MCID: PRT013
MIFTS: 49

Portal Hypertension malady

Rare diseases, Gastrointestinal diseases categories

Aliases & Classifications for Portal Hypertension

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Portal Hypertension, Aliases & Descriptions:

Name: Portal Hypertension 9 41 11
Hypertension, Portal 41 60
 
Hypertension Portal 43


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Gastrointestinal diseases


External Ids:

Disease Ontology9 DOID:10762
ICD9CM27 572.3
MeSH33 D006975
SNOMED-CT55 155821005, 34742003
NCIt38 C3119
ICD1025 K76.6

Summaries for Portal Hypertension

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NIH Rare Diseases:41 Portal hypertension is abnormally high blood pressure in branches of the portal vein, the large vein that brings blood from the intestine to the liver. portal hypertension itself does not cause symptoms, but complications from the condition can lead to an enlarged abdomen, abdominal discomfort, confusion, drowsiness and internal bleeding. it may be caused by a variety of conditions, but cirrhosis is the most common cause in western countries. treatment is generally directed toward the cause of the condition, although emergency treatment is sometimes needed for serious complications. last updated: 2/1/2012

MalaCards based summary: Portal Hypertension, also known as hypertension, portal, is related to liver cirrhosis and liver disease. An important gene associated with Portal Hypertension is UTS2 (urotensin 2), and among its related pathways are Agents Acting on the Renin-Angiotensin System Pathway, Pharmacodynamics and FOXA2 and FOXA3 transcription factor networks. The compounds n(g)-nitro-l-arginine methyl ester and lacidipine have been mentioned in the context of this disorder. Affiliated tissues include liver, spleen and endothelial, and related mouse phenotypes are immune system and normal.

Wikipedia:63 In medicine, portal hypertension is hypertension (high blood pressure) in the portal vein system, which... more...

Related Diseases for Portal Hypertension

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Diseases in the Portal Hypertension family:

Portal Hypertension Due to Infrahepatic Block

Diseases related to Portal Hypertension via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 238)
idRelated DiseaseScoreTop Affiliating Genes
1liver cirrhosis31.4UTS2, EDN1, F2
2liver disease31.2SST, F2
3hepatorenal syndrome30.3F2, EDN1
4vascular disease30.1AGTR1, CMA1, EDN1
5ischemia30.0G6PC, EDN1, AVPR1A
6pulmonary hypertension30.0EDN1, UTS2, G6PC
7atherosclerosis29.9AGTR1, UTS2, CMA1, EDN1
8hepatitis11.0
9hypersplenism10.8
10nodular regenerative hyperplasia10.7
11portal vein thrombosis10.7
12esophagitis10.7
13pancreatitis10.7
14aneurysm10.6
15splenomegaly10.6
16hepatopulmonary syndrome10.6
17hepatocellular carcinoma10.6
18splenic artery aneurysm10.6
19endotheliitis10.6
20gastric antral vascular ectasia10.6
21banti's syndrome10.5
22arteriovenous fistula10.5
23hepatitis c10.5
24tuberculosis10.5
25biliary atresia10.5
26congenital hepatic fibrosis10.5
27hepatoportal sclerosis10.5
28systemic mastocytosis10.4
29cystinosis10.4
30protein c deficiency10.4
31hepatitis c virus10.4
32cystic fibrosis10.4
33hepatitis b10.4
34hereditary hemorrhagic telangiectasia10.4
35mastocytosis10.4
36coach syndrome10.3
37primary biliary cirrhosis10.3
38choledochal cyst10.3
39felty's syndrome10.3
40abdominal tuberculosis10.3
41polycystic liver disease10.3
42polycythemia vera10.3
43alcoholic hepatitis10.3
44myelofibrosis10.3
45thrombocytopenia10.3
46dyskeratosis congenita10.3
47cystadenoma10.3
48fatty liver disease10.3
49peliosis hepatis10.3
50caroli disease10.3

Graphical network of the top 20 diseases related to Portal Hypertension:



Diseases related to portal hypertension

Symptoms for Portal Hypertension

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Drugs & Therapeutics for Portal Hypertension

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Drug clinical trials:

Search ClinicalTrials for Portal Hypertension

Search NIH Clinical Center for Portal Hypertension

Genetic Tests for Portal Hypertension

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Anatomical Context for Portal Hypertension

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MalaCards organs/tissues related to Portal Hypertension:

31
Liver, Spleen, Endothelial, Pancreas, Brain, Colon, Bone, Kidney, Thyroid, Breast, Testes, Myeloid, Bone marrow, Lymph node, Smooth muscle, Small intestine, Placenta

