The Formulation And Individualized Practice Research Of Dietary Management Program On Children With Glycogen Storage Disease Type Ⅰ

Background:Glycogen storage disease type I(GSD I)is a type of glucose metabolic disorder caused by deficiency of the glucose-6-phosphatase system involved in glycogenesis and glycogenolysis.At present,there is no specific treatment,the main methods are dietary management,raw cornstarch,and symptomatic treatment.Due to the deficiency of the glucose6-phosphatase system,glycogenesis and glycogenolysis pathways in children are blocked,and the digestion and absorption of food become the only source of blood glucose in children.Therefore,dietary therapy as a basic treatment is very important.Although the importance of dietary management has been mentioned in many reviews and the latest clinical guidelines,there is a lack of specific and targeted clinical practice on how to guide children with GSD I.Therefore,on the basis of literature evidence,this study formulated the dietary management program for children with GSD I,and implemented it individually in clinical practice,so as to preliminary evaluate its application effect.The purpose of this study is to help children with GSD I adjust their diet structure and eat scientifically and rationally,so as to facilitate the growth and development,improve metabolic control indicators,and prevent various long-term complications.Objective:To formulate dietary management program on children with GSD I and then evaluate its clinical effects.Methods:1.The Formulation of the dietary management program.On the basis of literature review,the framework and the first draft of the dietary management program was constructed.Two rounds of Delphi expert consultations were conducted among 11 experts and then formed the final version of dietary management program on children with GSD I.2.The evaluation of the individualized clinical practice effect of the dietary management program.This study is a case study.The subjects of this study were children with GSD I and their parents with poor dietary management.The intervention methods were carried out according to the dietary management program formed in the early stage.And the changes of metabolic control indexes,examination indexes of main organ functions(liver,kidney),growth and development(height,weight,body mass index)of the children were compared before and after dietary intervention.Results:1.The Formulation of the dietary management program.In the literature review,11 literatures were included,and 21 pieces of evidence were obtained.After two rounds of Delphi consulting,the final program includes two phases:the treatment period and the home period.During the treatment period,the first-level items are establishing multidisciplinary team,improving dietary management evaluation,making individualized diet plan and strengthening dietary health education.During the home period,the three first-level items are improving the children’s and their parents’ dietary treatment compliance,paying attention to blood glucose monitoring and adjusting the diet timely.2.The evaluation of the individualized clinical practice effect of the dietary management program.A total of 4 children were included and their indexes were compared before and after dietary adjustment.The results were as follows:(1)Case 1:This is a 4-year-10-month boy.The blood glucose level was basically maintained between 3.6 and 6.4mmol/L.After 11 months of dietary intervention,all the indicators(blood lipid,uric acid,lactic acid,liver enzymes)were basically within the normal range,especially the triglyceride decreased from 11.33mmol/L to 2.62mmol/L,and there was no higher risk of pancreatitis.In terms of growth and development indicators,the height increased by 12cm,the weight decreased by 0.6kg,and the BMI decreased by 5.1kg/m2.The results indicated that the height of the children increased rapidly and weight growth was effectively controlled on the basis of maintaining the stability of blood glucose.(2)Case 2:This is an 8-year-2-month boy.The blood glucose level was basically maintained between 3.8 and 6.3mmol/L.After 6 months of dietary intervention,all the indicators(blood lipid,uric acid,lactic acid,liver enzymes)were improved,especially the uric acid decreased from 433.9mmol/L to 320.7mmol/L.In terms of growth and development indicators,the height increased by 3cm,the weight decreased by 0.2kg,and the BMI decreased by 1.2kg/m2.On the basis of ensuring the growth and development of the child,the weight growth of the child was effectively controlled and the nutritional status of obesity was improved.(3)Case 3:This is a 14-year-10-month girl.During the dietary management,the blood glucose fluctuated significantly more stable than before,and the postprandial blood glucose decreased from 10mmol/L to 7mmol/L.After 7 months of dietary intervention,all the indexes(blood lipid,lactic acid,liver enzyme)were significantly decreased,TC and liver enzymes were reduced to the normal range,and there was no significant progress in the main organ function indexes.And the child’s height increased by 3cm,catching up to P50.(4)Case 4:This is an 18-year-old girl.The fluctuation of blood glucose during dietary management was significantly stable,and the postprandial blood glucose decreased from 15mmol/L to l0mmol/L.After 6 months of dietary intervention,all the indexes(blood lipid,lactic acid,liver enzymes)decreased significantly compared with before,especially the triglyceride decreased from 21.42mmol/L to 9.1mmol/L,and there was no significant progress in the main organ function indexes.And the child’s BMI increased by 21kg/m2,which improved her emaciated nutritional status.Conclusion:1.The dietary management program for children with GSD I was constructed in this study,which includes two stages:the treatment period and the home period.During the treatment period,the first-level items were establishing multidisciplinary team,improving dietary management evaluation,making individualized diet plan and strengthening dietary health education.During the home period,the three first-level items were to improve the children’s and their parents’ dietary treatment compliance,pay attention to blood glucose monitoring and timely dietary adjustment.2.After the implementation of the program,the blood glucose control,main metabolic indexes,main organ function examination indexes and growth and development of the four children were improved in varying degrees,indicating that the dietary management program can effectively help the children with GSD I to better maintain blood glucose stability,improve their metabolic control and growth and development,and prevent long-term complications.Therefore,it is worth further promotion and implementation in clinical.