| Hirschsprung's disease (HD) is commonly digestive malformation in pediatric surgery, with morbidity of 1/4000-1/5000 and male/female ratio of 4:1. The main reason is lack of intestinal ganglion cell because ganglion cell cannot migrate, focus and differentiation. The aganglionic(AG) intestines constrict constantly to occlude the passage of intestinal contents. At the ganglion(NG) segment, intestine become dilatation and hypertrophy. Delay to excrete meconium, refractory constipation and abdominal distension are commonly seen, also commonly accompanying with enterocolitis in small intestine and colon, lower intestine obstruction. It is unclear in etiology and pathogenesis of HD. And it is generally considered that it is a result of multiple factors, such as ischemia, hypoxia, virus, toxin and heredity. Each factor acts on the different phase of neuron development to inhibit development of ganglion cell. The pathogeny of HD is classified into three conditions (1) disable migration of ganglion cells in embryonic period due to stagnancy of neural crest cell differentiation and migrate to intestine wall caused by multiple gene heredity disease, gene mutation and /or gene malexpression. The current researches support the key role of RET protein and GDNF EDNR NCX etc. (2) Micro-environment inhibits development of ganglion cells. For example, extracellular matrix protein, change of neuron growth factor and its receptors affect on the survival and development of crest cells.(3) Some local factors to destroy intestine crest cells such as ischemia, hypoxia, immune factors, Epstein-Barr virus and parasite infection.In this research, immunohistochemical technique is used to observe expression of RET protein, NC AM in AG intestine and NG intestine, study their relationship and the role in pathogenesis. It provides a new thinking manner and diagnostic evidence to prevent and diagnose HD.Material and methods: The samples of 26 patients with HD fixed in 10%... |
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