糖皮质激素在八种儿童神经系统疾病中的应用分析

Analysis of the application of glucocorticoids in eight pediatric neurological disorders in China

目的了解儿童神经系统疾病中糖皮质激素的应用现况,为进一步规范化糖皮质激素的应用提供依据。方法横断面研究,2019年7月8日至28日通过中华医学会儿科学分会临床药理学组、神经学组和儿童药物专业委员会对全国1073家医院1850名儿科医生,针对8种儿童神经系统疾病的糖皮质激素应用情况进行电子问卷调研,病种包括细菌性脑膜炎、抗N-甲基-D天冬氨酸受体脑炎、视神经脊髓炎谱系疾病、多发性硬化、吉兰-巴雷综合征、重症肌无力、幼年型皮肌炎和Duchenne型肌营养不良,并与现行指南或共识进行比较以判断应用是否规范,规范性评价相关因素分析以医院等级、医生从业时间及有无小儿神经专科为自变量,应用χ^(2)检验及多因素Logistic回归分析进行统计学处理。结果收集到来自全国31个省、自治区、直辖市1073家医院1834名(99.1%)儿科医师的有效问卷。糖皮质激素应用规范率依次为幼年型皮肌炎5.8%(51/879)、重症肌无力13.7%(153/1115)、抗N-甲基-D天冬氨酸受体脑炎18.2%(142/781)、细菌性脑膜炎18.5%(310/1680)、Duchenne型肌营养不良23.0%(248/1079)、视神经脊髓炎谱系疾病或多发性硬化33.4%(322/964)、吉兰-巴雷综合征43.5%(477/1096),其中以使用疗程不规范最为常见,规范率36.8%(12.5%~62.2%)。糖皮质激素应用规范性多因素Logistic回归分析提示所在医院有儿童神经专科的医生在细菌性脑膜炎(OR=1.83,95%CI 1.39~2.43,P<0.01)、吉兰-巴雷综合征(OR=1.97,95%CI 1.50~2.59,P<0.01)、Duchenne型肌营养不良(OR=1.85,95%CI 1.38~2.48,P<0.01)、幼年皮肌炎(OR=2.08,95%CI 1.03~4.19,P=0.040)、抗N-甲基-D天冬氨酸受体脑炎(OR=0.302,95%CI 0.20~0.46,P<0.01)的诊治中糖皮质激素应用规范率较所在医院无儿科神经专业的医师更高。结论儿科医生在儿童神经系统疾病中糖皮质激素应用总体规范率不足一半,所在医院有儿童神经专科的医生在多种疾病中的糖皮质激素应用更为规范,需进一步加强指南或共识学习及儿童神经专科医师相关培训。

Objective To investigate the application of glucocorticoids in children with neurological diseases by pediatricians,and to provide baseline data for further standardization of glucocorticoids application.Methods This was a cross-sectional survey,an electronic questionnaire survey was conducted online from July 8,2019 to July 28,2019 through the Subspecialty Group of Clinical Pharmacology,the Subspecialty Group of Neurology,and Expert Committee on Rational Use of Medicines for Children in the Society of Pediatrics,Chinese Medical Association.This survey was conducted for the application of glucocorticoids in 8 neurological diseases in children,and compared with the current guidelines or consensus to judge whether the application was standard.A total of 1850 pediatricians from 1073 hospitals were surveyed.Surveyed diseases included bacterial meningitis,anti-N-methyl-D-aspartate receptor encephalitis,neuromyelitis optica spectrum disease(NMOSD),multiple sclerosis,Guillain-Barre syndrome,myasthenia gravis,juvenile dermatomyositis,and Duchenne muscular dystrophy.In order to explore the associated factors of standardized glucocorticoid application,the level of hospital,whether there was an independent department of pediatric neurology,and working experience of the doctor were used as independent variables.Theχ^(2) test and multivariate Logistic analysis was used for statistical analysis.Results A total of 1834(99.1%)valid questionnaires were collected from 1073 hospitals in 31 provinces,autonomous regions and municipalities.The coincidence rate with recommendation of the current guideline or consensus on glucocorticoids was 5.8%(51/879)in juvenile dermatomyositis,13.7%(153/1115)in myasthenia gravis,18.2%(142/781)in bacterial meningitis,18.5%(310/1680)in anti-N-methyl-D-aspartate receptor encephalitis,23.0%(248/1079)in Duchenne muscular dystrophy,33.4%(322/964)in NMOSD and multiple sclerosis,and 43.5%(477/1096)in Guillain-Barre syndrome.The most common one was the irregular course of treatment,and the coincidence rate was 36.8%(12.5%-62.2%).The multivariate Logistic regression analysis indicated that the use of glucocorticoids was more standardized in bacterial meningitis(OR=1.83,95%CI 1.39-2.43,P<0.01),Guillain-Barre syndrome(OR=1.97,95%CI 1.50-2.59,P<0.01),Duchenne muscular dystrophy(OR=1.85,95%CI 1.38-2.48,P<0.01),juvenile dermatomyositis(OR=2.08,95%CI 1.03-4.19,P=0.040)and N-methyl-D-aspartate receptor encephalitis(OR=0.302,95%CI 0.20-0.46,P<0.01)and by pediatricians with pediatric neurology specialty in the hospital.Conclusions The coincidence rate with recommendation of the current guideline or consensus on glucocorticoids is less than half in children with neurological diseases,and the use of glucocorticoids is more standardized in a variety of diseases by pediatricians with pediatric neurology specialty in the hospital,which indicates that the study of guidelines or consensus and the special training of pediatric neurologists should be further strengthened.