Finally, the ability of HSCR patient ENS cells to colonize autologous aneuronal colon muscle tissue was assessed.ĮNS-derived p75-sorted cells from patients expressed multiple NC progenitor and differentiation markers and proliferated in culture under conditions simulating Wnt signaling.

The ability of human HSCR postnatal ENS-derived cells to colonize the embryonic intestine was demonstrated by organ coculture with avian embryo gut, and the ability of human postnatal HSCR aneuronal colon muscle to support ENS formation was tested by organ coculture with embryonic mouse ENS cells. ENS cells were assessed for NC markers immunohistologically and by quantitative reverse-transcription polymerase chain reaction, and mitosis was detected by ethynyl-2'-deoxyuridine labeling. Aneuronal colon tissue was obtained from the distal resection margin (23 patients). We tested whether aneuronal (aganglionic) colon tissue from patients may be colonized by autologous ENS-derived cells.Ĭells were obtained and cryopreserved from 31 HSCR patients from the proximal resection margin of colon, and ENS cells were isolated using flow cytometry for the NC marker p75 (nine patients).

Treatment is by distal bowel resection, but neural cell replacement may be an alternative. Hirschsprung disease (HSCR) is caused by failure of cells derived from the neural crest (NC) to colonize the distal bowel in early embryogenesis, resulting in absence of the enteric nervous system (ENS) and failure of intestinal transit postnatally.

Victoria 2 how to colonize