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细胞外基质蛋白、细胞凋亡和 c-kit 阳性细胞在肾盂输尿管连接部梗阻发病机制中的作用。

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

机构信息

Fırat University Faculty of Medicine, Department of Pediatric Surgery, Elazığ, Turkey.

出版信息

J Pediatr Urol. 2010 Apr;6(2):125-9. doi: 10.1016/j.jpurol.2009.07.011. Epub 2009 Aug 28.

DOI:10.1016/j.jpurol.2009.07.011
PMID:19716769
Abstract

AIM

To investigate histopathological changes in ureteropelvic junction obstruction (UPJO) from an etiological perspective.

PATIENTS AND METHODS

Medical 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.

RESULTS

Control 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.

CONCLUSIONS

High 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.

摘要

目的

从病因学角度探讨肾盂输尿管连接部梗阻(UPJO)的组织病理学变化。

患者与方法

回顾性分析 UPJO 患者的病历资料并采集病理标本,同时选取法医尸检的肾切除标本作为对照。光镜下评估标本,免疫组化检测纤维连接蛋白、IV 型胶原、层粘连蛋白、Bax 和 Bcl-2 的表达情况,以及 c-kit 确定的 Cajal 间质细胞。半定量评估染色评分,采用 Mann-Whitney U 检验进行结果评估。

结果

对照组包括 14 例儿童(中位年龄 3.5 岁,6 个月至 17 岁),研究组包括 22 例 UPJO 患儿(中位年龄 9 个月,1 个月至 10 岁)。所有患者 UPJ 的平滑肌中均可见非特异性炎症、上皮增生和萎缩伴纤维化。纤维连接蛋白、IV 型胶原和层粘连蛋白在 UPJO 的平滑肌束内空间和基质中显著增加。Bax 和 Bcl-2 的表达在 UPJO 中增加。c-kit 无法对 Cajal 间质细胞进行染色,但对肥大细胞的染色显著。

结论

纤维连接蛋白、层粘连蛋白和 IV 型胶原的高表达可能与 UPJO 的发病机制有关。输尿管芽分支和小管形成过程中肾脏形态发生缺陷可能是导致这种先天性病变的原因。

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