目的 探讨及分析不同基因型TSC患儿CR发病情况及其位置、大小及随诊变化情况.方法 回顾性分析经临床确诊并行基因检测的TSC患儿88例,按其基因检测结果分为3组:TSC1组、TSC2组及TSC阴性组,对比分析各组CR的发病率、位置分布及其大小,并对部分随诊患儿肿瘤大小变化进行相关因素分析.结果 不同基因组间CR的发生率、在心脏各腔室占比及具体位置分布均无明显统计学差异(P＞0.05).TSC阴性组CR的最大径明显小于TSC1组及TSC2组(P＜0.05).随诊患儿中CR减小或消失与初诊年龄有统计学差异(P＜0.05),ROC曲线提示14.5个月龄之前初诊CR,肿瘤缩小或消失的可能性较大.结论 超声心动图可以明确诊断CR,并对其进行随访监测.TSC各基因组间CR的发生率及位置分布无明显差异,无基因突变组CR大小明显小于基因突变组.CR初诊年龄＜14.5个月龄可作为一项预测CR缩小或消失可能性的指标.

Objective:To explore the echocardiographic diagnosis of interruption of the aortic arch (IAA) and its accompanied cardiovascular malformations and syndromes.Methods:We retrospectively analyzed the imaging and clinical data of children with IAA confirmed by CTA and/or surgery at Beijing Children's Hospital, Capital Medical University from January 2014 to December 2019 to summarize the diagnostic characteristics of IAA and its accompanied cardiovascular malformations and syndromes and explore the rules of diagnosis.Results:A total of 38 cases were included, including 26 males and 12 females. The patients ranged in age from 2 days to 9 years old, with a median age of 3 months. Among the 38 cases, type A accounted for 87% (33/38), type B accounted for 13% (5/38), and type C accounted for 0. All the 38 cases were associated with other cardiovascular malformations. A total of six malformation combinations or syndromes were found, including the triad of interrupted aortic arch (55%, 21/33), Berry syndrome (8%, 3/38), combination with permanent fifth aortic arch (21%, 8/38), Shone syndrome (8%, 3/38), Phace syndrome (3%, 1/38), and simple combination with multiple collateral vessels (5%, 2/38). The abnormal blood vessels supplying the descending artery included patent ductus arteriosus, permanent fifth aortic arch, and collateral vessels. Of the 38 cases, 34 underwent CTA examination, 27 underwent cardiac surgical treatment, 37 were correctly diagnosed by echocardiography, and one patient with Shone syndrome was misdiagnosed with coarctation of the aorta.Conclusion:Echocardiography can be used to diagnose IAA and accompanied malformations and syndromes, which can improve the accuracy of comprehensive diagnosis, reduce misdiagnosis, and provide more information for surgery.

Objective:To assess the value of echocardiography in the diagnosis and treatment of left atrial appendage aneurysm (LAAA) in children.Methods:Nine children with LAAA diagnosed by echocardiography from March 2016 to March 2021 at Beijing Children's Hospital, Capital Medical University were retrospectively selected. We summarized and analyzed their clinical data, echocardiographic findings, treatment follow-up, and prognosis results.Results:Of the nine patients with LAAA, four were male and five were female. They ranged in age from 34 days to 12 years and 11 months, and the reasons for presentation were cardiomegaly, atrial tachycardia, heart murmur, and mediastinal mass. Two-dimensional ultrasound showed a cystic anechoic structure in the lateral of the left ventricle, communicating with the left atrium, with a long diameter of (32.3±12.9) mm and a short diameter of (16.4±6.9) mm for LAAA, a round trip between LAAA and left atrial blood flow signals was observed by color Doppler, and giant LAAA could compress the left ventricle. One of the nine patients had compression of the lateral wall of the left ventricle, two had congenital heart disease, two had space-occupying lesions (one mediastinal mass and one pulmonary tumor), and one had mitral stenosis with insufficiency. There was no thrombosis, pericardial defect, or coronary artery and pulmonary vein compression. All were intrapericardial LAAAs. In one patient, the left ventricle was compressed due to the tumor, and the LAAA was surgically removed, and the shape, size, and function of the left heart were normal after surgery. One patient was treated with antiarrhythmic drugs and one patient underwent mediastinal tumor resection, and the LAAA was smaller than before in both cases.Conclusion:LAAA is a rare cardiac malformation. Echocardiography can accurately diagnose LAAA in childhood, determine whether there is thrombosis and whether it compresses adjacent structures, and help to find the causes and evaluate the therapeutic effect.

