重度先天性上睑下垂的手术治疗

目的分析额肌筋膜瓣悬吊术和异体阔筋膜悬吊术矫正重度先天性上睑下垂的优缺点。方法对重度先天性上睑下垂66例87眼进行手术治疗,其中额肌筋膜瓣悬吊术30例45眼,异体阔筋膜悬吊术36例42眼,分析术后上睑下垂矫正效果、上睑弧度、双重睑成形及暴露性角膜炎发生情况。结果额肌筋膜瓣悬吊术取材方便、复发率较低、上睑弧度匀称;阔筋膜悬吊手术简单、重睑形成良好,但取材困难、复发率稍较高。结论额肌筋膜瓣悬吊术和阔筋膜悬吊术均能有效治疗重度先天性上睑下垂,前者无需特殊材料,术后效果较稳定。     

Echocardiogram‐gated multislice spiral CT in functionally univentricular heart following long‐lasting Waterston–Cooley anastomosis

Waterston–Cooley anastomosis may be carried out in patients with tricuspid atresia to provide pulmonary perfusion. It is associated with several complications, including preferential blood flow to the right lung, hypoplasia of the left pulmonary artery, obstruction of the anatomosis or rupture of pulmonary aneurysms. We study a patient with thrombosis in the pulmonary arteries following surgical construction of a Waterston shunt in childhood. Imaging findings and clinical symptoms are discussed with emphasis on echocardiogram‐gated multislice spiral CT.     

先天性肾盂输尿管连接部狭窄患儿平滑肌细胞凋亡及Bcl-xl,Bad表达检测

目的:检测先天性肾盂输尿管连接部狭窄患儿平滑肌细胞凋亡及Bcl-xl,Bad的表达.方法:采用免疫组化SABC法检测30例先天性肾盂输尿管连接部狭窄患儿肾盂输尿管连接部狭窄段(观察组)与其周围相应正常输尿管(对照组)平滑肌中凋亡调控基因Bcl-xl,Bad的表达;用末端脱氧核苷酸转移酶(TdT)介导的d-UTP生物素缺口末端标记技术(TUNn)检测2组平滑肌凋亡细胞.结果:观察组平滑肌细胞中Bad表达率高于对照组((15.54±3.18)%vs(11.00±2.07)%,P＜0.05),Bcl-xl在2组平滑肌细胞中均不表达;观察组平滑肌细胞凋亡指数高于对照组((21.60±3.12)%vs(10.20±2.18)%,P＜0.05).结论:先天性肾盂输尿管连接部狭窄平滑肌细胞减少与其细胞凋亡及Bad表达升高有关,与Bcl-xl表达无关.     

Recent progress of pediatric cardiac surgery in China

Pediatric cardiac surgery in China has madegreat progress since the first successful open heart operation for correction of ventricular septal defect （VSD） with an artificial heart-lung machine in 1958.1 In recent years, the state has paid much attention to both the basic research and the primary, secondary and tertiary prevention of congenital heart disease. The surgical treatment for congenital heart diseases in infancy was listed as one of the Family Planning Technology Specific Projects among the Key Tasks in the Tenth Five-Year Plan. Due to economic and social conditions in China primary or secondary prevention and treatment of congenital heart disease are still in the early phase of development. Therefore, tertiary prevention -- surgical treatment--is still of crucial importance. Here we review the current status of pediatric cardiac surgery in China and highlight the latest advances in this exciting field.     

Congenital hepatoportal arteriovenous fistula: Report of a case

(Received for publication on Nov. 20, 1998; accepted on Sept. 17, 1999)     

Trisomy 21 with XYY

A case of double aneuploidy involving chromosome 21 and Y is reported in an eight-month-old infant with developmental delay and failure to thrive. Patient had all classical phenotypical features of trisomy 21 except, absence of epicanthal folds. The diagnosis was confirmed by cytogenetic study performed on peripheral blood leucocyte culture using G-banding. Literature review revealed only 17 cases of XYY and trisomy 21 reported so far. No such case is reported in Indian literature. Relevant literature is reviwed and possible effects of trisomy 21 on XYY and that of XYY on trisomy 21 has been discussed. A routine chromosomal study even in patient with classical features of Down syndrome has been advocated. Interestingly, our patient also had left to right shunts at atrial and ductal level and tricuspid regurgitation. Given the rarity of the disorder and scanty published data the incidence, phenotype and recurrence risk are difficult to establish.     

先天性肾积水肾脏细胞凋亡的病理及其临床意义

目的　检测先天性肾积水肾脏细胞凋亡的发生 ,并探讨其临床意义。方法　应用电镜、DNA原位缺口末端标记法及DNA电泳检测 15例先天性肾积水患儿患肾细胞凋亡 ,其中轻、中、重度肾积水各 5例。结果　先天性肾积水肾脏有细胞凋亡发生 ,可见细胞核周边染色质凝集及凋亡小体。重度肾积水肾脏平均凋亡指数为 0 .0 94± 0 .0 17,中度肾积水为 0 .0 3 3± 0 .0 17,轻度肾积水为0 .0 0 2± 0 .0 0 3 ,三者相比具有显著性意义 ,并且随着肾积水程度的加重 ,凋亡细胞数增多。 1例中度肾积水和 5例重度肾积水肾组织电泳带型呈典型的“梯形结构”。结论　肾细胞凋亡可能参与了先天性肾积水肾脏的损伤     

Gd-DTPA增强动态核磁共振诊断输尿管不全梗阻的实验研究

目的：探讨Gd-DTPA增强动态核磁共振成像（MRI）诊断输尿管不全梗阻的意义。方法：将新生鼠输尿管交界处理于腰大肌中制成输尿管不全梗阻模型（n=10)。1年后用Gd-DTPA增强动态MRI检查梗阻组和对照组（n＝12）鼠的肾脏，并对不同时间记录的肾脏影像进行分析，计算出肾皮质，髓质和肾盂的相对信号强度。结果：使用Gd-DTPA后，对照组肾后质，髓质和肾盂信号强度相继减弱，30－60min后基本恢复，梗阻肾脏的变化明显较对照组慢，60min后仍未恢复。结论Gd-DTPA增强动态MRI可用于诊断输尿管不全梗阻并提供肾功能变化信息。     

先天性肾积水转化生长因子转录水平的研究

目的：检测先天性肾积水患肾转化生长因子β1（TGF－β1）mRNA表达的改变，探讨TGF－β1在小儿先天性肾积水肾损伤中的作用及其临床意义。方法：采用RT－PCR技术检测15例先天性肾积水患儿患肾实质组织，肾盂组织，肾盂输尿管狭窄段组织TGF－β1mRNA的表达，经凝胶分析系统半定量，和10例正常肾，肾盂及输尿管对照.结果：患肾组织，肾盂及肾盂输尿管狭窄段中TGF－β1mRNA的表达增高，结论：先天性肾积水患儿患肾组织TGF－β1表达增高，TGF－β1表达的增高可能和先天性肾积水所致的慢性肾损伤及间质纤维化有关。