MCID: ZLL002
MIFTS: 57

Zollinger-Ellison Syndrome malady

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

Aliases & Classifications for Zollinger-Ellison Syndrome

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Sources:
42NIH Rare Diseases, 48Orphanet, 61UMLS, 8Disease Ontology, 44Novoseek, 33MeSH, 34MESH via Orphanet, 26ICD10 via Orphanet, 62UMLS via Orphanet
See all sources

Aliases & Descriptions for Zollinger-Ellison Syndrome:

Name: Zollinger-Ellison Syndrome 8 42 44 48 61
Gastrinoma 42 48 61
Pancreatic Ulcerogenic Tumor Syndrome 42
 
Z E Syndrome 42
Zes 42


Classifications:



Characteristics (Orphanet epidemiological data):

48
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 Ontology8 DOID:0050782
MeSH33 D015043
Orphanet48 913
MESH via Orphanet34 D015043, D015408
ICD10 via Orphanet26 E16.4
UMLS via Orphanet62 C0017150, C0043515

Summaries for Zollinger-Ellison Syndrome

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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 based summary: Zollinger-Ellison Syndrome, also known as gastrinoma, is related to multiple endocrine neoplasia and gastrinoma. 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: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 (islet of Langerhans),... more...

Related Diseases for Zollinger-Ellison Syndrome

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

(show top 50)    (show all 182)
idRelated DiseaseScoreTop Affiliating Genes
1multiple endocrine neoplasia32.0GAST, CHGA, MEN1
2gastrinoma31.8GRP, MEN1, SST, CHGA, SCT, GAST
3esophagitis31.6HRH2, GAST, NTS
4pancreatitis31.6SCT, IL6, SST
5islet cell tumor31.6SST, GAST
6pernicious anemia31.5GAST
7duodenal ulcer31.5HRH2, GAST, GRP, SCT
8steatorrhea31.5SCT, SST
9cushing's syndrome31.4POMC, SST
10hyperparathyroidism31.4SCT, GAST, MEN1, CHGA
11gastritis31.3IL8, GAST, SCT
12apudoma31.2SST
13primary hyperparathyroidism31.1GAST, CHGA, MEN1, SCT
14diarrhea31.0MLN, CHGA, SST, GAST
15acromegaly30.9GHRH, SST, POMC, MEN1
16neuroendocrine tumor30.7SST, CHGA, POMC, GHRH
17multiple endocrine neoplasia 130.7SST, MEN1, GAST, CHGA
18lung cancer30.6CHGA, GRP, EGF
19gastric outlet obstruction30.6GAST, MLN
20hypokalemia30.6DNAH8, POMC
21hyperthyroidism30.5NTS, SST, GHRH, DNAH8
22colorectal cancer30.4NTS, EGF, GRP, GAST
23atrophic gastritis30.3GAST, MEN1, SST, CHGA, SCT
24peptic ulcer disease30.3SCT, IL8, DNAH8, GAST, SST, HRH2
25glucagonoma30.3GHRH, CHGA, SCT, GAST, MEN1, SST
26somatostatinoma30.3GAST, GHRH, SST, CHGA
27pneumonia30.2GRP, IL6, HRH2, IL8
28gastroesophageal reflux30.2NTS, MLN, SCT, DNAH8, GAST, HRH2
29prolactinoma30.1MEN1, SST, CHGA, POMC
30obesity30.0GRP, GHRH, IL6, MLN, POMC, NTS
31insulinoma29.9GHRH, GRP, GAST, SLC18A2, SCT, CHGA
32carcinoid syndrome29.8SLC18A2, GAST, GRP, GHRH, NTS, SCT
33adenocarcinoma29.6NTS, SCT, CHGA, SST, MEN1, DNAH8
34pheochromocytoma29.3SCT, SST, MEN1, NTS, SLC18A2, EGF
35duodenitis10.9
36hepatitis10.7
37cystadenocarcinoma10.7
38cystadenoma10.6
39duodenal gastrinoma10.5
40mucinous cystadenocarcinoma10.5
41angiodysplasia10.5SST
42gigantism10.5SST
43pituitary carcinoma10.5POMC
44pituitary hypoplasia10.5GHRH
45acute adrenal insufficiency10.5POMC
46pituitary-dependent cushing's disease10.5POMC
47hypoaldosteronism10.5POMC
48acinar cell carcinoma10.4GAST, CHGA
49marden-walker syndrome10.4
50crohn's disease10.4

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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FDA approved drugs:

id Drug Name Active Ingredient(s)13 Pharmaceutical Company Approval Date
1
SecreFlo13 SECRETIN SECRETIN SYNTHETIC PORCINE Repligen Approved April 2002
FDA Label: SecreFlo
Malady that Drug Treats: Pancreatic dysfunction and gastrinoma
Indications and Usage:13 SecreFlo is indicated for use in secretin stimulation testing for:; (1) Stimulation of pancreatic secretions, including bicarbonate, to aid in the diagnosis of; pancreatic exocrine dysfunction,; (2) Stimulation of gastrin secretion to aid in the diagnosis of gastrinoma.
DrugBank Targets:11 1. Secretin receptor
Mechanism of Action:13 
Target: secreted pancreatic juices
Action: increase volume and bicarbonate content
FDA: The primary action of SecreFlo is to increase the volume and bicarbonate content of; secreted pancreatic juices. The standard unit of activity used for SecreFlo is the clinical; unit defined by Jorpes & Mutt in 1966. (1) In the validated cat bioassay, which was used; to define and quantitate the biological activity of secretin and as the release test for the; biologically derived porcine secretin product, SecreFlo demonstrates a potency of; approximately 5000 clinical units (CU) per milligram of peptide as opposed to 3000 CU; per mg for biologically derived porcine secretin. As a pure peptide drug product,; SecreFlo"! dosing is expressed by weight in micrograms. The relationship of; micrograms of secretin to biological activity is 0.2 mcg = 1 CU.