Animal Models for Portal Hypertension or affiliated genes

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MGI Mouse Phenotypes related to Portal Hypertension:

35
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053878.0AVPR1A, F2, CMA1, SST, AGTR1
2MP:00028737.9AVPR1A, F2, EDN1, G6PC, SST
3MP:00036317.9SST, G6PC, EDN1, F2, AVPR1A
4MP:00053767.3AGTR1, AVPR1A, F2, EDN1, G6PC, SST

Publications for Portal Hypertension

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Articles related to Portal Hypertension:

(show top 50)    (show all 882)
idTitleAuthorsYear
1
Cirrhosis and portal hypertension: The importance of risk stratification, the role of hepatic venous pressure gradient measurement. (25866605)
2015
2
An autosomal dominant form of non-cirrhotic portal hypertension. (25796481)
2015
3
PRO-C3-levels in patients with HIV/HCV-Co-infection reflect fibrosis stage and degree of portal hypertension. (25265505)
2014
4
Massive gastrointestinal bleeding due to isolated jejunal varices in a patient without portal hypertension. (23528981)
2013
5
Alpha-adrenoceptor antagonists and chemical sympathectomy exacerbate anaphylaxis-induced hypotension, but not portal hypertension, in anesthetized rats. (23948775)
2013
6
Azathioprine induced serious portal hypertension: a case series of three IBD patients and review of the literature. (24261031)
2013
7
A woman with didanosine retinopathy and non-cirrhotic portal hypertension. (24400350)
2013
8
Features of portal hypertension are associated with major adverse events in Fontan patients: the VAST study. (23849105)
2013
9
Use of per rectal portal scintigraphy to detect portal hypertension in sinusoidal obstructive syndrome following unrelated cord blood transplantation. (23548502)
2013
10
Management of small hepatocellular carcinoma in cirrhosis: focus on portal hypertension. (23482437)
2013
11
In vivo validation of 4D flow MRI for assessing the hemodynamics of portal hypertension. (23148034)
2013
12
Portal hypertension related to light chain deposition disease of liver: an enlightening experience. (23723105)
2013
13
Gastric variceal bleeding from segmentary idiopathic splenic vein stenosis with left-sided portal hypertension: An unappreciated complication of pregnancy. (23607892)
2013
14
Effect of Helicobacter pylori infection on esophagogastric variceal bleeding in patients with liver cirrhosis and portal hypertension. (23577833)
2013
15
Use of serum vitamin B12 level as a marker to differentiate idiopathic noncirrhotic intrahepatic portal hypertension from cryptogenic cirrhosis. (22918688)
2013
16
Endoscopically proven case of rapid esophagogastric variceal progression and rupture as a result of portal hypertension with liver sarcoidosis. (24354353)
2013
17
Hepatic hemodynamics in 24 patients with nodular regenerative hyperplasia and symptomatic portal hypertension. (22554152)
2012
18
Mortality and complications in patients with portal hypertension who underwent transjugular intrahepatic portosystemic shunt (TIPS) - 12 years experience. (22767002)
2012
19
Splenic artery embolization as an adjunctive procedure for portal hypertension. (23729984)
2012
20
Transjugular intrahepatic portosystemic shunt for hepatitis C virus-related portal hypertension after liver transplantation. (22360603)
2012
21
Randomised clinical trial: the safety and efficacy of long-acting octreotide in patients with portal hypertension. (22380529)
2012
22
Transumbilical Single-Incision Laparoscopy Surgery Splenectomy Plus Pericaudial Devascularization in One Case With Portal Hypertension: The First Report. (22228758)
2012
23
Tracheal varix in portal hypertension. (21358342)
2012
24
Endoscopic management of portal hypertension. (22830037)
2012
25
Noncirrhotic portal hypertension in HIV infection. (21157331)
2011
26
Roux-en-Y loop varices in children with portal hypertension after liver transplantation: an unusual cause of "obscure" gastrointestinal bleeding. (20609173)
2011
27
Multiple splenic artery aneurysms resulting in infarction of the spleen and regional portal hypertension. (21654537)
2011
28
Kupffer cells are activated in cirrhotic portal hypertension and not normalised by TIPS. (21572121)
2011
29
Noncirrhotic portal hypertension in HIV-infected patients: a case control evaluation and review of the literature. (20969464)
2010
30
Long-term results of esophagogastric devascularization and splenectomy associated with endoscopic treatment in schistosomal portal hypertension. (20645097)
2010
31
A case of solid pseudopapillary neoplasm of the pancreas presenting with left-sided extrahepatic portal hypertension. (20720353)
2010
32
Altered basal and postprandial plasma melatonin, gastrin, ghrelin, leptin and insulin in patients with liver cirrhosis and portal hypertension without and with oral administration of melatonin or tryptophan. (19552764)
2009
33
Portal hypertension and hypersplenism in a patient with a Bochdalek hernia: a case report. (18398560)
2009
34
Portal hypertensive gastropathy (PHG). (15648238)
2004
35
Adverse effects of liver dysfunction and portal hypertension on intestinal adaptation in short bowel syndrome in children. (12488176)
2002
36
Choledocholithiasis in the Setting of Portal Hypertension. (11469971)
2001
37
Hepatopulmonary syndrome in noncirrhotic portal hypertension. (10836927)
2000
38
Transjugular intrahepatic portosystemic shunt for bleeding angiodysplasia-like lesions in portal-hypertensive colopathy. (9649472)
1998
39
Measurement of spleen size and its relation to hypersplenism and portal hemodynamics in portal hypertension due to hepatic cirrhosis. (8946990)
1996
40
Pharmacological treatment of portal hypertension. (8813485)
1996
41
Indications and role of albumin, plasma volume expansion excluded, in the preoperative or postoperative management of portal hypertension]. (8881492)
1996
42
Transjugular intrahepatic portosystemic shunt as a treatment for protein-losing enteropathy caused by portal hypertension. (8942750)
1996
43
Pharmacological therapy for portal hypertension: rationale and results. (7551972)
1995
44
Vasopressin, somatostatin and digestive hemorrhages in portal hypertension: the end of the tunnel?]. (7875444)
1994
45
Somatostatin and portal hypertensive bleeding: a safe therapeutic alternative? (1360437)
1992
46
Portal hypertension and ascites in systemic mastocytosis. (2608581)
1989
47
Idiopathic portal hypertension (noncirrhotic portal fibrosis), thrombosis in portal venous system and protein C deficiency. (2807175)
1989
48
Nodular regenerative hyperplasia of the liver with and without portal hypertension: a comparison. (3963734)
1986
49
Isoniazid-related liver disease. Occurrence with portal hypertension, hypoalbuminemia, and hypersplenism. (448941)
1979
50
Partial splenectomy and pleural splenic transposition, a new approach to congestive hypersplenism of portal hypertension. (5037265)
1972

