先天性巨结肠发病机制的研究进展

Recent developments in pathogenesis of Hirschsprung＇s disease

先天性巨结肠（HD）属于肠神经系统异常性疾病，发病与肠神经嵴细胞（GNCC）迁移障碍有关，研究发现调控GNCC迁移障碍的为一复杂基因网络，随着表观遗传学及干细胞领域的深入研究，上述调控网络取得了一定进展，通过修饰、完善HD中提取的肠神经嵴干细胞/肠神经嵴前体细胞（GNCSC/GNCP）移植成为HD的潜在治疗策略。现就相关研究进展进行综述，期待能促进对HD发病机制的研究。

Hirschsprung＇s disease（HD） is a conginental disorder of enteric nervous system（ENS） and is associated with the abnormal migration of gut neural cerst cell（GNCC）. Recent studies showed that the regulation of this migration is a complex gene network. By the development of epigenetic and stem ceils, this network is becoming much more clear. Transplantation of the modified gut neural crest stem cells/gut neural crest precursors（GNCSC/GNCP） acquired from the HD bowl is becoming the potential therapy of HD. This paper summerizes the recent advances and is to promote better understanding the pathogenesis of HD.