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== Causes ==
== Causes ==
*Budd-Chiari syndrome is associated  with a wide range of etiologies.
*Budd-Chiari syndrome is associated  with a wide range of etiologies.<ref name="pmid28834866">{{cite journal |vauthors=Lin M, Zhang F, Wang Y, Zhang B, Zhang W, Zou X, Zhang M, Zhuge Y |title=Liver cirrhosis caused by chronic Budd-Chiari syndrome |journal=Medicine (Baltimore) |volume=96 |issue=34 |pages=e7425 |year=2017 |pmid=28834866 |pmc=5571988 |doi=10.1097/MD.0000000000007425 |url=}}</ref><ref name="pmid26668741">{{cite journal |vauthors=Martens P, Nevens F |title=Budd-Chiari syndrome |journal=United European Gastroenterol J |volume=3 |issue=6 |pages=489–500 |year=2015 |pmid=26668741 |pmc=4669515 |doi=10.1177/2050640615582293 |url=}}</ref><ref name="pmid27258526">{{cite journal |vauthors=Merz WM, Rüland AM, Hippe V, Poetzsch B, Meyer C, Pollok JM, Gembruch U, Trebicka J |title=Pregnancy in Budd-Chiari Syndrome: Case Report and Proposed Risk Score |journal=Medicine (Baltimore) |volume=95 |issue=22 |pages=e3817 |year=2016 |pmid=27258526 |pmc=4900734 |doi=10.1097/MD.0000000000003817 |url=}}</ref>
*On the basis of underlying cause Budd- Chairi can be:
*On the basis of underlying cause Budd- Chairi can be:
** Primary (75%): thrombosis of the hepatic vein
** Primary (75%): thrombosis of the hepatic vein

Revision as of 16:44, 9 November 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

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Overview

Causes

  • Budd-Chiari syndrome is associated with a wide range of etiologies.[1][2][3]
  • On the basis of underlying cause Budd- Chairi can be:
    • Primary (75%): thrombosis of the hepatic vein
    • Secondary (25%): invasion/compression of the hepatic vein by an outside structure like(e.g. a tumor, abscess or cysts)

Causes include:

  • Myeloproliferative disorders:
    • Myeloproliferative and other hemotologic abnormalities are one of the most common causes of Budd Chiari Syndrome.
    • V617F mutation in Janus tyrosine kinase-2 (JAK2) is found in 80% of patients with polycythemia vera and 50% of patients with essential thrombocythemia or idiopathic myelofibrosis.Budd Chiari syndrome patients that test negative for this mutation should have bone marrow biopsy performed.
    • Other associated hematologic causes include:
      • Paroxysmal nocturnal hemoglobinuria
      • Antiphospholipid syndrome
      • factor V leiden mutation
      • prothrombin gene mutation
      • methylene tetrahydrofolate reductase gene mutation
  • Malignancy
    • Malignancy is commonly associated with compression or invasion of vessels and hypercoagulable state.
    • Budd-Chiari is commonly associated with
      • hepatocellular carcinoma(associated with membranous obstruction of inferior vena cava)
      • adrenal gland or kidney malignancy
      • right atrial sarcoma
      • pancreatic cancer
      • lung carcinoma
      • gastric carcinoma
  • Infections and benign liver lesions
    • Cause extrinsic compression of inferior venacava. May be associated with hypercoagylable state. These lesions include:
      • hepatic cysts and abscesses
      • hepatic adenoma
      • hepatic mucinous cystic neoplasm (cystadenoma)
      • syphilitic gumma
      • invasive aspergillosis
      • zygomycosis (mucormycosis)
      • aortic aneurysm
  • Oral contraceptives and pregnancy
    • Hypercoagulable state in women using oral contraceptives (for more than two weeks), pregnant , or those who have delivered a child within the previous two months accounts for nearly 20 percent of cases of the Budd-Chiari syndrome
  • Other hypercoagulable states

Hypercoagulable conditions associated with Budd-Chiari include:

    • G1691A factor V (Leiden) gene mutation associated with activated protein C resistance
    • G20210A factor II gene mutation
    • Antiphospholipid syndrome
    • Antithrombin deficiency
    • Protein C deficiency
    • Protein S deficiency
    • Paroxysmal nocturnal hemoglobinuria
  • Behçet's syndrome
    • Vasculitis in Behçet's syndrome can predispose to thrombosis.
  • Membranous webs are usually are found near the entrance of the right hepatic vein into the inferior vena cava, may be due to a congenital anomaly or a myeloproliferative disease.
    • More common in patients from South Africa, India, and Asia. Potentially treatable cause of Budd-chiari.
  • Miscellaneous
    • Miscellaneous causes of the Budd-Chiari syndrome include:
      • Systemic lupus erythematosus
      • Mixed-connective tissue disease
      • Sjögren's syndrome
      • inflammatory bowel disease
      • hypereosinophilic syndrome
      • idiopathic granulomatous venulitis
      • sarcoidosis
      • protein-losing enteropathy
      • minimal change nephrotic syndrome
      • neurofibromatosis
      • alpha-1 antitrypsin deficiency
      • trauma
  • Idiopathic
    • Upto 20 percent of cases of the Budd-Chiari syndrome are idiopathic.

References

  1. Lin M, Zhang F, Wang Y, Zhang B, Zhang W, Zou X, Zhang M, Zhuge Y (2017). "Liver cirrhosis caused by chronic Budd-Chiari syndrome". Medicine (Baltimore). 96 (34): e7425. doi:10.1097/MD.0000000000007425. PMC 5571988. PMID 28834866.
  2. Martens P, Nevens F (2015). "Budd-Chiari syndrome". United European Gastroenterol J. 3 (6): 489–500. doi:10.1177/2050640615582293. PMC 4669515. PMID 26668741.
  3. Merz WM, Rüland AM, Hippe V, Poetzsch B, Meyer C, Pollok JM, Gembruch U, Trebicka J (2016). "Pregnancy in Budd-Chiari Syndrome: Case Report and Proposed Risk Score". Medicine (Baltimore). 95 (22): e3817. doi:10.1097/MD.0000000000003817. PMC 4900734. PMID 27258526.


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