摘要
脊髓性肌萎缩症(spinal muscular atrophy,SMA)是一种罕见的遗传性神经肌肉疾病,临床表现为肢体近端进行性、对称性肌无力和肌萎缩。SMA患儿易合并吞咽功能障碍、胃肠道功能障碍、喂养困难、骨代谢异常和营养缺乏/过剩等营养相关问题。目前尚无标准化的SMA营养支持指南。患儿需定期进行生长和营养状况的监测与评估,并由专业营养师进行营养干预与治疗。个体化的营养管理在提高患儿生活质量、延缓病情、延长生存期中起着重要作用。
Spinal muscular atrophy(SMA)is a rare hereditary neuromuscular disease.Clinical manifestations of SMA include proximal limbs symmetrical and progressive muscle weakness and atrophy.Children with SMA are susceptible to swallowing dysfunction,gastrointestinal dysfunction,feeding difficulties,abnormal bone metabolism,and undernutrition/overnutrition.Currently,there is no standardized SMA nutrition support guideline.Patients need regular evaluations of growth and nutritional status.Nutritional interventions and treatments need to be done by professional dietitians.Individualized nutrition management plays an important role in improving the quality of life,delaying the illness,and prolonging the survival time of children with SMA.
作者
龚晓妍
卢忠英
李文辉
朱小妹
胡超平
周水珍
李西华
钱甜
王艺
GONG Xiao-yan;LU Zhong-ying;LI Wen-hui;ZHU Xiao-mei;HU Chao-ping;ZHOU Shui-zhen;LI Xi-hua;QIAN Tian;WANG Yi(Department of Clinical Nutrition,Children’s Hospital,Hehai University,Shanghai 201102,China;Department of Neurology,Children’s Hospital,Hehai University,Shanghai 201102,China)
出处
《中国临床医学》
2021年第4期699-704,共6页
Chinese Journal of Clinical Medicine
基金
复旦大学附属儿科医院儿童脊髓性肌萎缩症的多学科诊治与管理MDT项目,中国SMA诊治中心联盟(ASCC)项目.
关键词
脊髓性肌萎缩症
儿童
营养管理
spinal muscular atrophy
children
nutrition management
