结直肠离子通道与先天性巨结肠关系的研究进展

离子通道是细胞膜上一类特殊亲水性蛋白微孔道,也是肌肉、神经细胞等电活动的物质基础。目前研究通过生物学及离子通道膜片钳等新技术对离子通道有了进一步的认识,并逐步发掘离子通道的结构功能异常与疾病的发生存在的紧密关系。先天性巨结肠症(Hirschsprung's Disease,HD)又称无神经节细胞症,是小儿外科的常见疾病之一。HD临床表现为胎粪排出延迟、顽固性便秘及腹胀,常并发小肠结肠炎、低位肠梗阻等。目前研究尚未完全明确HD的发病机制,本文对HD的发生与结直肠离子通道功能间的关系作一综述。

Research Progress of Colorectum Ion Channels in Hirschsprung Disease

Ion channels are the hydrophilic proteins that are the micro channels on the cell membranes. It is the material basis of the electrical activity of muscle and nerve cells. The researches have made a further understanding of the channels by means of patch-clamp and new biology technologies, that the abnormal structure of the ion channels may be relative to some certain diseases. Hirschsprung disease that called the aganglionosis is one of the common diseases in pediatric surgery. The clinical manifestations of HD are the delayed meconiumexclusion, the intractable constipation and abdominal distension, which always be complicated with enterocolitis, lower intestinal obstruction, etc. The researches have not been entirely clear about the machenism of etiology. Thus, this article will review the relationship of colorectal ion channels and HD.