MCID: HPT002
MIFTS: 59

Hepatic Vein Thrombosis malady

Cardiovascular, Gastrointestinal, Liver, Blood categories

Summaries for Hepatic Vein Thrombosis

Sources:
43NIH Rare Diseases, 64Wikipedia, 47OMIM, 33MalaCards
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NIH Rare Diseases:43 Budd-chiari syndrome is a rare disorder characterized by narrowing and obstruction of the veins of the liver. this narrowing or obstruction slows or prevents blood from flowing out of the liver and back to the heart which can lead to liver damage. while some people experience no symptoms, many experience fatigue, abdominal pain, nausea, and jaundice. other associated findings include an accumulation of fluid in the abdomen (ascites), an enlarged spleen and/or liver, and severe bleeding in the esophagus. the severity of the disorder varies from case to case, depending on the site and number of affected veins. drugs may be used to dissolve or decrease the size of the obstruction (if it is a clot). in some cases surgery is performed. in most cases, the cause of budd-chiari syndrome is unknown. last updated: 10/30/2010

MalaCards: Hepatic Vein Thrombosis, also known as budd-chiari syndrome, is related to antiphospholipid syndrome and protein s deficiency, and has symptoms including ascitis, portal hypertension and splenomegaly. An important gene associated with Hepatic Vein Thrombosis is F5 (coagulation factor V (proaccelerin, labile factor)), and among its related pathways are Post-translational modification: synthesis of GPI-anchored proteins and Common Pathway. The drugs antithrombin iii, human and antithrombin iii,recombinant and the compounds aspartate and gamma-carboxyglutamic acid have been mentioned in the context of this disorder. Affiliated tissues include lymph node, spleen and heart, and related mouse phenotype integument.

Wikipedia:64 Budd–Chiari syndrome is the clinical picture caused by occlusion of the hepatic veins. It presents... more...

Description from OMIM:47 600880

Aliases & Classifications for Hepatic Vein Thrombosis

Sources:
8Disease Ontology, 9diseasecard, 43NIH Rare Diseases, 22GTR, 47OMIM, 10DISEASES, 45Novoseek, 49Orphanet, 61UMLS, 57SNOMED-CT, 35MeSH, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet, 25ICD10
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Cardiovascular, Gastrointestinal, Liver, Blood


Characteristics (Orphanet epidemiological data):

49
budd-chiari syndrome:
Inheritance: Multigenic/multifactorial; Prevalence: 1-9/100000; Age of onset: Variable; Age of death: Any age
hepatic veno-occlusive disease:
Inheritance: Sporadic; Prevalence: 1-5/10000; Age of onset: Variable; Age of death: Any age


Aliases & Descriptions:

hepatic vein thrombosis 8 10 61
budd-chiari syndrome 8 9 43 47 45 49 61
hepatic veno-occlusive disease 43 45 49 61
budd - chiari syndrome 8 22
sinusoidal obstruction syndrome 49
hepatic vein obstruction 43


External Ids:

Disease Ontology8 DOID:11512
MeSH35 D006502
OMIM47 600880
MESH via Orphanet36 D006502, D006504
SNOMED-CT57 38739001, 195436007
ICD10 via Orphanet26 I82.0, K76.5
SNOMED-CT via Orphanet58 82385007, 65617004
UMLS via Orphanet62 C0856761, C0019156
ICD1025 I82.0, K76.5

Related Diseases for Hepatic Vein Thrombosis

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases related to Hepatic Vein Thrombosis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 140)
idRelated DiseaseScoreTop Affiliating Genes
1antiphospholipid syndrome31.0F5, MTHFR, PROS1
2protein s deficiency30.9F5, PIGA
3protein c deficiency30.9SERPINE1, PROS1, MTHFR, F5
4portal vein thrombosis30.7F5, MTHFR, PROS1
5thrombocytosis30.6JAK2
6patent foramen ovale30.3F5
7sagittal sinus thrombosis30.3F5, PROS1
8thrombophilia30.2SERPINE1, PROS1, MTHFR, F5
9hyperhomocysteinemia30.2F5, MTHFR, PROS1, SERPINE1
10thrombocytopenia30.2F5, PIGA
11polycythemia vera30.2JAK2
12venous thrombosis30.1JAK2, F5, MTHFR, PROS1, SERPINE1
13hepatic veno-occlusive disease with immunodeficiency10.8
14hematopoietic stem cell transplantation10.6
15vein disease10.5
16hepatopulmonary syndrome10.5
17young syndrome10.5
18ulcerative colitis10.4
19familial mediterranean fever10.4
20essential thrombocythemia10.4
21nodular regenerative hyperplasia10.4
22n syndrome10.4
23acute leukemia10.4
24myeloproliferative disorder10.4
25relapsing polychondritis10.3
26alveolar echinococcosis10.3
27intravenous leiomyomatosis10.3
28polyarteritis nodosa10.3
29alcoholic liver cirrhosis10.3
30hemangioendothelioma10.3
31f5-related budd-chiari syndrome10.3
32jak2-related budd-chiari syndrome10.3
33epithelioid hemangioendothelioma10.3
34hemoglobinuria10.3
35acute promyelocytic leukemia10.3
36paroxysmal nocturnal hemoglobinuria10.3
37graft versus host disease10.3
38hemochromatosis10.3
39esophageal varix10.3
40central pontine myelinolysis10.3
41agammaglobulinemia10.3
42bone development disease10.3
43acute graft versus host disease10.3
44primary agammaglobulinemia10.3
45acute liver failure10.2
46thrombophlebitis migrans10.2
47erythropoietin polycythemia10.2
48hepatic tuberculosis10.2
49diabetic ketoacidosis10.2
50hepatitis c10.2

Graphical network of the top 20 diseases related to Hepatic Vein Thrombosis:



Diseases related to hepatic vein thrombosis

Clinical Features for Hepatic Vein Thrombosis

Sources:
47OMIM, 49Orphanet
See all sources

Clinical features from OMIM:

600880

Clinical synopsis from OMIM:

600880

Symptoms:

49 (show all 20)
  • ascitis
  • portal hypertension
  • splenomegaly
  • esophageal varices
  • acute abdominal pain/colic
  • cirrhosis
  • hepatocellular liver disease/hepatic failure
  • hepatomegaly/liver enlargement (excluding storage disease)
  • abnormal hepatic enzymes/transaminases
  • fever/chilling
  • malabsorption/chronic diarrhea/steatorrhea
  • gastrointestinal bleeding/hemorrhage/hematemesis/melena/rectorrhagia
  • intestinal obstruction/ileus
  • peritonitis/peritoneal abscess
  • biliary/gallbladder stones/lithiasis/cholecystitis
  • hepatitis/icterus/cholestasis
  • acute hepatic failure
  • acute ischemic syndrome
  • mesenteric/intestinal infarction
  • weight loss/loss of appetite/break in weight curve/general health alteration

Drugs & Therapeutics for Hepatic Vein Thrombosis

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

Approved drugs:

Search CenterWatch for Hepatic Vein Thrombosis

Drug clinical trials:

Search ClinicalTrials for Hepatic Vein Thrombosis

Search NIH Clinical Center for Hepatic Vein Thrombosis

Search CenterWatch for Hepatic Vein Thrombosis

Inferred drug relations via UMLS61/NDF-RT41:

Show with subtypes

Genetic Tests for Hepatic Vein Thrombosis

Sources:
22GTR
See all sources

Genetic tests related to Hepatic Vein Thrombosis:

id Genetic test Affiliating Genes
1 Budd-chiari Syndrome22

Anatomical Context for Hepatic Vein Thrombosis

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Hepatic Vein Thrombosis:

33
Lymph node, Spleen, Heart, Liver

Animal Models for Hepatic Vein Thrombosis or affiliated genes

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

MGI Mouse Phenotypes related to Hepatic Vein Thrombosis:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00107717.4SERPINE1, PROS1, MTHFR, F5, PIGA, JAK2

Publications for Hepatic Vein Thrombosis

Sources:
51PubMed
See all sources

Articles related to Hepatic Vein Thrombosis:

(show top 50)    (show all 103)
idTitleAuthorsYear
1
Does decreased fibrinolysis have a role to play in the development of non-neoplastic portal vein thrombosis in patients with hepatic cirrhosis? (23504244)
2013
2
Using ALPPS to induce rapid liver hypertrophy in a patient with hepatic fibrosis and portal vein thrombosis. (22996934)
2013
3
Splenectomy in chronic hepatic disorders: portal vein thrombosis and improvement of liver function. (21293126)
2011
4
VKORC1 and CYP2C9 genetic polymorphisms in hepatic or portal vein thrombosis. (20434758)
2010
5
Chronic portal vein thrombosis: transcapsular hepatic collateral vessels and communicating ectopic varices. (20829527)
2010
6
Treatment of hepatocellular carcinoma with portal vein thrombosis by sorafenib combined with hepatic arterial infusion chemotherapy. (20981227)
2010
7
Late acute celiac and hepatic artery thrombosis with portal vein thrombosis resulting in hepatic infarction 12 years post orthotopic liver transplantation. (20009144)
2009
8
The JAK2(V617F) tyrosine kinase mutation identifies clinically latent myeloproliferative disorders in patients presenting with hepatic or portal vein thrombosis. (19046316)
2008
9
Hepatic vein thrombosis as a complication of ulcerative colitis in a 12-year-old patient. (17372822)
2007
10
Infliximab in the treatment of hepatic vein thrombosis (Budd-Chiari syndrome) in three patients with Behcet's syndrome. (17478465)
2007
11
Tumor mimicking hepatic echinococcus alveolaris with portal vein thrombosis. (17917334)
2007
12
Hepatic arterial infusion chemotherapy with epirubicin in patients with advanced hepatocellular carcinoma and portal vein tumor thrombosis. (18097170)
2007
13
Microvascular thrombosis in the hepatic vein of a patient with paroxysmal nocturnal hemoglobinuria. (17988986)
2007
14
Noncirrhotic portal vein thrombosis exhibits neuropsychological and MR changes consistent with minimal hepatic encephalopathy. (16557541)
2006
15
Pivotal role of PAI-1 in a murine model of hepatic vein thrombosis. (16160004)
2006
16
Acute hepatic vein thrombosis after liver transplantation in a child with biliary atresia and absent inferior vena cava. (16797332)
2006
17
Favorable resolution of hepatic infarctions in transplanted liver after portal vein thrombosis treated by surgical thrombectomy: a case report. (17112919)
2006
18
Hemorrhage from ruptured hepatic artery pseudoaneurysm co-occurring with pancreatic head tumor and portal vein thrombosis: surgical and endovascular treatment. (16082320)
2005
19
Macronodular hepatic tuberculosis associated with portal vein thrombosis and portal hypertension. (16026440)
2005
20
Familial high factor VIII level associated with hepatic vein thrombosis. (15319659)
2004
21
Hepatic veno-occlusive disease and portal vein thrombosis; closer than we think? (15571947)
2004
22
Relationship between various patterns of transient increased hepatic attenuation on CT and portal vein thrombosis related to acute cholecystitis. (15269038)
2004
23
Portal vein thrombosis complicating neonatal hepatic abscess. (14581740)
2003
24
Hepatic vein thrombosis (Budd-Chiari syndrome). (11928075)
2002
25
Portal or hepatic vein thrombosis as the first presentation of a myeloproliferative disorder in patients with normal peripheral blood counts. (12181028)
2002
26
Hepatic vein thrombosis with ulcerative colitis. (10918734)
2000
27
Hepatic vein thrombosis in a patient with mutant prothrombin 20210A allele. (9759637)
1998
28
Proposal of a new nomenclature for Budd-Chiari syndrome: hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion. (9794901)
1998
29
Successful treatment of hepatic vein thrombosis following reduced-size liver transplantation. (9838415)
1998
30
Prospective evaluation of the prevalence of factor V Leiden mutation in portal or hepatic vein thrombosis. (9365003)
1997
31
Balloon occlusion portography to diagnose new-onset left hepatic vein thrombosis and widening of an existing Wallstent TIPS by Palmaz stents for recurrent portal hypertension and variceal bleeding. (8781163)
1996
32
Hepatic vein thrombosis in BehAset's disease. (2194924)
1990
33
Thrombotic cutaneous nodules and hepatic vein thrombosis in the anticardiolipin syndrome. (2591097)
1989
34
Hepatic vein thrombosis (Budd-Chiari syndrome). (6693073)
1984
35
Ultrasonography: hepatic vein thrombosis in sickle cell anemia. (7114404)
1982
36
Oral contraceptives and hepatic vein thrombosis. (7205808)
1981
37
Case report: hepatic vein thrombosis (acute Budd-Chiari syndrome) in acute leukemia. (6947690)
1981
38
"Dimple sign" in hepatic vein thrombosis. (465948)
1979
39
Acute hepatic vein thrombosis occurring during therapy for Hodgkin's disease: a case report. (509400)
1979
40
Activation of the Hageman factor-prekallikrein system in the pathogenesis of the generalized Shwartzman reaction and of the hepatic vein thrombosis phenomenon in the rat. (470342)
1979
41
Acute hepatic vein thrombosis in a patient with Behcet's disease. (829359)
1976
42
Hepatic vein thrombosis treated with streptokinase. (4439110)
1974
43
Hepatic vein thrombosis in children in the Near East. (5054003)
1972
44
Hepatic vein thrombosis (Budd-Chiari syndrome). (5445577)
1970
45
Hepatic vein thrombosis. Increased incidence in rats given methylcholanthrene and carbon tetrachloride. (5450122)
1970
46
Severe cirrhosis with hepatic vein thrombosis in rats given carbon tetrachloride and 3-methylcholanthrene. (5711263)
1968
47
Paroxysmal nocturnal haemoglobinuria parturition complicated by presumed hepatic vein thrombosis. (5684424)
1968
48
Hepatic vein thrombosis complicating polycythemia vera. Successful treatment with a portacaval shunt. (6028691)
1967
49
Chiari's disease, hepatic-vein thrombosis. (14941265)
1952
50
Case report of stenosis of the vena cava with vena caval and hepatic vein thrombosis reto trauma. (14933990)
1952

Genetic Variations for Hepatic Vein Thrombosis

Expression for genes affiliated with Hepatic Vein Thrombosis

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

Search GEO for disease gene expression data for Hepatic Vein Thrombosis.

