儿童肠神经元发育异常症研究进展  

Research advances of intestinal neuronal dysplasia in children

作　　者：纪福龙 康建宇 刘勇[1] 孙大庆 JI Fulong;KANG Jianyu;LIU Yong;SUN Daqing(Department of Pediatric Surgery,General Hospital of Tianjin Medical University,Tianjin 300052,China)

机构地区：[1]天津医科大学总医院儿外科,天津300052

出　　处：《妇儿健康导刊》2022年第9期14-16,25,共4页JOURNAL OF WOMEN AND CHILDREN'S HEALTH GUIDE

基　　金：国家自然科学基金资助项目(82070554)。

摘　　要：肠神经元发育异常症(IND)是一种常见的先天性肠神经发育畸形,发病率仅次于先天性巨结肠(HD)。顽固性便秘是IND的主要临床表现,IND分为A型和B型,其中IND-B占95%以上。目前该病发生的具体机制尚不明确,诊断标准复杂主观,即使在直肠活检的组织病理学分析中,关于IND-B的诊断标准仍然存在空白。已有多种免疫组织化学标志物被提出辅助肠道神经系统的组织病理学分析,这些标志物为该疾病的组织病理学诊断提供了新的可能性。本文重点从儿童IND-B的相关发病机制、诊断及治疗方法进行综述。Intestinal neuronal dysplasia(IND)is a common congenital intestinal neurodevelopmental malformation,and its incidence rate is second only to Hirschsprung’s disease(HD).Intractable constipation is the main clinical manifestation of IND.IND is divided into type A and type B,of which IND-B accounts for more than 95%.At present,the specific mechanism of the disease is not clear,and the diagnostic criteria are complex and subjective.Even in the histopathological analysis of rectal biopsy,the diagnostic criteria for IND-B are still blank.A variety of immunohistochemical markers have been proposed to assist the histopathological analysis of the intestinal nervous system,which provide new possibilities for the histopathological diagnosis of the disease.This article focuses on the pathogenesis,diagnosis and treatment of IND-B.

关 键 词：肠神经元发育异常症 慢性便秘 肠神经疾病 先天性巨结肠 

分 类 号：R726[医药卫生—儿科]