Medical Professional Publications

Research Now: Stem Cells Provide Future Treatment for Hirschsprung Disease

Dr. Cheryl Gariepy is working to isolate and transplant enteric nervous system stem cells to treat this surgery-dependent disease

Dr. Cheryl Gariepy, principal investigator in the Center for Molecular and Human Genetics, is using a mouse model of Hirschsprung disease to isolate stem cells of the enteric nervous system.  In doing so, she hopes to provide new treatments for Hirschsprung disease and other disorders of the enteric nervous system.
The enteric nervous system is the largest and most complex division of the peripheral and autonomic nervous systems and contains many different types of neurons and neurotransmitters. It is organized into interconnected networks that control intestinal motility, secretion of fluid into the gut lumen, local blood flow and the secretion of hormones.

Hirschsprung disease is the congenital absence of the enteric nervous system in a segment of intestine. It is the most common cause of neonatal intestinal obstruction.  While mortality is low with surgical resection, morbidity can be quite high.  Thirty percent of patients need a second operation due to a persistent or new segment of hypoganglionated bowel, an anastomotic leak or stricture. Fecal incontinence and constipation persist through adolescence in 16 to 32 percent of patients. The enteric nervous system is also implicated in a wide variety of other common disorders.  Most of these are the result of enteric nervous system damage later in life.

Dr. Gariepy’s team has found that stem cells for the enteric nervous system exist in the intestine in an animal model of Hirschsprung disease and that these stem cells can be isolated and transplanted to the gut after birth where they can form neurons. Her team is now working on techniques to harness these stem cells to treat individuals with Hirschsprung disease and other disorders of the enteric nervous system.  

Dr. Gariepy is also an attending physician in the Division of Gastroenterology Hepatology and Nutrition.

Select Citations
YG Tsai, N Murakami, CE Gariepy. 2011 Postnatal intestinal engraftment of prospectively selected enteric neural crest stem cells in a rat model of Hirschsprung disease. Neurogastroenterology and Motility, 23:362-369

E. Imseis, E, CE Gariepy. Hirschsprung Disease. In Pediatric Gastrointestinal Disease: Pathophysiology, Diagnosis, and Management. Fifth Edition. Edited by R.E. Kleinman, O.J. Goulet, G. Mieli-Vergani, I.R. Sanderson, P.M. Sherman and B.L Schneider, BC Decker Inc, Hamilton, Ontario Canada, 2008 pp. 683-693 (currently being revised for Sixth Edition)

YH Tsai, CE Gariepy. 2005. Dynamic changes in the proximal gut neural crest stem cell population are associated with successful development of the distal enteric nervous system in rats. Pediatric Res., 58: 636-643

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