Zhou Ying,Yang Shaobo,Huang Yanlei.Role of Shh/Bmp4 signal pathway in congenital anorectal malformation[J].Journal of Clinical Pediatric Surgery,2021,20(02):191-195.[doi:10.12260/lcxewkzz.2021.02.016]
Shh/Bmp4信号通路在先天性肛门直肠畸形末端肠壁神经系统中的作用
- Title:
- Role of Shh/Bmp4 signal pathway in congenital anorectal malformation
- Keywords:
- Rectal Diseases/CN; Rectal Diseases/ET; Anal Canal/AB; Bectum/AB
- 分类号:
- R726.1 R657.1
- 摘要:
- 肛门直肠畸形（anorectal malformation，ARM）是小儿外科常见的消化道畸形，手术是目前最主要的治疗方式，但仍有一部分ARM患者术后存在着不同程度的排便功能障碍，影响患者术后生活质量。虽然ARM的具体发病机制及术后排便功能障碍发生机制尚不明确，但有文献指出直肠末端肠壁神经系统（enteric nervous system，ENS）的发育与术后排便功能相关。目前有动物模型实验及相关临床研究证实，Shh/Bmp4信号通路可在ENS发生发展过程中发挥重要的调节作用。
- Abstract:
- Anorectal malformation (ARM) is a common digestive tract malformation in pediatric surgery.Currently surgery is the most important treatment.However,some ARM children have varying degrees of defecation dysfunction and it affects postoperative quality-of-life.Although the specific pathogenesis of ARM and the mechanism of postoperative defecation dysfunction have remained elusive,the development of nervous system (enteric nervous system,ENS) in intestinal wall of terminal rectum is associated with postoperative defecation function.Both animal model experiments and clinical trials have confirmed that Shh/Bmp4 signaling pathway plays an important regulatory role in the occurrence and development of ENS.
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备注/Memo
收稿日期:2019-07-08。
基金项目:国家自然科学基金项目（编号：81401243）；复旦大学"卓学计划"（编号：2015—2018）；上海市卫生健康委员会基金项目（编号：201744Y0018）
通讯作者:黄焱磊,Email:yanleihuang@163.com