Pharmacologic studies of Hirschsprung's disease on a murine model.

In studies of the pharmacology of a murine model of aganglionosis, the contracted aganglionic portion of the terminal bowel showed slow regular contractions of high amplitude in comparison to the rapid low-amplitude contractions of a normal segment of bowel. An absence of the nonadrenergic inhibitory system was demonstrated in the aganglionic, contracted portion of the bowel. This lack of the nonadrenergic system confirms previous findings in human tissue. It is proposed that absence of this inhibitory system in the aganglionic portion of the bowel results in the principal pathophysiologic findings in Hirschsprung's disease.