Variations for Portal Hypertension

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Expression for genes affiliated with Portal Hypertension

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Search GEO for disease gene expression data for Portal Hypertension.

Pathways for genes affiliated with Portal Hypertension

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Pathways related to Portal Hypertension according to GeneCards Suite gene sharing:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.6AGTR1, CMA1
29.1G6PC, F2
3
Show member pathways
9.1UTS2, AGTR1, F2
4
Show member pathways
Development EDNRB signaling58
Development ACM2 and ACM4 activation of ERK58
Cell adhesion Integrin inside out signaling58
Development G Proteins mediated regulation MARK ERK signaling58
Signal transduction IP3 signaling58
Development Angiotensin signaling via PYK258
Development EPO induced MAPK pathway58
9.0EDN1, F2, AGTR1
5
Show member pathways
8.2AGTR1, AVPR1A, F2, EDN1, UTS2
6
Show member pathways
7.9EDN1, UTS2, SST, AVPR1A, AGTR1, F2
7
Show member pathways
7.5AVPR1A, F2, EDN1, AGTR1, SST, CMA1

Compounds for genes affiliated with Portal Hypertension

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Compounds related to Portal Hypertension according to GeneCards Suite gene sharing:

(show top 50)    (show all 78)
idCompoundScoreTop Affiliating Genes
1n(g)-nitro-l-arginine methyl ester4310.0EDN1, UTS2
2lacidipine439.9AGTR1, EDN1
3trandolapril43 1210.9EDN1, AGTR1
4cilazapril43 1210.9AGTR1, EDN1
5quinapril43 49 1211.9AGTR1, EDN1
6hydralazine43 49 1211.8AGTR1, EDN1
7valsartan43 59 28 1212.8EDN1, AGTR1
8atenolol43 49 28 24 1213.8EDN1, AGTR1
9irbesartan43 28 49 1212.7AGTR1, EDN1
10terlipressin43 1210.7AVPR1A, F2, SST
11dihydropyridine439.6EDN1, AGTR1
12phenylephrine43 28 24 1212.6EDN1, AGTR1, UTS2
13nitroglycerin43 1210.6AGTR1, EDN1, SST
14captopril43 59 49 28 1213.6SST, AGTR1, EDN1
15chymostatin439.6AGTR1, CMA1, EDN1
16verapamil43 28 49 24 1213.6EDN1, UTS2, AGTR1
17sodium nitroprusside439.6AGTR1, EDN1, SST
18acth439.5SST, AVPR1A, AGTR1
19nifedipine43 28 49 1212.5SST, AGTR1, EDN1
205-hydroxytryptamine439.5SST, EDN1, F2
21isoproterenol43 1210.5EDN1, SST, AGTR1
22enalapril43 49 1211.5EDN1, AGTR1
23diltiazem43 28 49 1212.4F2, AGTR1, EDN1
24carvedilol43 49 28 1212.4AGTR1, EDN1
25histamine43 28 2411.3CMA1, EDN1, SST
26thromboxane a243 2410.3EDN1, AGTR1, CPOX
27norepinephrine43 24 1211.3EDN1, UTS2, AGTR1, SST
28acetylcholine43 49 28 24 1213.3AGTR1, SST, EDN1, UTS2
29desmopressin43 59 28 1212.2F2, AVPR1A
30n acetylcysteine439.2AGTR1, UTS2, F2
31prostacyclin439.2EDN1, CPOX, AGTR1
32indomethacin43 28 59 1212.1UTS2, CPOX, EDN1
33vegf439.1EDN1, AGTR1, SST, UTS2
34aspirin43 49 28 2412.1F2, CPOX, AGTR1
35epinephrine43 24 1211.1AGTR1, SST, F2, EDN1
36creatinine439.1EDN1, AGTR1, F2, UTS2
