MCID: HPT066
MIFTS: 32

Hepatoportal Sclerosis malady

Liver diseases, Rare diseases categories

Summaries for Hepatoportal Sclerosis

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34MalaCards
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MalaCards: Hepatoportal Sclerosis, also known as obliterative portal venopathy, is related to hypertension and portal hypertension. An important gene associated with Hepatoportal Sclerosis is ELN (elastin), and among its related pathways are p70S6K Signaling and Integrin Pathway. The compounds hydralazine and ang ii have been mentioned in the context of this disorder. Affiliated tissues include liver and endothelial, and related mouse phenotypes are homeostasis/metabolism and cardiovascular system.

Aliases & Classifications for Hepatoportal Sclerosis

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Sources:
50Orphanet, 27ICD10 via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Liver diseases
Orphanet: 50 
Rare hepatic diseases


Characteristics (Orphanet epidemiological data):

50
obliterative portal venopathy:
Age of onset: Variable


Aliases & Descriptions:

hepatoportal sclerosis 50
obliterative portal venopathy 50


External Ids:

ICD10 via Orphanet27 K74.1

Related Diseases for Hepatoportal Sclerosis

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Sources:
18GeneCards, 19GeneDecks
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Graphical network of the top 20 diseases related to Hepatoportal Sclerosis:



Diseases related to hepatoportal sclerosis

Symptoms for Hepatoportal Sclerosis

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Drugs & Therapeutics for Hepatoportal Sclerosis

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

Search CenterWatch for Hepatoportal Sclerosis

Drug clinical trials:

Search ClinicalTrials for Hepatoportal Sclerosis

Search NIH Clinical Center for Hepatoportal Sclerosis

Search CenterWatch for Hepatoportal Sclerosis

Genetic Tests for Hepatoportal Sclerosis

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Anatomical Context for Hepatoportal Sclerosis

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Sources:
34MalaCards
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MalaCards organs/tissues related to Hepatoportal Sclerosis:

34
Liver, Endothelial

Animal Models for Hepatoportal Sclerosis or affiliated genes

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Sources:
38MGI
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MGI Mouse Phenotypes related to Hepatoportal Sclerosis:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053768.5ACE, ELN, F2
2MP:00053858.2ACE, ELN, F2

Publications for Hepatoportal Sclerosis

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53PubMed
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Articles related to Hepatoportal Sclerosis:

(show all 28)
idTitleAuthorsYear
1
Liver failure and the need for transplantation in 6 patients with hepatoportal sclerosis. (23769069)
2013
2
Hepatoportal sclerosis in childhood: descriptive analysis of 12 patients. (24133357)
2013
3
Hepatoportal sclerosis (obliterative portal venopathy) and nodular regenerative hyperplasia in a patient with myasthenia gravis: A case report and review of the published work. (23675894)
2013
4
Mercaptopurine-induced hepatoportal sclerosis in a patient with Crohn's disease. (22841133)
2013
5
Hepatoportal sclerosis clinical different evolutionary stages: presentation of 3 cases and literature review. (22372805)
2012
6
Induction of elastin expression in vascular endothelial cells relates to hepatoportal sclerosis in idiopathic portal hypertension: possible link to serum anti-endothelial cell antibodies. (22288597)
2012
7
Hepatoportal sclerosis: CT and MRI appearance with histopathologic correlation. (22268180)
2012
8
Oxaliplatin-induced hepatoportal sclerosis, portal hypertension, and variceal bleeding successfully treated with transjugular intrahepatic portosystemic shunt. (22537609)
2012
9
The involvement of nitric oxide in the physiopathology of hepatoportal sclerosis. (22820432)
2012
10
Hepatoportal sclerosis in childhood: some presenting with cholestatic features (a re-evaluation of 12 children). (22150463)
2012
11
Elevated levels of circulating angiotensin converting enzyme in patients with hepatoportal sclerosis. (21290180)
2011
12
Hepatoportal sclerosis and extrahepatic portal venous obstruction associated with anti-phospholipid antibody syndrome in child. (20675717)
2011
13
The development of hepatoportal sclerosis and portal hypertension due to didanosine use in HIV. (21057809)
2011
14
Variceal bleeding in an adolescent with HIV diagnosed with hepatoportal sclerosis and nodular regenerative hyperplasia. (19841596)
2010
15
Liver failure and the need for transplantation in three patients with hepatoportal sclerosis. (19100429)
2008
16
Response to Schiano et al. Hepatoportal sclerosis as a cause of noncirrhotic portal hypertension in patients with HIV. (18341510)
2008
17
Hepatoportal sclerosis: a cause of portal hypertension to bear in mind. (18279649)
2008
18
Hepatoportal sclerosis as a cause of noncirrhotic portal hypertension in patients with HIV. (17640321)
2007
19
Liver failure and need for liver transplantation in patients with advanced hepatoportal sclerosis. (17414109)
2007
20
Clinical features, serum interleukin-6, and interferon-gamma levels of 34 turkish patients with hepatoportal sclerosis. (17404864)
2007
21
Association of Adams-Oliver syndrome and hepatoportal sclerosis: an additional case. (16532468)
2006
22
Adams-Oliver syndrome and hepatoportal sclerosis: occasional association or common mechanism? (15832360)
2005
23
Hepatoportal sclerosis in a child. (15316776)
2004
24
Hepatoportal sclerosis. (8578318)
1995
25
Hepatoportal sclerosis demonstrated by abdominal ultrasonography. (1658085)
1991
26
Hepatoportal sclerosis in childhood: a mimic of extrahepatic portal vein obstruction. (7252729)
1981
27
Hepatoportal sclerosis--its relationship to intrahepatic portal venous thrombosis. (429020)
1979
28
Extra- and intrahepatic portal hypertension without cirrhosis (hepatoportal sclerosis). (5833586)
1965

