PDF(Pubmed)
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.
摘要：
Hirschsprung病（HSCR）是一种先天性疾病，其特征是在妊娠期间神经c细胞无法迁移并填充远端肠，影响不同长度的肠，导致远端功能性梗阻。一旦通过证明受影响的肠段不存在神经节细胞或神经节病证实诊断，就需要手术治疗来纠正HSCR。Hirschsprung病相关小肠结肠炎（HAEC）是一种与HSCR相关的炎症并发症，可出现在术前或术后期间，并与发病率和死亡率增加有关。HAEC的发病机制仍然知之甚少，但是肠道动力障碍,菌群失调，粘膜防御和肠屏障功能受损似乎起着重要作用。对于HAEC没有明确的定义,但诊断主要是临床，根据严重程度指导治疗。这里,我们的目标是提供对临床表现的全面审查，病因学,病理生理学,以及目前HAEC的治疗选择。
