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Research progress of nutritional management of children with spinal muscular atrophy
Received:June 15, 2020  Revised:October 09, 2020  Click here to download the full text
Citation of this paper: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.Research progress of nutritional management of children with spinal muscular atrophy[J].Chinese Journal of Clinical Medicine,2021,28(4):699-704
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Author NameAffiliationE-mail
GONG Xiao-yan Department of Clinical Nutrition, Children's Hospital, Fudan University, Shanghai 201102, China  
LU Zhong-ying Department of Clinical Nutrition, Children's Hospital, Fudan University, Shanghai 201102, China  
LI Wen-hui Department of Neurology, Children's Hospital, Fudan University, Shanghai 201102, China  
ZHU Xiao-mei Department of Neurology, Children's Hospital, Fudan University, Shanghai 201102, China  
HU Chao-ping Department of Neurology, Children's Hospital, Fudan University, Shanghai 201102, China  
ZHOU Shui-zhen Department of Neurology, Children's Hospital, Fudan University, Shanghai 201102, China  
LI Xi-hua Department of Neurology, Children's Hospital, Fudan University, Shanghai 201102, China  
QIAN Tian Department of Clinical Nutrition, Children's Hospital, Fudan University, Shanghai 201102, China  
WANG Yi Department of Neurology, Children's Hospital, Fudan University, Shanghai 201102, China yiwang@shmu.edu.cn 
Abstract: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.
keywords:spinal muscular atrophy  children  nutrition management
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