MCID: ZLL002
MIFTS: 65

Zollinger-Ellison Syndrome malady

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

Summaries for Zollinger-Ellison Syndrome

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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 based summary: Zollinger-Ellison Syndrome, also known as gastrinoma, is related to gastrinoma and multiple endocrine neoplasia. An important gene associated with Zollinger-Ellison Syndrome is SCT (secretin), 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:10 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:65 Zollinger?Ellison Syndrome (ZES) is caused by a non?beta islet cell (islet of Langerhans),... more...

Aliases & Classifications for Zollinger-Ellison Syndrome

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Sources:
10Disease Ontology, 43NIH Rare Diseases, 45Novoseek, 49Orphanet, 62UMLS, 35MeSH, 36MESH via Orphanet, 28ICD10 via Orphanet, 63UMLS via Orphanet
See all sources

Zollinger-Ellison Syndrome, Aliases & Descriptions:

Name: Zollinger-Ellison Syndrome 10 43 45 49 62
Gastrinoma 43 49 62
Pancreatic Ulcerogenic Tumor Syndrome 43 62
 
Z E Syndrome 43
Zes 43


Classifications:



Characteristics (Orphanet epidemiological data):

49
zollinger-ellison syndrome:
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe),1-9/100000 (Europe); Age of onset: Adult; Age of death: normal life expectancy


External Ids:

Disease Ontology10 DOID:0050782
MeSH35 D015043
Orphanet49 913
MESH via Orphanet36 D015043, D015408
ICD10 via Orphanet28 E16.4
UMLS via Orphanet63 C0017150, C0043515

Related Diseases for Zollinger-Ellison Syndrome

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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 181)
idRelated DiseaseScoreTop Affiliating Genes
1gastrinoma31.8GRP, MEN1, SST, CHGA, SCT, GAST
2multiple endocrine neoplasia31.6GAST, CHGA, MEN1
3pancreatitis31.4SCT, IL6, SST
4esophagitis31.2HRH2, GAST, NTS
5duodenal ulcer31.1HRH2, GAST, GRP, SCT
6gastritis31.1IL8, GAST, SCT
7hyperparathyroidism31.1SCT, GAST, MEN1, CHGA
8islet cell tumor31.0SST, GAST
9steatorrhea30.9SCT, SST
10cushing's syndrome30.9POMC, SST
11primary hyperparathyroidism30.8GAST, CHGA, MEN1, SCT
12diarrhea30.8MLN, CHGA, SST, GAST
13pernicious anemia30.8GAST
14peptic ulcer disease30.7SCT, IL8, DNAH8, GAST, SST, HRH2
15acromegaly30.7GHRH, SST, POMC, MEN1
16carcinoid syndrome30.7SLC18A2, GAST, GRP, GHRH, NTS, SCT
17insulinoma30.6GHRH, GRP, GAST, SLC18A2, SCT, CHGA
18neuroendocrine tumor30.5SST, CHGA, POMC, GHRH
19apudoma30.5SST
20adenocarcinoma30.5NTS, SCT, CHGA, SST, MEN1, DNAH8
21multiple endocrine neoplasia 130.5SST, MEN1, GAST, CHGA
22lung cancer30.4CHGA, GRP, EGF
23hypokalemia30.4DNAH8, POMC
24hyperthyroidism30.4NTS, SST, GHRH, DNAH8
25colorectal cancer30.4NTS, EGF, GRP, GAST
26pneumonia30.4GRP, IL6, HRH2, IL8
27gastroesophageal reflux30.4NTS, MLN, SCT, DNAH8, GAST, HRH2
28obesity30.4GRP, GHRH, IL6, MLN, POMC, NTS
29glucagonoma30.3GHRH, CHGA, SCT, GAST, MEN1, SST
30pheochromocytoma30.3SCT, SST, MEN1, NTS, SLC18A2, EGF
31atrophic gastritis30.2GAST, MEN1, SST, CHGA, SCT
32gastric outlet obstruction30.0GAST, MLN
33somatostatinoma30.0GAST, GHRH, SST, CHGA
34prolactinoma30.0MEN1, SST, CHGA, POMC
35duodenitis10.9
36hepatitis10.7
37cystadenocarcinoma10.7
38cystadenoma10.6
39duodenal gastrinoma10.5
40mucinous cystadenocarcinoma10.5
41marden-walker syndrome10.4
42crohn's disease10.4
43gastroduodenitis10.4
44ovarian mucinous cystadenocarcinoma10.4
45ovarian cystadenocarcinoma10.4
46gastric gastrinoma10.3
47nasopharyngeal carcinoma10.3
48neurofibromatosis, type 110.3
49alcohol abuse10.3
50myelofibrosis10.3

