MCID: PRT013
MIFTS: 65

Portal Hypertension malady

Gastrointestinal category

Summaries for Portal Hypertension

Sources:
43NIH Rare Diseases, 64Wikipedia, 33MalaCards
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NIH Rare Diseases:43 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: Portal Hypertension, also known as hypertension, portal, is related to cystic fibrosis and insulin resistance. An important gene associated with Portal Hypertension is SST (somatostatin), and among its related pathways are Common Pathway and Agents Acting on the Renin-Angiotensin System Pathway, Pharmacodynamics. The drugs acebutolol and amiloride and the compounds terlipressin and captopril have been mentioned in the context of this disorder. Affiliated tissues include liver, brain and kidney, and related mouse phenotypes are normal and homeostasis/metabolism.

Wikipedia:64 In medicine, portal hypertension is hypertension (high blood pressure) in the portal vein system, which... more...

Aliases & Classifications for Portal Hypertension

Sources:
8Disease Ontology, 43NIH Rare Diseases, 10DISEASES, 45Novoseek, 61UMLS, 57SNOMED-CT, 35MeSH, 40NCIt, 27ICD9CM, 25ICD10
See all sources

Classifications:

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


Aliases & Descriptions:

portal hypertension 8 43 10
hypertension, portal 43 61
hypertensive disease 61
hypertension portal 45


External Ids:

Disease Ontology8 DOID:10762
MeSH35 D006975
NCIt40 C3119
SNOMED-CT57 34742003, 155821005
ICD9CM27 572.3
ICD1025 K76.6

Related Diseases for Portal Hypertension

Sources:
17GeneCards, 18GeneDecks
See all sources

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 241)
idRelated DiseaseScoreTop Affiliating Genes
1cystic fibrosis30.6CA2
2insulin resistance30.5G6PC, EDN1, AGTR1, SST, UTS2, ADRB3
3vascular disease30.2EDN3, EDN1, AGTR1, CMA1
4hepatorenal syndrome30.0EDN1, F2
5atherosclerosis30.0EDN1, AGTR1, CMA1, UTS2, ADRB3
6primary pulmonary hypertension30.0G6PC, EDN3, EDN1, UTS2
7hypertriglyceridemia30.0ADRB3, G6PC
8brain edema30.0BDKRB2
9adenoma30.0SST, EDN3, G6PC
10coronary heart disease29.7EDN1, AGTR1, ADRB3
11hypersplenism10.8
12nodular regenerative hyperplasia10.7
13portal vein thrombosis10.7
14hepatitis c10.6
15hepatopulmonary syndrome10.6
16splenic artery aneurysm10.5
17banti's syndrome10.5
18splenomegaly10.5
19gastric antral vascular ectasia10.5
20hepatitis a10.5
21n syndrome10.5
22tuberculosis10.5
23congenital hepatic fibrosis10.5
24hepatitis d10.4
25hepatoportal sclerosis10.4
26biliary atresia10.4
27hepatitis e10.4
28abdominal tuberculosis10.4
29systemic mastocytosis10.4
30hepatitis c virus10.4
31hereditary hemorrhagic telangiectasia10.3
32primary biliary cirrhosis10.3
33felty's syndrome10.3
34cystinosis10.3
35choledochal cyst10.3
36hepatic vein thrombosis10.3
37mastocytosis10.3
38dyskeratosis congenita10.2
39mixed connective tissue disease10.2
40fatty liver disease10.2
41splenic infarction10.2
42protein-losing enteropathy10.2
43hepatitis b10.2
44alcoholic liver cirrhosis10.2
45alcoholic hepatitis10.2
46polycystic liver disease10.2
47thrombocytopenia10.2
48mallory-weiss syndrome10.2
49pediatric hypertension10.2
50autonomic dysfunction10.2

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



Diseases related to portal hypertension

Clinical Features for Portal Hypertension

Drugs & Therapeutics for Portal Hypertension

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Portal Hypertension

Drug clinical trials:

Search ClinicalTrials for Portal Hypertension

Search NIH Clinical Center for Portal Hypertension

Search CenterWatch for Portal Hypertension

Inferred drug relations via UMLS61/NDF-RT41:

