Journal article

Surgical Intervention to Rescue Hirschsprung Disease in a Rat Model

Lincon A Stamp, Florian Obermayr, Louise Pontell, Heather M Young, Dan Xie, David H Croaker, Zan-Min Song, John B Furness

JOURNAL OF NEUROGASTROENTEROLOGY AND MOTILITY | KOREAN SOC NEUROGASTROENTEROLOGY & MOTILITY | Published : 2015

Abstract

BACKGROUND/AIMS: Rats with a spontaneous null mutation in endothelin receptor type B or Ednrb (sl/sl; spotting lethal) lack enteric neurons in the distal bowel and usually die within the first week after birth. This early postnatal lethality limits their use for examining the potential of cell therapy to treat Hirschsprung disease, and for studies of the influence of EDNRB on the mature CNS and vascular systems. METHODS: We have developed a surgical intervention to prolong the life of the spotting lethal sl/sl rat, in which we perform a colostomy on postnatal (P) day 4-6 rats to avoid the fatal obstruction caused by the lack of colonic enteric neurons. RESULTS: The stomas remained patent and..

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