Expression of beta-1 integrins in ganglionic and aganglionic segments of patients with Hirschsprung's disease.

Abstract

An immunocytochemical study was performed to assess the role of beta-1 integrins in the pathogenesis of Hirschsprung's disease. Fresh tissue samples from both aganglionic and ganglionic segments of five patients who were undergoing surgery for Hirschsprung's disease were obtained. Samples were rapidly frozen in liquid nitrogen. Sections were cut and stained using anti alpha-1, 2, 3, 4, 5, 6 and beta-1 monoclonals according to the indirect immunoperoxidase method. The evaluation did not reveal any significant change of pattern in the distribution of beta-1 integrins in the non-neural elements of both aganglionic and ganglionic segments of colon, such as the epithelium, muscularis mucosa, muscularis externa, connective tissues and blood vessels. Nerve fibres in both aganglionic and ganglionic segments strongly expressed the alpha-6 chain of very late activation antigen which led to their increase in the aganglionic segment. In addition to revealing the increase, alpha-6 monoclonals also had in situ positive control due to their presence in non-neural elements. Hence, immunostaining of the suction biopsies with anti alpha-6 monoclonals may be employed as a new and simple method in the diagnosis of Hirschsprung's disease. On the other hand, beta-1 integrins do not seem to play a role in the defective migration of ganglion cells occurring in Hirschsprung's disease.