TY - JOUR
T1 - Cutting-edge regenerative therapy for Hirschsprung disease and its allied disorders
AU - Yoshimaru, Koichiro
AU - Matsuura, Toshiharu
AU - Uchida, Yasuyuki
AU - Sonoda, Soichiro
AU - Maeda, Shohei
AU - Kajihara, Keisuke
AU - Kawano, Yuki
AU - Shirai, Takeshi
AU - Toriigahara, Yukihiro
AU - Kalim, Alvin Santoso
AU - Zhang, Xiu Ying
AU - Takahashi, Yoshiaki
AU - Kawakubo, Naonori
AU - Nagata, Kouji
AU - Yamaza, Haruyoshi
AU - Yamaza, Takayoshi
AU - Taguchi, Tomoaki
AU - Tajiri, Tatsuro
N1 - Publisher Copyright:
© The Author(s) under exclusive licence to Springer Nature Singapore Pte Ltd. 2023.
PY - 2024/9
Y1 - 2024/9
N2 - 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.
AB - 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.
KW - Allied disorders of Hirschsprung disease
KW - Chronic idiopathic intestinal pseudo-obstruction
KW - Hirschsprung disease
KW - Hypoganglionosis
KW - Regenerative therapy
KW - Stem cell therapy
UR - http://www.scopus.com/inward/record.url?scp=85169782116&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85169782116&partnerID=8YFLogxK
U2 - 10.1007/s00595-023-02741-6
DO - 10.1007/s00595-023-02741-6
M3 - Review article
C2 - 37668735
AN - SCOPUS:85169782116
SN - 0941-1291
VL - 54
SP - 977
EP - 994
JO - Surgery today
JF - Surgery today
IS - 9
ER -