Pathways for genes affiliated with Hepatic Vein Thrombosis

Sources:
54Reactome, 30KEGG, 52QIAGEN, 38NCBI BioSystems Database, 53R&D Systems
See all sources

Compounds for genes affiliated with Hepatic Vein Thrombosis

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

Compounds related to Hepatic Vein Thrombosis according to GeneCards/GeneDecks:

(show all 37)
idCompoundScoreTop Affiliating Genes
1aspartate4510.5JAK2
2gamma-carboxyglutamic acid459.9PROS1, F5
3batroxobin459.9SERPINE1, F5
4ancrod459.9SERPINE1, F5
5tibolone459.8SERPINE1, F5
6cyproteroneacetate459.8F5, SERPINE1
7desmopressin45 60 29 1112.8SERPINE1, F5
85-methyltetrahydrofolate45 1110.7MTHFR, F5
9cystathionine459.7F5, MTHFR
10hirudin459.7SERPINE1, F5
11vitamin-e459.6JAK2, F5, PROS1
12mycophenolate mofetil45 50 1111.5F5, SERPINE1
13vitamin b6459.5MTHFR, F5
14raloxifene45 29 50 1112.5SERPINE1, F5
15endotoxin459.5SERPINE1, F5, JAK2
16progestins459.5F5, JAK2
17phospholipid459.4JAK2, F5, PROS1
18protamine459.4SERPINE1, F5
19Drotrecogin alfa119.4SERPINE1, PROS1, F5
20prostacyclin459.3SERPINE1, PROS1, F5
21p00001459.3SERPINE1, MTHFR
22ascorbic acid45 2410.2JAK2, F5, MTHFR
23metformin45 50 1111.1SERPINE1, MTHFR, F5
24uric acid45 2410.1F5, MTHFR, SERPINE1
25homocysteine45 2410.1SERPINE1, MTHFR, F5
26fibrinogen458.9SERPINE1, MTHFR, F5
27vegf458.9SERPINE1, PROS1, F5, JAK2
28vitamin d458.8MTHFR, PROS1, SERPINE1
29aspirin45 50 29 2411.7SERPINE1, MTHFR, F5, JAK2
30cyclophosphamide45 50 1110.7PIGA, F5, MTHFR, SERPINE1
31creatinine458.5F5, MTHFR, PROS1, SERPINE1
32heparin45 29 11 2411.5F5, MTHFR, PROS1, SERPINE1
33testosterone45 60 11 2411.5F5, MTHFR, PROS1, SERPINE1
34estrogen458.4JAK2, F5, MTHFR, SERPINE1
35retinoic acid45 249.4SERPINE1, MTHFR, F5, JAK2
36cholesterol45 29 11 2411.2JAK2, F5, MTHFR, PROS1, SERPINE1
37serine458.1JAK2, F5, MTHFR, PROS1, SERPINE1

GO Terms for genes affiliated with Hepatic Vein Thrombosis

Sources:
16Gene Ontology
See all sources

Cellular components related to Hepatic Vein Thrombosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1platelet alpha granule lumenGO:0310939.0SERPINE1, PROS1, F5

Biological processes related to Hepatic Vein Thrombosis according to GeneCards/GeneDecks:

(show all 9)
idNameGO IDScoreTop Affiliating Genes
1GPI anchor biosynthetic processGO:0065069.7DPM1, PIGA
2C-terminal protein lipidationGO:0065019.5PIGA, DPM1
3fibrinolysisGO:0427309.5SERPINE1, PROS1
4positive regulation of inflammatory responseGO:0507299.5SERPINE1, JAK2
5platelet degranulationGO:0025769.2SERPINE1, PROS1, F5
6post-translational protein modificationGO:0436879.2PIGA, PROS1, DPM1
7blood circulationGO:0080159.0MTHFR, F5
8platelet activationGO:0301689.0SERPINE1, PROS1, F5
9blood coagulationGO:0075968.8JAK2, F5, PROS1, SERPINE1

Products for genes affiliated with Hepatic Vein Thrombosis

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Hepatic Vein Thrombosis

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