摘要
先天性巨结肠,是一种神经嵴细胞源性疾病和多基因遗传病,其病因至今虽然尚未完全清楚,但对该病病因和发病机制的研究已取得较大的进展。本文从遗传学、Cajal问质细胞、缝隙连接蛋白43和环境因素等方面阐述HD的发病原因。
Hirschsprung' s disease (HD) , a congenital disease, is characterized by the absence of ganglion cells in the ganglion plexuses of the caudal most gut. It stands as a model for complex multifactorial or multigenic disorders . In the last two decades, there has been a greater understanding of the etiopathogenisis and pathogenesy of hirschsprung' s disease. The goals of this review are to summarize progress in the study of etiological factor, such as genic mutation, the disorders of the interstitial cells of Cajal and connexin 43 ( Cx43 ) , the effect of environment.
出处
《中国临床新医学》
2009年第5期473-476,共4页
CHINESE JOURNAL OF NEW CLINICAL MEDICINE