MCID: HPT002
MIFTS: 65

Hepatic Vein Thrombosis malady

Cardiovascular diseases, Gastrointestinal diseases, Liver diseases, Blood diseases categories

Summaries for Hepatic Vein Thrombosis

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42NIH Rare Diseases, 63Wikipedia, 46OMIM, 32MalaCards
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NIH Rare Diseases:42 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 c deficiency, and has symptoms including intestinal obstruction/ileus, abnormal hepatic enzymes/transaminases and esophageal varices. 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 liver, heart and spleen, and related mouse phenotype integument.

Wikipedia:63 Budd–Chiari syndrome is a condition caused by occlusion of the hepatic veins that drain the liver. It... more...

Description from OMIM:46 600880

Aliases & Classifications for Hepatic Vein Thrombosis

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Sources:
8Disease Ontology, 10DISEASES, 60UMLS, 9diseasecard, 42NIH Rare Diseases, 46OMIM, 44Novoseek, 48Orphanet, 22GTR, 56SNOMED-CT, 34MeSH, 57SNOMED-CT via Orphanet, 35MESH via Orphanet, 26ICD10 via Orphanet, 61UMLS via Orphanet, 25ICD10
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Classifications:



Characteristics (Orphanet epidemiological data):

48
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 60
budd-chiari syndrome 8 9 42 46 44 48 60
hepatic veno-occlusive disease 42 44 48 60
budd - chiari syndrome 8 22
sinusoidal obstruction syndrome 48
hepatic vein obstruction 42


External Ids:

Disease Ontology8 DOID:11512
MeSH34 D006502
SNOMED-CT via Orphanet57 38739001, 82385007, 65617004
OMIM46 600880
SNOMED-CT56 38739001, 195436007
MESH via Orphanet35 D006502, D006504
ICD10 via Orphanet26 I82.0, K76.5
UMLS via Orphanet61 C0856761, C0019156
ICD1025 I82.0, K76.5

Related Diseases for Hepatic Vein Thrombosis

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17GeneCards, 18GeneDecks
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Diseases related to Hepatic Vein Thrombosis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 143)
idRelated DiseaseScoreTop Affiliating Genes
1antiphospholipid syndrome30.9PROS1, F5, MTHFR
2protein c deficiency30.9PROS1, MTHFR, SERPINE1, F5
3antithrombin iii deficiency30.7PROS1, MTHFR, F5
4portal vein thrombosis30.7PROS1, MTHFR, F5
5liver cirrhosis30.6PROS1
6thrombocytosis30.6JAK2
7protein s deficiency30.5PIGA, F5
8patent foramen ovale30.2F5
9sagittal sinus thrombosis30.2F5, PROS1
10pulmonary embolism30.2PROS1, F5, MTHFR
11thrombophilia30.2MTHFR, PROS1, SERPINE1, F5
12hyperhomocysteinemia30.2SERPINE1, PROS1, MTHFR, F5
13thrombocytopenia30.2PIGA, F5
14polycythemia vera30.1JAK2
15polycythemia30.1JAK2
16hypertension30.1MTHFR, SERPINE1, PROS1, F5
17hepatitis11.0
18hepatic veno-occlusive disease with immunodeficiency10.8
19hepatitis a10.8
20vein disease10.5
21hepatopulmonary syndrome10.5
22ulcerative colitis10.4
23leukemia10.4
24familial mediterranean fever10.4
25cholangiocarcinoma10.4
26echinococcosis10.4
27intrahepatic cholangiocarcinoma10.4
28nephroblastoma10.4
29acute leukemia10.4
30relapsing polychondritis10.3
31cerebritis10.3
32alveolar echinococcosis10.3
33intravenous leiomyomatosis10.3
34polyarteritis nodosa10.3
35alcoholic liver cirrhosis10.3
36hepatocellular carcinoma10.3
37leiomyomatosis10.3
38f5-related budd-chiari syndrome10.3
39jak2-related budd-chiari syndrome10.3
40hemoglobinuria10.3
41graft versus host disease10.2
42hemochromatosis10.2
43esophageal varix10.2
44central pontine myelinolysis10.2
45agammaglobulinemia10.2
46bone development disease10.2
47esophageal disease10.2
48esophagitis10.2
49liver disease10.2
50acute liver failure10.2

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



Diseases related to hepatic vein thrombosis

Clinical Features for Hepatic Vein Thrombosis

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Sources:
46OMIM, 48Orphanet
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Clinical features from OMIM:

600880

Clinical synopsis from OMIM:

600880

Symptoms:

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

Drugs & Therapeutics for Hepatic Vein Thrombosis

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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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 UMLS60/NDF-RT40:

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Genetic Tests for Hepatic Vein Thrombosis

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22GTR
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Genetic tests related to Hepatic Vein Thrombosis:

id Genetic test Affiliating Genes
1 Budd-Chiari Syndrome22

Anatomical Context for Hepatic Vein Thrombosis

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32MalaCards
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MalaCards organs/tissues related to Hepatic Vein Thrombosis:

32
Liver, Heart, Spleen, Lymph node

Animal Models for Hepatic Vein Thrombosis or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Hepatic Vein Thrombosis:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00107717.4SERPINE1, PROS1, MTHFR, F5, PIGA, JAK2

Publications for Hepatic Vein Thrombosis

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Sources:
50PubMed
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Articles related to Hepatic Vein Thrombosis:

(show top 50)    (show all 111)
idTitleAuthorsYear
1
High incidence of hepatitis B infection-associated cirrhosis and hepatocellular carcinoma in the Southeast Asian patients with portal vein thrombosis. (21658275)
2011
2
Portal vein thrombosis as complication of romiplostim treatment in a cirrhotic patient with hepatitis C-associated immune thrombocytopenic purpura. (21310200)
2011
3
Splenectomy in chronic hepatic disorders: portal vein thrombosis and improvement of liver function. (21293126)
2011
4
No-touch hepatic hilum technique to treat early portal vein thrombosis after pediatric liver transplantation. (20887425)
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
Pulmonary embolism and portal vein thrombosis in an immunocompetent adolescent with acute cytomegalovirus hepatitis. (19116696)
2009
8
Late acute celiac and hepatic artery thrombosis with portal vein thrombosis resulting in hepatic infarction 12 years post orthotopic liver transplantation. (20009144)
2009
9
Infliximab in the treatment of hepatic vein thrombosis (Budd-Chiari syndrome) in three patients with Behcet's syndrome. (17478465)
2007
10
Enhancing and expansile portal vein thrombosis: value in the diagnosis of hepatocellular carcinoma in patients with multiple hepatic lesions. (17449777)
2007
11
Tumor mimicking hepatic echinococcus alveolaris with portal vein thrombosis. (17917334)
2007
12
Portal and hepatic vein thrombosis in liver abscess: CT findings. (17161932)
2007
13
Hepatic arterial infusion chemotherapy with epirubicin in patients with advanced hepatocellular carcinoma and portal vein tumor thrombosis. (18097170)
2007
14
Microvascular thrombosis in the hepatic vein of a patient with paroxysmal nocturnal hemoglobinuria. (17988986)
2007
15
Pivotal role of PAI-1 in a murine model of hepatic vein thrombosis. (16160004)
2006
16
Severe thrombophilic diathesis starting with hepatic vein thrombosis (BUDD-CHIARI syndrome) in a family with a new Protein S gene mutation. (16673558)
2006
17
Acute hepatic vein thrombosis after liver transplantation in a child with biliary atresia and absent inferior vena cava. (16797332)
2006
18
Favorable resolution of hepatic infarctions in transplanted liver after portal vein thrombosis treated by surgical thrombectomy: a case report. (17112919)
2006
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
Hepatic vein thrombosis (Budd-Chiari syndrome). (11928075)
2002
24
Segmental hyperattenuation in the liver as a result of right hepatic vein thrombosis: an unusual complication of central venous catheterization. (12196241)
2002
25
Hepatic vein thrombosis with ulcerative colitis. (10918734)
2000
26
Hepatic vein thrombosis in a patient with mutant prothrombin 20210A allele. (9759637)
1998
27
Prevalence of the factor V Leiden mutation in portal and hepatic vein thrombosis. (9771422)
1998
28
Successful treatment of hepatic vein thrombosis following reduced-size liver transplantation. (9838415)
1998
29
Prospective evaluation of the prevalence of factor V Leiden mutation in portal or hepatic vein thrombosis. (9365003)
1997
30
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
31
Successful systemic thrombolysis of hepatic vein thrombosis in a patient with promyelocytic leukemia treated with all-trans retinoic acid. (7717385)
1995
32
Hepatic vein thrombosis in a teenager: a case report. (2007158)
1991
33
Thrombotic cutaneous nodules and hepatic vein thrombosis in the anticardiolipin syndrome. (2591097)
1989
34
Hepatic vein thrombosis (Budd Chiari syndrome): possible association with the use of oral contraceptives. (3296214)
1987
35
Idiopathic hypereosinophilia associated with hepatic vein thrombosis. (3689079)
1987
36
Risk of hepatic vein thrombosis in relation to recent use of oral contraceptives. A case-control study. (3949113)
1986
37
Primary myeloproliferative disorder and hepatic vein thrombosis. A prospective study of erythroid colony formation in vitro in 20 patients with Budd-Chiari syndrome. (4026081)
1985
38
Hepatic vein thrombosis (Budd-Chiari syndrome). (6693073)
1984
39
Ultrasonography: hepatic vein thrombosis in sickle cell anemia. (7114404)
1982
40
Case report: hepatic vein thrombosis (acute Budd-Chiari syndrome) in acute leukemia. (6947690)
1981
41
Acute hepatic vein thrombosis occurring during therapy for Hodgkin's disease: a case report. (509400)
1979
42
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
43
Hepatic vein thrombosis in mice ingesting chlorinated hydrocarbons. (578719)
1977
44
Hepatic vein thrombosis treated with streptokinase. (4439110)
1974
45
Hepatic vein thrombosis (Budd-Chiari syndrome). (5445577)
1970
46
Hepatic vein thrombosis. Increased incidence in rats given methylcholanthrene and carbon tetrachloride. (5450122)
1970
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
MYOMA-ERYTHROCYTOSIS SYNDROME, PRESENTING WITH ACUTE HEPATIC VEIN THROMBOSIS. (14269124)
1965
50
Chiari's disease, hepatic-vein thrombosis. (14941265)
1952

Genetic Variations for Hepatic Vein Thrombosis

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Expression for genes affiliated with Hepatic Vein Thrombosis

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1BioGPS, 15Gene Expression Omnibus DataSets
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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

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Sources:
53Reactome, 29KEGG, 51QIAGEN, 37NCBI BioSystems Database, 52R&D Systems
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Compounds for genes affiliated with Hepatic Vein Thrombosis

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Sources:
44Novoseek, 59Tocris Bioscience, 28IUPHAR, 11DrugBank, 49PharmGKB, 24HMDB
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Compounds related to Hepatic Vein Thrombosis according to GeneCards/GeneDecks:

(show all 37)
idCompoundScoreTop Affiliating Genes
1aspartate4410.5JAK2
2gamma-carboxyglutamic acid449.9PROS1, F5
3batroxobin449.9SERPINE1, F5
4ancrod449.9SERPINE1, F5
5tibolone449.8SERPINE1, F5
6cyproteroneacetate449.8F5, SERPINE1
7desmopressin44 59 28 1112.8SERPINE1, F5
85-methyltetrahydrofolate44 1110.7MTHFR, F5
9cystathionine449.7F5, MTHFR
10hirudin449.7SERPINE1, F5
11vitamin-e449.6JAK2, F5, PROS1
12mycophenolate mofetil44 49 1111.5F5, SERPINE1
13vitamin b6449.5MTHFR, F5
14raloxifene44 28 49 1112.5SERPINE1, F5
15endotoxin449.5SERPINE1, F5, JAK2
16progestins449.5F5, JAK2
17phospholipid449.4JAK2, F5, PROS1
18protamine449.4SERPINE1, F5
19Drotrecogin alfa119.4SERPINE1, PROS1, F5
20prostacyclin449.3SERPINE1, PROS1, F5
21p00001449.3SERPINE1, MTHFR
22ascorbic acid44 2410.2JAK2, F5, MTHFR
23metformin44 49 1111.1SERPINE1, MTHFR, F5
24uric acid44 2410.1F5, MTHFR, SERPINE1
25homocysteine44 2410.1SERPINE1, MTHFR, F5
26fibrinogen448.9SERPINE1, MTHFR, F5
27vegf448.9SERPINE1, PROS1, F5, JAK2
28vitamin d448.8MTHFR, PROS1, SERPINE1
29aspirin44 49 28 2411.7SERPINE1, MTHFR, F5, JAK2
30cyclophosphamide44 49 1110.7PIGA, F5, MTHFR, SERPINE1
31creatinine448.5F5, MTHFR, PROS1, SERPINE1
32heparin44 28 11 2411.5F5, MTHFR, PROS1, SERPINE1
33testosterone44 59 11 2411.5F5, MTHFR, PROS1, SERPINE1
34estrogen448.4JAK2, F5, MTHFR, SERPINE1
35retinoic acid44 249.4SERPINE1, MTHFR, F5, JAK2
36cholesterol44 28 11 2411.2JAK2, F5, MTHFR, PROS1, SERPINE1
37serine448.1JAK2, F5, MTHFR, PROS1, SERPINE1

GO Terms for genes affiliated with Hepatic Vein Thrombosis

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16Gene Ontology
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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

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Hepatic Vein Thrombosis

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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
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet