Inflammatory response in enterocolitis in the piebald lethal mouse model of Hirschsprung's disease.

Mucosal immune defense responses in two clinical forms of enterocolitis complicating congenital megacolon in the piebald lethal mouse model of Hirschsprung's disease were investigated. Significant histological and immunocytochemical differences were seen in the ganglionic segment of colon between piebald mice with early clinical onset of acute illness and piebald mice with late onset enterocolitis. The number of IgA-containing plasma cells infiltrating the lamina propria of the gut in the former group were significantly increased (p less than 0.001) compared to the latter group of mice and healthy controls. This study suggests that the two forms of enterocolitis complicating congenital megacolon in piebald mice have different etiologies.