MCID: HPT066
MIFTS: 26

Hepatoportal Sclerosis

Categories: Liver diseases, Rare diseases

Aliases & Classifications for Hepatoportal Sclerosis

MalaCards integrated aliases for Hepatoportal Sclerosis:

Name: Hepatoportal Sclerosis 59
Obliterative Portal Venopathy 59

Characteristics:

Orphanet epidemiological data:

59
hepatoportal sclerosis
Age of onset: All ages;

Classifications:

Orphanet: 59  
Rare hepatic diseases


External Ids:

Orphanet 59 ORPHA64743
ICD10 via Orphanet 34 K74.1

Summaries for Hepatoportal Sclerosis

MalaCards based summary : Hepatoportal Sclerosis, also known as obliterative portal venopathy, is related to portal hypertension and infantile liver failure syndrome 1. An important gene associated with Hepatoportal Sclerosis is ACE (Angiotensin I Converting Enzyme). Affiliated tissues include liver, endothelial and heart.

Related Diseases for Hepatoportal Sclerosis

Diseases related to Hepatoportal Sclerosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 72)
# Related Disease Score Top Affiliating Genes
1 portal hypertension 30.6 F2 GPT
2 infantile liver failure syndrome 1 30.4 F2 GPT
3 liver cirrhosis 30.1 F2 GPT
4 liver disease 29.9 F2 GPT
5 cystic fibrosis 10.4
6 castleman disease 10.2
7 poems syndrome 10.2
8 protein s deficiency 10.2
9 biliary tract disease 10.2
10 splenomegaly 10.2
11 adams-oliver syndrome 1 10.2
12 oliver syndrome 10.2
13 adams-oliver syndrome 10.2
14 nodular regenerative hyperplasia 10.2
15 hepatic tuberculosis 10.0 F2 GPT
16 kwashiorkor 10.0 F2 GPT
17 hepatic coma 10.0 F2 GPT
18 hepatitis d 10.0 F2 GPT
19 hepatitis 10.0 F2 GPT
20 nonalcoholic steatohepatitis 10.0 F2 GPT
21 hemorrhagic fever 10.0 F2 GPT
22 hepatorenal syndrome 10.0 ACE F2
23 choledocholithiasis 10.0 F2 GPT
24 retinal artery occlusion 10.0 ACE F2
25 analbuminemia 10.0 F2 GPT
26 arteritic anterior ischemic optic neuropathy 10.0 ACE F2
27 ischemic optic neuropathy 10.0 ACE F2
28 acute liver failure 10.0 F2 GPT
29 hepatitis e 10.0 F2 GPT
30 intermittent claudication 10.0 ACE F2
31 nonarteritic anterior ischemic optic neuropathy 10.0 ACE F2
32 heart conduction disease 10.0 ACE F2
33 bile duct disease 10.0 F2 GPT
34 hepatitis a 10.0 F2 GPT
35 cholecystitis 10.0 F2 GPT
36 bilirubin metabolic disorder 10.0 F2 GPT
37 obstructive jaundice 10.0 F2 GPT
38 pulmonary edema 10.0 ACE F2
39 thrombophilia due to thrombin defect 10.0
40 myasthenia gravis 10.0
41 crohn's disease 10.0
42 thrombosis 10.0
43 adenoma 10.0
44 banti's syndrome 10.0
45 myasthenia gravis congenital 10.0
46 hepatic encephalopathy 10.0 F2 GPT
47 alcoholic hepatitis 10.0 F2 GPT
48 wilson disease 10.0 F2 GPT
49 peripheral vascular disease 10.0 ACE F2
50 migraine with aura 10.0 ACE F2

Graphical network of the top 20 diseases related to Hepatoportal Sclerosis:



Diseases related to Hepatoportal Sclerosis

Symptoms & Phenotypes for Hepatoportal Sclerosis

Drugs & Therapeutics for Hepatoportal Sclerosis

Search Clinical Trials , NIH Clinical Center for Hepatoportal Sclerosis

Genetic Tests for Hepatoportal Sclerosis

Anatomical Context for Hepatoportal Sclerosis

MalaCards organs/tissues related to Hepatoportal Sclerosis:

41
Liver, Endothelial, Heart

Publications for Hepatoportal Sclerosis

Articles related to Hepatoportal Sclerosis:

