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{{Budd-Chiari syndrome}}
{{Budd-Chiari syndrome}}
{{CMG}}; {{AE}}{{Mazia}}
{{CMG}}; {{AE}}{{Mazia}}
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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 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.
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 ==
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**[[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 [[Hepatic vein|right hepatic vein]] into the [[Inferior vena cavae|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|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|Protein-losing enteropathy]]
***minimal change nephrotic syndrome
***[[Minimal change disease]]
***[[neurofibromatosis]]
***[[Neurofibromatosis]]
***[[alpha-1 antitrypsin deficiency]]
***[[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]].



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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