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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Drug clinical trials:

Search ClinicalTrials for Zollinger-Ellison Syndrome

Search NIH Clinical Center for Zollinger-Ellison Syndrome

Inferred drug relations via UMLS62/NDF-RT41:

Genetic Tests for Zollinger-Ellison Syndrome

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Anatomical Context for Zollinger-Ellison Syndrome

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MalaCards organs/tissues related to Zollinger-Ellison Syndrome:

33
Pancreas, Liver, Lymph node, Ovary, Heart, Small intestine, Kidney, Testes, Colon, Pancreatic islet, Lung

Animal Models for Zollinger-Ellison Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Zollinger-Ellison Syndrome:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053868.3SCT, POMC, NTS, IL6, HRH2, SLC18A2
2MP:00053818.1SLC18A2, HRH2, GAST, IL6, MEN1, EGF
3MP:00053797.9POMC, CHGA, MEN1, IL6, GHRH, GAST
4MP:00036317.9HRH2, SCT, SLC18A2, GHRH, IL6, NTS
5MP:00053787.6POMC, SLC18A2, CHGA, GHRH, IL6, NTS
6MP:00053877.3POMC, SST, MEN1, IL8, GHRH, EGF
7MP:00053767.2SCT, SLC18A2, GAST, HRH2, GHRH, IL6

Publications for Zollinger-Ellison Syndrome

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Articles related to Zollinger-Ellison Syndrome:

(show top 50)    (show all 513)
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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Cosmic variations for Zollinger-Ellison Syndrome:

8
id Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Conf
122579MEN1small intestine,duodenum,carcinoid-endocrine tumour,gastrinoma1

Expression for genes affiliated with Zollinger-Ellison Syndrome

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

Pathways for genes affiliated with Zollinger-Ellison Syndrome

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Compounds for genes affiliated with Zollinger-Ellison Syndrome

