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{{Budd-Chiari syndrome}}
{{Budd-Chiari syndrome}}
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{{CMG}}; {{AE}}{{Mazia}}


==Overview==
==Overview==
Medical therapy can be used for short-term [[Symptomatic|symptomatic relief]] in Budd-Chiari syndrome. However, the use of such medical therapy alone is associated with a high [[Mortality rate|2-year mortality rate]]. A low-[[sodium]] diet is recommended for [[patients]] with Budd-Chiari syndrome. This helps in effective control of [[ascites]]. [[Symptomatic]] treatment includes [[diuretics]] to control [[ascites]], [[anticoagulants]] such as [[heparin]] and [[warfarin]] for [[Hypercoagulable states|hypercoagulable state]] and [[Antifibrinolytic Agent|antifibrinolytic agents]].


A minority of patients can be treated medically with [[sodium]] restriction, [[diuretics]] to control ascites, anticoagulants such as [[heparin]] and [[warfarin]], and general symptomatic management. The majority of patients require further intervention. Milder forms of Budd-Chiari may be treated with surgical shunts to divert blood flow around the obstruction or the liver itself. Shunts must be placed early after diagnosis for best results. The [[transjugular intrahepatic portosystemic shunt]] (TIPS) is similar to a surgical shunt. It accomplishes the same goal but has a lower procedure-related mortality, which has led to a growth in its popularity. Patients with stenosis or vena caval obstruction may benefit from [[angioplasty]]. <ref>Fisher NC, McCafferty I, Dolapci M, Wali M, Buckels JA, Olliff SP, Elias E. Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting. Gut. 1999 Apr;44(4):568-74.</ref> Limited studies on [[thrombolysis]] with direct infusion of [[urokinase]] and [[tissue plasminogen activator]] (tPA) into the obstructed vein have shown moderate success in treating Budd-Chiari syndrome; however, it is not routinely attempted.
==Medical Therapy==
*The therapy for Budd-Chiari syndrome is aimed at alleviating the [[obstruction]]. Underlying conditions are aggressively treated.<ref name="pmid24923240">{{cite journal |vauthors=Copelan A, Remer EM, Sands M, Nghiem H, Kapoor B |title=Diagnosis and management of Budd Chiari syndrome: an update |journal=Cardiovasc Intervent Radiol |volume=38 |issue=1 |pages=1–12 |year=2015 |pmid=24923240 |doi=10.1007/s00270-014-0919-9 |url=}}</ref><ref name="pmid23742773">{{cite journal |vauthors=Sun YL, Fu Y, Zhou L, Ma XX, Wang ZW, Wu Y |title=Staged management of Budd-Chiari syndrome caused by co-obstruction of the inferior vena cava and main hepatic veins |journal=HBPD INT |volume=12 |issue=3 |pages=278–85 |year=2013 |pmid=23742773 |doi= |url=}}</ref>
*Patients with Budd-Chiari syndrome are treated according to the severity of the [[disease]]. Treatment options include:
**Initial medical therapy
**[[Endovascular surgery|Endovascular procedure]] to restore [[vessel]] patency include:
***[[Angioplasty]]
***[[Stenting]]
***Local [[thrombolysis]]
**[[Transjugular intrahepatic portosystemic shunts|Transjugular intrahepatic portosystemic shunt]] ([[Transjugular intrahepatic portosystemic shunt|TIPS]])
**[[Liver transplantation]]
 
===Medical therapy===
*Medical therapy can be used for short-term [[Symptomatic|symptomatic relief]]. However, the use of such medical therapy alone is associated with a high [[Mortality rate|2-year mortality rate]].
*A [[Low sodium diet|low-sodium diet]] is recommended for [[patients]] of Budd-Chiari syndrome. This helps in effective control of [[ascites]].
*[[Symptomatic treatment]] includes [[diuretics]] to control [[ascites]], [[anticoagulants]] such as [[heparin]] and [[warfarin]] for [[Hypercoagulable states|hypercoagulable state]] and [[Antifibrinolytic Agent|antifibrinolytic agents]].
===Anticoagulation===
*[[Anticoagulation]] is recommended in all patients of BCS to [[Prevention|prevent]] progression of the [[thrombosis]].
*[[Anticoagulation]] with [[Low molecular weight heparin|low molecular weight heparin LMWH]] should be initiated without delay soon after [[diagnosis]]. The risk of associated [[bleeding]] [[complications]] is comparable to [[patients]] with [[anticoagulation therapy]] for other indications.
*[[Anticoagulation]] is maintained with a target value of Anti-[[Factor Xa|Xa]] between 0.5 and 0.8 IU/ml.
*The goal is to maintain [[INR]] between 2.5 and 3 monitored by regular [[INR]] testing.
*[[Prothrombin time (PT)|Prothrombin time]] and [[activated partial thromboplastin time]] are measured once [[anticoagulation]] is started and should be maintained within the [[therapeutic range]].
*Before switching from [[low molecular weight heparin]] ([[LMWH]]) to [[Anticoagulants|oral anticoagulants]], all [[contraindications]] have to be ruled out and a complete [[diagnostic]] workup has to be completed.
===Thrombolysis===
*[[Thrombolytic|Thrombolytic agents]] include [[streptokinase]], [[urokinase]], [[Rt-PA|recombinant tissue-type plasminogen activator (rt-PA)]].
*[[Thrombolysis|Local thrombolysis]] performed by an [[interventional radiologist]] is preferable over [[Thrombolysis|systemic thrombolysis]].
*[[Systemic]] or intra-[[arterial]] [[thrombolysis]] in BCS has to be administered locally into the [[hepatic vein]], [[inferior vena cava]], and [[Transjugular intrahepatic portosystemic shunt|TIPS]] in case of [[Thrombosis|acute thrombosis]].


==References==
==References==
{{Reflist|2}}


{{Reflist|2}}
[[Category:Disease]]
[[Category:Gastroenterology]]
[[Category:Gastroenterology]]
[[Category:Hepatology]]
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Latest revision as of 19:04, 27 December 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

Medical therapy can be used for short-term symptomatic relief in Budd-Chiari syndrome. However, the use of such medical therapy alone is associated with a high 2-year mortality rate. A low-sodium diet is recommended for patients with Budd-Chiari syndrome. This helps in effective control of ascites. Symptomatic treatment includes diuretics to control ascites, anticoagulants such as heparin and warfarin for hypercoagulable state and antifibrinolytic agents.

Medical Therapy

Medical therapy

Anticoagulation

Thrombolysis

References

  1. Copelan A, Remer EM, Sands M, Nghiem H, Kapoor B (2015). "Diagnosis and management of Budd Chiari syndrome: an update". Cardiovasc Intervent Radiol. 38 (1): 1–12. doi:10.1007/s00270-014-0919-9. PMID 24923240.
  2. Sun YL, Fu Y, Zhou L, Ma XX, Wang ZW, Wu Y (2013). "Staged management of Budd-Chiari syndrome caused by co-obstruction of the inferior vena cava and main hepatic veins". HBPD INT. 12 (3): 278–85. PMID 23742773.