肠神经畸形肠壁神经递质表达的对比研究

目的：检测先天性巨结肠症(HD)、肠神经发育不良(1ND)、肠神经元减少症(HYPG)等各类肠神经畸形(NIM)的不同肠段的一氧化氮合酶(nNOS)、血红素氧合酶-2(HO-2)和血管活性肠肽(VIP)的表达与分布，探讨它们的表达、分布差异以及对临床的指导意义。方法：使用SP免疫组织化学方法，观察12例HD、15例IND和3例HYPG患儿的不同肠段中nNOS、HO-2和VIP的分布，并将吻合口处直肠肠段与10例正常儿童结肠对比。结果：HD组中直肠的nNOS和HO-2染色阳性的纤维偶见。但VIP在黏膜下层的染色在不同病例中不尽相同；IND组直肠和扩张结肠中nNOS和VIP阳性的神经元和神经纤维有不同程度增多；HYPG组中直肠三种神经递质表达均有不同程度减少。结论：不同神经递质的表达在各类NIM中并不一致，提示HD、IND及HYPG的发病原因不完全相同。检测直肠远端的神经递质，特别是nNOS的含量和分布有助于临床鉴别各类NIM。

Intestinal NANC neuropeptides in neuronal intestinal malformations

Objective To assess the expression patterns of nitric oxide synthase(nNOS),heme oxygenase-2(HO-2) and vasoactive intestinal polypeptide(VIP) in neuronal intestinal malformations(NIM),including Hirschsprung's disease(HD),intestinal neuronal dysplasia(IND) and hypoganglionsis(HYPG).Methods Rectal specimens from 12 HD,15 IND and 3 HYPG patients were immunochemically stained for NOS,HO-2 and VIP. Control group consisted of colon specimens from 10 normal children.Results In the HD group,the fibers were found to be significantly reduced in rectum using nNOS and HO-2 staining. In IND group,ganglion cells and fibers were found to be increased in rectum and distended segment of colon using nNOS and VIP staining. In HYPG group,all the 3 transmitters were reduced.Conclusions Discrepancies were detected in the 3 neuronal transmitters in different types of NIM,suggesting different neuronal development in HD,IND and HYPG. The neural transmitters may be helpful in differentiating the diagnoses of NIM.