To summarize the effectiveness and nursing experience of enzyme replacement therapy in children with late-onset pompe disease(LOPD),and provide reference for clinical practice on November 2021.Care focused on adequate evaluation,medication observation and care,respiratory and skeletal muscle function exercise,high pro-tein and low carbohydrate diets,personalized psychological support,and discharge education and follow-up.Two patients received standard medication 25 times without any infusion related adverse reactions,increased body mass index,and improved clinical symptoms.The care of children with LOPD undergoing enzyme replacement therapy should be emphasized,and personalized treatment and nursing plans for medication,functional exercise,diet,and psychology can be developed,so as to effectively promote patient health and improve quality of life.
glycogen storage disease type Ⅱpompe diseaseenzyme replacement therapychildrennursing
万方数据
维普
