Hirschsprung disease (HSCR) is a congenital disease that is characterized by the absence of intrinsic ganglion cells in the submucosal and myenteric plexuses of the distal colon and is the most common cause of congenital intestinal obstruction. Hirschsprung-associated enterocolitis (HAEC) is a life-threatening complication of HSCR, which can occur either before or after surgical resection of the aganglionic bowel. Even though HAEC is a leading cause of death in HSCR patients, its etiology and pathophysiology remain poorly understood. Various factors have been associated with HAEC, including the mucus barrier, microbiota, immune function, obstruction of the colon, and genetic variations. In this review, we examine our current mouse model of HAEC and how it informs our understanding of the disease. We also describe current emerging research that highlights the potential future of HAEC treatment.

先天性巨结肠症 (HSCR) 是一种先天性疾病，其特征是远端结肠粘膜下层和肌间丛中缺乏内在神经节细胞，是先天性肠梗阻的最常见原因。先天性巨结肠相关性小肠结肠炎 (HAEC) 是 HSCR 的危及生命的并发症，可能发生在手术切除神经节肠管之前或之后。尽管 HAEC 是 HSCR 患者死亡的主要原因，但其病因和病理生理学仍然知之甚少。多种因素与 HAEC 相关，包括粘液屏障、微生物群、免疫功能、结肠梗阻和遗传变异。在这篇综述中，我们检查了我们目前的 HAEC 小鼠模型，以及它如何帮助我们了解这种疾病。