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Hepatic vein thrombosis

MedGen UID:
9217
Concept ID:
C0019154
Disease or Syndrome
Synonyms: Hepatic Vein Thromboses; Hepatic Vein Thrombosis; Thromboses, Hepatic Vein; Thrombosis, Hepatic Vein; Vein Thromboses, Hepatic; Vein Thrombosis, Hepatic
SNOMED CT: Hepatic vein thrombosis (38739001)
 
HPO: HP:0030243
Monarch Initiative: MONDO:0006786

Definition

An obstruction in the veins of the liver caused by a blood clot (thrombosis). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHepatic vein thrombosis

Conditions with this feature

Protein-losing enteropathy
MedGen UID:
19522
Concept ID:
C0033680
Disease or Syndrome
Complement hyperactivation, angiopathic thrombosis, and protein-losing enteropathy (CHAPLE) is characterized by abdominal pain and diarrhea, primary intestinal lymphangiectasia, hypoproteinemic edema, and malabsorption. Some patients also exhibit bowel inflammation, recurrent infections associated with hypogammaglobulinemia, and/or angiopathic thromboembolic disease. Patient T lymphocytes show increased complement activation, causing surface deposition of complement and generating soluble C5a (Ozen et al., 2017).
Hypercoagulability syndrome due to glycosylphosphatidylinositol deficiency
MedGen UID:
1684821
Concept ID:
C5201145
Disease or Syndrome
Glycosylphosphatidylinositol is a glycolipid that anchors more than 150 proteins to the cell surface, and these proteins, termed GPI-anchored proteins (GPI-APs), perform a variety of functions as enzymes, adhesion molecules, complement regulators, and coreceptors in signal transduction pathways. Reduced surface levels of GPI-APs or abnormal GPI-AP structure can therefore result in variable manifestations. Glycosylphosphatidylinositol biosynthesis defect-1 (GPIBD1) is characterized predominantly by portal hypertension due to portal vein thrombosis. Most patients have absence seizures, cerebral thrombosis, and macrocephaly. Some patients have mildly to moderately impaired intellectual development (summary by Makrythanasis et al., 2016; Pode-Shakked et al., 2019). Genetic Heterogeneity of Glycosylphosphatidylinositol Biosynthesis Defects Also see GPIBD2 (239300), caused by mutation in the PIGV gene (610274); GPIBD3 (614080), caused by mutation in the PIGN gene (606097); GPIBD4 (300868), caused by mutation in the PIGA gene (311770); GPIBD5 (280000), caused by mutation in the PIGL gene (605947); GPIBD6 (614749), caused by mutation in the PIGO gene (614730); GPIBD7 (615398), caused by mutation in the PIGT gene (610272); GPIBD8 (614207), caused by mutation in the PGAP2 gene (615187); GPIBD9 (615802), caused by mutation in the PGAP1 gene (611655); GPIBD10 (615716), caused by mutation in the PGAP3 gene (611801); GPIBD11 (616025), caused by mutation in the PIGW gene (610275); GPIBD12 (616809), caused by mutation in the PIGY gene (610662); GPIBD13 (616917), caused by mutation in the PIGG gene (616918); GPIBD14 (617599), caused by mutation in the PIGP gene (605938); GPIBD15 (617810), caused by mutation in the GPAA1 gene (603048); GPIBD16 (617816), caused by mutation in the PIGC gene (601730); GPIBD17 (618010), caused by mutation in the PIGH gene (600154); GPIBD18 (618143), caused by mutation in the PIGS gene (610271); GPIBD19 (618548), caused by mutation in the PIGQ gene (605754); GPIBD20 (618580), caused by mutation in the PIGB gene (604122); GPIBD21 (618590), caused by mutation in the PIGU gene (608528); GPIBD22 (618879), caused by mutation in the PIGK gene (605087); GPIBD23 (617020), caused by mutation in the ARV1 gene (611647); GPIBD24 (619356), caused by mutation in the PIGF gene (600153); and GPIBD25 (619985), caused by mutation in the C18ORF32 gene (619979).

Professional guidelines

PubMed

Naymagon L
Transl Res 2020 Nov;225:54-69. Epub 2020 May 11 doi: 10.1016/j.trsl.2020.04.012. PMID: 32407789
Barbui T, De Stefano V
Curr Opin Hematol 2017 Mar;24(2):108-114. doi: 10.1097/MOH.0000000000000312. PMID: 27875375
Riva N, Donadini MP, Dentali F, Squizzato A, Ageno W
Thromb Res 2012 Oct;130 Suppl 1:S1-3. doi: 10.1016/j.thromres.2012.08.259. PMID: 23026649

Recent clinical studies

Etiology

Venier LM, Clerici B, Bissola AL, Modi D, Jevtic SD, Radford M, Mahamad S, Nazy I, Arnold DM
Int J Hematol 2023 Mar;117(3):341-348. Epub 2022 Dec 27 doi: 10.1007/s12185-022-03516-4. PMID: 36574172Free PMC Article
Zanetto A, Cossiga V, Shalaby S, Guarino M, Invernizzi F, Lapenna L, Becchetti C, Morisco F, Morelli MC, Merli M, Toniutto P, Burra P; Special Interest Group Gender in Hepatology of the Italian Association for the Study of the Liver (AISF)
Dig Liver Dis 2023 Feb;55(2):178-186. Epub 2022 Jul 27 doi: 10.1016/j.dld.2022.07.005. PMID: 35906168
Mähringer-Kunz A, Meyer FI, Hahn F, Müller L, Düber C, Pinto Dos Santos D, Galle PR, Weinmann A, Kloeckner R, Schotten S
United European Gastroenterol J 2021 Jun;9(5):590-597. Epub 2021 Jun 2 doi: 10.1002/ueg2.12098. PMID: 34077613Free PMC Article
Naymagon L
Transl Res 2020 Nov;225:54-69. Epub 2020 May 11 doi: 10.1016/j.trsl.2020.04.012. PMID: 32407789
Barbui T, De Stefano V
Curr Opin Hematol 2017 Mar;24(2):108-114. doi: 10.1097/MOH.0000000000000312. PMID: 27875375

Diagnosis

Venier LM, Clerici B, Bissola AL, Modi D, Jevtic SD, Radford M, Mahamad S, Nazy I, Arnold DM
Int J Hematol 2023 Mar;117(3):341-348. Epub 2022 Dec 27 doi: 10.1007/s12185-022-03516-4. PMID: 36574172Free PMC Article
Mähringer-Kunz A, Meyer FI, Hahn F, Müller L, Düber C, Pinto Dos Santos D, Galle PR, Weinmann A, Kloeckner R, Schotten S
United European Gastroenterol J 2021 Jun;9(5):590-597. Epub 2021 Jun 2 doi: 10.1002/ueg2.12098. PMID: 34077613Free PMC Article
Barbui T, De Stefano V
Curr Opin Hematol 2017 Mar;24(2):108-114. doi: 10.1097/MOH.0000000000000312. PMID: 27875375
Riva N, Donadini MP, Dentali F, Squizzato A, Ageno W
Thromb Res 2012 Oct;130 Suppl 1:S1-3. doi: 10.1016/j.thromres.2012.08.259. PMID: 23026649
Zimmerman HJ
Drugs 1978 Jul;16(1):25-45. doi: 10.2165/00003495-197816010-00002. PMID: 352664

Therapy

Venier LM, Clerici B, Bissola AL, Modi D, Jevtic SD, Radford M, Mahamad S, Nazy I, Arnold DM
Int J Hematol 2023 Mar;117(3):341-348. Epub 2022 Dec 27 doi: 10.1007/s12185-022-03516-4. PMID: 36574172Free PMC Article
Mimier MK, Janczak DT, McBane RD, Houghton DE, Wysokinski WE
Pol Arch Intern Med 2018 Oct 31;128(10):604-608. Epub 2018 Sep 20 doi: 10.20452/pamw.4333. PMID: 30233080
Barbui T, De Stefano V
Curr Opin Hematol 2017 Mar;24(2):108-114. doi: 10.1097/MOH.0000000000000312. PMID: 27875375
Riva N, Donadini MP, Dentali F, Squizzato A, Ageno W
Thromb Res 2012 Oct;130 Suppl 1:S1-3. doi: 10.1016/j.thromres.2012.08.259. PMID: 23026649
Maddrey WC
Semin Liver Dis 1987 Feb;7(1):32-9. doi: 10.1055/s-2008-1040561. PMID: 3296214

Prognosis

Barbui T, De Stefano V
Curr Opin Hematol 2017 Mar;24(2):108-114. doi: 10.1097/MOH.0000000000000312. PMID: 27875375
Brière JB
Semin Thromb Hemost 2006 Apr;32(3):208-18. doi: 10.1055/s-2006-939432. PMID: 16673275
Valla DC
Semin Liver Dis 2002 Feb;22(1):5-14. doi: 10.1055/s-2002-23202. PMID: 11928075
Dourakis SP, Tolis G
Eur J Contracept Reprod Health Care 1998 Mar;3(1):7-16. doi: 10.3109/13625189809167479. PMID: 9678067
Zimmerman HJ
Drugs 1978 Jul;16(1):25-45. doi: 10.2165/00003495-197816010-00002. PMID: 352664

Clinical prediction guides

Shankar S, Rammohan A, Gunasekaran V, Narasimhan G, Rajalingam R, Palaniappan K, Kanagavelu R, Rajkumar A, Kaliamoorthy I, Rela M
Am J Transplant 2023 Jun;23(6):786-793. Epub 2023 Mar 10 doi: 10.1016/j.ajt.2023.03.004. PMID: 36906229
Mähringer-Kunz A, Meyer FI, Hahn F, Müller L, Düber C, Pinto Dos Santos D, Galle PR, Weinmann A, Kloeckner R, Schotten S
United European Gastroenterol J 2021 Jun;9(5):590-597. Epub 2021 Jun 2 doi: 10.1002/ueg2.12098. PMID: 34077613Free PMC Article
Naymagon L
Transl Res 2020 Nov;225:54-69. Epub 2020 May 11 doi: 10.1016/j.trsl.2020.04.012. PMID: 32407789
Priyadarshi RN, Kumar P, Kumar R, Anand U, Shyama
Abdom Radiol (NY) 2020 Mar;45(3):652-660. doi: 10.1007/s00261-020-02409-6. PMID: 31955219
Zimmerman HJ
Drugs 1978 Jul;16(1):25-45. doi: 10.2165/00003495-197816010-00002. PMID: 352664

Recent systematic reviews

Zhu Y, Jiang H, Chen Z, Lu B, Wu J
Medicine (Baltimore) 2017 Nov;96(47):e8856. doi: 10.1097/MD.0000000000008856. PMID: 29381999Free PMC Article

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