The Distribution Of C-kit～+ Interstitial Cells Of Cajal And The Expression Of Synaptophysin In Hirschsprung's Disease And Allied Hirschsprung's Disorder

Hirschsprung's disease (HD) and Allied Hirschsprung's disorders (HAD) are disorders affecting bowel motility. HD is characterized by aganglionosis of the affected intestinal segments. HAD are characterized by morphological and numerical abnormalities of the intestinal intramural ganglia, and show clinical and X-rays features like those of HD, although ganglia remain even in the colon which include hypoganglionosis (HYPG), immature ganglia (IMG), and neuronal intestinal dysplasia (NID). Previous studies have revealed that aganglionic or morphological and numerical abnormalities of the intestinal intramural ganglia may contribute to the pathogenesis of the intestine motility obstacle of them. And the para-sympathesis,sympathesis,non-cholinergic and non-adrenergic nerve systems are all altered. But the clinical syndrome is not relative to the length of the aganglionosis interstine. So other factors must play a part in their motility obstacles. Recently, people pay more attention on ICCs labeled by using c-kit single-clone antibody.The interstitial cells of Cajal are a species of cells distributed between the end of gastrointestinal nerve and smooth muscle cells, and first described by a Spain neuroanatomist Cajal. They are not neurons or glial cells, but special interstitial cells with independent functions. There are three major functions of ICCS:â‘ they are pacemakers cells in gastrointestinal smooth muscles, and they are pacemakers;â‘¡they facilitate active propagation of electrical events;â‘¢they mediate neurotransmission.Enteric nervous system,smooth muscle cells and ICCs coordinate with each other to facilitate gastrointesine motility. Therefore it is important that the synapses they form are in good condition. Synaptophysin is the specific protein on the membrane of synaptic vesicle, so it can be regarded as one marker of synapses.Observing results: In the first part, the density of c-kit+ICCs in submucosa,muscular layers and around myenteric plexuses of HD and HAD proximal colonic wall was reduced compared to that of normal control (P<0.05), and there was no significant difference between them (P>0.05). The density of c-kit+ICCs in submucosa, circular layers and around the myenteric plexuses in HD distal colonic wall was reduced compared to that of HAD and more reduced compared to that of normal control. Statistics showed the difference between HD and HAD (P<0.05) and significant difference between HD and normal control (P<0.001). But there was no significant difference in the density of c-kit+ICCs in the longitudinal muscular layers among them (P>0.05). In the second part, the synaptophysin immunoreaction was strong positive in the normal colon intestine and the proximal segment of HD and HAD (P>0.05).Compared the distal segment of HD and HAD to the normal colon, the synaptophysin expressions within the muscular layers were fewer (P<0.05) and the synaptophysin immunoreaction in submucosa and myenteric plexuses was markedly reduced (P<0.001).To sum up, we draw the conclusion that the altered distribution of c-kit+ICCs and the abnormal expression of synaptophysin in colonic wall of HD and HAD may relate to the pathogenesis of the intestine motility obstacle of them.