Progress on the etiology of the Hirschsprung's disease / 国际儿科学杂志

ABSTRACT

The major progress on the study of etiology of the Hirschsprung's disease is the genetic factots and the aherations of microenvironment in primarily embryogenic period.The genetic factors comprise of RETGDNF system.EDNRB.EDN3 system,and many other genes involving in the formulation of the tormer two svstems.such as SOX10,NRG-1, FoxD3,PHOX2B,HOXB5,ZFHXIB,all of which file essencial for the migration and differentiation of neural crest cells(NCC).The alterations of microenvironment in primarily embryogenic period include interstitial ceLls of cajal(ICC),cell adhesion molecules(CAMLl),Ca2+ and so on.Definitely,CAML1 plays as the basis of NCC's migration and differentiation,and the decline of the Ca2+dependent channel's expression brings about the anolnaloug contraction of smooth muscle.