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

(show top 50)    (show all 210)
idCompoundScoreTop Affiliating Genes
1pantoprazole45 51 26 1312.0SST, DNAH8, GAST, HRH2
2pepsinogen459.0HRH2, GAST, SCT, CHGA, GRP
3pirenzepine45 30 1310.9SST, GAST, HRH2, GRP, GHRH
4loxiglumide458.9SCT, GAST, GRP, NTS, SST
5amine458.9HRH2, SLC18A2, SCT, CHGA, NTS, GAST
6c-peptide458.9GAST, CHGA, SST, NTS, SCT
7peptidylglycine458.8GRP, CHGA, POMC, GAST
8lafutidine458.8HRH2, GAST, SST, SCT, MLN
9amphetamine45 51 1310.8POMC, GRP, NTS, SST, SLC18A2
10clonidine45 51 30 1311.7SCT, POMC, SST, NTS, DNAH8, GHRH
11ranitidine45 30 26 1311.7MLN, CHGA, GAST, SCT, HRH2
12propranolol45 30 51 26 1312.7SCT, CHGA, SST, GHRH, GRP, GAST
13opiate458.6POMC, NTS, DNAH8, GRP, GHRH, HRH2
14bethanechol45 30 1310.4SST, GAST, GRP, MLN
15isoproterenol45 139.4GAST, NTS, CHGA, HRH2, SCT, GRP
16domperidone45 30 1310.3SST, MLN, HRH2, GAST
17carbachol45 30 1310.3SST, GRP, CHGA, NTS, GAST, EGF
18forskolin45 51 1310.2NTS, SCT, GHRH, HRH2, EGF, POMC
19tetrodotoxin45 61 3010.2MLN, HRH2, NTS, SCT, GRP
20omeprazole45 51 30 26 1312.0MLN, SCT, SST, DNAH8, GAST, HRH2
21gaba458.0POMC, CHGA, SCT, SST, NTS, GRP
22gnrh457.9GRP, NTS, EGF, GAST, GHRH, SST
23indomethacin45 30 61 1310.9IL6, GRP, EGF, DNAH8, SCT, POMC
24oxyntomodulin45 618.9MLN, SCT, CHGA, SST, NTS, GRP
25hydrocortisone45 3 61 1310.9POMC, SST, IL8, IL6, GAST, EGF
26nifedipine45 30 51 1310.8MLN, NTS, SST, GHRH, GRP, GAST
27thymidine45 268.8MEN1, IL6, GRP, SCT, GAST, NTS
28cycloheximide457.7IL8, IL6, GRP, GHRH, POMC, EGF
29atropine45 30 139.7GRP, GAST, HRH2, GHRH, NTS, MLN
30thyroxine45 268.6GAST, DNAH8, GHRH, SCT, POMC, SST
31hydrogen45 268.6SCT, MLN, DNAH8, GAST, CHGA, SLC18A2
32glutamate457.4GRP, SST, CHGA, SCT, GHRH, DNAH8
33retinoic acid45 268.3HRH2, CHGA, SCT, IL6, DNAH8, SST
34vegf457.3GRP, IL6, IL8, MEN1, EGF, POMC
35pge2457.2HRH2, EGF, GRP, IL6, IL8, NTS
36arginine457.2SST, SCT, MLN, POMC, CHGA, DNAH8
37dexamethasone45 51 30 1310.1CHGA, IL8, EGF, IL6, GHRH, SST
38glucose457.0SLC18A2, MLN, SCT, POMC, SST, NTS
39octreotide45 61 30 1310.0EGF, NTS, SST, GHRH, CHGA, POMC
40dopamine45 30 26 1310.0SLC18A2, GAST, MLN, SCT, POMC, CHGA
41acth456.9POMC, MLN, SCT, CHGA, SST, NTS
42epinephrine45 26 138.8SCT, POMC, CHGA, SST, NTS, IL6
43norepinephrine45 26 138.8GHRH, GRP, GAST, SLC18A2, DNAH8, IL6
44testosterone45 61 26 139.8GAST, GRP, GHRH, DNAH8, IL6, EGF
455-hydroxytryptamine456.7SLC18A2, HRH2, GAST, GRP, DNAH8, NTS
46acetylcholine45 51 30 26 1310.5SLC18A2, HRH2, GAST, GRP, DNAH8, NTS
47histamine45 30 268.4NTS, IL8, IL6, GRP, GAST, SST
48adenylate456.2MLN, SCT, POMC, CHGA, SST, NTS
49calcium45 51 26 139.1SCT, MLN, SLC18A2, CHGA, MEN1, NTS
50serine456.0MLN, SCT, NTS, SLC18A2, HRH2, EGF

GO Terms for genes affiliated with Zollinger-Ellison Syndrome

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

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:00056157.8SST, IL8, IL6, GHRH, GRP, EGF
2extracellular regionGO:00055765.3EGF, MLN, SCT, POMC, CHGA, SST

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

idNameGO IDScoreTop Affiliating Genes
1response to amino acidGO:00432009.7IL6, SST
2cell-cell signalingGO:00072678.8MLN, POMC, SST, GHRH
3negative regulation of cell proliferationGO:00082858.8SST, MEN1, IL8, IL6
4G-protein coupled receptor signaling pathwayGO:00071868.3MLN, SST, IL8, GAST
5signal transductionGO:00071658.0EGF, POMC, NTS, IL8, GRP, GAST

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

idNameGO IDScoreTop Affiliating Genes
1neuropeptide hormone activityGO:00051849.6GRP, NTS
2hormone activityGO:00051798.0GAST, 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
15ExPASy
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet