摘要
Abstract
Inherited metabolic diseases(IMD),also known as congenital metabolic abnormalities(IEM),refer to a class of single gene genetic diseases due to biochemical metabolic abnormalities that lead to multiple organ damage.For the treatment of IMD,although gene and enzyme replacement therapy have received much attention,the majority of effective treatment methods are nutritional interventions.Nutritional therapy is widely available and has excellent therapeutic effects,so it is crucial for the management of many IMD.The research and clinical practice of IMD nutritional therapy mostly focused on different dietary compositions,and the treatment goal is to maintain stable and normal metabolism.These diseases require continuous enteral feeding during the catabolic stage to provide sufficient energy to meet metabolic needs,and prevent decompensation related to protein hydrolysis and lipolysis.The treatment goal of IMD is to maintain metabolic stability and normalcy.ETF is a key method in treating these diseases in children.Regardless of the type of the disease,home Enteral tube feeding(ETF)programs have increasingly been implemented globally to improve the quality of life.However,there is almost no implementation of tube feeding for patients with IMD in our country,which should be taken seriously and implemented.In this article,a brief introduction is given to the basic concepts and technique of ETF.关键词
儿童/遗传代谢病/管饲/营养治疗Key words
child/inherited metabolic diseases/enteral tube feeding/dietary treatment分类
医药卫生
