTS, DNAH8
26carbachol45 29 1110.2SCT, EGF, GAST, GRP, CHGA, NTS
27nifedipine45 50 29 1111.2SST, GHRH, GRP, CHGA, GAST, NTS
28hydrocortisone45 2 60 1111.2IL6, IL8, POMC, SST, GRP, EGF
29cyclic amp45 249.1NTS, HRH2, EGF, GHRH, CHGA, GRP
30thymidine45 249.0SCT, IL6, GAST, MEN1, EGF, GRP
31thyroxine45 249.0SST, NTS, GHRH, GAST, SCT, POMC
32indomethacin45 60 29 1110.9POMC, EGF, NTS, GRP, IL6, CDH1
33glucose457.8MLN, GHRH, NTS, SLC18A2, POMC, GAST
34gaba457.7SLC18A2, GAST, NTS, GRP, SCT, CHGA
35creatinine457.6GRP, CDH1, DNAH8, HRH2, CHGA, POMC
36norepinephrine45 11 249.5SLC18A2, DNAH8, POMC, NTS, GRP, CHGA
37octreotide45 60 29 1110.4EGF, MLN, GHRH, CHGA, GRP, NTS
38vegf457.4IL6, MEN1, GAST, CHGA, GRP, SST
39acth457.3POMC, MLN, GAST, SCT, GHRH, CHGA
40serine457.3HRH2, SLC18A2, IL8, POMC, NTS, GHRH
41arginine457.3CDH1, SCT, MLN, GHRH, CHGA, GRP
42retinoic acid45 247.8EGF, DNAH8, GRP, IL6, CDH1, CHGA
43testosterone45 60 11 249.8IL6, CDH1, DNAH8, POMC, SCT, GAST
445-hydroxytryptamine456.6SLC18A2, HRH2, DNAH8, POMC, SST, NTS
45epinephrine45 11 248.6POMC, SST, NTS, GRP, CHGA, GAST
46acetylcholine45 50 29 11 2410.4SLC18A2, GAST, MLN, CHGA, GRP, NTS
47adenylate456.3SCT, GAST, EGF, MLN, GHRH, CHGA
48histamine45 29 248.1SLC18A2, GAST, MLN, CHGA, GRP, NTS
49calcium45 50 11 248.7GHRH, SLC18A2, MLN, NTS, DNAH8, CDH1
50tyrosine455.7GRP, MLN, EGF, CDH1, NTS, IL6

GO Terms for genes affiliated with Zollinger-Ellison Syndrome

Sources:
16Gene Ontology
See all sources

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

  • Antibodies
  • Proteins
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Sources for Zollinger-Ellison Syndrome

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet