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对于仅接受药物治疗的布加综合征患者,就诊时存在腹水与缺乏长期缓解相关:一项单中心真实世界经验。

Presence of Ascites at Presentation is Associated With Absence of Long-Term Response Amongst Patients With Budd-Chiari Syndrome When Treated With Medical Therapy Alone: A Single Centre Real-Life Experience.

作者信息

Agrawal Dhiraj, Gupta Deepak, Nathani Rohit, Dhore Prashant, Meshram Megha, Bhatia Shobna J, Shukla Akash

机构信息

Department of Gastroenterology, Seth GSMC and KEM Hospital, Mumbai 400 012, India.

出版信息

J Clin Exp Hepatol. 2022 May-Jun;12(3):861-870. doi: 10.1016/j.jceh.2021.10.148. Epub 2021 Nov 3.

Abstract

BACKGROUND

There is lack of data on long-term outcomes of patients with Budd-Chairi Syndrome (BCS) treated with medical therapy including anticoagulation alone.

METHODS

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.

RESULTS

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%],  = 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%],  = 0.001) and PR (8 [30.8%],  = 0.001). On binary logistic regression analysis, presence of ascites at baseline was associated with LoR (OR 0.303 [0.098-0.931]).

CONCLUSION

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

背景

关于布加综合征(BCS)患者单纯接受包括抗凝治疗在内的药物治疗的长期预后数据匮乏。

方法

纳入连续的138例BCS患者(平均[标准差,SD]年龄29.3[12.9]岁;66例男性),这些患者仅接受包括抗凝治疗在内的药物治疗,且随访时间至少为12个月。初始反应分为完全缓解(CR)、部分缓解(PR)或无反应(NR),随访时分为反应丧失(LoR)或反应维持(MoR)。评估基线、临床和生化参数与不同反应之间的关联。

结果

76例患者(55.1%)达到CR,26例(18.8%)达到PR,36例(26.1%)为NR。PR或NR患者均未随后达到CR。在中位随访40(范围12 - 174)个月时,PR组LoR比CR组更常见(12例[46.2%]对18例[23.7%],P = 0.03)。LoR与基线时存在腹水(比值比[OR]1.5;95%置信区间[CI]0.06 - 0.71)、胃肠道出血(OR 1.33;95%CI 0.09 - 0.82)或黄疸(OR 1.01;95%CI 0.11 - 0.97)以及随访持续时间(OR 0.018;95%CI 1.006 - 1.030)相关。NR组死亡率(28例[77.8%])高于CR组(15例[19.7%],P = 0.001)和PR组(8例[30.8%],P = 0.001)。二元逻辑回归分析显示,基线时存在腹水与LoR相关(OR 0.303[0.098 - 0.931])。

结论

初始达到CR患者的生存率高于无反应者。三分之一患者在随访时出现LoR。基线时存在腹水与LoR相关。

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Etiology, management, and outcome of the Budd-Chiari syndrome.布加综合征的病因、治疗及预后
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