Austin Kelly Miller
Division of Pediatric General and Thoracic Surgery, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA 15224, USA.
Semin Pediatr Surg. 2012 Nov;21(4):319-27. doi: 10.1053/j.sempedsurg.2012.07.006.
Hirschsprung's disease-associated enterocolitis (HAEC) remains the most life-threatening complication in Hirschsprung disease (HD) patients. The pathogenesis of HAEC has not been determined and many hypotheses regarding the etiology of HAEC have been proposed. These include a possible causal relationship between the abnormal enteric nervous system development in HD and the development of enterocolitis. Based on the complex genetic causes of HD that have been discovered and the resultant heterogeneous group of patients that exists, the causes of HAEC are likely multiple. New insights regarding the relationship of the role of the enteric nervous system and its interaction between intestinal barrier function, innate host immunity, and commensal microflora have been discovered, which may shed light on this perplexing problem. This review presents current known risk factors of HAEC and the proposed theories and supporting evidence for the potential etiologies of HAEC.
先天性巨结肠相关小肠结肠炎（HAEC）仍然是先天性巨结肠（HD）患者最危及生命的并发症。HAEC的发病机制尚未明确，关于其病因已提出了许多假说。这些假说包括HD中异常的肠道神经系统发育与小肠结肠炎发生之间可能存在因果关系。基于已发现的HD复杂遗传病因以及由此产生的异质性患者群体，HAEC的病因可能是多方面的。关于肠道神经系统的作用及其与肠道屏障功能、宿主固有免疫和共生微生物群之间相互作用的关系，已有新的见解，这可能有助于阐明这个令人困惑的问题。本文综述了目前已知的HAEC危险因素以及关于HAEC潜在病因的提出的理论和支持证据。
