Meijers J H, Molenaar J C
Department of Paediatric Surgery, Erasmus University, Rotterdam, The Netherlands.
Neth J Surg. 1991;43(6):230-5.
Hirschsprung's disease is characterized by the absence of enteric neurons from the distal colon and rectum. We reviewed the history of Hirschsprung's disease from its first description till the experimental approaches of its pathogenesis today. In our laboratory we introduced the use of monoclonal antibodies directed against neurofilament epitopes as useful tools in the diagnosis of Hirschsprung's disease and allied disorders. One particular monoclonal antibody (2FII) enables to distinguish between classical Hirschsprung's disease, long segment aganglionosis, hypoganglionosis, hyperganglionosis and chronic constipation. We also used monoclonal antibodies in experimental studies concerning the formation and malformation of the enteric nervous system in murine and chicken embryos. One particular antibody (HNK-I) was found to be a marker for very early precursors of enteric neurons in chicken (and human) embryos. In chicken embryos HNK-I visualizes cephalic neural crest cells, the area in the embryo that gives rise to all neurons in the gut. Using a microsurgical technique, we developed a model for Hirschsprung's disease in the chicken embryo.
先天性巨结肠症的特征是远端结肠和直肠缺乏肠神经元。我们回顾了先天性巨结肠症从首次被描述到如今其发病机制的实验研究方法的历史。在我们实验室，我们引入了针对神经丝表位的单克隆抗体，将其作为诊断先天性巨结肠症及相关疾病的有用工具。一种特定的单克隆抗体（2FII）能够区分典型的先天性巨结肠症、长段无神经节症、神经节减少症、神经节增多症和慢性便秘。我们还将单克隆抗体用于关于小鼠和鸡胚胎中肠神经系统形成和畸形的实验研究。发现一种特定抗体（HNK-1）是鸡（和人类）胚胎中肠神经元非常早期前体的标志物。在鸡胚胎中，HNK-1可使头部神经嵴细胞可视化，神经嵴细胞是胚胎中产生肠道所有神经元的区域。我们利用显微外科技术，在鸡胚胎中建立了先天性巨结肠症模型。
