Division of Pediatric Surgery, Department of Surgery, University of Tennessee Health Sciences Center, 50 N. Dunlap Street, Suite 320, Memphis, TN 38105, USA; Children's Foundation Research Institute, Le Bonheur Children's Hospital, Memphis, TN, USA.
Division of Pediatric Surgery, Department of Surgery, University of Tennessee Health Sciences Center, 50 N. Dunlap Street, Suite 320, Memphis, TN 38105, USA; Children's Foundation Research Institute, Le Bonheur Children's Hospital, Memphis, TN, USA.
Semin Pediatr Surg. 2022 Apr;31(2):151162. doi: 10.1016/j.sempedsurg.2022.151162. Epub 2022 Apr 8.
Hirschsprung-associated enterocolitis (HAEC) was described in 1886 by Harald Hirschsprung and is a potentially deadly complication of Hirschsprung Disease. HAEC is classically characterized by abdominal distension, fever, and diarrhea, although there can be a variety of other associated symptoms, including colicky abdominal pain, lethargy, and the passage of blood-stained stools. HAEC occurs both pre-operatively and post-operatively, is the presenting symptom of HSCR in up to 25% of infants and varies in overall incidence from 20 to 60%. This article reviews our current understanding of HAEC pathogenesis, diagnosis, and treatment with discussion of areas of ongoing research, controversy, and future investigation.
先天性巨结肠相关性结肠炎（HAEC）于 1886 年由 Harald Hirschsprung 描述，是先天性巨结肠症的一种潜在致命并发症。HAEC 的典型特征是腹胀、发热和腹泻，尽管可能存在各种其他相关症状，包括绞痛性腹痛、嗜睡和便血。HAEC 既发生在术前，也发生在术后，是高达 25%婴儿先天性巨结肠症的首发症状，总发病率从 20%到 60%不等。本文回顾了我们目前对 HAEC 发病机制、诊断和治疗的理解，并讨论了正在进行的研究、争议和未来调查的领域。
