Impaired gastrointestinal mucosal defense in Hirschsprung's disease: a clue to the pathogenesis of enterocolitis?

Twelve patients with Hirschsprung's disease were studied to investigate why some children develop enterocolitis. Previous studies have failed to explain this adequately; they have also failed to explain why enterocolitis can occur many years after definitive surgery. Six of the 12 patients had enterocolitis. By assessing immunological mucosal defense, it was shown that these patients had a marked deficiency in transfer of secretory IgA across the gastrointestinal mucosal cell, and thus were prone to mucosal invasion of both pathogenic and commensal organisms. The abnormalities were detected upon initial investigation (at presentation) prior to the onset of enterocolitis, and persisted into later life. The significance of these findings with reference to predicting which children are prone to develop enterocolitis and their long-term susceptibility to recurrence are discussed.