Cajal细胞在先天性肾盂输尿管连接部梗阻中的分布及意义

目的研究Cajal细胞(interstitial cells of Cajal,ICC)在先天性肾盂输尿管连接部梗阻(congenital ureteropelvic junction obstruction,UPJO)中的分布及意义。方法以UPJO患儿术中UPJ组织标本为实验组,因非肾积水原因引产或死胎患儿UPJ组织标本为对照组,应用ICC特异的C-kit抗体行免疫组织化学染色,运用t检验比较两组C-kit阳性ICC细胞的分布情况。结果 C-kit阳性细胞主要存在于组织肌层。与对照组相比,UPJO组C-kit阳性细胞的数量及密度均明显减少,两组C-kit阳性细胞数及平均光密度值差异有统计学意义(P值均0.05)。结论与对照组相比,新生儿UPJO的C-kit阳性细胞数量及密度明显减少,提示ICC减少可能与UPJO的发生有关。     

Cajal间质细胞在先天性肾盂输尿管连接部梗阻的表达及意义

目的探讨Cajal间质细胞（ICC）在先天性肾盂输尿管连接部梗阻（ureteropelvic junction obstruction，UPJO）中的表达及意义。方法病例组22例，均为非管腔外压迫所致的先天性UPJO患儿梗阻段标本。对照组15例为肾盂输尿管连接部（ureteropelvic junction，UPJ）切缘无肿瘤细胞浸润的Wilms瘤患儿标本。应用c-kit抗体免疫组织化学SABC法检测2组UPJ组织ICC的表达，并用图像进行定量分析比较。结果ICC主要表达于UPJ肌层。UPJO组肌层ICC表达的面积为（14．968±1.473）×10^4，低于对照组的（16．303±1.576）×10^4，P〈0．01；UPJO组肌层ICC表达的平均光密度为0．227±0．021，低于对照组的0．242±0．024，P〈0．05。组间比较差异均有统计学意义。结论ICC减少在UPJO的病因和发病机制中起重要作用。     

维生素D及其受体与肾盂输尿管连接部狭窄相关性的初步研究

目的初步分析维生素D及其受体与肾盂输尿管连接部狭窄(ureteropelvic junction obstruction,UPJO)发生的相关性。方法选择2016年3月1日至2016年10月31日上海交通大学医学院附属上海儿童医学中心小儿泌尿外科收治的因UPJO行离断式肾盂输尿管成形术的患儿为UPJO组(n=10),因肾脏肿瘤行肾与输尿管切除术的患儿为对照组(n=8)。收集两组术前血标本检测维生素D三项(25-羟维生素D 2、25-羟维生素D 3及总25-羟维生素D的含量)。取两组术中标本采用ELISA法,检测维生素D受体(vitamin D receptor,VDR)、转化生子因子(transforming growth factor-β,TGF-β)及钙黏蛋白E的含量。结果两组术前血标本中25-羟维生素D 2、25-羟维生素D 3及体内总25-羟维生素D的含量均无统计学差异(P>0.05)。UPJO组VDR、TGF-β、钙黏蛋白E的平均值分别为(771.6±70.57)ng/每克总蛋白,(131.0±10.33)ng/每克总蛋白及(125.5±10.98)ng/每克总蛋白;对照组分别为(2128±251.8)ng/每克总蛋白,(82.3±6.834)ng/每克总蛋白及(386.6±78.71)ng/每克总蛋白。两组UPJ标本中VDR、TGF-β、钙黏蛋白E水平的差异有统计学意义(P<0.05)。结论UPJO组VDR表达量显著低于对照组,提示VDR可能与UPJO发生相关,初步推测其机制可能是肾盂输尿管连接部VDR表达量减少引起TGF-β表达量明显升高,使得肾盂输尿管连接部的组织纤维化加重,进而导致或加重UPJO。     

先天性肾盂输尿管连接部梗阻上皮屏障缺陷表型及其分子机制研究

目的:通过检测先天性肾盂输尿管连接部梗阻(ureteropelvic junction obstruction, UPJO)患儿梗阻段组织上皮屏障结构分子、缺氧诱导因子和血管内皮标志分子的分布和表达情况,了解UPJO梗阻段上皮屏障缺陷和缺氧表型,探讨尿路上皮细胞屏障缺陷和缺氧的相关性。方法:收集2019年1～8月复旦大...     

先天性肾盂输尿管连接部梗阻突触素和神经丝蛋白表达的研究

目的 通过先天性肾盂输尿管连接部梗阻(UPJO)突触素和神经丝蛋白的表达,对UPJO神经肌肉连接进行研究,探讨UPJ0发病机制.方法 应用免疫组化技术对20例先天性.肾盂输尿管连接部梗阻患儿和10例肾母细胞瘤患儿的标本在神经肌肉连接处行突触素和神经丝蛋白标记,光镜下观察其免疫反应表达.结果 对照组突触素和神经丝蛋白的免疫反应阳性表达,实验组突触素和神经丝蛋白免疫反应呈阴性表达.结论 UPJO狭窄部位突触素和神经丝蛋白的异常表达与UPJO的发生有关,病变段输尿管平滑肌处于失神经支配状态,Cannon定律是UPJO病理生理合理的解释.     

儿童先天性肾盂输尿管连接部梗阻性肾积水手术后分肾功能变化的短期随访研究

目的 了解单侧肾盂输尿管连接部梗阻(ureteropelvic junction obstruction,UPJO)手术后短期患侧分肾功能(differential renal function,DRF)缓解效果. 方法 选择2019年3月至2019年10月首都医科大学附属北京儿童医院收治的单侧UPJO行肾盂成形术患者...     

先天性肾盂输尿管连接部梗阻的临床病理学分析

目的采用病例对照研究的方法来探讨肾盂输尿管连接部Cajal间质细胞(interstitial cell of cajal,ICC)以及代表信息传递的神经组织的改变与先天性肾积水发病机制的关系及其临床意义。方法收集2013年01月至2014年12月由本院确诊的30例非管腔压迫或新生物所致先天性肾盂输尿管连接部梗阻(Ureteropelvic junction obstruction,UPJO)患儿为病例组;选择同期11例无肿瘤细胞浸润肾盂输尿管连接部的肾肿瘤患儿作为对照组。所有标本观察组织学改变、神经纤维和ICC标记物的变化。结果病例组肌层肥厚,排列紊乱、稀疏,其间可见大量增粗胶原纤维。与对照组相比,管腔明显狭窄;与对照组比较,病例组平滑肌神经纤维丝蛋白大部分标本呈阴性表达,仅少部分标本呈弱阳性表达,且神经纤维异常增粗。病例组神经纤维分布密度为(1.51±0.39),对照组为(3.79±0.48),差异有统计学意义(t=15.36,P0.01);病例组UPJ肌层仅见少量ICC呈C-kit阳性表达,甚至部分标本C-kit免疫反应呈阴性表达。病例组ICC标记物分布密度为(1.70±1.24),对照组为(9.09±1.76),差异有统计学意义(t=15.099,P0.01)。结论神经纤维与Cajal间质细胞减少在先天性肾盂输尿管连接部梗阻的病因和发病机制中起重要作用,术中应彻底切除肾盂输尿管连接部病变段,以减少术后复发。     

分层次多点悬吊技术在腹腔镜肾盂成形术中的应用研究

目的 探讨分层次多点悬吊技术在腹腔镜肾盂成形术(laparoscopic pyeloplasty,LP)治疗肾盂输尿管连接部梗阻(ureteropelvic junction obstruction,UPJO)中的应用价值.方法 以山东大学齐鲁医院2017年3月至2020年12月期间接受LP治疗的138例单侧UPJO患...     

儿童肾盂输尿管连接部梗阻患者尿液中MMP-7和MMP-9的临床意义

目的 探讨尿液中MMP-7和MMP-9对儿童肾盂输尿管连接部梗阻(ureteropelvic junction obstruction,UPJO)的诊断价值.方法 本研究共纳入新疆维吾尔自治区人民医院24例单侧UPJO患者作为病例组,其中左侧18例、右侧6例,平均年龄5.8岁(37天至15岁),20例完成肾动态显像.1...     

肾盂输尿管连接部梗阻合并同侧膀胱输尿管连接部梗阻的诊断与治疗

目的 肾盂输尿管连接部梗阻(ureteropelvic junction obstruction,UPJO)和膀胱输尿管连接部梗阻(ureterovesical junction obstruction,UVJO)这两个最常见的儿童泌尿系统病理状态同时存在较少见.本文对单侧UPJO合并同侧UVJO的诊断与治疗进行探讨.方法 回顾性分析上海交通大学医学院附属新华医院2012年1月至2015年7月间手术治疗的UPJO合并UVJO患儿.术前常规行泌尿系超声、同位素利尿肾图(DR)及排泄性膀胱尿道造影(VCUG)等检查.结果 我院共诊治单侧UPJO合并UVJO的43例患儿,失访2例.11例患儿术前明确诊断;41例患儿先行肾盂成形术+肾造瘘术,其中10例术后复查发现输尿管末端狭窄自行缓解,28例再次接受输尿管膀胱再植术,3例患儿行肾盂成形术+肾造瘘术后复查核素提示分肾功能低于10％,给予患肾切除;38例患儿术复查肾积水程度和肾脏功能均得到显著改善.结论 单侧UPJO合并UVJO的术前诊断非常困难,术中应仔细检查,避免遗漏同时合并存在的病理改变.术前尽量通过超声、DR及MR等影像检查作出明确诊断,进而制定个体化的治疗方案,能够获得满意的治疗效果.如术前明确有UPJO的存在,应选择首先进行肾盂成形术,术后根据检查结果决定是否行输尿管膀胱再植术.     

先天性肾盂输尿管连接处梗阻性肾积水的神经性因素研究

目的探讨神经性因素在先天性肾盂输尿管连接处梗阻性肾积水发病中的作用。方法将31例患儿手术切除的肾盂输尿管连接处（实验组）、同一患儿输尿管切缘（切缘对照组）、产前排除泌尿系异常、孕32周以上引产并进行尸体解剖的胎儿30例（阴性对照组）进行病理分析。显微镜下观察神经节细胞及神经纤维的分布。分析神经节细胞和神经纤维在各组中的分布及差异。结果切缘对照组以及阴性对照组的神经纤维分布密度均多于实验组,而切缘对照组与实验对照组比较,差异无统计学意义。结论神经性因素是先天I生。肾盂输尿管连接处梗阻性肾积水的重要原因。临床应充分切除输尿管连接处狭窄段,以减少术后复发。     

Changes in Structure,Interstitial Cajal-like Cells and Apoptosis of Smooth Muscle Cells in Congenital Ureteropelvic Junction Obstruction

Objective: The goal of this study is to evaluate some structural changes in muscular, collagenous and neural components as well as expression of Cajal-like cells and apoptosis of smooth muscle cells in congenital ureteropelvic junction obstruction (UPJO). Methods: Tissue specimens were obtained from 25 patients with UPJO and compared with normal ureteropelvic junction regions of 19 autopsies. In paraffin embedded sections the amount of Cajal-like cells, density of nerve fibers and smooth muscle cell apoptosis (using immunohistochemical staining) were determined. Collagen deposition and muscular components were stained by Trichrome-Masson staining and evaluated by image analysis techniques. Arrangement of muscular bundles was also evaluated qualitatively. Findings : The number of Cajal-like cells was significantly lower in patients than in controls. The apoptotic score and mean number of nerve fibers were not statistically different for the two groups. Arrangement of muscular fibers was more irregular in patients than in controls (P<0.001). Collagen deposition was significantly higher in patients than in controls (P<0.001). The mean amount of muscular component was lower in patients than in normal ones. (P= 0.09) Conclusion: We found significant pathologic changes in congenital ureteropelvic junction obstruction such as decrease in Cajal-like cells, increase in collagen deposition and irregular arrangement of muscle fibers.Key Words: Uretero-Pelvic Junction Stenosis, Image Analysis, Immunohistochemistry, Interstitial Cell of Cajal-Like Cells     

The roles of extracellular matrix proteins,apoptosis and c-kit positive cells in the pathogenesis of ureteropelvic junction obstruction

AimTo investigate histopathological changes in ureteropelvic junction obstruction (UPJO) from an etiological perspective.Patients and methodsMedical records of patients with UPJO were reviewed and pathological specimens collected. Nephrectomy materials from forensic autopsies were taken as controls. Specimens were assessed with light microscopy. Fibronectin, type 4 collagen, laminin, Bax and Bcl-2 expression for apoptosis, together with interstitial cells of Cajal determination with c-kit were determined immunohistochemically. Staining scores were evaluated semiquantitatively. Results were evaluated using Mann–Whitney U-test.ResultsControl group comprised 14 children (median age, 3.5 years; 6 months–17 years). Study group comprised 22 children with UPJO (median age, 9 months; 1 month–10 years). Light microscopy revealed non-specific inflammation, epithelial proliferation and atrophy with fibrosis in the smooth muscle of the UPJ in all patients. Fibronectin, type 4 collagen and laminin were found to be significantly increased in UPJO at the intrafascicular space of smooth muscle and the matrix of stroma. Bcl-2 expression was increased in UPJO. c-Kit was unable to stain interstitial cells of Cajal, but staining for mast cells was significant.ConclusionsHigh expression of fibronectin, laminin and type 4 collagen may indicate a relation to the pathogenesis of UPJO. Defective kidney morphogenesis, during branching and tubulogenesis of ureteric bud, may be responsible for this congenital pathology.     

Acquired ureteropelvic junction obstruction in childhood

Ureteropelvic junction (UPJ) obstruction is generally considered to be a congenital lesion or occasionally an intermittent phenomena relating to periods of increased urine flow. Acquired ureteropelvic junction obstruction without a recognizable secondary cause is quite unusual. We present a 5.5-year-old boy who, 4 years after repair of a right sided UPJ obstruction, developed a significant left-sided UPJ obstruction in a system which previously had been normal.     

Congenital solitary kidney with obstructive uropathy

Two cases of unilateral renal agenesis presenting as obstructive uropathy in the congenital solitary kidney are reported here. One patient had anuria, hypertension urinary tract infection (UTI) and hydronephrosis due to ureteropelvic junction (UPJ) obstruction on the left side. Ultrasonography was helpful in diagnosing the condition. The second patient had hydronephrosis and hydroureter due to ureterovesical junction (UVJ) obstruction on the right side. Both recovered following corrective surgery in the form of pyeloplasty and ureteroneocystostomy respectively and continued to do well on follow up.     

Reconstructive surgery for lower pole ureteropelvic junction obstruction associated with incomplete ureteral duplication

IntroductionThe lower moiety of duplex kidney can be associated with ureteropelvic junction obstruction (UPJO). Surgical correction can be challenging in cases of incomplete duplication where the junction of the lower and upper pole ureters is proximal. We review our experience with this unusual entity with an emphasis on surgical techniques employed in reconstruction.MethodsWe retrospectively reviewed the charts of eight patients with lower pole UPJO who underwent surgery in 2002–2008. The surgical approach, specifically the utilization of the non-obstructed upper pole ureter, used in the reconstruction was noted.ResultsFour of eight patients were symptomatic at presentation with either infection or pain. UPJO was at least in part secondary to lower pole crossing vessels in four patients and was treated with dismembered pyeloplasty. Lower pole to upper pole pyeloureterostomy was necessary in four patients due to short ureteral length between the UPJ and junction of lower and upper pole ureters. No complications or obstruction of either moiety developed during 1 year of follow up.ConclusionsLower pole UPJO in incomplete renal duplication mandates individualized surgical treatment dependent upon anatomy encountered. We have found that pyeloureterostomy is a safe alternative to drainage of the obstructed lower pole.     

Fibroepithelial polyps causing ureteropelvic junction obstruction in children--a case report and review article.

Fibroepithelial polyps are extremely rare benign mesodermal tumours in children that can cause ureteropelvic junction (UPJ) obstruction. We report on a 10-year-old boy presenting with UPJ obstruction due to a fibroepithelial polyp, and review 28 similar published paediatric cases.     

Xanthogranulomatous pyelonephritis is associated with higher tissue expression of monocyte chemotactic protein-1.

TOPIC: Xanthogranulomatous pyelonephritis (XGP) is a chronic inflammation of the kidney characterized by destruction and replacement of its parenchyma with granulomatous tissue. It is associated with both chronic urinary obstruction and urinary tract infection (UTI). METHODS: We studied two children with chronic ureteropelvic junction obstruction (UPJO) and recurrent UTI nephrectomized for poor kidney function. An intraoperative renal biopsy was taken to relate the presence of infiltrating monocytes plus tubular atrophy to tissue expression of monocyte chemotactic protein-1 (MCP-1) and epidermal growth factor (EGF). XGP was diagnosed by a pathologist in both cases. RESULTS: MCP-1 expression was significantly higher in the two patients compared with the controls or patients with uncomplicated UPJO. It also correlated with the extent of monocyte infiltration, whereas EGF was only significantly downregulated when compared with the controls. CONCLUSIONS: MCP-1 would seem to play a key role in the pathogenesis of XGP by mediating the recruitment of circulating monocytes or by cells resident in the interstitial space.     

Anderson-Hynes pyeloplasty in horseshoe kidney in children: is it effective without symphysiotomy?

Contemporary reports on surgery for horseshoe kidney (HK) still recommend isthmotomy and lateropexy to complete an open pyeloplasty. To evaluate whether simple Anderson-Hynes pyeloplasty without symphysiotomy is effective for relief of ureteropelvic junction obstruction (UPJO) in HK, we studied the records of ten children, two of whom had bilateral UPJO. Only one child presented with calculi; 11 units were operated upon for UPJO, 1 needed a partial nephrectomy. The surgical outcome was evaluated with emphasis on the changes in renal drainage and function assessed by ultrasonography and diuretic renal scans. Associated vesicoureteral reflux was observed more often (25%) than with UPJO in normal kidneys. Obstruction was caused by a crossing lower-pole vessel in three cases, a high ureteral insertion in two and narrowing of the UPJ 7. Postoperative follow-up (mean 5.5 years) revealed improved renal function and good drainage in all cases. Hydronephrosis vanished in 7, whereas grade 2 hydronephrosis remained in two children with former refluxive megaureter and grade 3 in one. All children are doing well and have no symptoms due to the persistent isthmus (Rovsing syndrome). It is concluded that simple Anderson-Hynes pyeloplasty via a flank incision is a highly effective and safe procedure for treating UPJO in HK. Accepted: 13 October 1998