摘要: The management of the Budd-Chiari syndrome improved dramatically during last 10 years and includes less invasive diagnostic modalities using modern imaging, identification a myeloproliferative disorder in 20 to 50% patients V617F JAK2 mutation, graduate therapeutic strategy. common association with thrombotic is reason for thorough work-up (myeloproliferative disorder, defect C or S protein, factor V Leiden, II antiphosholipid syndrome, other disorders). Ultrasonography should be performed by an experimented examiner, informed suspicion. 5-year survival rate treated this contemporary approach (anticoagulation, treatment underlying cause, recanalization, transjugular intrahepatic portosystemic shunting, liver transplantation) above 80%.
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