关键词: 先天性巨结肠, 结肠炎, 发病机制, 治疗进展

Abstract: Hirschsprungassociated enterocolitis (HAEC) is the most common complication of Hirschsprung's disease (HSCR). The main clinical manifestations are diarrhea, bloating, fever and sepsis. Because the initial research was relatively simple, it was initially thought that HAEC was caused by the disturbance of the intestinal water and electrolyte balance in the digestive system. As the research continued to deepen, it was later considered that HAEC is a type of colon inflammation caused by mechanical obstruction. Studies over the past years have shown that the pathogenicity of HAEC is related to factors such as abnormal intestinal immune function, abnormal intestinal barrier function, changes in intestinal flora and intestinal mechanical obstruction.On the treatment of this disease method, surgical treatment is still the first choice. Therefore, this article summarizes the research progress of the pathogenesis and treatment of HAEC, and provides a certain reference for firstline clinical diagnosis and treatment.

Key words: hirschsprung's disease, enterocolitis, pathogenesis, treatment progress