Correlation Studies Of Glucocorticoids Long-term Effects On Duchenne Muscle Dystrophy

Objective:To explore the effect of glucocorticoid therapy to30Duchenne muscledystrophy(DMD) patients, to explore the effective dosage and schedule ofglucocorticoid therapy to DMD.Methods:1. With reference “shenjing xitong yichuanxing jibing”, publishedfrom People’s medical publishing house, the first edition in July2001,diagnosis criteria about DMD,30male patients aged from2to11anddiagnosed as DMD in outpatient and inpatient department of Children’sHospital of Chongqing Medical University from2005to2011, are includedin our clinical research.2.30patients will have the gene detection before treatment. Thetreatment will last fifteen month, they will have a follow-up everythree-month. Each follow-up the dose of drugs will be adjusted and patients will get some related function such as muscle strength, the changes ofpseudohypertrophy of gastrocnemius, Gower’s sign, myocardial enzymeand laboratory testing.3. The main drug is prednisone, the specific dosage as follows:1.5mg/kg/day in the first3-month after treatment,1.0mg/kg/day in thesecond3-month,0.75mg/kg/day in the third3-month,0.5mg/kg/day in theforth3-month，and it needs oral in the morning. One year after treatment,the dosage has turned to0.5mg/kg/day. In order to prevent side effectsprednisone may bring, we also add the calcium(calcium carbonate andvitamin tablets)500mg, immunity regulator(mannatide tablets)5-15mg andlong-term vitamin(fursultimine tablets)25mg every or every other day.4. Before and one year after the treatment, each patient will have thelimbs long bone radiography to know if he has developed the osteoporosis.5. All data were analyzed by SPSS software, vesion17.0andstastistical significance was set at P<0.05.Results:1. The positive rate of gene deletion was76.67%. From the results ofgenetic testing, we can see different exons, the proportion of missing is alsodifferent: exons51(30%)、exons48(26.67%)、exons45(20%)、exons50(16.67%)、exons49(13.33%)、exons17(10%)、exons8(6.67%)、exons12(6.67%)、exons44(6.67%)、exons19(3.33%). 2. There is no statistical significance between the number of missingDMD gene exons and30fasting patients serum CK, CK MB and LDHlevel before and one year after the treatment, that means there is nocorrelation between them.However, glucocorticoid therapy is related to CK,CK MB and LDH.3. Before treatment, the mean muscle strength score of30patients was46.1points, after3month, the score has up to49.8points, then from51.8points up to53.0points, until a year after treatment, the score hasamounted to53.6points. Muscle strength is gradually rising trend.4. In different period after treatment, mean value of serum CK, CKMB and LDH level are different degree of decline.5. From statistical results, the hardness of gastrocnemius haveobviously improved in76.7%of patients, for the performance of hardnesseased and there is part of sense of flexibility.6. Before treatment, each patient has the positive Gower’s sign, until afull year after treatment, only eight patients has the positive one in30patients, the other22patients have been converted into negative, thenegative rate is83.3%.7. Before treatment, there are eight patients have a mildly abnormalbased lung function, treatment after a year, seven patients have returned tonormal lung function, the negative rate is62.5%.8. Before and after a year treatment, every patient would have the limbs long bone radiography, and their own two results will be compared toknow whether there is bone change or not. Results shows that there are nosignificantly changes such as osteoporosis before and after traeatment in30patients.9. There is no obvious decline in immunity of patients before and aftertreatment.10. There is no patients have had ocular complaint such as blurredvision.11. One patient appeared mild Cushing syndrome manifestations forhe had doubled the dosage of prednisone and gradually restored afterdosage adjustment.Conclusion:1. Prednisone administration is effective for the treatment ofDuchenne muscle dystrophy muscle dystrophy.2. Most of patients have not experienced obvious side effects ofglucocorticoids in our study.