关键词: AC-BCS, Acute on chronic BCS BCS, Budd-Chairi Syndrome CR, Complete response CTP, Child-Pugh score EVL, Endoscopic variceal band ligation GAVE, Gastric antral vascular ectasia GI, gastrointestinal GOV1/2, Gastroesophageal varices 1 and 2 HVOTO, Hepatic venous outflow tract obstruction INR, International Normalised Ratio IVC, inferior vena cava thrombosis LMWH, low-molecular-weight heparin LoR, Loss of response MELD, Model for end stage liver disease MoR, Maintenance of response NR, Nonresponse PHG, portal hypertensive gastropathy PR, Partial response SBP, Spontaneous bacterial peritonitis Warfarin budd-chiari syndrome cirrhosis portal hypertension

来  源:   DOI:10.1016/j.jceh.2021.10.148   PDF(Pubmed)

Abstract:
UNASSIGNED: There is lack of data on long-term outcomes of patients with Budd-Chairi Syndrome (BCS) treated with medical therapy including anticoagulation alone.
UNASSIGNED: Consecutive patients (N = 138, mean [standard deviation, SD] age 29.3 [12.9] years; 66 men) with BCS, treated with medical therapy alone including anticoagulation, with minimum follow-up of 12 months were included. Initial response was classified as complete (CR), partial (PR) or nonresponse (NR) and on follow-up as loss of response (LoR) or maintenance of response (MoR). The association of baseline, clinical and biochemical parameters with different responses was evaluated.
UNASSIGNED: Seventy-six patients (55.1%) had CR, 26 (18.8%) had PR and 36 (26.1%) had NR. None with PR or NR had CR later. At a median follow-up of 40 (range 12-174) months, LoR was more common in PR group than in CR group (12 [46.2%] vs 18 [23.7%], P = 0.03). LoR was associated with presence of ascites (odds ratio [OR] 1.5; 95% confidence interval [CI] 0.06-0.71), gastrointestinal bleed (OR 1.33; 95% CI 0.09-0.82) or jaundice (OR 1.01; 95% CI 0.11-0.97) at baseline and duration of follow-up (OR 0.018; 95% CI 1.006-1.030). Mortality was higher in NR (28 [77.8%]) compared with CR (15 [19.7%], P = 0.001) and PR (8 [30.8%], P = 0.001). On binary logistic regression analysis, presence of ascites at baseline was associated with LoR (OR 0.303 [0.098-0.931]).
UNASSIGNED: Patients with initial CR have better survival than nonresponders. One-third had LoR on follow-up. The presence of ascites at baseline is associated with LoR.
摘要:
缺乏接受包括单独抗凝在内的药物治疗的Budd-Chairi综合征(BCS)患者的长期预后数据。
连续患者(N=138,平均值[标准差,SD]年龄29.3[12.9]岁;66名男性)患有BCS,仅接受药物治疗,包括抗凝治疗,纳入最少随访12个月.初始反应被分类为完全(CR),部分(PR)或无应答(NR),并作为应答丧失(LoR)或应答维持(MoR)进行随访。基线的关联,评估了具有不同反应的临床和生化参数.
76例患者(55.1%)有CR,26例(18.8%)有PR,36例(26.1%)有NR。具有PR或NR的人后来都没有CR。在中位随访40(范围12-174)个月时,LoR在PR组比CR组更常见(12[46.2%]vs18[23.7%],P=0.03)。LoR与腹水的存在相关(比值比[OR]1.5;95%置信区间[CI]0.06-0.71),基线和随访期间的胃肠道出血(OR1.33;95%CI0.09-0.82)或黄疸(OR1.01;95%CI0.11-0.97)(OR0.018;95%CI1.006-1.030)。NR(28[77.8%])死亡率高于CR(15[19.7%],P=0.001)和PR(8[30.8%],P=0.001)。在二元逻辑回归分析中,基线时腹水的存在与LoR相关(OR0.303[0.098-0.931]).
初始CR患者的生存率优于无反应者。三分之一的人在后续行动中有LoR。基线时腹水的存在与LoR相关。
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