Budd-Chiari syndrome

(World J Hepatol 2016;8:691)

詳見Redefining Budd-Chiari syndrome: A systematic review.

• 病因是陣發性睡眠性血紅蛋白尿症 (PNH)。 PNH 是一種造血幹細胞獲得性疾病，

  • 血管內溶血
  • 靜脈血栓

• 所有 BCS 患者都應考慮對這種血液病進行檢測。

• introduction of Budd-Chiari syndrome

• Hepatic outflow obstruction

• 2/2 occlusion of hepatic vein(s) or IVC → sinusoidal congestion and portal HTN.

• Can be 1° (eg, thrombosis) or 2° (eg, extravascular compression).

• ~50% due to myeloprolif

• a/w JAK2 mutations (P. vera, etc.),

• hypercoag states (systemic, OCP, pregnancy),

• tumor invasion (HCC, renal, adrenal),

• trauma,

• idiopathic

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• hepatomegaly, RUQ pain, ascites, dilated venous collaterals, acute liver failure
• diagnostic test of Budd-Chiari syndrome:
• Treatment: Rx underlying condition, anticoag (LMWH → warfarin); consider thrombolysis if acute; angioplasty & stent if short stenosis; consider TIPS or DIPS (U/S-guided direct intrahepatic portosystemic shunt) (if other methods fail to treat sx of portal HTN); liver transplant if ALF or failed other options