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{{Budd-Chiari syndrome}}
{{Budd-Chiari syndrome}}
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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 syndrome can be primary (75%) caused by [[thrombosis]] of the [[Hepatic veins|hepatic vein]] or secondary (25%) caused by [[invasion]]/compression of the [[Hepatic veins|hepatic vein]] by an outside structure such as 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>
*On the basis of underlying cause Budd- Chairi can be:
** Primary (75%): [[thrombosis]] of the [[Hepatic veins|hepatic vein]]
**Secondary (25%): [[invasion]]/compression of the [[Hepatic veins|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 disease|Myeloproliferative disorders]]:
 
**[[Myeloproliferative disease|Myeloproliferative]] and other hemotologic abnormalities are one of the most common causes of [[Budd Chiari Syndrome]].
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.
**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.
'''Genetic causes'''
**Other associated [[hematologic]] causes include:
 
***[[Paroxysmal nocturnal hemoglobinuria]]
* [[Protein C]] deficiency
***[[Antiphospholipid syndrome]]
 
***[[Factor V Leiden mutation|factor V leiden mutation]]
* [[Protein S]] deficiency
***[[Prothrombin gene mutation G20210A|prothrombin gene mutation]]  
 
***methylene tetrahydrofolate reductase [[gene mutation]]  
* [[Factor V Leiden]] mutation
 
'''Other causes'''
 
* [[Antiphospholipid syndrome]]
 
* [[Aspergillosis]]
 
* [[Behcet's disease]]


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


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


*[[Ethynodiol diacetate and ethinyl estradiol]]
*[[Oral contraceptives]] and [[pregnancy]]
**[[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]]  


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


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.
*[[Behçet's syndrome]]
**[[Vasculitis]] in [[Behçet's syndrome]] can predispose to [[thrombosis]].
*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.
*Miscellaneous:
**Miscellaneous causes of the Budd-Chiari syndrome include:
***[[Systemic lupus erythematosus]]
***[[Mixed connective tissue disease|Mixed-connective tissue disease]]
***[[Sjögren's syndrome]]
***[[Inflammatory bowel disease]]
***[[Hypereosinophilic syndrome]]
***Idiopathic granulomatous venulitis
***[[Sarcoidosis]]
***[[Protein losing enteropathy|Protein-losing enteropathy]]
***[[Minimal change disease]]
***[[Neurofibromatosis]]
***[[Alpha 1-antitrypsin deficiency|Alpha-1 antitrypsin deficiency]]
***[[Trauma]]
*[[Idiopathic]]:
**Upto 20 percent of cases of the Budd-Chiari syndrome are [[idiopathic]].


==References==
==References==

Latest revision as of 17:00, 30 November 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mazia Fatima, MBBS [2]

Overview

Budd-Chiari syndrome is associated with a wide range of etiologies. On the basis of underlying cause Budd- Chairi syndrome can be primary (75%) caused by thrombosis of the hepatic vein or secondary (25%) caused by invasion/compression of the hepatic vein by an outside structure such as 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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