关键词: BCS, Budd-Chiari Syndrome CT, Computed Tomography CTP, Child-Turcotte-Pugh INR, International Normalized Ratio IVC, Inferior Vena Cava JAK-2, Janus Kinase-2 MELD, Model for End-Stage Liver Disease MRI, Magnetic Resonance Imaging MTHFR, Methylene tetrahydrofolatereductase PNH, Paroxysmal Nocturnal Hemoglobinuria PT, Prothrombin Time TEG, Thromboelastography aPTT, Partial Thromboplastin Time cirrhosis gastrointestinal hemorrhage hepatic venous outflow tract obstruction portal hypertension varices

来  源:   DOI:10.1016/j.jceh.2018.10.002   PDF(Sci-hub)   PDF(Pubmed)

Abstract:
UNASSIGNED: Budd-Chiari Syndrome (BCS) is considered a thrombophilic state, and most patients with BCS have thrombophilic disorder. Liver dysfunction-related coagulopathy makes coagulation function unpredictable in BCS. Thromboelastography (TEG) assesses the dynamics, strength, and stability of clot formation. We conducted a pilot study using TEG to evaluate coagulation status in patients with BCS.
UNASSIGNED: Fifty-one patients with newly diagnosed BCS (age 32.3 [10.7] years; 23 men) underwent TEG (TEG®5000 Hemostasis Analyzer®, USA), and its components were analyzed and correlated with clinical profile and thrombophilic disorders. Patients who had received anticoagulation, antiplatelet drugs, or radiological intervention were excluded.
UNASSIGNED: Twenty-nine patients had normal TEG, 11 had procoagulant TEG, and 11 had hypocoagulant TEG. Among patients with hypocoagulant TEG, Coagulation Index (CI) was < -3 in 11 patients, R was >8 min in 6 patients, K was >3 min in 9 patients, alpha <55 in 9 patients, and MA <51 in 7 patients; among those with hypercoagulant TEG, CI was >3 in 3 patients, R < 2 min in 2 patients, K <1 min in 2 patients, alpha >78 in none, and MA >69 mm in 7 patients. TEG findings were similar in patients with and without thrombophilic disorder. The mean platelet count (1.75, 2.22, and 1.79 × 105/mm3; P = 0.13) and international normalized ratio (1.27, 1.34, and 1.28, P = 0.69) were similar in those with procoagulant, normal, and hypocoagulant TEG. Two patients in Rotterdam class-III had abnormal LY30. Other clinical parameters did not correlate with TEG findings.
UNASSIGNED: Patients with BCS are heterogeneous with respect to coagulation status, with one-fifth of patients are hypocoagulant on TEG. Patients with advanced disease may have accelerated fibrinolysis.
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