37cycloheximide438.9EDN1, CPOX, SST, AGTR1
38nitric oxide43 24 1210.8EDN1, AGTR1, CPOX, UTS2
39phosphoinositide438.8AGTR1, EDN1, G6PC, UTS2
40phosphatidylinositol438.7AGTR1, CPOX, EDN1, AVPR1A
41lactate438.6G6PC, F2, CPOX, SST
42h2o2438.5CPOX, UTS2, AGTR1, G6PC
43dexamethasone43 49 28 1211.5AGTR1, SST, G6PC, CPOX
44glutamate438.4CPOX, G6PC, SST, AGTR1
45cholesterol43 28 24 1211.3G6PC, CMA1, UTS2, AGTR1, CPOX
46glucose438.2AGTR1, UTS2, G6PC, CPOX, SST
47alanine438.2CPOX, F2, G6PC, AGTR1
48oxygen43 249.2UTS2, CPOX, G6PC, EDN1, AGTR1
49arginine437.7G6PC, SST, AGTR1, EDN1, AVPR1A, CPOX
50calcium43 49 24 1210.4AVPR1A, AGTR1, UTS2, CMA1, G6PC, CPOX

GO Terms for genes affiliated with Portal Hypertension

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Cellular components related to Portal Hypertension according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:00056158.8SST, UTS2, EDN1, F2
2extracellular regionGO:00055768.2SST, UTS2, CMA1, EDN1, F2

Biological processes related to Portal Hypertension according to GeneCards Suite gene sharing:

(show all 17)
idNameGO IDScoreTop Affiliating Genes
1response to testosteroneGO:00335749.9UTS2, EDN1
2positive regulation of renal sodium excretionGO:00358159.9AVPR1A, EDN1
3positive regulation of collagen biosynthetic processGO:00329679.8UTS2, F2
4response to amino acidGO:00432009.8EDN1, SST
5positive regulation of angiogenesisGO:00457669.8UTS2, CMA1
6positive regulation of cytosolic calcium ion concentration involved in phospholipase C-activating G-protein coupled signaling paGO:00514829.8EDN1, AGTR1
7regulation of vasoconstrictionGO:00192299.7EDN1, AGTR1
8regulation of inflammatory responseGO:00507279.6CMA1, AGTR1
9positive regulation of heart rateGO:00104609.6UTS2, EDN1, AVPR1A
10positive regulation of reactive oxygen species metabolic processGO:020003799.5AGTR1, F2
11cell surface receptor signaling pathwayGO:00071669.4SST, EDN1, F2
12calcium-mediated signalingGO:00197229.4AGTR1, EDN1, AVPR1A
13positive regulation of cell growthGO:00303079.2F2, AVPR1A
14glucose transportGO:00157589.1EDN1, G6PC
15positive regulation of cell proliferationGO:00082849.1EDN1, F2, AVPR1A
16positive regulation of cytosolic calcium ion concentrationGO:00072049.1AVPR1A, EDN1, UTS2, AGTR1
17G-protein coupled receptor signaling pathwayGO:00071869.0AVPR1A, EDN1, SST, AGTR1

Molecular functions related to Portal Hypertension according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1hormone activityGO:00051799.4SST, UTS2, EDN1

Products for genes affiliated with Portal Hypertension

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Sources for Portal Hypertension

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3CDC
13ExPASy
14FMA
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23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet