肛门直肠畸形直肠末端肠壁组织蛋白酶D及外周蛋白的表达  

Expression of cathepsin D and peripherin in the rectal end of anorectal malformations

作　　者：李薇[1] 耿其明[1] 张杰[1] 陈焕[1] 吕小逢[1] 路长贵[1] 蒋维维[1] 唐维兵[1] 徐小群[1] 

机构地区：[1]南京医科大学附属南京儿童医院新生儿外科,江苏南京210008

出　　处：《江苏大学学报（医学版）》2011年第4期353-356,362,F0003,共6页Journal of Jiangsu University：Medicine Edition

摘　　要：目的:研究肛门直肠畸形患儿直肠末端肠壁内神经节细胞的发育情况,探讨术后排便障碍的病理机制。方法:肛门直肠畸形患儿直肠末端标本72例,其中高位无肛12例,中间位无肛36例,低位无肛24例,分别对应为高位组、中间位组和低位组;选择非肠神经相关疾病行结肠手术的患儿10例,取其结肠壁作为对照标本。采用免疫组织化学法检测直肠末端组织蛋白酶D(cathepsin D,CD)和外周蛋白在肠壁中的表达情况。结果:高位组CD的表达(0.0040±0.00082)明显低于对照组(0.049±0.0045,P<0.01);中间位组、低位组与对照组比较,CD表达亦有减少,但差异无统计学意义;高位组CD的表达较低位组(0.043±0.0094)显著减少(P<0.05);高位组与中间位组及中间位组与低位组分别比较,差异无统计学意义。高位组、中间位组及低位组外周蛋白的表达均显著低于对照组,尤以高位组最著;高位组外周蛋白的表达(0.0047±0.00055)较中间位组(0.020±0.0029)、低位组(0.030±0.0032)显著减少(P<0.01);中间位组与低位组比较,外周蛋白的表达差异无统计学意义。结论:肛门直肠畸形患儿直肠末端肠壁内神经节细胞的发育程度与直肠盲端位置高低密切相关,直肠盲端位置越高,肠壁神经节细胞发育越差,这可能是中、高位无肛畸形术后排便障碍的发生机制之一。Objective： To study the ganglion cells in the rectal end with different types of anorectal malformations（ARMs） and explore the possible pathological mechanisms of defecation disorders after operations.Methods： Seventy-two cases of rectal end samples came from the patients with ARMs of which high-type was in 12 cases,intermediate-type in 36 cases,and low-type in 24 cases.Ten cases of colon coming from the patients without enteric nervous-related diseases after colonic operations were as the control group.The expression of cathepsin D（CD） and peripherin in above samples were visualized by immunohistochemistry.Results： The expression of CD in the high-type anomalies was obviously lower than that in the control group（0.0040±0.00082 vs 0.049±0.0045,P0.01）.The average percentage of positive areain both intermediate-type and low-type was lower than the control group,but with no significant differences.The expression of CD in the high-type was obviously lower than the low-type（0.0040±0.00082 vs 0.043±0.0094,P0.05）.No statistical differences can be seen between the high-type and intermediate-type,as well as between the intermediate-type and low-type.The expression of peripherin in the high-type,intermediate-type and low-type was obviously lower than that in the control group,especially in the high-type anomalies.The expression of peripherin in the high-type was significantly lower than the intermediate-type and low-type（0.0047±0.00055 vs 0.020±0.0029,P0.01;0.0047±0.00055 vs 0.030±0.0032,P0.01）.There were no marked differences between the intermediate-type and low-type in the expression of peripherin.Conclusion： The development of the ganglion cells in the rectal end of ARMs was affected to a certain extent,compared with the normal.There is a close relation between the developmental extent of the ganglion cells and the abnormal position of the ARMs,which may be one of the pathological mechanisms of postoperative defecation disorders in the high-type anomalies.

关 键 词：肛门直肠畸形 神经节细胞 组织蛋白酶D 外周蛋白 

分 类 号：R726.2[医药卫生—儿科]