Hirschsprung's disease associated enterocolitis: A comprehensive review.

Eric M Gershon, Leonel Rodriguez, Ricardo A Arbizu
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Abstract

Hirschsprung's disease (HSCR) is a congenital disorder characterized by failure of the neural crest cells to migrate and populate the distal bowel during gestation affecting different lengths of intestine leading to a distal functional obstruction. Surgical treatment is needed to correct HSCR once the diagnosis is confirmed by demonstrating the absence of ganglion cells or aganglionosis of the affected bowel segment. Hirschsprung's disease associated enterocolitis (HAEC) is an inflammatory complication associated with HSCR that can present either in the pre- or postoperative period and associated with increased morbidity and mortality. The pathogenesis of HAEC remains poorly understood, but intestinal dysmotility, dysbiosis and impaired mucosal defense and intestinal barrier function appear to play a significant role. There is no clear definition for HAEC, but the diagnosis is primarily clinical, and treatment is guided based on severity. Here, we aim to provide a comprehensive review of the clinical presentation, etiology, pathophysiology, and current therapeutic options for HAEC.

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赫氏病相关性小肠结肠炎:全面回顾。
赫氏肠病(HSCR)是一种先天性疾病,其特征是神经嵴细胞在妊娠期间无法迁移并填充到远端肠道,影响不同长度的肠道,导致远端功能性梗阻。一旦确诊受影响的肠段没有神经节细胞或无神经节细胞,就需要通过手术治疗来矫正 HSCR。Hirschsprung's disease associated enterocolitis(HAEC)是一种与 HSCR 相关的炎症并发症,可在术前或术后出现,并会增加发病率和死亡率。HAEC 的发病机制仍不十分清楚,但肠道运动障碍、菌群失调以及粘膜防御和肠道屏障功能受损似乎在其中发挥了重要作用。HAEC 目前尚无明确定义,主要依靠临床诊断,并根据严重程度指导治疗。在此,我们旨在全面回顾 HAEC 的临床表现、病因学、病理生理学和当前的治疗方案。
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