This is fantastic news for sufferers of Hirschprungs Disease and their offspring and future treatment. Noo won’t be eligible for this treatment, however, his children may be.
Researcher: Richard Lindley, University of Liverpool
Fellowship: CORE/Royal College of Surgeons One Year Research Award
Grant Total: £40,140 – Completed
This research is aimed at developing a new treatment for Hirschsprung’s disease. The researcher team planned to grow new nerve cells from the bowel of children with Hirschsprung’s disease and transplant them back into the child to improve the outcome of surgery.
Final Report
In Hirschsprung’s disease children are born with missing nerve cells from the final part of the bowel. This means that the bowel does not function properly and without surgery the condition can be life-threatening. Although it can be treated with surgery, the long-term results are often disappointing and 1 in 5 children with Hirschsprung’s disease will suffer from severe incontinence or need a colostomy. To improve this outcome, the research team intended to grow new nerve cells from the bowel of children with Hirschsprung’s disease and discover if transplanting them back into the child would improve the outcome of surgery.
The research showed that it is possible to isolate cells (called progenitor cells) which can then be grown in the laboratory. They also demonstrated that the cells are capable of forming new nerve cells (neurons) and the cells which support them, called glial cells. The progenitor cells are grown as collections of cells called neurospheres, which contain both the progenitor cells and also neurons and glia.
These progenitor cells can be found in the bowel of normal children and also in children with Hirschsprung’s disease.
The research team then showed, using animal models, that it is possible to transplant the neurospheres, and after transplantation neurons and glia move out of the neurosphere and produce a new network of nerves.
In order to investigate whether the transplanted nerve cells have an effect on the contractions of the bowel, the research team developed a system that uses a video camera and computer program to analyse the movement of the bowel. Initial results from these experiments suggest that the transplanted bowel behaves in a similar fashion to bowel that has developed normally.
It is hoped that eventually, this work will result in a treatment for Hirschspung’s disease based on transplantation of these progenitor cells.