Kobayashi H, O'Briain D S, Puri P
Children's Research Centre, Our Lady's Hospital For Sick Children, Dublin, Ireland.
J Pediatr Surg. 1994 Feb;29(2):301-4. doi: 10.1016/0022-3468(94)90336-0.
The authors studied expression of NADPH-diaphorase and NCAM in colon from 16 patients with Hirschsprung's disease (HD) and eight age-matched controls, using immunohistochemical techniques. Myenteric ganglia in the ganglionic bowel and hypertrophic nerve trunks in the aganglionic bowel displayed strong NADPH-diaphorase and NCAM reactivity. Quantitative assessment of immunoreactivity in colonic muscle demonstrated a selective absence of NADPH-diaphorase and NCAM-positive nerve fibers in the circular as well as longitudinal muscle of aganglionic bowel, whereas these immunoreactive nerve fibers were present in abundance in the muscle of ganglionic colon of HD patients as well as in the colon of controls. The lack of expression of NCAM on nerve fibers within the muscle of HD patients suggests a developmental abnormality of innervation of the muscle, and the selective absence of NADPH-diaphorase-positive nerves in the muscle is most likely responsible for the spasticity of the aganglionic segment.
作者采用免疫组化技术，研究了16例先天性巨结肠（HD）患者及8例年龄匹配的对照者结肠中NADPH黄递酶和神经细胞黏附分子（NCAM）的表达。神经节段肠管中的肌间神经节和无神经节肠管中的肥厚神经干显示出强烈的NADPH黄递酶和NCAM反应性。对结肠肌肉中免疫反应性的定量评估表明，无神经节肠管的环形肌和纵形肌中选择性缺乏NADPH黄递酶和NCAM阳性神经纤维，而这些免疫反应性神经纤维在HD患者的神经节段结肠肌肉以及对照者的结肠中大量存在。HD患者肌肉内神经纤维上NCAM表达的缺乏提示肌肉神经支配存在发育异常，而肌肉中NADPH黄递酶阳性神经的选择性缺失很可能是无神经节段痉挛的原因。
