【作者】 孙劲松；

【导师】 林涛；

【作者基本信息】 重庆医科大学 ， 儿科学， 2010， 硕士

【摘要】 目的:对比胶原纤维、神经纤维与Cajal间质细胞在正常肾盂输尿管连接部(UPJ)及先天性肾盂输尿管连接部梗阻(UPJO)所致肾积水患者肾盂输尿管连接部狭窄段的分布,探讨UPJO所致肾积水的发病机制及其临床意义。方法:1.标本采集:2008.4-2009.5期间我院住院病人,30例肾盂输尿管连接部狭窄段组织标本,术前均经临床诊断为肾盂输尿管连接部梗阻所致肾积水,且术中证实为肾盂输尿管连接部狭窄,术后病理诊断符合先天性肾盂输尿管连接部狭窄所致肾积水。患儿年龄7个月～11岁,平均4.7岁,男18例,女12例。同时收集11例肾肿瘤患儿的无肿瘤细胞浸润的肾盂输尿管连接部作为对照。年龄9月～8岁,平均3.2岁,男7例,女4例。全部标本常规固定石蜡包埋组织切片备用。2.每例标本均进行HE染色及Masson染色,观察组织学变化。采用石蜡切片SP免疫组化方法检测两组标本中的神经丝蛋白及C-Kit的表达。3.观察神经纤维和Cajal间质细胞的分布并计数。用SPSS 11.5统计软件包对比分析两组表达情况。结果:1.对照组HE染色及Masson染色见平滑肌内纵外横排列规则,无纤维组织增生。而病变段UPJ肌层纤维组织明显增生,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。2.对照组的平滑肌层可见较密集的神经纤维丝蛋白免疫阳性表达,神经纤维呈横向或纵向排列。在病变段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,弯曲。统计分析其分布密度与对照组相比,其差异具有显著性(P<0.01)。3.对照组UPJ肌层可见大量C-kit表达阳性的Cajal间质细胞,细胞胞体较大,呈纺锤状或星形,有2个或2个以上的细长突起,胞质内可见棕色颗粒样物质,细胞核大,核周胞质少。应用甲苯胺蓝染色后不呈现异染性。而病变段UPJ肌层仅见少量Cajal间质细胞呈C-kit阳性表达,甚至部分标本C-kit免疫反应呈阴性表达。统计分析其分布密度与正常对照组存在差异性(P<0.01)。结论:1.胶原纤维、神经纤维与Cajal间质细胞分布异常是先天性肾盂输尿管连接部梗阻的重要病理改变。2.神经因素(神经纤维与Cajal间质细胞减少)可能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。3.临床治疗上,应彻底切除肾盂输尿管连接部病变段,减少术后复发。更多还原

【Abstract】 OBJECTIVE:Comparing the distribution of collagen fibers, nerve fibers and interstitial cells of Cajal in the ureteropelvic junction (UPJ) between congenital hydronephrosis patients and control children, discussed the pathogenesis and the clinical significance of congenital ureteropelvic junction obstruction (UPJO) in the etiology of congenital hydronephrosis.MATERIALS AND METHODS:1. Between 2008.4-2009.5, 30 specimen of ureteropelvic junction stenosis tissue were collected. All patients were clinically diagnosed as UPJO, confirmed by surgery and pathological diagnosis. Patients aged from 7 months to 11 years old, with an average 4.7-year-old, 18 males and 12 females. At the same time, 11 children of renal tumors (found no tumor cells infiltrate in UPJ) selected as controls, whose aged from 9 months to 8 years old, with an average 3.2-year-old, 7 males and 4 females. All samples were fixed and paraffin-embedded.2. HE and Masson staining were carried out to investigate the changes of anatomy and collagen fibers. By using immunohistochemical SP method, neurofilament protein and C-Kit expression were detected.3. Observed the distribution and counted the nerve fibers and the interstitial cells of Cajal. Used the SPSS 11.5 Statistical Package to compared the difference between two groups.RESULTS:1. The muscle arrange as inner longitudinal and outer horizontal, and no significant fibrous proliferation in the control group. In UPJ stenosis tissue, fibrous hyperplasia and muscular hypertrophy were found by HE and Masson staining, among which existing obvious thicker collagen hyperplasia, and the diameter of lumen is markly shorter than that of control.2. Nerve fibers exist widespread in control group, in which a stronger expression of microfilaments protein in the smooth muscle layer, nerve fibers were arranged horizontally or vertically. However, microfilaments proteins were negative in most specimens of the lesion segment, only a few samples showed weak positive expression with abnormal morphology. Compared with the control group, the difference was significant (P <0.01).3. In control group, there exsit a large number of C-kit-positive interstitial cells of Cajal. Cajal cells showing spindle-shaped or star-shaped, with two or more than 2 slender processes, brown particles in cytoplasm, large nucleus and small perinuclear cytoplasm. Application of toluidine blue staining, it did not showed as metachromatic. Only a few interstitial cells of Cajal were C-kit expression in muscular lesion segment of UPJ. Compared with the control group, the difference was significant (P <0.01).CONCLUSIONS:1. Collagen fibers, nerve fibers and abnormal distribution of interstitial cells of Cajal are all pathological changes in congenital UPJO.2. Neural elements (decreased nerve fibers and interstitial cells of Cajal) may play an important role in the etiology and pathogenesis of congenital UPJO. 3. Clinically, in order to reduce recurrence, ureteropelvic junction stenosis should be totally removed.更多还原