Cutting-edge regenerative therapy for Hirschsprung disease and its allied disorders

Koichiro Yoshimaru, Toshiharu Matsuura, Yasuyuki Uchida, Soichiro Sonoda, Shohei Maeda, Keisuke Kajihara, Yuki Kawano, Takeshi Shirai, Yukihiro Toriigahara, Alvin Santoso Kalim, Xiu Ying Zhang, Yoshiaki Takahashi, Naonori Kawakubo, Kouji Nagata, Haruyoshi Yamaza, Takayoshi Yamaza, Tomoaki Taguchi, Tatsuro Tajiri

Research output: Contribution to journalReview articlepeer-review

1 Citation (Scopus)

Abstract

Hirschsprung disease (HSCR) and its associated disorders (AD-HSCR) often result in severe hypoperistalsis caused by enteric neuropathy, mesenchymopathy, and myopathy. Notably, HSCR involving the small intestine, isolated hypoganglionosis, chronic idiopathic intestinal pseudo-obstruction, and megacystis-microcolon-intestinal hypoperistalsis syndrome carry a poor prognosis. Ultimately, small-bowel transplantation (SBTx) is necessary for refractory cases, but it is highly invasive and outcomes are less than optimal, despite advances in surgical techniques and management. Thus, regenerative therapy has come to light as a potential form of treatment involving regeneration of the enteric nervous system, mesenchyme, and smooth muscle in affected areas. We review the cutting-edge regenerative therapeutic approaches for managing HSCR and AD-HSCR, including the use of enteric nervous system progenitor cells, embryonic stem cells, induced pluripotent stem cells, and mesenchymal stem cells as cell sources, the recipient intestine's microenvironment, and transplantation methods. Perspectives on the future of these treatments are also discussed.

Original languageEnglish
Pages (from-to)977-994
Number of pages18
JournalSurgery today
Volume54
Issue number9
DOIs
Publication statusPublished - Sept 2024

All Science Journal Classification (ASJC) codes

  • Surgery

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