OBJECTIVES:We described morphologic characteristics, particularly pulmonary an atomy, and determined the prevalence of definitive end states and their determin ants in children with pulmonary atresia associated with v...OBJECTIVES:We described morphologic characteristics, particularly pulmonary an atomy, and determined the prevalence of definitive end states and their determin ants in children with pulmonary atresia associated with ventricular septal defec t(PAVSD). BACKGROUND: Pulmonary atresia associated with ventricular septal defec t represents a broad morphologic spectrum that greatly influences management and outcomes. METHODS: From 1975 to 2004, 220 children with PAVSD presented to our institution. Blinded angiographic review(n=171) characterized bronchopulmonary s egment arterial supply. RESULTS:A total of 185 patients underwent surgery,and re pair was definitive in 75%. Initial operations included systemic-pulmonary art ery shunt in 57%, complete primary repair in 31%, or right ventricular outflow tract reconstruction in 12%. Based on angiographic review,118 patients had sim ple PAVSD and 53 patients had PAVSD with major aortopulmonary collateral arterie s(MAPCAs). Overall survival from initial operation was 71%at 10 years. Risk fac tors for death after initial operation included younger age at repair, earlier b irth cohort, fewer bronchopulmonary segments supplied by native pulmonary arteri es, and initial placement of a systemic-pulmonary artery shunt. Competing risks analysis for initially palliated patients predicted that after 10 years, 68%ac hieved complete repair(with associated factors including later birth cohort and more bronchopulmonary segments supplied by native pulmonary arteries), 22%died without repair, and 10%remained alive without repair. Reoperations after comple te repair occurred in 38 children(27%), with risk factors including older age a t palliation, MAPCAs, and more segments supplied by collaterals. CONCLUSIONS: Ou tcomes in children with PAVSD have improved over time, and are better in complet ely repaired cases. Bronchopulmonary arterial supply is an important determinant of mortality, achievement of definitive repair, and post-repair reoperation.展开更多
目的探讨儿童上尿路结石术后复发现状,分析结石术后复发的危险因素,并建立列线图预测儿童上尿路结石复发风险。方法回顾性分析2014年6月至2020年12月在首都医科大学附属北京友谊医院泌尿外科接受手术治疗的218例上尿路结石患儿临床资料...目的探讨儿童上尿路结石术后复发现状,分析结石术后复发的危险因素,并建立列线图预测儿童上尿路结石复发风险。方法回顾性分析2014年6月至2020年12月在首都医科大学附属北京友谊医院泌尿外科接受手术治疗的218例上尿路结石患儿临床资料,采用多因素Cox回归模型筛选患儿的临床资料及随访资料,使用Kaplan-Meier法绘制结石复发曲线,依据独立危险因素构建列线图预测模型,并评价模型的预测效能。结果218例中,男157例、女61例,年龄(4.1±3.4)岁,随访(48.3±21.8)个月,总体复发率为16.1%(35/218)。Cox多因素回归分析结果显示:3岁以上较1岁以下患儿的复发风险高(HR=8.685,95%CI:1.144~65.930);肥胖较消瘦患儿复发风险高(HR=7.903,95%CI:1.046~59.684);结石质量>2000 Hu·cm^(2)较结石质量<1000 Hu·cm^(2)患儿复发风险高(HR=2.842,95%CI:1.171~6.895);术前尿白细胞阳性较术前尿白细胞阴性患儿复发风险高(HR=5.172,95%CI:1.681~15.913)。以上述4项独立危险因素为输入变量建立列线图预测模型,经计算该模型C-index为0.853,1年未复发率预测模型的受试者操作特征曲线下面积(area under curve,AUC)为0.98,3年未复发率预测模型的AUC为0.96,5年未复发率预测模型的AUC为0.84,校正曲线贴近于理想曲线。结论儿童上尿路结石术后具有较高的复发率,高龄、肥胖、结石质量大及术前尿白细胞阳性患儿具有较高的复发风险。本研究建立的儿童上尿路结石术后复发风险列线图模型有助于临床医师更精准地识别高危患儿,制定个性化结石防治方案。展开更多
文摘OBJECTIVES:We described morphologic characteristics, particularly pulmonary an atomy, and determined the prevalence of definitive end states and their determin ants in children with pulmonary atresia associated with ventricular septal defec t(PAVSD). BACKGROUND: Pulmonary atresia associated with ventricular septal defec t represents a broad morphologic spectrum that greatly influences management and outcomes. METHODS: From 1975 to 2004, 220 children with PAVSD presented to our institution. Blinded angiographic review(n=171) characterized bronchopulmonary s egment arterial supply. RESULTS:A total of 185 patients underwent surgery,and re pair was definitive in 75%. Initial operations included systemic-pulmonary art ery shunt in 57%, complete primary repair in 31%, or right ventricular outflow tract reconstruction in 12%. Based on angiographic review,118 patients had sim ple PAVSD and 53 patients had PAVSD with major aortopulmonary collateral arterie s(MAPCAs). Overall survival from initial operation was 71%at 10 years. Risk fac tors for death after initial operation included younger age at repair, earlier b irth cohort, fewer bronchopulmonary segments supplied by native pulmonary arteri es, and initial placement of a systemic-pulmonary artery shunt. Competing risks analysis for initially palliated patients predicted that after 10 years, 68%ac hieved complete repair(with associated factors including later birth cohort and more bronchopulmonary segments supplied by native pulmonary arteries), 22%died without repair, and 10%remained alive without repair. Reoperations after comple te repair occurred in 38 children(27%), with risk factors including older age a t palliation, MAPCAs, and more segments supplied by collaterals. CONCLUSIONS: Ou tcomes in children with PAVSD have improved over time, and are better in complet ely repaired cases. Bronchopulmonary arterial supply is an important determinant of mortality, achievement of definitive repair, and post-repair reoperation.
文摘目的探讨儿童上尿路结石术后复发现状,分析结石术后复发的危险因素,并建立列线图预测儿童上尿路结石复发风险。方法回顾性分析2014年6月至2020年12月在首都医科大学附属北京友谊医院泌尿外科接受手术治疗的218例上尿路结石患儿临床资料,采用多因素Cox回归模型筛选患儿的临床资料及随访资料,使用Kaplan-Meier法绘制结石复发曲线,依据独立危险因素构建列线图预测模型,并评价模型的预测效能。结果218例中,男157例、女61例,年龄(4.1±3.4)岁,随访(48.3±21.8)个月,总体复发率为16.1%(35/218)。Cox多因素回归分析结果显示:3岁以上较1岁以下患儿的复发风险高(HR=8.685,95%CI:1.144~65.930);肥胖较消瘦患儿复发风险高(HR=7.903,95%CI:1.046~59.684);结石质量>2000 Hu·cm^(2)较结石质量<1000 Hu·cm^(2)患儿复发风险高(HR=2.842,95%CI:1.171~6.895);术前尿白细胞阳性较术前尿白细胞阴性患儿复发风险高(HR=5.172,95%CI:1.681~15.913)。以上述4项独立危险因素为输入变量建立列线图预测模型,经计算该模型C-index为0.853,1年未复发率预测模型的受试者操作特征曲线下面积(area under curve,AUC)为0.98,3年未复发率预测模型的AUC为0.96,5年未复发率预测模型的AUC为0.84,校正曲线贴近于理想曲线。结论儿童上尿路结石术后具有较高的复发率,高龄、肥胖、结石质量大及术前尿白细胞阳性患儿具有较高的复发风险。本研究建立的儿童上尿路结石术后复发风险列线图模型有助于临床医师更精准地识别高危患儿,制定个性化结石防治方案。