目的 探讨Shone's综合征的超声心动图特点、诊断方法及应用价值.方法 以Shone's综合征21例为研究对象,分析超声心动图特点.将左心系统畸形分为3个梗阻水平,比较超声及CT的诊断价值.术后超声随访,与术前比较,观察疗效.结果 21例中完全型2例,不完全型19例,二尖瓣瓣上环14例,降落伞型二尖瓣(PMV) 11例,主动脉瓣下狭窄10例,主动脉缩窄(COA) 15例.9例行CT检查及手术治疗,超声在左室流入道水平、主动脉瓣下及瓣膜水平诊断比CT更有优势(P＜0.05),在主动脉瓣上及主动脉弓降部水平诊断与CT无明显差异.术后超声随访,梗阻解除满意(P＜0.05).结论 超声心动图诊断Shone's综合征比CT更有优势.评估梗阻程度,需同时关注压差及解剖异常.应用超声早期诊断、准确评估对本病预后有重要意义.

目的:探讨超声心动图在新生儿持续性肺动脉高压(PPHN)诊疗评价中的应用价值.方法:选取北京儿童医院2009年7月至2020年2月临床确诊的201例PPHN患儿,回顾性分析临床及超声心动图资料.根据治疗后转归分为生存组(n=170)和死亡组(n=31),对比两组基线资料,分析影响治疗结局的相关因素.对生存组患儿进行超声心动图随访,观察疗效.结果:201例PPHN患儿中,190例(94.5％)患者的肺动脉收缩压(PASP)>50 mmHg(1mmHg=0.133 kPa).Logistic回归分析显示,酸中毒、右心室舒张末期内径/左心室舒张末期内径(RVDd/LVDd)是影响治疗转归的独立危险因素,即当PPHN患儿存在酸中毒(OR=8.288,95％CI:3.126～21.969)、RVDd/LVDd越大(OR=132.326,95％CI:3.009～5819.371),其死亡可能性越大.PPHN患者的超声心动图主要表现为右心房室增大,右心室流出道增宽,右心室壁增厚,主肺动脉及分支内径增宽;卵圆孔或动脉导管水平分流方向为"右向左"或"双向",三尖瓣反流;除外发绀型先天性心脏病.201例PPHN患儿超声心动图提示主肺动脉内径(MPAD)增宽[(10.5±1.5)mm]、RVDd增大[(9.8±1.9)mm],RVDd/LVDd增大(0.57±0.13).超声心动图随访发现,生存组PPHN患儿治疗后MPAD、RVDd及RVDd/LVDd均较治疗前减小(P<0.05),差异有统计学意义.结论:PPHN是新生儿期死亡率较高的危重症疾病,超声心动图结合临床可及时准确做出诊断,监测治疗效果.RVDd/LVDd可作为一项预测PPHN预后的超声指标应用于临床.

Objective:To study the classification of persistent fifth aortic arch (PFAA) and the value of echocardiography in the diagnosis of PFAA.Methods:A total of 16 cases (male 6, female 10, at ages from 7 days to 4 years and 2 months old, the median age was 3 months) diagnosed with PFAA in Beijing Children′s Hospital Affiliated to Capital Medical University from January 2013 to June 2019 were studied retrospectively. The diagnosis standard, differential methods and misdiagnosed analysis of different subtypes of PFAA by echocardiography were summarized and analyzed.Results:The 16 cases included 1 case of type A1 double lumen aortic arch, 8 cases of type A2 single-lumen aortic arch, 3 cases of type B1 with pulmonary atresia and 4 cases of type B3 pulmonary artery branch arising from the distal end of ascending aorta. Only one patient of double lumen aortic arch missed diagnosis by echocardiography, and the rest were accurately diagnosed by echocardiography. CTA was performed in 13 cases, including 9 cases of type A, 1 case of type B1 and 3 cases of type B3, which confirmed the echocardiography diagnosis. Seven cases of Type A2 were operated.Conclusions:PFAA is a rare and complicated aortic arch malformation, which is divided into four major classification and multiple subtypes. Echocardiography can diagnose the PFAA and its classification, it is of great clinical significance for the early diagnosis, treatment and prognosis of children.

目的 探讨超声心动图在儿童肺动脉高压(PH)病因分析中的应用.方法 以9 219例经超声心动图诊断PH的息儿为研究对象,回顾性分析临床病因.分为先天性心脏病(CHD)组和非CHD组,对比两组间PH程度分级的构成.结果 9 219例中,CHD组占77.7％:其中左向右分流型89.0％、肺静脉压力升高型7.5％、紫绀型2.9％、先天性肺动静脉异常型0.6％;非CHD组占22.3％:其中肺部疾病和(或)低氧所致PH 68.8％、动脉性PH 25.0％、左心疾病所致PH4.8％、未明多因素机制所致PH 1.3％、慢性血栓栓塞性PH 0.1％.CHD组PH分级轻度22.5％、中度46.2％、重度31.3％,以中、重度为主;非CHD组PH分级轻度49.2％、中度33.6％、重度17.2％,以轻、中度为主.两组间分级构成差异有统计学意义.结论 儿童PH主要为多种疾病引起的继发性PH,左向右分流型CHD是最常见病因,新生儿持续性肺动脉高压(PPHN)是儿童PH特有的病因.

目的 观察桂枝茯苓丸加味保留灌肠联合超声药物导入治疗盆腔炎(pelvic inflammatory disease,PID)的临床效果.方法 选取2015年1月至2017年12月首都医科大学附属北京妇产医院理疗科盆腔炎患者62例,分为两组,每组31例.对照组采用超声药物导入治疗;观察组采用桂枝茯苓加味保留灌肠联合超声药物导入治疗,10次为1个疗程,两组均治疗3个疗程.比较治疗前后两组的疼痛视觉模拟评分(visual analogue score,VAS)、中医证候评分、CRP及血液流变学指标.结果 治疗后,观察组的总显效率优于对照组(61.29％比32.26％,P＜0.05),VAS评分低于对照组[1.3(0.0,2.2)比2.3(2.1,3.3),P＜0.05],中医证候评分低于对照组[2(0,3)比3(3,5),P＜0.05].治疗后,观察组CRP、ESR、血浆黏度及红细胞比容水平明显降低,并低于对照组(P＜0.05).结论 桂枝茯苓加味保留灌肠联合超声药物导入能治疗盆腔炎,减轻炎症反应,缓解疼痛,改善血液高凝状态,临床疗效显著.

Objective To assess the clinical efficacy of acupuncture and moxibustion for luteinized unruptured follicle syndrome (LUFS) with randomized controlled trials (RCTs).Methods With the methods of evidence-based medicine,through retrieval of literatures about acupuncture and moxibustion for the treatment of LUFS,those which abided by the inclusion criteria were given qualitative evaluation and Meta-analysis by RevMan5.2.Results 13 RCTs met the inclusion criteria were included with 824 patients involved.The results of Meta analysis suggested acupuncture and moxibustion could be better than that of the control group treated with western medicine in clinical effectiveness [RR =1.60,95％ CI(1.45,1.77),P ＜ 0.01].Conclusion Acupuncture and moxibustion may have advantages in improving LUFS,but because the quality of included literatures in the study was poor,more high quality studies are needed to make the evidence stronger.

慢性盆腔炎(chronic pelvic infectious diseases,CPID)是妇科常见病,发病率为30%,好发年龄为30～40岁的妇女.多因经期或性生活不洁,分娩、流产或宫腔手术时消毒不严,感染后迁延不愈,导致盆腔结缔组织增厚、粘连甚至形成包块.临床表现主要为下腹及肛门坠胀疼痛、腰骶部酸胀疼痛、带下量增多、月经失调,重者可致不孕.

ObjectiveTo study the effect of ultrasound transdermal drug delivery combining with semiconductor laser on chronic pelvic inflammation disease(CPID).Methods80 CPID patients were divided into two groups randomly: 40 cases for treating group who accepted ultrasound transdermal drug delivery and semiconductor laser. 40 cases for the control group who accepted ultrasound transdermal drug delivery only. After 30 days of treating, a Condition-Score was assessed. ResultsIn the treating group, 18 cured, 19 remarkable effective, 3 effective, which was significant improved compared with that in the control group, 6 cases cured, 21 cases remarkably effect, 10 cases effective, 3 case ineffective(P<0.01).ConclusionUltrasound transdermal drug delivery combined with semiconductor laser is more effective on chronic pelvic inflammation disease.