| Objective:To analyze the clinical features and serum-related antibodies of myasthenia gravis(MG)in children and initially explore the correlation between serum-related antibodies and clinical features of MG in children,and provide a basis for the choice of diagnosis and treatment plan and prognosis judgment.Methods:The clinical data of 127 MG patients in the Department of Neurology of the Children’s Hospital of Chongqing Medical University from September 2017 to August 2019 was collected,and all the patients completed4 serum-related antibody tests for MG.Clinical data like age of onset,gender,clinical symptoms,other combined autoimmune diseases,treatments,drug side effects,clinical outcomes was all included.Summarize the main clinical features of MG in children and comparative analysis of the clinical features of the 4 groups:only AchR-Ab(+),AchR-Ab(+)with Titin-Ab(+)orRyR-Ab(+),AchR-Ab(-)with Titin-Ab(+)andRyR-Ab(+),all antibody negative.Results:1.127 MG patients were included with a 1:1.65 ratio of men to women.The average age of onset was 3.89±3.09 years old,most of them were 1-3 years old(63.78%).2.A total of 107 patients(84.25%)were classified as MG type I,and 19 patients were classified as MG type II,including 8 cases of MG type IIa(6.29%)and 11 cases of MG type IIb(8.67%),and 1 patient was classified as MG type III(0.79%).3.The positive rate of the Neostigmine test andRepetitive Neuroelectric Stimulation was98.43%(125/127)and 18.89%(24/127).The positive rate of the AchR-Ab was 70.86%(90/127).The positive rate of the Titin-Ab andRyR-Ab were both 24.42%(31/127).No one was positive for Mu SK-Ab and 20.47%(26/127)cases were negative for all the 4 antibodies.4.The rate of abnormal thyroid function was 25.19%(32/127),the rate of abnormal autoantibody detection was 25.98%(33/127).Enlarged thymus with uneven density was found in 2 patients(1.57%).5.The remission rate of patients treated with pyridostigmine monotherapy or pyridostigmine combined with glucocorticoid was 74.47%,but the therapeutic effect of pyridostigmine combined with glucocorticoid is better than that of pyridostigmine monotherapy(P=0.002).The prognosis of patients who added glucocorticoid after the failure of pyridostigmine monotherapy was better than those who did not add glucocorticoid(P=0.000).6.There was statistically significant difference in age of onset in the 4 groups(P=0.000),but there was no statistically significant difference in gender,clinical classification,auxiliary examination,treatment and prognosis(P>0.05).7.In the AchR-Ab positive group,the AchR-Ab titer showed a positive correlation with disease course(r_s=0.29,P=0.018),there is a significant difference between AchR-Ab titer andRepetitive Neuroelectric Stimulation.Conclusions:1.MG in children was more common in females and the peak onset age was 1-3 years old.Type I(ocular MG)was more common and generalized type was rare.2.MG in children may be accompanied by abnormal thyroid function while combined with other autoimmune diseases were rare.3.Thymoma was rare in MG in children.In this study,no thymoma patients were found in Titin-Ab andRyR-Ab positive patients.4.Children with MG are sensitive to glucocorticoid,and when pyridostigmine monotherapy is not effective,it can be combined with glucocorticoid.5.No significant differences were found in the clinical features and treatment response of serum-related antibody subtypes in children with MG.The AchR-Ab titer showed a positive correlation with disease course and was related toRepetitive Neuroelectric Stimulation. |
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