Genetic Tests for Portal Hypertension

Anatomical Context for Portal Hypertension

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Portal Hypertension:

33
Liver, Brain, Kidney, Lymph node, Spleen, Bone marrow, Smooth muscle, Small intestine, Colon, Pancreas, Thyroid, Breast, Placenta, Myeloid, T cells, B cells, Endothelial, Pancreatic islet

Animal Models for Portal Hypertension or affiliated genes

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

MGI Mouse Phenotypes related to Portal Hypertension:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000287310.3G6PC, EDN1, SST, AVPR1A, F2, ADRBK1
2MP:000537610.0ADRB3, BDKRB2, CA2, CTC1, G6PC, EDN1

Publications for Portal Hypertension

Sources:
51PubMed
See all sources

Articles related to Portal Hypertension:

(show top 50)    (show all 801)
idTitleAuthorsYear
1
Invasive and non-invasive techniques for detecting portal hypertension and predicting variceal bleeding in cirrhosis: A review. (24328372)
2014
2
Gastric variceal bleeding from segmentary idiopathic splenic vein stenosis with left-sided portal hypertension: An unappreciated complication of pregnancy. (23607892)
2013
3
Portal vein omentin is increased in patients with liver cirrhosis but is not associated with complications of portal hypertension. (23855493)
2013
4
Transplantation of endothelial progenitor cells ameliorates vascular dysfunction and portal hypertension in carbon tetrachloride-induced rat liver cirrhotic model. (22849788)
2013
5
Clinical effects and complications of TIPS for portal hypertension due to cirrhosis: a single center. (24307804)
2013
6
Influence of clinically significant portal hypertension on survival after hepatic resection for hepatocellular carcinoma in cirrhotic patients. (23654354)
2013
7
Unilateral Giant Varicocele Mimicking Inguinal Hernia Resulting from Portosystemic Shunt without Evidence of Portal Hypertension: An Unusual Case Report. (23533916)
2013
8
The risk association between experimental portal hypertension and an aortic atherosclerosis-like disease. (22730048)
2013
9
Transjugular intrahepatic portosystemic shunt for symptomatic portal hypertension in hepatocellular carcinoma with portal vein tumor thrombosis. (23679937)
2013
10
Differentiation and Migration of Bone Marrow Mesenchymal Stem Cells Transplanted through the Spleen in Rats with Portal Hypertension. (24340101)
2013
11
Hepatic and splenic stiffness augmentation assessed with MR elastography in an in vivo porcine portal hypertension model. (23418135)
2013
12
Antioxidant properties of glutamine and its role in VEGF-Akt pathways in portal hypertension gastropathy. (23901221)
2013
13
Aquaporin-1 is associated with arterial capillary proliferation and hepatic sinusoidal transformation contributing to portal hypertension in primary biliary cirrhosis. (23949237)
2013
14
Ascitic microbiota composition is correlated with clinical severity in cirrhosis with portal hypertension. (24086392)
2013
15
Can the development of cardiac dysfunction in patients with liver cirrhosis and portal hypertension be predicted? (23199558)
2013
16
Morphological and biomechanical remodeling of the hepatic portal vein in a swine model of portal hypertension. (22192237)
2012
17
Spleno-parametrial ectopic varices: an unusual porto-systemic shunt in portal hypertension. (22270437)
2012
18
Small intestinal transit in patients with liver cirrhosis and portal hypertension: a descriptive study. (23216853)
2012
19
Portal hypertension caused by postoperative superior mesenteric arteriovenous fistula. (22838176)
2012
20
Massive enlargement of a paraumbilical vein in a patient with advanced portal hypertension. (21890161)
2012
21
Role of hepatic vein catheterisation and transient elastography in the diagnosis of idiopathic portal hypertension. (22721839)
2012
22
Colonic venous malformation and portal hypertension: association, management, and review of the literature. (22212493)
2012
23
Sinistral portal hypertension: clinical features and surgical treatment of chronic splenic vein occlusion. (22024761)
2012
24
Delayed liver laceration following transjugular intrahepatic portosystemic shunt for portal hypertension. (23326153)
2012
25
Predicting portal hypertension as assessed by acoustic radiation force impulse: correlations with the Doppler ultrasound. (22815421)
2012
26
Adams-Oliver syndrome and portal hypertension: fortuitous association or common mechanism? (22307742)
2012
27
Management of hypersplenism in non-cirrhotic portal hypertension: a surgical series. (22484585)
2012
28
Long-term low-dose maintenance pegylated interferon may prevent decompensation in cirrhotic patients with portal hypertension: are we there yet? (22287292)
2012
29
A Idiopathic portal hypertension with multiple hepatic hyperplastic nodules supplied by portal vein. (22700643)
2012
30
Disseminated sarcoidosis resulting in portal hypertension and gastrointestinal bleeding: a rare presentation. (22891173)
2012
31
Assessment of intrahepatic blood flow by Doppler ultrasonography: relationship between the hepatic vein, portal vein, hepatic artery and portal pressure measured intraoperatively in patients with portal hypertension. (21767412)
2011
32
Influence of the inversion of the portal/splenic vein diameter in the results of the surgical treatment of schistossomotic portal hypertension. (21537741)
2011
33
Kupffer cell activation and portal hypertension. (21708827)
2011
34
MR findings of the brain in children and adolescents with portal hypertension and the relationship with blood manganese levels. (20571985)
2010
35
Hepatic endothelial dysfunction and abnormal angiogenesis: new targets in the treatment of portal hypertension. (20561700)
2010
36
Investigating the vascular influences of sepsis in portal hypertension: better late than never? (21093821)
2010
37
Treatment of a patient with obstructive jaundice and extrahepatic portal hypertension. (19666415)
2009
38
The usefulness of distal splenorenal shunt in children with portal hypertension for the treatment of severe thrombocytopenia and leukopenia. (18196322)
2008
39
Experimental investigation of the role of endothelin-1 in idiopathic portal hypertension. (17608860)
2007
40
Effect of somatostatin versus octreotide on portal haemodynamics in patients with cirrhosis and portal hypertension. (15647641)
2005
41
Congenital hepatic fibrosis and portal hypertension in pregnancy. (15694091)
2005
42
Choledocholithiasis in the Setting of Portal Hypertension. (11469971)
2001
43
Esophageal variceal ligation for esophageal variceal hemorrhage in a patient with portal and primary pulmonary hypertension complicating myelofibrosis. (11330433)
2001
44
Effect of somatostatin on portal and hepatic hemodynamics and portal venous pressure in cirrhotic patients with portal hypertension]. (11825434)
1998
45
Portal vein calcification and associated biliary stricture in idiopathic portal hypertension (Banti's syndrome). (9516510)
1998
46
Hemoptysis secondary to bronchial varices associated with alcoholic liver cirrhosis and portal hypertension. (8079940)
1994
47
Portal hypertension as presenting feature of a myeloproliferative disorder. Diagnosis and therapeutic dilemmas. (8016576)
1993
48
"Endemic" idiopathic portal hypertension: report on 32 patients with non-cirrhotic portal fibrosis. (1290252)
1992
49
Carbohydrate metabolism enzymes in children with extrahepatic portal hypertension]. (1722840)
1991
50
Portal hypertension: serotonin and pathogenesis. (2285648)
1990

Genetic Variations for Portal Hypertension

Expression for genes affiliated with Portal Hypertension

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Portal Hypertension

Search GEO for disease gene expression data for Portal Hypertension.

Pathways for genes affiliated with Portal Hypertension

Sources:
54Reactome, 52QIAGEN, 50PharmGKB, 38NCBI BioSystems Database, 30KEGG, 12EMD Millipore
See all sources

Pathways related to Portal Hypertension according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
10.4F2, FGB
2
Hide members
10.4CMA1, AGTR1, BDKRB2
3
Hide members
10.4F2, FGB, BDKRB2
4
Hide members
10.4AGTR1, F2, ADRBK1
510.4BDKRB2, AGTR1, AVPR1A, ADRB3
6
Hide members
10.4UTS2R, AVPR1A, AGTR1, BDKRB2
7
Hide members
10.4EDN3, EDN1, AGTR1, FGB, F2
8
Hide members
10.3ADRBK1, BDKRB2, EDN3, EDN1, AGTR1, AVPR1A
9
Hide members
10.3BDKRB2, EDN3, EDN1, AGTR1, SST, AVPR1A
10
Hide members
10.2SST, AGTR1, EDN1, EDN3, BDKRB2, FGB

Compounds for genes affiliated with Portal Hypertension

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

Compounds related to Portal Hypertension according to GeneCards/GeneDecks:

(show top 50)    (show all 103)
idCompoundScoreTop Affiliating Genes
1terlipressin45 1111.9F2, AVPR1A, SST
2captopril45 29 50 60 1114.9BDKRB2, EDN3, EDN1, SST
3norepinephrine45 11 2412.8CA2, EDN3, UTS2, ADRBK1, ADRB3
4thromboxane a245 2411.8BDKRB2, EDN3, EDN1, AGTR1, CPOX
5sodium nitroprusside4510.8BDKRB2, EDN3, EDN1, AGTR1, SST
6nifedipine45 50 29 1113.8BDKRB2, EDN3, EDN1, AGTR1, SST
7chymostatin4510.8CMA1, AGTR1, EDN1
8phenylephrine45 29 11 2413.8UTS2, AGTR1, EDN1, EDN3
9l-nmma4510.8BDKRB2, EDN3, EDN1, AGTR1
10epinephrine45 11 2412.8CA2, EDN1, AGTR1, SST, F2, ADRBK1
11isoproterenol45 1111.8BDKRB2, EDN1, AGTR1, SST, ADRBK1, ADRB3
12cgmp45 2911.8BDKRB2, EDN3, EDN1, AGTR1, ADRB3
13heparin45 29 11 2413.8ADRBK1, CMA1
14acetylcholine45 50 29 11 2414.8EDN3, EDN1, AGTR1, SST, UTS2, ADRBK1
15n(g)-nitro-l-arginine methyl ester4510.8BDKRB2, EDN1, UTS2
16histamine45 29 2412.8BDKRB2, EDN3, EDN1, SST, CMA1, ADRBK1
17inositol 1,4,5 trisphosphate4510.8BDKRB2, EDN3, EDN1, AGTR1, ADRBK1
18dexamethasone45 50 29 1113.8BDKRB2, G6PC, EDN3, ADRB3
19creatinine4510.8BDKRB2, CA2, EDN1, MDH2, AGTR1, F2
20phosphoinositide4510.8BDKRB2, G6PC, EDN3, EDN1, AGTR1, UTS2
21pge24510.8BDKRB2, CA2, EDN3, EDN1, SST, CPOX
22urotensin ii (human)6010.8UTS2R, UTS2
23[orn8]-urotensin ii6010.7UTS2R, UTS2
24bq 4854510.7EDN1, EDN3
25lactate4510.7CA2, G6PC, MDH2, SST, F2, CPOX
26indomethacin45 60 29 1113.7BDKRB2, EDN3, EDN1, UTS2, CPOX
27(+-)-ac 7954 hydrochloride6010.7UTS2, UTS2R
28glutamate4510.7BDKRB2, CA2, G6PC, EDN3, MDH2, AGTR1
29arginine4510.7CPOX, BDKRB2, CA2, G6PC, EDN3, EDN1
30nitric oxide45 11 2412.7BDKRB2, EDN3, EDN1, AGTR1, UTS2, ADRB3
31atp45 2911.7BDKRB2, CA2, G6PC, MDH2, AGTR1, ADRBK1
32sb 6575106010.7UTS2, UTS2R
33glucose4510.7G6PC, EDN3, MDH2, AGTR1, SST, UTS2
34alanine4510.7BDKRB2, CA2, G6PC, EDN3, MDH2, AGTR1
35sb 7063756010.7UTS2, UTS2R
36aspartate4510.7CA2, G6PC, EDN3, MDH2, F2, ADRBK1
37palosuran45 2911.7UTS2, UTS2R
38adenylate4510.7BDKRB2, CA2, G6PC, EDN3, SST, ADRB3
39calcium45 50 11 2413.6MDH2, EDN1, EDN3, G6PC, CA2, BDKRB2
40lipid4510.6BDKRB2, CA2, G6PC, AGTR1, UTS2, ADRBK1
41diltiazem45 29 1112.6F2, AGTR1, EDN1
42lead4510.6CA2, G6PC, ADRBK1, CPOX
43carvedilol45 50 29 1113.6EDN1, AGTR1, ADRB3
44sarafotoxin s6a6010.6EDN3, EDN1
45oxygen45 2411.5CA2, G6PC, EDN1, AGTR1, UTS2, ADRB3
46endothelin 2 (human)6010.5EDN3, EDN1
47endothelin 3 (human, rat)6010.4EDN3, EDN1
48carbachol45 29 1112.4ADRBK1, SST, EDN1, EDN3
49endothelin 1 (human, porcine)6010.3EDN3, EDN1
50citrate4510.1CA2, MDH2, F2, CPOX