Drug clinical trials:

Search ClinicalTrials for Zollinger-Ellison Syndrome

Search NIH Clinical Center for Zollinger-Ellison Syndrome

Inferred drug relations via UMLS61/NDF-RT40:

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:

31
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:

35
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:

6
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 Suite gene sharing:

(show top 50)    (show all 210)
idCompoundScoreTop Affiliating Genes
1pantoprazole44 50 24 1112.0SST, DNAH8, GAST, HRH2
2pepsinogen449.0HRH2, GAST, SCT, CHGA, GRP
3pirenzepine44 28 1110.9SST, GAST, HRH2, GRP, GHRH
4loxiglumide448.9SCT, GAST, GRP, NTS, SST
5amine448.9HRH2, SLC18A2, SCT, CHGA, NTS, GAST
6c-peptide448.9GAST, CHGA, SST, NTS, SCT
7peptidylglycine448.8GRP, CHGA, POMC, GAST
8lafutidine448.8HRH2, GAST, SST, SCT, MLN
9amphetamine44 50 1110.8POMC, GRP, NTS, SST, SLC18A2
10clonidine44 50 28 1111.7SCT, POMC, SST, NTS, DNAH8, GHRH
11ranitidine44 28 24 1111.7MLN, CHGA, GAST, SCT, HRH2
12propranolol44 28 50 24 1112.7SCT, CHGA, SST, GHRH, GRP, GAST
13opiate448.6POMC, NTS, DNAH8, GRP, GHRH, HRH2
14bethanechol44 28 1110.4SST, GAST, GRP, MLN
15isoproterenol44 119.4GAST, NTS, CHGA, HRH2, SCT, GRP
16domperidone44 28 1110.3SST, MLN, HRH2, GAST
17carbachol44 28 1110.3SST, GRP, CHGA, NTS, GAST, EGF
18forskolin44 50 1110.2NTS, SCT, GHRH, HRH2, EGF, POMC
19tetrodotoxin44 60 2810.2MLN, HRH2, NTS, SCT, GRP
20omeprazole44 50 28 24 1112.0MLN, SCT, SST, DNAH8, GAST, HRH2
21gaba448.0POMC, CHGA, SCT, SST, NTS, GRP
22gnrh447.9GRP, NTS, EGF, GAST, GHRH, SST
23indomethacin44 28 60 1110.9IL6, GRP, EGF, DNAH8, SCT, POMC
24oxyntomodulin44 608.9MLN, SCT, CHGA, SST, NTS, GRP
25hydrocortisone44 1 60 1110.9POMC, SST, IL8, IL6, GAST, EGF
26nifedipine44 28 50 1110.8MLN, NTS, SST, GHRH, GRP, GAST
27thymidine44 248.8MEN1, IL6, GRP, SCT, GAST, NTS
28cycloheximide447.7IL8, IL6, GRP, GHRH, POMC, EGF
29atropine44 28 119.7GRP, GAST, HRH2, GHRH, NTS, MLN
30thyroxine44 248.6GAST, DNAH8, GHRH, SCT, POMC, SST
31hydrogen44 248.6SCT, MLN, DNAH8, GAST, CHGA, SLC18A2
32glutamate447.4GRP, SST, CHGA, SCT, GHRH, DNAH8
33retinoic acid44 248.3HRH2, CHGA, SCT, IL6, DNAH8, SST
34vegf447.3GRP, IL6, IL8, MEN1, EGF, POMC
35pge2447.2HRH2, EGF, GRP, IL6, IL8, NTS
36arginine447.2SST, SCT, MLN, POMC, CHGA, DNAH8
37dexamethasone44 50 28 1110.1CHGA, IL8, EGF, IL6, GHRH, SST
38glucose447.0SLC18A2, MLN, SCT, POMC, SST, NTS
39octreotide44 60 28 1110.0EGF, NTS, SST, GHRH, CHGA, POMC
40dopamine44 28 24 1110.0SLC18A2, GAST, MLN, SCT, POMC, CHGA
41acth446.9POMC, MLN, SCT, CHGA, SST, NTS
42epinephrine44 24 118.8SCT, POMC, CHGA, SST, NTS, IL6
43norepinephrine44 24 118.8GHRH, GRP, GAST, SLC18A2, DNAH8, IL6
44testosterone44 60 24 119.8GAST, GRP, GHRH, DNAH8, IL6, EGF
455-hydroxytryptamine446.7SLC18A2, HRH2, GAST, GRP, DNAH8, NTS
46acetylcholine44 50 28 24 1110.5SLC18A2, HRH2, GAST, GRP, DNAH8, NTS
47histamine44 28 248.4NTS, IL8, IL6, GRP, GAST, SST
48adenylate446.2MLN, SCT, POMC, CHGA, SST, NTS
49calcium44 50 24 119.1SCT, MLN, SLC18A2, CHGA, MEN1, NTS
50serine446.0MLN, 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 Suite gene sharing:

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 Suite gene sharing:

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

Molecular functions related to Zollinger-Ellison Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1neuropeptide hormone activityGO:00051849.6GRP, NTS
2hormone activityGO:00051798.0GAST, SST, POMC, SCT, MLN

Sources for Zollinger-Ellison Syndrome

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2CDC
12ExPASy
13FDA
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
57SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet