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

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 Hirschsprun...

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Bibliographic Details
Published in:Journal of pediatric surgery 2022-09, Vol.57 (9), p.61-68
Main Authors: Pan, Weikang, Goldstein, Allan M., Hotta, Ryo
Format: Article
Language:English
Subjects:
Cell therapy
Enteric nervous system
Hirschsprung disease
Neuronal stem cells
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Summary: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.
ISSN:0022-3468
1531-5037
DOI:10.1016/j.jpedsurg.2021.10.049