IF 0.8 4区 医学 Q4 PEDIATRICS
World Journal of Pediatric Surgery Pub Date : 2025-03-31 eCollection Date: 2025-01-01 DOI:10.1136/wjps-2024-000860
Jessica L Mueller, Ryo Hotta
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引用次数: 0

摘要

肠道神经系统(ENS)由神经元和神经胶质细胞组成,控制着胃肠道的许多复杂功能。赫氏肠病(HSCR)是一种先天性疾病,其特征是由于胚胎发育过程中神经嵴衍生细胞未能在远端肠道定植,导致不同长度的远端肠道缺乏肠神经系统。HSCR 患者通常在新生儿期出现严重便秘,通过直肠抽吸活检确诊,然后通过拉通手术切除受影响的肠段,并将近端神经节肠道与肛门重新连接。拉通手术后的效果并不理想,许多患者持续存在运动障碍和排便功能障碍的问题,这表明这种疾病的治疗仍有优化的空间。本综述将重点讨论最近在更好地了解 HSCR 方面取得的进展,并利用这些进展进行更准确、可能更微创的诊断。我们还讨论了 HSCR 未来的潜在治疗方法,尤其是基于细胞的 HSCR 治疗方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Current and future state of the management of Hirschsprung disease.

The enteric nervous system (ENS) consists of a network of neurons and glia that control numerous complex functions of the gastrointestinal tract. Hirschsprung disease (HSCR) is a congenital disorder characterized by the absence of ENS along variable lengths of distal intestine due to failure of neural crest-derived cells to colonize the distal intestine during embryonic development. A patient with HSCR usually presents with severe constipation in the neonatal period and is diagnosed by rectal suction biopsy, followed by pull-through procedure to surgically remove the affected segment and reconnect the proximal ganglionated intestine to the anus. Outcomes after pull-through surgery are suboptimal and many patients suffer from ongoing issues of dysmotility and bowel dysfunction, suggesting there is room for optimizing the management of this disease. This review focuses on discussing the recent advances to better understand HSCR and leverage them for more accurate and potentially less invasive diagnosis. We also discuss the potential future management of HSCR, particularly cell-based approaches for the treatment of HSCR.

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来源期刊
CiteScore
1.40
自引率
12.50%
发文量
38
审稿时长
13 weeks
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