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== Causes ==
== Causes ==
*Budd-Chiari syndrome is associated  with a wide range of etiologies.
*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)


* Primary (75%): [[thrombosis]] of the hepatic vein
Causes include:
* Secondary (25%): compression of the hepatic vein by an outside structure (e.g. a [[tumor]])
*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


Often, the patient is known to have a tendency towards [[thrombosis]], although Budd-Chiari syndrome can also be the first symptom of such a tendency.
*Malignancy
'''Genetic causes'''
**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


* [[Protein C]] deficiency
*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


* [[Protein S]] deficiency
*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


* [[Factor V Leiden]] mutation
*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


'''Other causes'''
*Behçet's syndrome
 
**Vasculitis in Behçet's syndrome can predispose to thrombosis.
* [[Antiphospholipid syndrome]]
*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.
* [[Aspergillosis]]
*Miscellaneous
 
**Miscellaneous causes of the Budd-Chiari syndrome include:
* [[Behcet's disease]]
***Systemic lupus erythematosus
 
***Mixed-connective tissue disease
* [[Dacarbazine]]
***Sjögren's syndrome
 
***inflammatory bowel disease
*[[Desogestrel and Ethinyl Estradiol]]
***hypereosinophilic syndrome
 
***idiopathic granulomatous venulitis
*[[Ethynodiol diacetate and ethinyl estradiol]]
***sarcoidosis
 
***protein-losing enteropathy
* [[Physical trauma|Trauma]]
***minimal change nephrotic syndrome
* [[polycythemia|Polycythemia vera]] <ref>Patel RK, Lea NC, Heneghan MA, Westwood NB, Milojkovic D, Thanigaikumar M, Yallop D, Arya R, Pagliuca A, Gaken J, Wendon J, Heaton ND, Mufti GJ. Prevalence of the activating JAK2 tyrosine kinase mutation V617F in the Budd-Chiari syndrome. Gastroenterology. 2006 Jun;130(7):2031-8.</ref>
***neurofibromatosis
 
***alpha-1 antitrypsin deficiency
A related condition is [[veno-occlusive disease]], which occurs in recipients of [[bone marrow transplant]]s as a complication of their medication. Although its mechanism is similar, it is not considered a form of Budd-Chiari syndrome.
***trauma  
*Idiopathic
**Upto 20 percent of cases of the Budd-Chiari syndrome are idiopathic.


==References==
==References==

Revision as of 09:31, 6 November 2017

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Overview

Causes

  • Budd-Chiari syndrome is associated with a wide range of etiologies.
  • 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


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