MCID: HPT066
MIFTS: 20

Hepatoportal Sclerosis

Categories: Liver diseases, Rare diseases

Aliases & Classifications for Hepatoportal Sclerosis

MalaCards integrated aliases for Hepatoportal Sclerosis:

Name: Hepatoportal Sclerosis 58
Obliterative Portal Venopathy 58

Characteristics:

Orphanet epidemiological data:

58
hepatoportal sclerosis
Age of onset: All ages;

Classifications:

Orphanet: 58  
Rare hepatic diseases


External Ids:

ICD10 via Orphanet 33 K74.1
Orphanet 58 ORPHA64743

Summaries for Hepatoportal Sclerosis

MalaCards based summary : Hepatoportal Sclerosis, also known as obliterative portal venopathy, is related to varicose veins and esophageal varix. An important gene associated with Hepatoportal Sclerosis is ACE (Angiotensin I Converting Enzyme). Affiliated tissues include liver and endothelial.

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 208)
# Related Disease Score Top Affiliating Genes
1 varicose veins 30.3 F2 ELN
2 esophageal varix 30.3 GPT F2
3 ascending cholangitis 30.1 GPT F2
4 biliary atresia 30.1 GPT F2
5 portal hypertension 30.1 GPT F2 ELN ACE
6 sclerosing cholangitis 30.0 GPT F2
7 hypersplenism 30.0 GPT F2
8 cholangitis, primary sclerosing 30.0 GPT F2
9 polycythemia 29.9 F2 ACE
10 autoimmune hepatitis 29.9 GPT F2
11 biliary tract disease 29.9 GPT F2
12 cholangitis 29.8 GPT F2
13 hepatic coma 29.8 GPT F2
14 hepatic encephalopathy 29.8 GPT F2
15 liver cirrhosis 29.7 GPT F2 ACE
16 vascular disease 29.3 F2 ELN ACE
17 splenomegaly 10.4
18 fibrosis of extraocular muscles, congenital, 1 10.4
19 cystic fibrosis 10.3
20 protein s deficiency 10.3
21 thrombophilia 10.3
22 portal vein thrombosis 10.3
23 nodular regenerative hyperplasia 10.3
24 incomplete septal cirrhosis 10.3
25 liver disease 10.2
26 banti's syndrome 10.2
27 hepatocellular carcinoma 10.2
28 immune deficiency disease 10.2
29 polycythemia vera 10.2
30 cholangiocarcinoma 10.2
31 autosomal recessive disease 10.2
32 autoimmune cholangitis 10.2
33 castleman disease 10.2
34 bacterial infectious disease 10.2
35 protein-losing enteropathy 10.2
36 primary biliary cholangitis 10.2
37 poems syndrome 10.2
38 thrombocytopenia 10.2
39 thrombocytosis 10.2
40 iron metabolism disease 10.2
41 intrahepatic cholangiocarcinoma 10.2
42 47,xyy 10.2
43 48,xyyy 10.2
44 congenital hepatic fibrosis 10.2
45 secondary sclerosing cholangitis 10.2
46 oliver syndrome 10.1
47 adams-oliver syndrome 10.1
48 cholestasis 10.1
49 cryptogenic cirrhosis 10.1
50 pancreas lymphoma 10.1 GPT ACE

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:

40
Liver, Endothelial

Publications for Hepatoportal Sclerosis

Articles related to Hepatoportal Sclerosis:

(show top 50) (show all 132)
# Title Authors PMID Year
1
Key histopathologic features in idiopathic noncirrhotic portal hypertension: an interobserver agreement study and proposal for diagnostic criteria. 61
32958831 2021
2
Hepatocellular carcinoma in obliterative portal venopathy. 61
33189587 2021
3
Interobserver study on histologic features of idiopathic non-cirrhotic portal hypertension. 61
33097074 2020
4
Living donor liver transplantation for idiopathic portal hypertension with extrahepatic portal vein stenosis and splenic artery aneurysms: a case report and review of the literature. 61
33121468 2020
5
Biliary Atresia Patients With Successful Kasai Portoenterostomy Can Present With Features of Obliterative Portal Venopathy. 61
32187144 2020
6
Aberrant von Willebrand factor expression of sinusoidal endothelial cells and quiescence of hepatic stellate cells in nodular regenerative hyperplasia and obliterative portal venopathy. 61
31994248 2020
7
Progressive Splenomegaly and Hypersplenism: An Unusual Case of Splenic Vein Stenosis with Histologic Findings of Hepatoportal Sclerosis. 61
31870607 2020
8
Obliterative Portal Venopathy Without Cirrhosis Is Prevalent in Pediatric Cystic Fibrosis Liver Disease With Portal Hypertension. 61
30404037 2019
9
Portal Cavernoma Cholangiopathy: Histologic Features and Differential Diagnosis. 61
30357333 2019
10
The Implications of Liver Biopsy Results in Patients with Myeloproliferative Neoplasms Being Treated with Ruxolitinib. 61
30723558 2019
11
CONGENITAL HEPATIC FIBROSIS AND OBLITERATIVE PORTAL VENOPATHY WITHOUT PORTAL HYPERTENSION - A REVIEW OF LITERATURE BASED ON AN ASYMPTOMATIC CASE. 61
30785513 2018
12
Non-cirrhotic portal hypertension: an imaging review. 61
29564495 2018
13
Portal Vein Thrombosis: Imaging the Spectrum of Disease With an Emphasis on MRI Features. 61
29792748 2018
14
Hepatobiliary and Pancreatic: Obliterative portal venopathy found in Castleman disease variant of POEMS syndrome. 61
29691905 2018
15
Hepatic morphology abnormalities: beyond cirrhosis. 61
29043403 2018
16
Ten-year experience of transjugular intrahepatic portosystemic shunt for noncirrhotic portal hypertension. 61
29324586 2018
17
Spectrum of histopathological changes in patients with non-cirrhotic portal fibrosis. 61
29651703 2018
18
Obliterative portal venopathy: A histopathologic finding associated with chronic antibody-mediated rejection in pediatric liver allografts. 61
29363222 2018
19
Mutations in the novel gene FOPV are associated with familial autosomal dominant and non-familial obliterative portal venopathy. 61
28792652 2018
20
Liver transplantation in adult cystic fibrosis: Clinical, imaging, and pathological evidence of obliterative portal venopathy. 61
28816015 2017
21
Prevalence of histological features of idiopathic noncirrhotic portal hypertension in general population: a retrospective study of incidental liver biopsies. 61
28597108 2017
22
POEMS syndrome and idiopathic portal hypertension: a possible association. 61
28247771 2017
23
Adams-Oliver syndrome review of the literature: Refining the diagnostic phenotype. 61
28160419 2017
24
Nodular Regenerative Hyperplasia in HIV-positive patients : a case series and review of the literature. 61
29364092 2017
25
Liver transplantation for a patient with Turner syndrome presenting severe portal hypertension: a case report and literature review. 61
27358061 2016
26
 Hepatoportal sclerosis related to the use of herbals and nutritional supplements. Causality or coincidence? 61
27740529 2016
27
Impact of Obliterative Portal Venopathy Associated With Human Immunodeficiency Virus. 61
26986141 2016
28
Obliterative portal venopathy without portal hypertension: an underestimated condition. 61
26264219 2016
29
Idiopathic Noncirrhotic Portal Hypertension: An Appraisal. 61
26563701 2016
30
Idiopathic noncirrhotic portal hypertension: current perspectives. 61
27555800 2016
31
Progression of Hepatic Adenoma to Carcinoma in the Setting of Hepatoportal Sclerosis in HIV Patient: Case Report and Review of the Literature. 61
27812395 2016
32
Hepatocellular nodules expressing markers of hepatocellular adenomas in Budd-Chiari syndrome and other rare hepatic vascular disorders. 61
26119687 2015
33
[Vascular liver diseases]. 61
26298892 2015
34
Multimodality imaging of obliterative portal venopathy: what every radiologist should know. 61
25514699 2015
35
Hepatic proliferation and angiogenesis markers are increased after portal deprivation in rats: a study of molecular, histological and radiological changes. 61
26020934 2015
36
An association of myeloproliferative neoplasms and obliterative portal venopathy. 61
24474573 2014
37
Obliterative portal venopathy: a study of 48 children. 61
24768253 2014
38
Chronic immune-mediated reaction syndrome as the cause of late graft mortality in living-donor liver transplantation for primary biliary cirrhosis. 61
24836838 2014
39
Obliterative portal venopathy: a clinical and histopathological review. 61
23812828 2013
40
Hepatoportal sclerosis in childhood: descriptive analysis of 12 patients. 61
24133357 2013
41
Hepatoportal sclerosis (obliterative portal venopathy) and nodular regenerative hyperplasia in a patient with myasthenia gravis: A case report and review of the published work. 61
23675894 2013
42
Mercaptopurine-induced hepatoportal sclerosis in a patient with Crohn's disease. 61
22841133 2013
43
Liver failure and the need for transplantation in 6 patients with hepatoportal sclerosis. 61
23769069 2013
44
An unusual cause of oesophageal variceal bleeding in a Chinese human immunodeficiency virus-infected patient. 61
23378360 2013
45
Role of endothelial-mesenchymal transition in idiopathic portal hypertension. 61
23275299 2013
46
The involvement of nitric oxide in the physiopathology of hepatoportal sclerosis. 61
22820432 2012
47
Oxaliplatin-induced hepatoportal sclerosis, portal hypertension, and variceal bleeding successfully treated with transjugular intrahepatic portosystemic shunt. 61
22537609 2012
48
Case 183: Obliterative portal venopathy. 61
22723566 2012
49
Obliterative portal venopathy: findings at CT imaging. 61
22474672 2012
50
Hepatoportal sclerosis in childhood: some presenting with cholestatic features (a re-evaluation of 12 children). 61
22150463 2012

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 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
Content
Loading form....