Abstract
A breeding colony of piebald lethal mice was established in order to study the natural history of congenital megacolon in this mouse model of Hirschsprung's disease and to investigate mucosal defense mechanisms and secretory functions in enterccolitis complicating congenital msgacolon. In experiment 1, 214 mice Were studied, 53 of which had congenital itegacolon (S1/S1). All S1/S1 mice died at 3-11 wks of age showing two distinct patterns of mortality. 64% of mice became acutely ill at 3-4 wks of age and died while the remainder died at 9-11 wks. The former group of mice exhibited clinical & histopathological evidence of severe enterccolitis while the latter group had massive abdominal distension & classical megacolon. In experiment 2, S1/S1 mice were sacrificed at the time of acute illness. Significant histological & immunohistochemical differences were seen in the ganglionic colon between piebald mice with early clinical onset of acute illness & piebald mice with the classical clinical picture of congenital megacolon. In the former group of mice the number of imunocytes in lamina propria was significantly higher than control mice (p<0.001), imnunoglobulin producing cells were equally distributed throughout the lamina propria & IgA containing cells were the most abundant cell type in the colon. In the latter group of mice, immunocyte responses were significantly low & the distribution of immunocytes markedly different with the immunoglobulin producing cells being located only at the deep layer of lamina propria. While there was marked depletion of neutral mucin & sulphomucins in both groups of mice, depletion of sulphomucin which is the dominant mucin of distal colon was more severe in piebald mice with the classical clinical picture of congenital megacolon. The existence of two such populations each of which reflects different clinical form of enterocolitis provides an exciting model for investigation of the causes of development of enterocolitis complicating congenital megacolon.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Purl, P., Fujinpto, T. NATURAL HISTORY & PATHOPHYSIOLOGY OF ENIERCOOLITIS IN PIEBALD LEIHAL MOUSE MODEL OF HIRSCHSPRUNG'S DISEASE. Pediatr Res 21 (Suppl 4), 276 (1987). https://doi.org/10.1203/00006450-198704010-00651
Issue Date:
DOI: https://doi.org/10.1203/00006450-198704010-00651