左肾静脉压迫综合征的临床随访

左肾静脉压迫综合征是指行走于腹主动脉和肠系膜上动脉之间夹角处的左肾静脉受压,从而引起血尿、蛋白尿等一系列临床表现的综合征。本文从临床随访的角度,对该病的一些共识问题进行总结,以期引起临床的重视。     

小儿血尿与左肾静脉压迫综合征

左肾静脉压迫综合征又称胡桃夹症是指左肾静脉在穿越肠系膜上动脉和主动脉之间受压而导致左肾静脉高压和扩张。这是引起儿童非肾小球性血尿的一个重要原因,其产生血尿的机制为扩张的左肾静脉与肾盂肾盏粘膜下血管有交通支相通,当左肾静脉瘀血时粘膜下静脉窦内压力上升而破裂,血液进入肾集合系统。     

儿童左肾静脉压迫综合征的临床诊断

左肾静脉压迫综合征是指由于左肾静脉于腹主动脉和肠系膜上动脉之间受压迫而引起的某些临床症状及尿液检查异常。它可引起血尿、蛋白尿及生殖静脉综合征等临床表现。目前对该症的诊断尚无统一意见。腹部B超对其诊断有意义。本症的诊断应结合临床症状及其他有关实验室检查以排除其他血尿/蛋白尿的原因,尤其不应忽视肾实质性病变,以免延误诊断和治疗。     

左肾静脉压迫综合征1例

患儿，男。3岁4个月，以反复肉眼血尿半年余为主诉来诊。半年前无明显诱因出现肉眼血尿，感冒后症状加重，无其他伴随表现。在当地医院治疗，仍反复出现肉眼血尿。查体：一般情况可，血压正常，全身及眼睑无水肿，心肺听诊无异常。腹软，肝脾不大，无压痛及反跳痛，神经系统检查未见异常。实验室检查：血常规正常，尿红细胞（＋＋＋），蛋白微量。左肾静脉彩超示：腹主动脉前方的左肾静脉内径倦主动脉左侧的肾静脉内径〉3。诊断为左肾静脉压迫综合征。嘱其定期来医院复查，随访至今，患儿病情较稳定。     

重症左肾静脉压迫综合征的手术治疗

重症左肾静脉压迫综合征需要外科治疗。外科治疗包括手术治疗和介入治疗。介入治疗为左肾静脉狭窄处支架置放术。手术治疗有2种方法,肠系膜上动脉移位术和左肾静脉移位术。前者指切断肠系膜上动脉后下移至左肾静脉下方与腹主动脉行端侧吻合;后者指切断左肾静脉后下移5cm,与下腔静脉端侧吻合。本院行左肾静脉移位术治疗3例,均获成功,在手术方法上有所创新,取得一定经验。     

左肾静脉压迫综合征与血尿关系探讨

单纯性血尿是儿科肾脏病常见症状之一，临床上可有多种病因，其中部分非肾小球性血尿可由于左肾静脉压迫综合征又称胡桃夹现象所致，本症临床表现为血尿或蛋白尿，病程迁延，反复发作，但多有自愈倾向，无需特殊治疗。然而，我们在临床工作中发现，不少左。肾静脉受压患者同时存在其他。肾脏疾患，现对我院70例经B超检查存在左。肾静脉受压患儿进行回顾性分析，就如何评价左。肾静脉受压与血尿的关系进行研讨。     

左肾静脉压迫综合征50例彩色多普勒超声表现

目的　探讨彩色多普勒超声对左肾静脉压迫综合征 (NCS)的诊断价值。方法　利用彩色多普勒血流显像对 5 0例NCS患儿进行检查 ,并总结其声像图特点。结果　本组 5 0例均见左肾静脉受压征象 ,受压部位前可见扩张 ,仰卧位时扩张部位前后径 (a) /受压部位前后径 (b) >3.2 ,扩张部位血流速度 (V1) >受压部位血流速度 (V2 )。脊柱后伸位 15min后a/b >5 ,V1>V2 ,俯卧位 15min后扩张消失或减轻 ,V2 增加 ,呈动态变化。彩超检测狭窄部位血流信号变细甚至缺失 ,狭窄前方左肾静脉 (LRV)血流信号呈花色血流。结论　彩色多普勒超声检查可直观观察左肾静脉受压征象 ,为临床提供准确信息     

中西医结合治疗左肾静脉压迫综合征30例

目的探讨中西医结合治疗左肾静脉压迫综合征的效果。方法左肾静脉压迫综合征患儿50例随机分为二组。对照组20例常规口服VitE,1、2次/d,每次20～50mg,同时予对症处理,避免劳累及受凉;治疗组30例在上述基础上加用中成药通心络胶囊口服,每次1、2粒,1、2次/d。二组均治疗0.5年为1个疗程。结果对照组治愈1例,显效5例,无效14例,总有效率为30%;治疗组治愈15例,显效12例,无效3例,总有效率为90%,二组总有效率比较差异显著（χ^2=14.39P〈0.01）。结论中西医结合治疗左肾静脉压迫综合征可缩短疗程,大部分可避免手术痛苦。     

左肾静脉压迫综合征误诊分析

左肾静脉综合征又称胡桃夹现象，是左肾静脉在腹主动脉和肠系膜上静脉形成的夹角内受到机械性挤压而引起的反复发作性血尿和（或）蛋白尿。该病临床表现缺乏特异性，临床上常被误诊为肾小球肾炎或不能明确诊断，故对胡桃夹现象认识非常重要。现对误诊的病例做如下分析。[第一段]     

Outcomes of the Modified Norwood Procedure: Hypoplastic Left Heart Syndrome Versus Other Single-Ventricle Malformations

Recent studies have suggested that survival after the modified Norwood procedure is influenced by anatomy and is worse for patients with hypoplastic left heart syndrome (HLHS) than for patients with other forms of functional single ventricle. This study aimed to compare the outcomes of the modified Norwood procedure for these two groups at a single center. A retrospective chart review compared modified Norwood procedure outcomes between children with HLHS (n = 40) and children with other single-ventricle malformations (n = 19) from January 2002 to August 2012. The HLHS patients had a lower mean age (10.0 ± 13.2 days) at the time of operation than the non-HLHS patients (16.5 ± 18.5 days). The difference was not significant. The number of patients with a Sano modification was significantly higher in the HLHS group (HLHS 85 % vs non-HLHS 32 %; p < 0.001). The follow-up data did not differ between the groups (HLHS 3.6 ± 3.2 years vs non-HLHS 4.8 ± 3.3 years, nonsignificant difference). The overall operative survival was 85 %: 83 % for the patients with HLHS and 89 % for the patients with non-HLHS (nonsignificant difference). The Kaplan–Meier transplant-free survival estimate at 10 years was 73 % and did not differ between the HLHS patients (67 %) and the non-HLHS patients (84 %, nonsignificant difference). The timing of stages 2 and 3 palliation (bidirectional Glenn and external conduit Fontan) did not differ significantly (HLHS, 7.0 ± 2.8 months and 3.0 ± 0.5 years vs non-HLHS 6.1 ± 1.7 months and 2.8 ± 0.8 years). The two groups were similar with respect to the need for post-Norwood extracorporeal membrane oxygenation support, aortic arch reinterventions, atrioventricular valve repair, pulmonary artery reconstruction, pacemaker implantation, and progression to bidirectional Glenn and Fontan completion. The modified Norwood reconstruction provides good palliation for HLHS as well as other forms of functional single-ventricle anatomy. Survival, postoperative morbidity, and progression to Glenn and Fontan are not significantly different.     

Clinical Course and Renal Function in Minimal Change Nephrotic Syndrome

Thirteen unselected children with the minimal change nephrotic syndrome were followed for 11 months-7.5 years, during which time 32 renal function tests were performed. Glomerular filtration rate (GFR) was determined as clearance of inulin using a standard clearance technique. The children had between zero and 34 relapses and three developed steroid resistance. Three patients had severe but reversible complications related to the corticosteroid therapy. Chlorambucil was given to four patients, of whom three with steroid resistance had lasting remissions, whereas one patient with frequent relapses had only transient improvement. The GFR was decreased at the first episode in six patients, whose clinical course did not differ from that of the others. None had a decreased GFR later in the course of the disease. It is concluded that reduced GFR at the onset of the minimal change nephrotic syndrome is reversible and does not imply an unfavourable outcome.     

Exercise Performance Following Repair of Hypoplastic Left Heart Syndrome: A Comparison with Other Types of Fontan Patients

Reports of exercise performance after Fontan surgery for hypoplastic left heart syndrome (HLHS) are lacking. We compared the exercise performance of total cavopulmonary connection type (TCPC) of Fontan subjects with HLHS (group 1, n= 7) to those not requiring a Norwood procedure having a systemic right ventricle (group 2, n= 6) or a systemic left ventricle (group 3, n= 8). The subjects underwent assessment of resting pulmonary mechanics followed by maximal exercise testing with a bicycle or treadmill protocol. ECG, oxygen consumption, and carbon dioxide production were measured continuously. There was not a significant difference seen between HLHS and the comparison groups for the following parameters: maximum heart rate, maximum oxygen consumption, respiratory exchange ratio, breathing reserve, and arterial oxygen saturation at rest or exercise. Exercise performance in the TCPC type of Fontan patients was comparable regardless of ventricular morphology or surgical approach.     

A Case of Wolf-Hirschhorn Syndrome and Hypoplastic Left Heart Syndrome

Wolf-Hirschhorn Syndrome (WHS) is a genetic syndrome that includes a typical facial appearance, mental retardation, growth delay, seizures, and congenital cardiac defects. A deletion of the terminal band of the short arm of chromosome 4, with a breakpoint at the 4p15 to 4p16 region, is the most common genetic mutation causing WHS. Congenital heart disease associated with WHS typically includes atrial and ventricular septal defects, though there are a few case reports of associated complex congenital heart disease. Here we report a case of an infant with a large 4p deletion, with a breakpoint at the 4p12 region, and hypoplasic left heart syndrome. We discuss a possible link between the size of the chromosomal deletion in WHS and the severity of the cardiac defect.