MCID: HPT066
MIFTS: 32

Hepatoportal Sclerosis malady

Liver diseases, Rare diseases categories
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Summaries for Hepatoportal Sclerosis

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MalaCards based summary: 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:
48Orphanet, 26ICD10 via Orphanet
See all sources

Hepatoportal Sclerosis, Aliases & Descriptions:

Name: Hepatoportal Sclerosis 48
 
Obliterative Portal Venopathy 48


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Liver diseases
Orphanet: 48 
Rare hepatic diseases


Characteristics (Orphanet epidemiological data):

48
obliterative portal venopathy:
Age of onset: Variable


External Ids:

ICD10 via Orphanet26 K74.1

Related Diseases for Hepatoportal Sclerosis

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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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Drug clinical trials:

Search ClinicalTrials for Hepatoportal Sclerosis

Search NIH Clinical Center for Hepatoportal Sclerosis

Genetic Tests for Hepatoportal Sclerosis

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Anatomical Context for Hepatoportal Sclerosis

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MalaCards organs/tissues related to Hepatoportal Sclerosis:

32
Liver, Endothelial

Animal Models for Hepatoportal Sclerosis or affiliated genes

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MGI Mouse Phenotypes related to Hepatoportal Sclerosis:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053768.5ACE, ELN, F2
2MP:00053858.2ACE, ELN, F2

Publications for Hepatoportal Sclerosis

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Articles related to Hepatoportal Sclerosis:

(show all 29)
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. (2421592)
1986
27
Hepatoportal sclerosis in childhood: a mimic of extrahepatic portal vein obstruction. (7252729)
1981
28
Hepatoportal sclerosis--its relationship to intrahepatic portal venous thrombosis. (429020)
1979
29
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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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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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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Compounds related to Hepatoportal Sclerosis according to GeneCards/GeneDecks:

(show all 22)
idCompoundScoreTop Affiliating Genes
1hydralazine44 50 1111.7ACE, ELN
2ang ii449.6ACE, ELN
3losartan44 50 28 1112.6ELN, ACE
4nifedipine44 28 50 1112.5ELN, ACE
5amiodarone44 50 28 1112.5F2, ACE
6diltiazem44 28 50 1112.5F2, ACE
7clopidogrel44 50 24 1112.5ACE, F2
8digoxin44 61 50 24 1113.4F2, ACE
9warfarin44 50 24 1112.4F2, ACE
10kininogen449.4ACE, F2
11dermatan sulfate449.3ELN, F2
12polyethylene glycol449.3ELN, F2
13suramin44 28 1111.3ELN, F2
14aspirin44 50 28 2412.2F2, ACE
15hyaluronic acid44 2410.2ELN, F2
16phosphatidylcholine449.2ELN, F2
17n acetylcysteine449.1ELN, F2
18fibrinogen449.0ELN, F2
19heparin44 28 24 1112.0ELN, F2
20lactate448.9F2, ELN
21creatinine448.9ACE, ELN, F2
22aspartate448.7ELN, F2

GO Terms for genes affiliated with Hepatoportal Sclerosis

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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

Sources for Hepatoportal Sclerosis

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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