Research And Clinical Significance Of Markers Related To The Hirschsprung’s Disease

Objective: To provide theoretical basis for the length of bowel resection，revealthe pathogenesis of Hirschsprung’s disease (HD) by investigating the pathologicalchanges of enteric nerves system (ENS)、smooth muscle、 interstitial cells of Cajal(ICCs)、Cathepsin D(CAD)、laminin (LN) in HD with immunochemically stainedand real time quantitative PCR.Methods: Resected intestines were obtained from34patients with HD, elevensamples in the control group were obtained from postmortem neonates withoutgastrointestinal or nervous system diseases． Imrnunohistoehemical staining forS-100,-SMA、c-kit、CAD、LN was done in the aganglionic, transitionalandnormal segment.The quantitative expressions of laminin gene in the three segmentswere detected by real time quantitative PCR. And all the data was analyzed by SPSSSoftwar.Results:1.Positive expression of S-100,-SMA and c-kit protein in HD weredecreased significantly than that in control group. However，these alterations becameless obvious at8cm from transitional segments in our study.2. From aganglionic segment to normal segment the mature ganglion cellsincreased, immature cells decreased.3. Stained area、protein and mRNA levels of laminin in aganglionie segmentshowed a significant increase compared with the normal segment.Conclusions:1.Pathological changes of enteric nerves system，smooth muscleand ICCs had been observed in HD．The length of the intestinal resection should bebeyond8cm from transitional segments．2.CAD can be used to distinguish the mature ganglion cells and immatureganglion cells.3. The increased expression of laminin in aganglionic segment may be related tothe cause of HD.