先天性肛门直肠畸形直肠末端PGP905和S-100蛋白表达的临床研究

目的研究先天性肛门直肠畸形末端肠壁内神经系统发育情况，探讨术后排便功能障碍的病理机制。方法收集2001年1月至2005年12月先天性肛门直肠畸形结肠盲端标本76例（包括高位11例，中位26例，低位39例）。记录平均胎龄、出生体重及取得直肠标本时的年龄。采用免疫组织化学方法检测PGP9．5和S-100蛋白在上述标本中的表达水平。结果不同类型先天性肛门直肠畸形患儿出生时胎龄和平均体重比较无统计学意义。PGP9．5和S-100在高位畸形患儿中的表达明显低于中位畸形（高位VS中位，PGP9．5：0．422±0．008VS0．447±0．006，P值=0．0226；S-100：0．417±0．009VS0．442±0．007，P值=0．0401）和低位畸形（高位VS低位，PGP9．5：0．422±0．008VS0．450±0．009，P值=0．0331；S-100：0．417±0．009VS0．447±0．010，P值=0．0436）；中位和低位畸形比较，差异无统计学意义（P〉0．05）；中位畸形一期手术组直肠末端PGP9．5和S-100的表达明显低于分期手术组（PGP9．5：0．421±0．010vs0．453±0．006，P值：0．0128；S-100：0．413±0．010vs0．449±0．006，P值=0．0048）；低位畸形26例男性患儿取得直肠盲端标本时的平均年龄为（0．11±0．08）个月，13例女性患儿平均年龄为（6．15±0．29）个月（P〈0．0001），但直肠末端PGP9．5和S-100的表达差异无统计学意义（P〉0．05）。结论先天性肛门直肠畸形末端肠壁内神经系统的发育程度与畸形位置密切相关，这可能是高位畸形术后排便障碍的发生机制之一，中位畸形盲端肠壁内神经系统的发育可随年龄逐渐趋向于成熟。

Clinical study on the expression of PGP 9.5 and S-100 protein in the rectal end with congenital anorectal malformations

Objective To study the enteric nervous system （ENS） in the rectal end with ctinical different types of anorectal malformations （ARMs） and explore the possible pathological mechanisms of postoperative defecation disorders. Methods 76 cases of rectal end samples came from the patients with ARMs from January 2001 to December 2005, of which high-type was in 11 cases, intermediate-type in 26 cases, and low-type in 39 cases. The mean fetal age, birth weight and the age acquiring the rectal samples were analyzed and the expression of PGP 9.5 and S-100 protein in above samples were visualized by immunohistochemistry. Results There were no significant differences in the mean fetal age and birth weight among the different types of ARMS （p 〉 0.05）. The expression of PGP 9.5 and S-100 protein in the high-type anomalies was obviously lower than that in the intermediate- （high-type vs intermediate-type, PGP9.5：0.422 ± 0.008 vs 0.447 ± 0.006, P = 0.0226; S-100：0.417 ± 0.009 vs 0.442 ± 0.007, P=0.0401） and low-type anomalies （high-type vs low-type, PGP9.5：0.422 ± 0.008 vs 0.450± 0.009, P = 0.0331; S-100：0.417 ± 0.009 vs 01447 ± 0.010, P = 0.0436）, but there were no marked differences between the intermediate- and low-type anomalies （P 〉 0.05）. The expression of PGP 9.5 and S-100 protein in the one-stage procedure group of the intermediate-type anomalies was obviously lower than that in the staged-procedure group （PGP＇ 9.5：0.421 ± 0.010 vs 0.453 ± 0.006, P = 0.0128; S-100：0.413 ± 0.010 vs 0.449 ± 0.006, P = 0.0048）. There was a significant difference in the age acquiring the rectal samples between the male group and the female group of the low-type anomalies （0,11 ± 0.08 months vs 6.15 ±0.29 months, P 〈 0.0001）, but there were no statistical differences in the expression of PGP 9.5 and S-100 protein among the male group and the female group （P 〉 0.05）. Conclusions There is a close relation between the developmental extent of the ENS and the abnormal position