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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 | 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 | ***[[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 | **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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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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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
- Budd-Chiari syndrome is associated with a wide range of etiologies.[1][2][3][4]
- 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 renal malignancy
- right atrial sarcoma
- pancreatic cancer
- lung carcinoma
- gastric carcinoma
- Infections and benign liver lesions
- Cause extrinsic compression of inferior vena cava. May be associated with hypercoagulable state. These lesions include:
- 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:
- 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
- Miscellaneous causes of the Budd-Chiari syndrome include:
- Idiopathic
- Upto 20 percent of cases of the Budd-Chiari syndrome are idiopathic.
References
- ↑ 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.
- ↑ 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.
- ↑ Martens P, Nevens F (2015). "Budd-Chiari syndrome". United European Gastroenterol J. 3 (6): 489–500. doi:10.1177/2050640615582293. PMC 4669515. PMID 26668741.
- ↑ 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.