Budd-Chiari综合征介入术后复发临床分析

目的观察Budd-Chiari综合征(BCS)介入术后复发患者的临床特点。方法回顾性分析49例复发BCS患者的临床资料及治疗预后情况。根据预后分为死亡组(n=11)和生存组(n=38),再将生存组分为再次复发组(n=14)与未再次复发组(n=24),分别计算各组病例Child-Pugh评分、Clichy指数、Rotterdam指数和New-Clichy指数,以ROC曲线衡量各指标预测患者预后的价值。结果根据ROC曲线,New-Clichy指数的截断点为4.37,New-Clichy指数>4.37者死亡率为62.50%(10/16),New-Clichy指数<4.37者死亡率为3.03%(1/33),死亡组New-Clichy指数显著高于生存组(P=0.038)。再次复发组平均年龄(P=0.016)、胆红素水平(P=0.014)、Clichy指数(P=0.042)明显低于未再次复发组。结论对于多数BCS介入术后复发患者,介入治疗仍属安全、有效、可行。New-Clichy指数对预测复发BCS介入术后死亡事件有较高价值。

Validation of prognostic indices in Egyptian Budd-Chiari syndrome patients: A single-center study

AIM To compare predictive ability of Budd-Chiari syndrome(BCS) prognostic indices(PIs) for one-year survival and Transjugular intrahepatic portosystemic shunt(TIPS) patency.METHODS This retrospective study enrolled 194 Egyptian patients with primary BCS who presented to the BuddChiari Study Group of Ain Shams University Hospital. Calculation of the available PIs was performed using Child-Pugh and model for end-stage liver disease scores, BCS-specific PIs(Clichy, New Clichy andRotterdam) for all patients, and BCS-TIPS PI only for patients who underwent TIPS. The overall one-year survival rate and the one-year shunt patency rate for TIPS were reported.RESULTS The overall one-year survival rate was 69.6%, and the New Clichy PI revealed the best validity for its prediction at a cut-off value of 3.75, with sensitivity and specificity of 78% and 73.3%, respectively [area under receiver operating characteristic curve(AUC) = 0.806]. The one-year survival rate post-TIPS was 89.7%, and the BCS-TIPS score demonstrated validity for its prediction at a cut-off value of 3.92(sensitivity and specificity were 71.4% and 64.5%, respectively)(AUC = 0.715). Logistic regression analysis revealed that the New Clichy PI(P = 0.030), high serum total bilirubin(P = 0.047) and low albumin(P < 0.001) were independent factors for predicting mortality within one year. The one-year shunt patency rate in TIPS was 80.2%, and none of the PIs exhibited significant validity for its prediction. CONCLUSION The New Clichy score could independently predict the one-year survival in Egyptian BCS patients.

蕈中毒:综述

尽管很罕见,但暴露于鹅膏蕈碱对人体健康有很大的影响,其罕见性也给临床医生的诊断也带来了挑战,医疗工作者很少对这一疾病进行报道。蕈中毒目前并无特定的解药。或许是因为罕见,又或者是缺少关注,美国在蕈中毒的诊断、治疗及研究方面落后于欧洲近30年。因此,美国有必要成立一个中心培训机构,并加强相关研究和数据收集工作,从而为蕈中毒患者提供更好的治疗。