MCID: HPT002
MIFTS: 66

Hepatic Vein Thrombosis malady

Rare diseases, Cardiovascular diseases, Gastrointestinal diseases, Liver diseases, Blood diseases categories
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Summaries for Hepatic Vein Thrombosis

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Sources:
43NIH Rare Diseases, 65Wikipedia, 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 portal vein thrombosis 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 G protein signaling Ras family GTPases in kinase cascades scheme and Collagen biosynthesis and modifying enzymes. The drugs antithrombin iii, human and antithrombin iii,recombinant and the compounds batroxobin and ancrod have been mentioned in the context of this disorder. Affiliated tissues include liver, heart and spleen, and related mouse phenotypes are tumorigenesis and liver/biliary system.

Wikipedia:65 Budd?Chiari syndrome is a condition caused by occlusion of the hepatic veins that drain the liver. It... more...

Description from OMIM:47 600880

Aliases & Classifications for Hepatic Vein Thrombosis

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8Disease Ontology, 10DISEASES, 62UMLS, 9diseasecard, 43NIH Rare Diseases, 47OMIM, 45Novoseek, 49Orphanet, 22GTR, 35MeSH, 58SNOMED-CT, 59SNOMED-CT via Orphanet, 36MESH via Orphanet, 26ICD10 via Orphanet, 63UMLS via Orphanet, 25ICD10
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Classifications:

Orphanet: 49 
Rare hepatic diseases


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 62
budd-chiari syndrome 8 9 43 47 45 49 62
hepatic veno-occlusive disease 43 45 49 62
budd - chiari syndrome 8 22
sinusoidal obstruction syndrome 49
hepatic vein obstruction 43


External Ids:

Disease Ontology8 DOID:11512
MeSH35 D006502
SNOMED-CT via Orphanet59 38739001, 82385007, 65617004
OMIM47 600880
SNOMED-CT58 195436007, 38739001
MESH via Orphanet36 D006502, D006504
ICD10 via Orphanet26 I82.0, K76.5
UMLS via Orphanet63 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 126)
idRelated DiseaseScoreTop Affiliating Genes
1portal vein thrombosis31.4PROS1, F5
2protein c deficiency30.8PROS1, SERPINE1, F5
3antithrombin iii deficiency30.8PROS1, F5
4antiphospholipid syndrome30.8PROS1, F5
5protein s deficiency30.6PIGA, F5
6venous thrombosis30.5SERPINE1, JAK2, PROS1, F5
7sagittal sinus thrombosis30.4F5, PROS1
8lupus erythematosus30.4PROS1, F5
9thrombocytopenia30.3PIGA, F5
10thrombophilia30.3SERPINE1, PROS1, F5
11hyperhomocysteinemia30.3SERPINE1, PROS1, F5
12hypertension30.2SERPINE1, F5, PROS1
13hepatitis11.1
14hepatic veno-occlusive disease with immunodeficiency10.8
15hematopoietic stem cell transplantation10.6
16hepatopulmonary syndrome10.5
17ulcerative colitis10.5
18leukemia10.5
19familial mediterranean fever10.5
20thrombocytosis10.5
21cholangiocarcinoma10.5
22echinococcosis10.5
23intrahepatic cholangiocarcinoma10.5
24liver cirrhosis10.5
25essential thrombocythemia10.5
26nodular regenerative hyperplasia10.5
27nephroblastoma10.4
28myeloproliferative disorder10.4
29relapsing polychondritis10.4
30polyarteritis nodosa10.4
31cerebritis10.4
32alveolar echinococcosis10.4
33intravenous leiomyomatosis10.4
34alcoholic liver cirrhosis10.4
35hepatocellular carcinoma10.4
36leiomyomatosis10.4
37hemangioendothelioma10.4
38f5-related budd-chiari syndrome10.4
39jak2-related budd-chiari syndrome10.4
40epithelioid hemangioendothelioma10.4
41hemoglobinuria10.3
42paroxysmal nocturnal hemoglobinuria10.3
43graft versus host disease10.3
44esophageal varix10.3
45hemochromatosis10.3
46central pontine myelinolysis10.3
47agammaglobulinemia10.3
48esophagitis10.3
49acute graft versus host disease10.3
50primary agammaglobulinemia10.3

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



Diseases related to hepatic vein thrombosis

Symptoms for Hepatic Vein Thrombosis

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47OMIM, 49Orphanet
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Symptoms by clinical synopsis from OMIM:

600880

Clinical features from OMIM:

600880

Symptoms:

49 (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
  • gastrointestinal bleeding/hemorrhage/hematemesis/melena/rectorrhagia
  • 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
  • hepatomegaly/liver enlargement (excluding storage disease)

Drugs & Therapeutics for Hepatic Vein Thrombosis

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Sources:
42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Hepatic Vein Thrombosis

Search NIH Clinical Center for Hepatic Vein Thrombosis

Inferred drug relations via UMLS62/NDF-RT41:

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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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33MalaCards
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MalaCards organs/tissues related to Hepatic Vein Thrombosis:

33
Liver, Heart, Spleen

Animal Models for Hepatic Vein Thrombosis or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Hepatic Vein Thrombosis:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020068.8PIGA, SERPINE1, JAK2
2MP:00053708.6F5, PROS1, SERPINE1
3MP:00053808.2PROS1, F5, JAK2, PIGA
4MP:00053768.2SERPINE1, JAK2, PROS1, F5
5MP:00053788.1F5, PIGA, SERPINE1, JAK2
6MP:00053978.1PIGA, SERPINE1, PROS1, JAK2
7MP:00107717.7SERPINE1, PIGA, JAK2, PROS1, F5
8MP:00107687.7F5, PIGA, PROS1, JAK2, SERPINE1

Publications for Hepatic Vein Thrombosis

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52PubMed
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Articles related to Hepatic Vein Thrombosis:

(show top 50)    (show all 70)
idTitleAuthorsYear
1
Japanese case of Budd-Chiari syndrome due to hepatic vein thrombosis successfully treated with liver transplantation. (22248193)
2012
2
Subcapsular hepatic hematoma with right hepatic vein thrombosis: a complication of shock wave lithotripsy. (18542735)
2008
3
Hepatic vein thrombosis as a complication of ulcerative colitis in a 12-year-old patient. (17372822)
2007
4
Infliximab in the treatment of hepatic vein thrombosis (Budd-Chiari syndrome) in three patients with Behcet's syndrome. (17478465)
2007
5
Portal and hepatic vein thrombosis in liver abscess: CT findings. (17161932)
2007
6
Pivotal role of PAI-1 in a murine model of hepatic vein thrombosis. (16160004)
2006
7
Severe thrombophilic diathesis starting with hepatic vein thrombosis (BUDD-CHIARI syndrome) in a family with a new Protein S gene mutation. (16673558)
2006
8
Acute hepatic vein thrombosis after liver transplantation in a child with biliary atresia and absent inferior vena cava. (16797332)
2006
9
Familial high factor VIII level associated with hepatic vein thrombosis. (15319659)
2004
10
Hepatic vein thrombosis (Budd-Chiari syndrome). (11928075)
2002
11
Portal or hepatic vein thrombosis as the first presentation of a myeloproliferative disorder in patients with normal peripheral blood counts. (12181028)
2002
12
Hepatic vein thrombosis with ulcerative colitis. (10918734)
2000
13
Segmental hepatic vein thrombosis associated with heparin-induced thrombocytopenia II. (10664167)
2000
14
Hepatic vein thrombosis in a patient with mutant prothrombin 20210A allele. (9759637)
1998
15
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
16
Prevalence of the factor V Leiden mutation in portal and hepatic vein thrombosis. (9771422)
1998
17
Reassessing the role of medical therapy in the management of hepatic vein thrombosis. (9346773)
1997
18
Prospective evaluation of the prevalence of factor V Leiden mutation in portal or hepatic vein thrombosis. (9365003)
1997
19
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
20
Budd-Chiari syndrome caused by hepatic vein thrombosis in a patient with myeloproliferative disorder. (8968799)
1996
21
Successful systemic thrombolysis of hepatic vein thrombosis in a patient with promyelocytic leukemia treated with all-trans retinoic acid. (7717385)
1995
22
Tissue plasminogen activator for hepatic vein thrombosis in paroxysmal nocturnal haemoglobinuria. (8283166)
1994
23
Paroxysmal nocturnal hemoglobinuria with hepatic vein thrombosis presenting as hepatic encephalopathy. (2307506)
1990
24
Thrombotic cutaneous nodules and hepatic vein thrombosis in the anticardiolipin syndrome. (2591097)
1989
25
Hepatic vein thrombosis (Budd-Chiari syndrome) in the microgranular variant of acute promyelocytic leukemia. (2773853)
1989
26
Recovery from hepatic vein thrombosis (Budd-Chiari syndrome) complicating ulcerative colitis. (3197587)
1988
27
Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria. A spectrum from asymptomatic occlusion of hepatic venules to fatal Budd-Chiari syndrome. (3609666)
1987
28
Risk of hepatic vein thrombosis in relation to recent use of oral contraceptives. A case-control study. (3949113)
1986
29
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
30
Ultrasonography: hepatic vein thrombosis in sickle cell anemia. (7114404)
1982
31
Oral contraceptives and hepatic vein thrombosis. (7205808)
1981
32
Veno-occlusive disease and primary hepatic vein thrombosis in Israeli Arabs. (7263190)
1981
33
Case report: hepatic vein thrombosis (acute Budd-Chiari syndrome) in acute leukemia. (6947690)
1981
34
"Dimple sign" in hepatic vein thrombosis. (465948)
1979
35
Acute hepatic vein thrombosis occurring during therapy for Hodgkin's disease: a case report. (509400)
1979
36
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
37
Hepatic vein thrombosis: a case presentation and retrospective study. (225035)
1979
38
Inferior vena cava and hepatic vein thrombosis as a rare complication of ventriculoatrial shunt. Case report. (619017)
1978
39
Hepatic vein thrombosis in a woman taking oral contraceptives: a case report. (903688)
1977
40
Acute hepatic vein thrombosis in a patient with Behcet's disease. (829359)
1976
41
Hepatic vein thrombosis in Buffalo strain female rats ingesting dimethylnitrosamine. (1187201)
1975
42
Activation of Hageman factor and initiation of hepatic vein thrombosis in the hyperlipemic rat. (4843381)
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
Hepatic vein thrombosis complicating polycythemia vera. Successful treatment with a portacaval shunt. (6028691)
1967
48
MYOMA-ERYTHROCYTOSIS SYNDROME, PRESENTING WITH ACUTE HEPATIC VEIN THROMBOSIS. (14269124)
1965
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

Variations for Hepatic Vein Thrombosis

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Expression for genes affiliated with Hepatic Vein Thrombosis

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2BioGPS, 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:
50PathCards, 60Thomson Reuters, 38NCBI BioSystems Database, 55Reactome, 53QIAGEN, 54R&D Systems, 30KEGG
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Pathways related to Hepatic Vein Thrombosis according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
MAPK Cascade38
Immune response Oncostatin M signaling via MAPK in human cells60
Oncostatin M Signaling Pathway38
9.4SERPINE1, JAK2
2
Show member pathways
9.2F5, PROS1
3
Show member pathways
8.7SERPINE1, PROS1, F5
4
Show member pathways
8.7F5, PROS1, SERPINE1
5
Show member pathways
Complement Activation, Classical Pathway38
Complement and Coagulation Cascades38
8.7SERPINE1, PROS1, F5
6
Show member pathways
8.2SERPINE1, JAK2, PROS1, F5

Compounds for genes affiliated with Hepatic Vein Thrombosis

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Sources:
45Novoseek, 61Tocris Bioscience, 29IUPHAR, 11DrugBank, 51PharmGKB, 24HMDB
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Compounds related to Hepatic Vein Thrombosis according to GeneCards/GeneDecks:

(show all 50)
idCompoundScoreTop Affiliating Genes
1batroxobin459.9F5, SERPINE1
2ancrod459.9F5, SERPINE1
3tibolone459.9SERPINE1, F5
4cyproteroneacetate459.9F5, SERPINE1
5desmopressin45 61 29 1112.9F5, SERPINE1
6hirudin459.9SERPINE1, F5
7protamine459.9F5, SERPINE1
8mycophenolate mofetil45 51 1111.9SERPINE1, F5
9progestins459.9JAK2, F5
10raloxifene45 51 29 1112.9F5, SERPINE1
11dextran sulfate459.9F5, JAK2
12kininogen459.9SERPINE1, F5
13metformin45 51 1111.9SERPINE1, F5
14pge1459.8F5, SERPINE1
15aprotinin45 1110.8F5, SERPINE1
16uric acid45 2410.8F5, SERPINE1
17progestin459.8SERPINE1, F5
1815-deoxy-delta-12,14-prostaglandin j2459.8JAK2, SERPINE1
19ag 1478459.8JAK2, SERPINE1
20glucosylceramide45 2410.8F5, PIGA
21sodium dodecylsulfate459.8F5, SERPINE1
22homocysteine45 2410.7F5, SERPINE1
23sp 60012545 6110.7JAK2, SERPINE1
24atorvastatin45 51 29 24 1113.7F5, SERPINE1
25mannitol45 1110.7SERPINE1, JAK2
26gamma-carboxyglutamic acid459.6PROS1, F5
27rosiglitazone29 45 51 24 1113.6JAK2, SERPINE1
28simvastatin45 51 61 29 24 1114.5SERPINE1, F5
29tacrolimus45 51 1111.5F5, SERPINE1
30endotoxin459.4F5, SERPINE1, JAK2
31aspirin45 51 29 2412.4F5, SERPINE1, JAK2
32aspartate459.4SERPINE1, JAK2, F5
33cyclophosphamide45 51 1111.3PIGA, SERPINE1, F5
34nitric oxide45 24 1111.3JAK2, SERPINE1, F5
35dihydrotestosterone45 29 24 1112.2SERPINE1, PROS1
36nicotine45 29 51 1112.1JAK2, SERPINE1
37vitamin-e459.1PROS1, JAK2, F5
38estrogen459.1F5, JAK2, SERPINE1
39prostacyclin459.1PROS1, SERPINE1, F5
40phospholipid459.1F5, PROS1, JAK2
41creatinine459.1PROS1, SERPINE1, F5
42heparin45 29 24 1112.1PROS1, F5, SERPINE1
43retinoic acid45 2410.0F5, SERPINE1, JAK2
44testosterone45 61 24 1112.0SERPINE1, F5, PROS1
45steroid459.0JAK2, PROS1, F5
46dexamethasone45 51 29 1111.9SERPINE1, PROS1, JAK2
47cholesterol45 29 24 1111.6PROS1, F5, JAK2, SERPINE1
48vegf458.6SERPINE1, JAK2, PROS1, F5
49serine458.6JAK2, F5, PROS1, SERPINE1
50glucose458.5JAK2, SERPINE1, PROS1

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:0310938.7F5, PROS1, SERPINE1

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

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of inflammatory responseGO:0507299.3JAK2, SERPINE1
2fibrinolysisGO:0427309.1PROS1, SERPINE1
3post-translational protein modificationGO:0436879.0PROS1, PIGA
4negative regulation of endopeptidase activityGO:0109518.8SERPINE1, PROS1
5platelet degranulationGO:0025768.8F5, PROS1, SERPINE1
6platelet activationGO:0301688.7SERPINE1, PROS1, F5
7blood coagulationGO:0075968.3F5, PROS1, JAK2, SERPINE1

Products for genes affiliated with Hepatic Vein Thrombosis

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  • Antibodies
  • Proteins
  • Lysates
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Sources for Hepatic Vein Thrombosis

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4CDC
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
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet