| Co-Investigator(Kenkyū-buntansha) |
TSUCHIDA Yoshiaki Faculty of Medicine, University of Tokyo, 医学部, 教授 (80010164)
SHIOTANI Yahei Osaka University Medical School, 医学部, 教授 (60028347)
SUZUKI Hiroshi Faculty of Medicine, Mie University, 医学部, 教授 (20004632)
OKADA Akira Osaka University Medical School, 医学部, 教授 (40028569)
OKABE Ikuo Nihon Univercity, School of Medicine, 医学部, 助教授 (20059017)
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| Research Abstract |
On hundred and thirty cases congenital intestinal motor dysfunction were collocted by number of Grant-in Aid for Co-operative Research (A) (No.03304037) from the Ministry of Education, Science and Culture, Japan during the post 20 years. The disorder was defined as a congenital, non-mechanical obstruction of the intestine with the presence of intramural ganglia in the triminal rectum. Intramural ganglia were abnormal in 86 cases, normal in 37, and could not be determined in 8. Of those with abnormal intramural ganglia, 27 had immature ganglia, 29 had hypoganglionosis, 20 had both the immature ganglia and hypoganglionosis, 5 had neuronal intestinal dysplasia, and 2 had a segmental anomaly. Of those with a normal myenteric plexus, 15 had chronic idiopathic intestinal pseudo-obstruction syndrome (CIIPS) and 11 had megacytis-microcolon intestinal hyperristalsis syndrome (MMIHS). Most cases congenital intestinal motor dysfunction without aganglionosis had normal acetylcholinesterase activity. While cases with normal intramural ganglia had normal rectoanal reflex, a significantly greater number of patients with hypoganglionosis or immature ganglionosis locked rectoanal reflex. While patients with immature ganglionosis had good prognosis, patients with hypoganglionosis, CIIPS and MMIHS had poor prognosis. We define pseudo-Hirschsprung's disease as abnormal group of intramural ganglia among cases with congential intestinal motor dysfunction. These findings indicate that psudo-Hirschsprung's disease remains a serious disease of childhood.
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