ヒルシュスプルング病無神経節腸管の単クローン抗ニューロフィラメント抗体による免疫組織化学的研究

概要

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Immunohistochemical studies were carried out on tissue sections obtained from 5 patients with Hirschsprung's disease, aged 3 months to 7 months, using monoclonal anti-neurofilament antibodies. The following antibodies were used with Avidin-Biotin-peroxidase Complex method : monoclonal anti-neurofilament antibody "H" (molecular weight 200,000), "M"(160,000), "L" (68,000) [Bohringer Mannheim, West Germany]. Numerous fibers immunoreactive for anti-neurofilament "H" and "M" antibodies were present in both intermuscular and submucosal layers of the aganglionic segment in all of the 5 cases. These positively stained nerve bundles were hypertrophic and randomly distributed. The ganglionic segment showed, in the inter-muscular and submucosal layers, a reduced number of thin fibers that were positively stained by anti-neurofilament "H" and "M" antibodies. The immunoreactivity of the nerve fibers to anti-neurofilament "L" antibody was weak not only in the aganglionic segment but also in the ganglionic segment. These results indicate that the fundamental pathology in Hirschsprung's disease is not only the absence of ganglion cells but also the abundance of nerve fibers which are immunoreactive to monoclonal antineurofilament antibodies.
日本小児外科学会の論文
1989-12-20