Eur J Pediatr Surg 2018; 28(03): 215-221
DOI: 10.1055/s-0038-1646925
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Update on the Role of Stem Cells in the Treatment of Hirschsprung Disease

Kathy Nga-Chu Lui
1   Department of Surgery, The University of Hong Kong, Hong Kong, Hong Kong
,
Paul Kwong Hang Tam
1   Department of Surgery, The University of Hong Kong, Hong Kong, Hong Kong
,
Elly Sau-Wai Ngan
1   Department of Surgery, The University of Hong Kong, Hong Kong, Hong Kong
› Institutsangaben
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Publikationsverlauf

27. März 2018

03. April 2018

Publikationsdatum:
24. April 2018 (online)

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Abstract

Stem cells possess the ability of self-renewal and the potency to differentiate into multiple cell lineages. Somatic stem cells are present in adult tissues, but they usually exhibit limited differentiation capacity and life span. On the other hand, somatic cells from adult tissues can be reprogrammed into induced pluripotent stem cells (iPSCs) that retain a full differentiation capacity with unlimited self-renewal ability. Autologous origin of iPSCs makes them an ideal source of cells for regenerative medicine to replenish the missing or damaged cells in the patients. iPSCs nowadays have also been widely used to build human disease models to study pathological mechanisms of the diseases. Hirschsprung disease (HSCR) is a congenital disorder caused by defects in the development of enteric neural crest stem cells. The failures of the ENCCs to proliferate, differentiate, and/or migrate lead to the absence of enteric neurons in the distal colon, resulting in colonic motility dysfunction. The lack of effective treatment for HSCR urges continuous efforts to develop new therapies for this congenital disorder. In this review, we will discuss the potential applications of somatic stem cells and iPSCs for the cell-based therapy of HSCR. We will also highlight the recent advances in stem cell research for the establishment of human HSCR models for the development of novel therapies.