(show all 50)
# Title Authors Year
1
Hepatobiliary and Pancreatic: Obliterative portal venopathy found in Castleman disease variant of POEMS syndrome. ( 29691905 )
2018
2
Obliterative Portal Venopathy without Cirrhosis is Prevalent in Pediatric Cystic Fibrosis Liver Disease with Portal Hypertension. ( 30404037 )
2018
3
Mutations in the novel gene FOPV are associated with familial autosomal dominant and non-familial obliterative portal venopathy. ( 28792652 )
2018
4
Obliterative portal venopathy: A histopathologic finding associated with chronic antibody-mediated rejection in pediatric liver allografts. ( 29363222 )
2018
5
Liver transplantation in adult cystic fibrosis: Clinical, imaging, and pathological evidence of obliterative portal venopathy. ( 28816015 )
2017
6
A Hepatoportal sclerosis related to the use of herbals and nutritional supplements. Causality or coincidence? ( 27740529 )
2016
7
Progression of Hepatic Adenoma to Carcinoma in the Setting of Hepatoportal Sclerosis in HIV Patient: Case Report and Review of the Literature. ( 27812395 )
2016
8
Obliterative portal venopathy without portal hypertension: an underestimated condition. ( 26264219 )
2016
9
Impact of Obliterative Portal Venopathy Associated With Human Immunodeficiency Virus. ( 26986141 )
2016
10
Multimodality imaging of obliterative portal venopathy: what every radiologist should know. ( 25514699 )
2015
11
An association of myeloproliferative neoplasms and obliterative portal venopathy. ( 24474573 )
2014
12
Obliterative portal venopathy: a study of 48 children. ( 24768253 )
2014
13
Mercaptopurine-induced hepatoportal sclerosis in a patient with Crohn's disease. ( 22841133 )
2013
14
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
15
Hepatoportal sclerosis in childhood: descriptive analysis of 12 patients. ( 24133357 )
2013
16
Liver failure and the need for transplantation in 6 patients with hepatoportal sclerosis. ( 23769069 )
2013
17
Obliterative portal venopathy: a clinical and histopathological review. ( 23812828 )
2013
18
Oxaliplatin-induced hepatoportal sclerosis, portal hypertension, and variceal bleeding successfully treated with transjugular intrahepatic portosystemic shunt. ( 22537609 )
2012
19
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
20
Hepatoportal sclerosis: CT and MRI appearance with histopathologic correlation. ( 22268180 )
2012
21
Hepatoportal sclerosis clinical different evolutionary stages: presentation of 3 cases and literature review. ( 22372805 )
2012
22
The involvement of nitric oxide in the physiopathology of hepatoportal sclerosis. ( 22820432 )
2012
23
Hepatoportal sclerosis in childhood: some presenting with cholestatic features (a re-evaluation of 12 children). ( 22150463 )
2012
24
Obliterative portal venopathy: findings at CT imaging. ( 22474672 )
2012
25
Case 183: Obliterative portal venopathy. ( 22723566 )
2012
26
Hepatoportal sclerosis and extrahepatic portal venous obstruction associated with anti-phospholipid antibody syndrome in child. ( 20675717 )
2011
27
The development of hepatoportal sclerosis and portal hypertension due to didanosine use in HIV. ( 21057809 )
2011
28
Elevated levels of circulating angiotensin converting enzyme in patients with hepatoportal sclerosis. ( 21290180 )
2011
29
Obliterative portal venopathy: portal hypertension is not always present at diagnosis. ( 21087805 )
2011
30
Variceal bleeding in an adolescent with HIV diagnosed with hepatoportal sclerosis and nodular regenerative hyperplasia. ( 19841596 )
2010
31
Acquired protein S deficiency leads to obliterative portal venopathy and to compensatory nodular regenerative hyperplasia in HIV-infected patients. ( 19512859 )
2009
32
Response to Schiano et al. Hepatoportal sclerosis as a cause of noncirrhotic portal hypertension in patients with HIV. ( 18341510 )
2008
33
Hepatoportal sclerosis: a cause of portal hypertension to bear in mind. ( 18279649 )
2008
34
Liver failure and the need for transplantation in three patients with hepatoportal sclerosis. ( 19100429 )
2008
35
Liver failure and need for liver transplantation in patients with advanced hepatoportal sclerosis. ( 17414109 )
2007
36
Hepatoportal sclerosis as a cause of noncirrhotic portal hypertension in patients with HIV. ( 17640321 )
2007
37
Clinical features, serum interleukin-6, and interferon-gamma levels of 34 turkish patients with hepatoportal sclerosis. ( 17404864 )
2007
38
Liver transplantation in precirrhotic biliary tract disease: Portal hypertension is frequently associated with nodular regenerative hyperplasia and obliterative portal venopathy. ( 17063088 )
2006
39
Association of Adams-Oliver syndrome and hepatoportal sclerosis: an additional case. ( 16532468 )
2006
40
Adams-Oliver syndrome and hepatoportal sclerosis: occasional association or common mechanism? ( 15832360 )
2005
41
Hepatoportal sclerosis in a child. ( 15316776 )
2004
42
Hepatoportal sclerosis. ( 8578318 )
1995
43
Hepatoportal sclerosis demonstrated by abdominal ultrasonography. ( 1658085 )
1991
44
Hepatoportal sclerosis in childhood. ( 2421592 )
1986
45
Hepatoportal sclerosis in childhood: a mimic of extrahepatic portal vein obstruction. ( 7252729 )
1981
46
Nodular regenerative hyperplasia of the liver in hematologic disorders: a possible response to obliterative portal venopathy. A morphometric study of nine cases with an hypothesis on the pathogenesis. ( 7432153 )
1980
47
Hepatoportal sclerosis--its relationship to intrahepatic portal venous thrombosis. ( 429020 )
1979
48
Obliterative portal venopathy of the liver. Associated with so-called idiopathic portal hypertension or tropical splenomegaly. ( 5766763 )
1969
49
Obliterative portal venopathy of the liver and "idiopathic portal hypertension". ( 5809690 )
1969
50
Extra- and intrahepatic portal hypertension without cirrhosis (hepatoportal sclerosis). ( 5833586 )
1965

Variations for Hepatoportal Sclerosis

Expression for Hepatoportal Sclerosis

Search GEO for disease gene expression data for Hepatoportal Sclerosis.

Pathways for Hepatoportal Sclerosis

GO Terms for Hepatoportal Sclerosis

Sources for Hepatoportal Sclerosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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