GO Terms for genes affiliated with Portal Hypertension

Sources:
16Gene Ontology
See all sources

Cellular components related to Portal Hypertension according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:00557610.4EDN1, SST, FGB, F2, CMA1, UTS2
2extracellular spaceGO:00561510.2UTS2, CA2, EDN3, EDN1, SST, PCSK1N
3plasma membraneGO:00588610.0BDKRB2, CA2, MDH2, AGTR1, FGB, AVPR1A

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

(show all 27)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of heart rateGO:01046010.8EDN3, EDN1, AVPR1A, UTS2
2positive regulation of circadian sleep/wake cycle, wakefulnessGO:01084110.8UTS2R, UTS2
3positive regulation of cellular pH reductionGO:03284910.8AVPR1A, CA2
4regulation of vasoconstrictionGO:01922910.8BDKRB2, EDN3, EDN1, AGTR1
5vasoconstrictionGO:04231010.8EDN1, EDN3, BDKRB2
6positive regulation of circadian sleep/wake cycle, REM sleepGO:04600510.8UTS2R, UTS2
7cell surface receptor signaling pathwayGO:00716610.8BDKRB2, EDN3, EDN1, SST, F2
8blood circulationGO:00801510.8UTS2R, AVPR1A, EDN3, BDKRB2
9elevation of cytosolic calcium ion concentrationGO:00720410.8UTS2R, UTS2, AVPR1A, AGTR1, EDN1, BDKRB2
10G-protein coupled receptor signaling pathwayGO:00718610.7BDKRB2, EDN1, AGTR1, SST, AVPR1A
11calcium-mediated signalingGO:01972210.7AVPR1A, AGTR1, EDN1
12positive regulation of fibroblast proliferationGO:04814610.7CTC1, UTS2, UTS2R
13negative regulation of renal sodium excretionGO:03581410.7UTS2, UTS2R
14peptide hormone secretionGO:03007210.7EDN3, EDN1
15vein smooth muscle contractionGO:01482610.7EDN3, EDN1
16negative regulation of glomerular filtrationGO:00310510.7UTS2, UTS2R
17negative regulation of urine volumeGO:03581110.7UTS2, UTS2R
18regulation of systemic arterial blood pressure by endothelinGO:00310010.7EDN3, EDN1
19artery smooth muscle contractionGO:01482410.6EDN1, EDN3
20positive regulation of cell growthGO:03030710.6AVPR1A, F2, UTS2R
21inositol phosphate-mediated signalingGO:04801610.6EDN1, EDN3
22positive regulation of hormone secretionGO:04688710.6EDN1, EDN3
23regulation of vasodilationGO:04231210.5AGTR1, UTS2R
24positive regulation of blood pressureGO:04577710.4UTS2R, UTS2
25positive regulation of angiogenesisGO:04576610.4CMA1, UTS2, UTS2R
26positive regulation of renal sodium excretionGO:03581510.4AVPR1A, EDN1
27elevation of cytosolic calcium ion concentration involved in phospholipase C-activating G-protein coupled signaling pathwayGO:05148210.1AGTR1, EDN1

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

idNameGO IDScoreTop Affiliating Genes
1receptor bindingGO:00510210.4UTS2, F2, FGB, PCSK1N, EDN3
2hormone activityGO:00517910.1UTS2, SST, EDN1, EDN3

Products for genes affiliated with Portal Hypertension

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  • Proteins
  • Lysates
  • Antibodies

Sources for Portal Hypertension

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