先天性巨结肠组织中SOX-10、GDNF表达及意义

Expression and Significance of SOX-10 and GDNF in Hirschsprung's Disease

【目的】探讨先天性巨结肠(HD)中SOX-10、GDNF两种神经标记物在患儿不同肠管表达的免疫组化特征。【方法】选取本院收治的30例HD患儿的临床资料(观察组),分别取其扩张段、移行段及狭窄段肠管组织标本,选择同期本院收治的10例因肛门闭锁行结肠造瘘还纳术患儿作为对照组,行常规病理学检查,采用免疫组化检测两组肠管的SOX-10、GDNF蛋白的表达,并分析其表达与其临床病理特征的关系。【结果】30例HD患儿中有28例狭窄段黏膜下和肌间神经丛未见明显SOX-10阳性细胞,2例仅见少量表达,正常组肠管、HD扩张段和移行段均分别可见SOX-10弱阳性、阳性或强阳性表达的神经节及胶质细胞,但各组间表达强度亦有不同。SOX-10各组神经丛表达面积正常组大于移行段组,而狭窄段未见表达。HD扩张段、HD移行段、HD狭窄段SOX-10阳性表达累积光密度显著低于正常对照组,差异均具有统计学意义(P<0.05)。GDNF抗体主要表达于神经节细胞、神经纤维和平滑肌细胞。GDNF在神经节细胞及平滑肌细胞表达阳性,但神经节细胞表达强度高于平滑肌细胞,所有标本均可见神经节细胞或平滑肌细胞阳性表达,但神经节细胞GDNF表达强度均比平滑肌表达强。在狭窄段中可见GDNF阳性表达神经纤维增生,形态扭曲呈波浪状,神经纤维半径也比其余组别神经纤维大。【结论】HD患儿病变段结肠的SOX-10及GDNF蛋白较正常肠管及扩张段表达明显降低,GDNF、SOX-10联合检测有助于辅助诊断HD,对识别病变段肠管具有重要意义,且手术切除范围至少大于移行段。

【objective】To investigate the immunohistochemical characteristics of SOX-10 and GDNF in different intestinal tracts of children with Hirschsprung's disease(HD).【Methods】The clinical data of 30 children with HD from our Hospital were selected.The specimens of dilated segment,transitional segment and narrow segment were taken from the observation group.At the same time,10 children who underwent colostomy due to anal atresia in the same period were selected as the control group,and routine pathological examination was performed.The expression of SOX-10,GDNF protein in the intestine of the two groups was detected by immunohistochemistry.The relationship between's expression and clinicopathological features was analyzed.【Results】Among 30 cases of HD,SOX-10 positive cells were not found in 28 cases of narrow segment submucosal and myenteric plexus,but only a little expression was found in 2 cases.In the normal group,the weakly positive,positive or strongly positive ganglia and glial cells were observed in the intestinal canal,HD dilated segment and transitional segment,respectively,but the expression intensity of SOX-10 was also different among the groups.The expression area of SOX-10 nerve plexus in the normal group was larger than that in the transitional group?but the expression of SOX-10 nerve plexus in narrow segment was not clear.The cumulative optical density of SOX-10 positive expression in HD dilated segment,HD transitional segment and HD narrow segment was significantly lower than that in the normal control group(P<0.05).GDNF antibodies were mainly expressed in ganglion cells,nerve fibers and smooth muscle cells.GDNF was positive in ganglion cells and smooth muscle cells,but the expression intensity of ganglion cells was higher than that of smooth muscle cells.Ganglion cells or smooth muscle cells were positive in all specimens,but the expression intensity of GDNF in ganglion cells was stronger than that in smooth muscle cells.In the narrow segment,the positive expression of GDNF was seen in nerve fibers proliferation.The shape of nerve fibers was wavy and the radius of nerve fibers was larger than that of other groups.【Conclusions】The expression of SOX-10 and GD-NF protein in diseased colon of children with HD was significantly lower than that in normal and dilated colon.