Opportunities for novel diagnostic and cell-based therapies for Hirschsprung disease.

Opportunities for novel diagnostic and cell-based therapies for Hirschsprung disease.
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DOI:
10.1016/j.jpedsurg.2021.10.049
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发表时间:
2022-09
影响因子:
2.4
通讯作者:
Hotta, Ryo
Hotta, Ryo
中科院分区:
医学3区
文献类型:
--
作者:
Pan, Weikang;Goldstein, Allan M.;Hotta, Ryo

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尽管我们对巨结肠病(HSCR)的病因和病理生理学的理解取得了重大进展,但早期准确的诊断和手术治疗仍然具有挑战性。此外,手术后的长期发病率,包括大便失禁、便秘和先天性巨结肠相关小肠结肠炎(HAEC),仍然存在问题。最近的进展应用最先进的成像技术来可视化肠神经系统,并利用神经干细胞来替代缺失的肠神经元和神经胶质细胞,为HSCR患者提供了一个有希望的新未来的可能性。在这篇综述中,我们总结了最近的研究进展,可能有一天会为这种疾病的诊断和治疗提供新的方法。
Despite significant progress in our understanding of the etiology and pathophysiology of Hirschsprung disease (HSCR), early and accurate diagnosis and operative management can be challenging. Moreover, long-term morbidity following surgery, including fecal incontinence, constipation, and Hirschsprung-associated enterocolitis (HAEC), remains problematic. Recent advances applying state-of-the art imaging for visualization of the enteric nervous system and utilizing neuronal stem cells to replace the missing enteric neurons and glial cells offer the possibility of a promising new future for patients with HSCR. In this review, we summarize recent research advances that may one day offer novel approaches for the diagnosis and management of this disease.
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