Variations for Hepatoportal Sclerosis

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Expression for genes affiliated with Hepatoportal Sclerosis

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Sources:
2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Hepatoportal Sclerosis

Search GEO for disease gene expression data for Hepatoportal Sclerosis.

Pathways for genes affiliated with Hepatoportal Sclerosis

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Sources:
51PathCards, 54QIAGEN
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Pathways related to Hepatoportal Sclerosis according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.1ACE, F2
2
Show member pathways
9.0ELN, F2

Compounds for genes affiliated with Hepatoportal Sclerosis

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Sources:
46Novoseek, 52PharmGKB, 12DrugBank, 30IUPHAR, 25HMDB, 62Tocris Bioscience
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Compounds related to Hepatoportal Sclerosis according to GeneCards/GeneDecks:

(show all 22)
idCompoundScoreTop Affiliating Genes
1hydralazine46 52 1211.7ACE, ELN
2ang ii469.6ACE, ELN
3losartan46 52 30 1212.6ELN, ACE
4nifedipine46 30 52 1212.5ELN, ACE
5amiodarone46 52 30 1212.5F2, ACE
6diltiazem46 30 52 1212.5F2, ACE
7clopidogrel46 52 25 1212.5ACE, F2
8digoxin46 62 52 25 1213.4F2, ACE
9warfarin46 52 25 1212.4F2, ACE
10kininogen469.4ACE, F2
11dermatan sulfate469.3ELN, F2
12polyethylene glycol469.3ELN, F2
13suramin46 30 1211.3ELN, F2
14aspirin46 52 30 2512.2F2, ACE
15hyaluronic acid46 2510.2ELN, F2
16phosphatidylcholine469.2ELN, F2
17n acetylcysteine469.1ELN, F2
18fibrinogen469.0ELN, F2
19heparin46 30 25 1212.0ELN, F2
20lactate468.9F2, ELN
21creatinine468.9ACE, ELN, F2
22aspartate468.7ELN, F2

GO Terms for genes affiliated with Hepatoportal Sclerosis

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Sources:
17Gene Ontology
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Cellular components related to Hepatoportal Sclerosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:0055768.5ACE, ELN, F2

Biological processes related to Hepatoportal Sclerosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cellular protein metabolic processGO:0442679.1ACE, F2
2blood vessel remodelingGO:0019749.0ACE, ELN

Products for genes affiliated with Hepatoportal Sclerosis

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Hepatoportal Sclerosis

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet