摘要
Prader-Willi综合征(PWS)是一种印记基因缺陷疾病,主要特征包括智力低下、肥胖、代谢紊乱和行为异常等。关于其代谢紊乱、行为异常等病理机制尚不明确,目前治疗效果也并不理想。近年来肠道微生态菌群在代谢调节中的作用逐渐被认识,本文将对PWS肠道微生态菌群的研究进展进行系统介绍。相比于健康人群或其他单纯肥胖人群,PWS患者的肠道菌群存在一定改变,且这些改变与PWS患者代谢紊乱和炎症状态相关。通过饮食或益生菌干预肠道菌群,在PWS患者中起到了一定的控制体重和改善行为异常的疗效。
Prader-Willi syndrome(PWS)is a genetically imprinted disorder characterized by intellectual impairment,obesity,metabolic disorders,and behavioral abnormalities.The pathological mechanisms of metabolic disorders and behavioral abnormalities are not yet clear,and the treatment effect up to now is not satisfactory.In recent years,the role of gut microbiota in metabolic regulation has gradually been recognized.This article will systematically review the research progress of gut microbiota in PWS.Compared to healthy individuals or other simply obese individuals.PWS patients show changes in the gut microbiota.The changes are related to metabolic disorders and inflammatory status in PWS patients.Intervention of gut microbiota through diet or probiotics has played a certain role in controlling weight and improving behavioral abnormalities in PWS patients.
作者
周煜
马明圣
ZHOU Yu;MA Mingsheng(Department of Pediatrics,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100730,China)
出处
《罕见病研究》
2024年第3期375-380,共6页
Journal of Rare Diseases