MCID: PRT013
MIFTS: 66

Portal Hypertension malady

Rare diseases, Gastrointestinal diseases, Cardiovascular diseases categories
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Summaries for Portal Hypertension

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NIH Rare Diseases:42 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 hypertension and liver cirrhosis. 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 drugs diltiazem and felodipine and 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:65 In medicine, portal hypertension is hypertension (high blood pressure) in the portal vein system, which... more...

Aliases & Classifications for Portal Hypertension

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Portal Hypertension, Aliases & Descriptions:

Name: Portal Hypertension 8 42 10
Hypertension, Portal 42 62
 
Hypertensive Disease 62
Hypertension Portal 44


Classifications:



External Ids:

Disease Ontology8 DOID:10762
NCIt39 C3119
ICD9CM27 572.3
SNOMED-CT57 34742003, 155821005
MeSH34 D006975
ICD1025 K76.6

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/GeneDecks gene sharing:

(show top 50)    (show all 236)
idRelated DiseaseScoreTop Affiliating Genes
1hypertension32.0UTS2, F2, AGTR1, EDN1
2liver cirrhosis31.4UTS2, EDN1, F2
3vascular disease30.1AGTR1, CMA1, EDN1
4ischemia30.0G6PC, EDN1, AVPR1A
5pulmonary hypertension30.0EDN1, UTS2, G6PC
6insulin resistance29.9UTS2, AGTR1, G6PC, EDN1, SST
7atherosclerosis29.9AGTR1, UTS2, CMA1, EDN1
8hepatitis11.0
9hypersplenism10.8
10liver disease10.7
11nodular regenerative hyperplasia10.7
12portal vein thrombosis10.7
13esophagitis10.7
14pancreatitis10.7
15aneurysm10.6
16hepatopulmonary syndrome10.6
17splenomegaly10.6
18splenic artery aneurysm10.6
19banti's syndrome10.6
20hepatocellular carcinoma10.6
21gastric antral vascular ectasia10.5
22endotheliitis10.5
23arteriovenous fistula10.5
24tuberculosis10.5
25congenital hepatic fibrosis10.5
26hepatitis c10.5
27hepatoportal sclerosis10.5
28cystic fibrosis10.4
29biliary atresia10.4
30protein c deficiency10.4
31systemic mastocytosis10.4
32sarcoidosis10.4
33mastocytosis10.4
34hepatitis b10.3
35primary biliary cirrhosis10.3
36cystinosis10.3
37choledochal cyst10.3
38fatty liver disease10.3
39felty's syndrome10.3
40abdominal tuberculosis10.3
41hepatitis c virus10.3
42esophageal varix10.3SST, F2
43alcoholic hepatitis10.3
44hereditary hemorrhagic telangiectasia10.3
45myelofibrosis10.3
46thrombocytopenia10.3
47dyskeratosis congenita10.3
48cystadenoma10.3
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

Inferred drug relations via UMLS62/NDF-RT40:

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:

32
Liver, Spleen, Endothelial, Pancreas, Brain, Colon, Bone, Kidney, Thyroid, Breast, Testes, Myeloid, Bone marrow, Lymph node, Smooth muscle, Small intestine, Placenta, T cells

Animal Models for Portal Hypertension or affiliated genes

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MGI Mouse Phenotypes related to Portal Hypertension:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053878.0AVPR1A, F2, CMA1, SST, AGTR1
2MP:00028737.9SST, G6PC, EDN1, F2, AVPR1A
3MP:00036317.9SST, G6PC, EDN1, F2, AVPR1A
4MP:00053767.3AVPR1A, F2, EDN1, G6PC, SST, AGTR1

Publications for Portal Hypertension

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Articles related to Portal Hypertension:

(show top 50)    (show all 858)
idTitleAuthorsYear
1
PRO-C3-levels in patients with HIV/HCV-Co-infection reflect fibrosis stage and degree of portal hypertension. (25265505)
2014
2
Massive gastrointestinal bleeding due to isolated jejunal varices in a patient without portal hypertension. (23528981)
2013
3
Alpha-adrenoceptor antagonists and chemical sympathectomy exacerbate anaphylaxis-induced hypotension, but not portal hypertension, in anesthetized rats. (23948775)
2013
4
Azathioprine induced serious portal hypertension: a case series of three IBD patients and review of the literature. (24261031)
2013
5
A woman with didanosine retinopathy and non-cirrhotic portal hypertension. (24400350)
2013
6
Features of portal hypertension are associated with major adverse events in Fontan patients: the VAST study. (23849105)
2013
7
Use of per rectal portal scintigraphy to detect portal hypertension in sinusoidal obstructive syndrome following unrelated cord blood transplantation. (23548502)
2013
8
Management of small hepatocellular carcinoma in cirrhosis: focus on portal hypertension. (23482437)
2013
9
In vivo validation of 4D flow MRI for assessing the hemodynamics of portal hypertension. (23148034)
2013
10
Portal hypertension related to light chain deposition disease of liver: an enlightening experience. (23723105)
2013
11
Gastric variceal bleeding from segmentary idiopathic splenic vein stenosis with left-sided portal hypertension: An unappreciated complication of pregnancy. (23607892)
2013
12
Effect of Helicobacter pylori infection on esophagogastric variceal bleeding in patients with liver cirrhosis and portal hypertension. (23577833)
2013
13
Use of serum vitamin B12 level as a marker to differentiate idiopathic noncirrhotic intrahepatic portal hypertension from cryptogenic cirrhosis. (22918688)
2013
14
Endoscopically proven case of rapid esophagogastric variceal progression and rupture as a result of portal hypertension with liver sarcoidosis. (24354353)
2013
15
Hepatic hemodynamics in 24 patients with nodular regenerative hyperplasia and symptomatic portal hypertension. (22554152)
2012
16
Mortality and complications in patients with portal hypertension who underwent transjugular intrahepatic portosystemic shunt (TIPS) - 12 years experience. (22767002)
2012
17
Splenic artery embolization as an adjunctive procedure for portal hypertension. (23729984)
2012
18
Transjugular intrahepatic portosystemic shunt for hepatitis C virus-related portal hypertension after liver transplantation. (22360603)
2012
19
Randomised clinical trial: the safety and efficacy of long-acting octreotide in patients with portal hypertension. (22380529)
2012
20
Transumbilical Single-Incision Laparoscopy Surgery Splenectomy Plus Pericaudial Devascularization in One Case With Portal Hypertension: The First Report. (22228758)
2012
21
Tracheal varix in portal hypertension. (21358342)
2012
22
Endoscopic management of portal hypertension. (22830037)
2012
23
Congenital hepatic fibrosis and portal hypertension in autosomal dominant polycystic kidney disease. (21694639)
2012
24
Noncirrhotic portal hypertension in HIV infection. (21157331)
2011
25
Roux-en-Y loop varices in children with portal hypertension after liver transplantation: an unusual cause of "obscure" gastrointestinal bleeding. (20609173)
2011
26
Multiple splenic artery aneurysms resulting in infarction of the spleen and regional portal hypertension. (21654537)
2011
27
Kupffer cells are activated in cirrhotic portal hypertension and not normalised by TIPS. (21572121)
2011
28
Noncirrhotic portal hypertension in HIV-infected patients: a case control evaluation and review of the literature. (20969464)
2010
29
Long-term results of esophagogastric devascularization and splenectomy associated with endoscopic treatment in schistosomal portal hypertension. (20645097)
2010
30
A case of solid pseudopapillary neoplasm of the pancreas presenting with left-sided extrahepatic portal hypertension. (20720353)
2010
31
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
32
Portal hypertension and hypersplenism in a patient with a Bochdalek hernia: a case report. (18398560)
2009
33
Portal hypertensive gastropathy (PHG). (15648238)
2004
34
Laparoscopic suture repair of a perforated gastric ulcer in a severely cirrhotic patient with portal hypertension: first case report. (14626407)
2003
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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Expression patterns in normal tissues for genes affiliated with Portal Hypertension

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Pathways for genes affiliated with Portal Hypertension

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Pathways related to Portal Hypertension according to GeneCards/GeneDecks:

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 signaling60
Development ACM2 and ACM4 activation of ERK60
Cell adhesion Integrin inside out signaling60
Development G Proteins mediated regulation MARK ERK signaling60
Signal transduction IP3 signaling60
Development Angiotensin signaling via PYK260
Development EPO induced MAPK pathway60
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/GeneDecks:

(show top 50)    (show all 78)
idCompoundScoreTop Affiliating Genes
1n(g)-nitro-l-arginine methyl ester4410.0EDN1, UTS2
2lacidipine449.9AGTR1, EDN1
3trandolapril44 1110.9EDN1, AGTR1
4cilazapril44 1110.9AGTR1, EDN1
5quinapril44 50 1111.9AGTR1, EDN1
6hydralazine44 50 1111.8AGTR1, EDN1
7valsartan44 61 28 1112.8EDN1, AGTR1
8atenolol44 50 28 24 1113.8EDN1, AGTR1
9irbesartan44 28 50 1112.7AGTR1, EDN1
10terlipressin44 1110.7AVPR1A, F2, SST
11dihydropyridine449.6EDN1, AGTR1
12phenylephrine44 28 24 1112.6EDN1, AGTR1, UTS2
13nitroglycerin44 1110.6AGTR1, EDN1, SST
14captopril44 61 50 28 1113.6SST, AGTR1, EDN1
15chymostatin449.6AGTR1, CMA1, EDN1
16verapamil44 28 50 24 1113.6EDN1, UTS2, AGTR1
17sodium nitroprusside449.6AGTR1, EDN1, SST
18acth449.5SST, AVPR1A, AGTR1
19nifedipine44 28 50 1112.5SST, AGTR1, EDN1
205-hydroxytryptamine449.5SST, EDN1, F2
21isoproterenol44 1110.5EDN1, SST, AGTR1
22enalapril44 50 1111.5EDN1, AGTR1
23diltiazem44 28 50 1112.4F2, AGTR1, EDN1
24carvedilol44 50 28 1112.4AGTR1, EDN1
25histamine44 28 2411.3CMA1, EDN1, SST
26thromboxane a244 2410.3EDN1, AGTR1, CPOX
27norepinephrine44 24 1111.3EDN1, UTS2, AGTR1, SST
28acetylcholine44 50 28 24 1113.3AGTR1, SST, EDN1, UTS2
29desmopressin44 61 28 1112.2F2, AVPR1A
30n acetylcysteine449.2AGTR1, UTS2, F2
31prostacyclin449.2EDN1, CPOX, AGTR1
32indomethacin44 28 61 1112.1UTS2, CPOX, EDN1
33vegf449.1EDN1, AGTR1, SST, UTS2
34aspirin44 50 28 2412.1F2, CPOX, AGTR1
35epinephrine44 24 1111.1AGTR1, SST, F2, EDN1
36creatinine449.1EDN1, AGTR1, F2, UTS2
37cycloheximide448.9EDN1, CPOX, SST, AGTR1
38nitric oxide44 24 1110.8EDN1, AGTR1, CPOX, UTS2
39phosphoinositide448.8AGTR1, EDN1, G6PC, UTS2
40phosphatidylinositol448.7AGTR1, CPOX, EDN1, AVPR1A
41lactate448.6G6PC, F2, CPOX, SST
42h2o2448.5CPOX, UTS2, AGTR1, G6PC
43dexamethasone44 50 28 1111.5AGTR1, SST, G6PC, CPOX
44glutamate448.4CPOX, G6PC, SST, AGTR1
45cholesterol44 28 24 1111.3G6PC, CMA1, UTS2, AGTR1, CPOX
46glucose448.2AGTR1, UTS2, G6PC, CPOX, SST
47alanine448.2CPOX, F2, G6PC, AGTR1
48oxygen44 249.2UTS2, CPOX, G6PC, EDN1, AGTR1
49arginine447.7G6PC, SST, AGTR1, EDN1, AVPR1A, CPOX
50calcium44 50 24 1110.4AVPR1A, 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/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:0056158.8SST, UTS2, EDN1, F2
2extracellular regionGO:0055768.2SST, UTS2, CMA1, EDN1, F2

Biological processes related to Portal Hypertension according to GeneCards/GeneDecks:

(show all 17)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of renal sodium excretionGO:0358159.9EDN1, AVPR1A
2response to testosteroneGO:0335749.9UTS2, EDN1
3positive regulation of collagen biosynthetic processGO:0329679.8F2, UTS2
4response to amino acidGO:0432009.8EDN1, SST
5positive regulation of angiogenesisGO:0457669.8CMA1, UTS2
6positive regulation of cytosolic calcium ion concentration involved in phospholipase C-activating G-protein coupled signaling paGO:0514829.8AGTR1, EDN1
7regulation of vasoconstrictionGO:0192299.7AGTR1, EDN1
8regulation of inflammatory responseGO:0507279.6CMA1, AGTR1
9positive regulation of heart rateGO:0104609.6UTS2, AVPR1A, EDN1
10positive regulation of reactive oxygen species metabolic processGO:20003799.5AGTR1, F2
11cell surface receptor signaling pathwayGO:0071669.4SST, F2, EDN1
12calcium-mediated signalingGO:0197229.4EDN1, AVPR1A, AGTR1
13positive regulation of cell growthGO:0303079.2F2, AVPR1A
14glucose transportGO:0157589.1G6PC, EDN1
15positive regulation of cell proliferationGO:0082849.1F2, AVPR1A, EDN1
16positive regulation of cytosolic calcium ion concentrationGO:0072049.1EDN1, UTS2, AGTR1, AVPR1A
17G-protein coupled receptor signaling pathwayGO:0071869.0EDN1, AGTR1, AVPR1A, SST

Molecular functions related to Portal Hypertension according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1hormone activityGO:0051799.4SST, UTS2, EDN1

Products for genes affiliated with Portal Hypertension

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Sources for Portal Hypertension

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet