| Objective: Duchenne muscular dystrophy(DMD)is a serious degenerative muscular disease affecting males.Characteristic progressive muscle wasting becomes noticeable in early childhood and,by their early teens,boys have become wheelchair bound.Premature death in their early twenties usually results from failure of the respiratory or cardiac musculature.Accompanying the disease are several nutrition-related concerns: growth,body composition,energy and protein requirements,bone health,constipation,swallowing difficulties,etc.It should be taken seriously to conduct nutritional assessment,nutrition risk screening and provide appropriate nutritional interventions after DMD diagnosis.This study is mainly for the children with DMD in walking stage in the third hospital of Hebei Medical University.The survey included growth,malnutrition,malnutrition risk,body composition and blood index in the children with DMD.Method:1 The 118 cases were selected from Department of Neuromuscular disease,the Third Hospital of Hebei Medical University.The time is from November 2015 to December 2016.All the patients were male who came from inpatient or outpatient,aged 5-16 years old.All subjects were in line with the diagnostic criteria of walking stage and the hormone treatment history is greater than 12 months.According to clinical symptoms,the children with DMD in walking stage was divided into early ambulatory stage and late ambulatory stage.In early ambulatory stage,the performance is easy to walking or running down and abnormal walking posture or gait.In late ambulatory stage,the performance is walking hard and the children is hard upstairs or to squatting.All the cases were divided into 2 groups according to the clinical symptoms,56 cases were in the early walking stage,aged 5~8 years old,mean age(7.07±1.16)years old.62 cases in late walking stage,aged 9~16 years old,the average age(10.82±1.76)years old.According to age they were divided into 5 groups.There were 21 children in 5~6 years old group,35 cases in 7~8 years old group;34 cases in 9~10 years old group;19 cases in the 11~12 years old group;9 cases in the 13~16 years old group.2 QuestionnairesThe survey includes name,age,date of birth,actual age,date of admission,disease diagnosis,course of disease,history of hormone medication,clinical symptoms,muscle strength,cultural level,primary caregivers,Caregiver's cultural level,caregiver-ship,family income,place of residence.3 Z-score methodRefer to the recommended height and weight standard of all age groups children,which was the survey data from 2005 nine cities.Z score was be calculated.Z score is calculated as: Z score = [child measurement(height or weight)value reference standard(height or weight)median] / reference standard(height or weight)standard deviation,Including the weight-for-age Z-score(WAZ),the height-for-age Z-score,the HAZ,the weight-Score,WHZ),body mass index-for-age(BAZ).Any of the above indicators <-2 can be determined to be undernourished,WAZ or BAZ<-2 is low body weight,HAZ <-2 is delayed growth,WHZ<-2 is weight loss,BAZ>1(for 5~18 years old)is overweight.4 Screening Tool for the Assessment of Malnutrition in Pediatrics(STAMP)score methodSTAMP score is based on the diagnosis with disease,the food intake and growth.First,it need to evaluate the existence of nutritional risk according to the diagnosis of children disease,there is no recorded as 0 points,there may be recorded as 2 points,there must be recorded as 3 points.And then to record the dietary changes through the questionnaire,the 0 points is no dietary change and good intake,the 2 points is less than half of dietary intake,no nutritional intake recorded as 3 points.When the patient?5 years old,STAMP score was need to be determined by BAZ score.When-2 <BAZ<+2,the nutritional risk score was 0 points,when-3 <BAZ?-2 or+2?BAZ<+3,the nutritional risk score was 1 point,when BAZ?-3 or BAZ?+3,the nutrition risk score was 3 points.The STAMP total score was the sum of above score.When STAMP total score was 0~1 points,it was low or without risk of malnutrition,when STAMP total score was 2~3 points,there was a moderate risk,when?4 points,there was a high risk of malnutrition.5 Body composition analysisThe Inbody220 Human Body Composition Analyzer(manufactured by Korea)was used to detect children.The data including age,height,weight,moisture content,inorganic salt content,fat content,muscle content,body mass index(BMI),body fat percentage(PBF%),obesity degree(OD),basal metabolic rate(BMR),growth fraction,etc.6 Laboratory inspectionThe patients' peripheral blood samples were obtained on the morning In fasting condition.Biochemical markers include serum total protein(TP),serum albumin(ALB),serum globulin(GLOB),serum potassium(K+),serum calcium(CA),Serum phosphorus(P)and creatinine(CREA),urea(UREA).Which were measured by OLYMPUS AU5400 biochemical test.The total protein was measured by biuret method,albumin and globulin were measured by bromine cresol green method,serum potassium method was determined by direct electrode method,serum calcium determination spectrophotometry,creatinine determination by dry film method.Blood routine indicators include red blood cell count(RBC),hemoglobin(HB),the United States produced COULTER ACT 5diff AL instrument,detection method with VCS method.7 Statistical analysisAll the data were analyzed by SPSS20.0 statistical analysis software.The count data statistical method includes Pearson chi-square test and continuous correction chi-square test and Fisher's exact test.The measurement data use independent samples t test.Results:1 Basic information118 cases were males,the steroid treatment history is greater than 12 months.The average age was(9.04±2.41)years old,average body weight(30.24±10.93)kg,average height(1.24±0.12)meter,average body mass index(BMI)(19.19±5.20)Kg/m2.The proportion of annual family income was 55.93% in 5~10 million.The children in the rural areas is accounting for 69.49%,The parents as major caregivers is 84.75% and 66.95% major caregivers received high school education.Compare with the early and late stage children with DMD,there was no significant difference in these aspects(P <0.05).Fifty-six children was in early walking stage and 5~8 years old,the mean age(7.07±1.16)years old,the mean body mass index(BMI)(16.64±2.64)Kg/m2.There were 62 cases in late walking stage,aged 9~16 years,the mean age was(10.82±1.76)years old,mean body mass index(BMI)21.5±5.84 Kg/m2.Control obesity or overweight BMI threshold of Chinese children aged 2 to 18 years old,there are 52(44.07%)cases to reach the overweight or obesity threshold,the cases include 19(33.93%)children in early walkingd stage and 33(53.23%)children with late walking stage.The incidence of obesity in children with DMD in late walking stage was significantly higher than that in early walking stage(P <0.05).Children with DMD were divided into 5 groups according to age.There were 21 cases aged 5~6 years old,35 cases aged 7~8 years old,34 cases aged 9~10 years old,19 cases aged 11~12years old,9 cases aged 13~16 years old.To reach the level of overweight or obesity BMI threshold,there were 7(33.33%)cases in 5~6 age group,12(34.29%)cases in 7~8 age group,18(52.94%)cases in 9~10 age group,9(47.37%)cases in 11~12 age group,6(66.67%)cases in 13~16 age group.Among them,13(38.24%)cases in 9~10 age group and 6(31.58%)in 11~12 age group and 4(11.43%)in 7~8 age group reached the obesity standard.2 Growth in children with DMD in walking stageThe incidence of delayed growth,overweight,obesity and low weight was respectively 67.80%,50.85%,28.82% and 16.10%.Compared with DMD children in the early walking stage,the incidence of delayed growth was higher in the late walking stage(P <0.05);the abnormality of BAZ score(overweight and low weight)in the late walking stage was higher(P <0.05);the abnormality of BAZ score(obesity and weight loss)in the late walking stage was higher(P <0.05).Compared with overweight and obesity,the incidence of low weight and weight loss was lower in children with DMD,which were 16.10% and 2.54%.The cases were divided into 5 age groups.The incidence of overweight were up to 60% in the late walking stage,of which,the children in 13~16 years old is as high as 66.67%.The high incidence of delayed growth was in all age groups,the highest incidence 84.21% was in 11~12 age group.The incidence of obesity in 9~10 age group is higher than others.3 The result of STAMP scoreThe incidence of malnutrition was high in children with DMD in walking stage.Compare with the early and late stage children,there was no significant difference in the incidence of moderate and high risk of malnutrition among children with early and late DMD(P>0.05).The incidence of high risk malnutrition was 54.24%.Of which,it is accounted for 57.81% in the late walking stage and 42.19% in the early walking stage.There was no significant difference in all age groups(P>0.05).The incidence of high risk of malnutrition was up to 60% in the 9~10 age group and 13~16 age group,it is respectively 61% and 66.67%.The 11~12 age group was accounting for 52.63%.4 Body composition analysis resultsThe study found that children with DMD in walking stage were experiencing overweight,high body fat content,reduced muscle mass and insufficient basal metabolic rate.The average amount of fat need to reduce(6.25±8.05)kg,and the average amount of muscle need to increase(2.33±3.80)kg.The children in late walking stage need to reduce the amount of fat and increase the amount of muscle more(P<0.05).The growth score was lower in late walking stage(P<0.05).Compared with the children in early walking stage,the abnormality of BMI(severe over,over and under)in late walking stage was higher(P<0.05),the abnormal levels of obesity(severe excess,excess and weakness)were higher(P<0.05),the insufficient incidence in the basal metabolic rate were higher(P<0.05).Compared with overweight and obesity,the incidence of the lack of body mass index,lack of body fat and weak was low and respectively 4.08%,8.16% and 12.24%.5 Blood test indicatorsThe abnormal items in blood test indicators are lower in the children with DMD in walking stage.The low incidence of Total protein was 36.44%.The low incidence of Albumin was 14.41%.The low incidence of Red blood cell count count was 11.02%.The low incidence of Hemoglobin was 28.81%.All children with Creatinine indicators were lower than normal,the incidence was 100.00%.Conclusion:1 The obesity rate was higher in children with DMD in late walking stage.2 Malnutrition includes delayed growth,overweight,obesity and low weight in children with DMD in walking stage.The incidence of delayed growth was higher in late walking stage.3 The insufficient of basal metabolic rate,decreased muscle mass,the exceeded body mass index and body fat percentage were serious problems in children with DMD in walking stage.The basal metabolic rate was lower in late walking stage. |
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