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==Overview==
==Overview==
Budd-Chiari syndrome is associated with a wide range of etiologies.On the basis of underlying cause Budd- Chairi can be Primary(75%) caused bythrombosis of the hepatic vein and Secondary (25%) caused by invasion/compression of the hepatic vein by an outside structure like(e.g. a tumor, abscess or cysts).Causes include myeloproliferative disorders, malignancy, paroxysmal nocturnal hemoglobinuria, antiphospholipid syndrome, factor V leiden mutation, infections and benign liver lesions, oral contraceptives and pregnancy, hypercoagulable states, Behçet's syndrome, membranous webs.
Budd-Chiari syndrome is associated with a wide range of [[etiologies]].On the basis of underlying cause Budd- Chairi can be Primary(75%) caused by [[thrombosis]] of the [[Hepatic veins|hepatic vein]] and Secondary (25%) caused by [[invasion]]/compression of the [[Hepatic veins|hepatic vein]] by an outside structure like(e.g. a [[tumor]], [[abscess]] or [[cysts]]). Causes include [[Myeloproliferative disease|myeloproliferative disorders]], [[malignancy]], [[paroxysmal nocturnal hemoglobinuria]], [[antiphospholipid syndrome]], [[Factor V Leiden mutation|factor V leiden mutation]], [[infections]] and [[Liver lesions|benign liver lesions]], [[oral contraceptives]] and [[pregnancy]], [[hypercoagulable states]], [[Behçet's syndrome]], membranous webs.


== Causes ==
== Causes ==
*Budd-Chiari syndrome is associated  with a wide range of etiologies.<ref name="pmid25937867">{{cite journal |vauthors=Afredj N, Guessab N, Nani A, Faraoun SA, Ouled Cheikh I, Kerbouche R, Hannoun D, Amir ZC, Ait Kaci H, Bentabak K, Plessier A, Valla DC, Cazals-Hatem V, Denninger MH, Boucekkine T, Debzi N |title=Aetiological factors of Budd-Chiari syndrome in Algeria |journal=World J Hepatol |volume=7 |issue=6 |pages=903–9 |year=2015 |pmid=25937867 |pmc=4411532 |doi=10.4254/wjh.v7.i6.903 |url=}}</ref><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>
*Budd-Chiari syndrome is associated  with a wide range of [[etiologies]].<ref name="pmid25937867">{{cite journal |vauthors=Afredj N, Guessab N, Nani A, Faraoun SA, Ouled Cheikh I, Kerbouche R, Hannoun D, Amir ZC, Ait Kaci H, Bentabak K, Plessier A, Valla DC, Cazals-Hatem V, Denninger MH, Boucekkine T, Debzi N |title=Aetiological factors of Budd-Chiari syndrome in Algeria |journal=World J Hepatol |volume=7 |issue=6 |pages=903–9 |year=2015 |pmid=25937867 |pmc=4411532 |doi=10.4254/wjh.v7.i6.903 |url=}}</ref><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 veins|hepatic vein]]
**Secondary (25%): invasion/compression of the hepatic vein by an outside structure like(e.g. a tumor, abscess or cysts)
**Secondary (25%): [[invasion]]/compression of the [[Hepatic veins|hepatic vein]] by an outside structure like(e.g. a [[tumor]], [[abscess]] or [[cysts]])


Causes include:
[[Causes]] include:
*Myeloproliferative disorders:
*[[Myeloproliferative disease|Myeloproliferative disorders]]:
**Myeloproliferative and other hemotologic abnormalities are one of the most common causes of Budd Chiari Syndrome.  
**[[Myeloproliferative disease|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.
**V617F [[Mutations|mutation]] in [[Tyrosine kinases|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 [[Mutations|mutation]] should have [[bone marrow biopsy]] performed.
**Other associated hematologic causes include:
**Other associated [[hematologic]] causes include:
***Paroxysmal nocturnal hemoglobinuria
***[[Paroxysmal nocturnal hemoglobinuria]]
***Antiphospholipid syndrome
***[[Antiphospholipid syndrome]]
***factor V leiden mutation
***[[Factor V Leiden mutation|factor V leiden mutation]]
***prothrombin gene mutation  
***[[Prothrombin gene mutation G20210A|prothrombin gene mutation]]
***methylene tetrahydrofolate reductase gene mutation  
***methylene tetrahydrofolate reductase [[gene mutation]]


*Malignancy
*[[Malignancy]]
**Malignancy is commonly associated with compression or invasion of vessels and hypercoagulable state.  
**[[Malignancy]] is commonly associated with compression or [[invasion]] of [[vessels]] and [[Hypercoagulable state|hypercoagulable]] state.  
**Budd-Chiari is commonly associated with  
**Budd-Chiari is commonly associated with  
***hepatocellular carcinoma(associated with membranous obstruction of inferior vena cava)
***[[hepatocellular carcinoma]](associated with membranous obstruction of [[inferior vena cava]])
***adrenal gland or kidney malignancy
***[[adrenal gland]] or [[renal]] [[malignancy]]
***right atrial sarcoma
***right [[atrial]] [[sarcoma]]
***pancreatic cancer
***[[pancreatic cancer]]
***lung carcinoma
***[[lung carcinoma]]
***gastric carcinoma
***[[gastric carcinoma]]


*Infections and benign liver lesions
*[[Infections]] and [[Liver lesions|benign liver lesions]]
**Cause extrinsic compression of inferior venacava. May be associated with hypercoagylable state. These lesions include:
**Cause extrinsic compression of [[inferior vena cava]]. May be associated with [[hypercoagulable state]]. These lesions include:
***hepatic cysts and abscesses
***[[hepatic cysts]] and [[abscesses]]
***hepatic adenoma
***[[hepatic adenoma]]
***hepatic mucinous cystic neoplasm (cystadenoma)
***[[hepatic]] [[mucinous]] [[cystic]] [[neoplasm]] ([[cystadenoma]])
***syphilitic gumma
***[[syphilitic]] [[gumma]]
***invasive aspergillosis
***[[invasive aspergillosis]]
***zygomycosis (mucormycosis)
***[[zygomycosis]] ([[Mucormycosis|mucormycosis)]]
***aortic aneurysm
***[[aortic aneurysm]]


*Oral contraceptives and pregnancy
*[[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  
**[[Hypercoagulable states|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
*Other [[hypercoagulable states]]
Hypercoagulable conditions associated with Budd-Chiari include:
[[Hypercoagulable conditions]] associated with Budd-Chiari include:
**G1691A factor V (Leiden) gene mutation associated with activated protein C resistance  
**[[Factor V Leiden mutation|G1691A factor V (Leiden) gene mutation]] associated with [[activated protein C]] [[resistance]]
**G20210A factor II gene mutation
**[[Factor II deficiency|G20210A factor II gene mutation]]
**Antiphospholipid syndrome  
**[[Antiphospholipid syndrome]]
**Antithrombin deficiency
**[[Antithrombin deficiency]]
**Protein C deficiency  
**[[Protein C deficiency]]
**Protein S deficiency
**[[Protein S deficiency]]
**Paroxysmal nocturnal hemoglobinuria  
**[[Paroxysmal nocturnal hemoglobinuria]]


*Behçet's syndrome
*[[Behçet's syndrome]]
**Vasculitis in Behçet's syndrome can predispose to thrombosis.
**[[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.
*Membranous webs are usually are found near the entrance of the [[Hepatic vein|right hepatic vein]] into the [[Inferior vena cavae|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.
** More common in [[patients]] from South Africa, India, and Asia. Potentially treatable cause of Budd-chiari.
*Miscellaneous
*Miscellaneous
**Miscellaneous causes of the Budd-Chiari syndrome include:
**Miscellaneous causes of the Budd-Chiari syndrome include:
***Systemic lupus erythematosus
***[[Systemic lupus erythematosus]]
***Mixed-connective tissue disease
***Mixed-connective tissue disease
***Sjögren's syndrome
***[[Sjögren's syndrome]]
***inflammatory bowel disease
***[[inflammatory bowel disease]]
***hypereosinophilic syndrome
***[[hypereosinophilic syndrome]]
***idiopathic granulomatous venulitis
***idiopathic granulomatous venulitis
***sarcoidosis
***[[sarcoidosis]]
***protein-losing enteropathy
***[[protein-losing enteropathy]]
***minimal change nephrotic syndrome
***minimal change nephrotic syndrome
***neurofibromatosis
***[[neurofibromatosis]]
***alpha-1 antitrypsin deficiency
***[[alpha-1 antitrypsin deficiency]]
***trauma  
***[[trauma]]
*Idiopathic
*[[Idiopathic]]
**Upto 20 percent of cases of the Budd-Chiari syndrome are idiopathic.
**Upto 20 percent of cases of the Budd-Chiari syndrome are [[idiopathic]].


==References==
==References==

Revision as of 19:41, 13 November 2017

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Overview

Budd-Chiari syndrome is associated with a wide range of etiologies.On the basis of underlying cause Budd- Chairi can be Primary(75%) caused by thrombosis of the hepatic vein and Secondary (25%) caused by invasion/compression of the hepatic vein by an outside structure like(e.g. a tumor, abscess or cysts). Causes include myeloproliferative disorders, malignancy, paroxysmal nocturnal hemoglobinuria, antiphospholipid syndrome, factor V leiden mutation, infections and benign liver lesions, oral contraceptives and pregnancy, hypercoagulable states, Behçet's syndrome, membranous webs.

Causes

Causes include:

Hypercoagulable conditions associated with Budd-Chiari include:

References

  1. Afredj N, Guessab N, Nani A, Faraoun SA, Ouled Cheikh I, Kerbouche R, Hannoun D, Amir ZC, Ait Kaci H, Bentabak K, Plessier A, Valla DC, Cazals-Hatem V, Denninger MH, Boucekkine T, Debzi N (2015). "Aetiological factors of Budd-Chiari syndrome in Algeria". World J Hepatol. 7 (6): 903–9. doi:10.4254/wjh.v7.i6.903. PMC 4411532. PMID 25937867.
  2. 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.
  3. Martens P, Nevens F (2015). "Budd-Chiari syndrome". United European Gastroenterol J. 3 (6): 489–500. doi:10.1177/2050640615582293. PMC 4669515. PMID 26668741.
  4. 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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