A Retrospective Evaluation of Children Diagnosed with Dermatomyositis: A Single-center Study

IF 0.2 Q4 MEDICINE, GENERAL & INTERNAL Medical Journal of Bakirkoy Pub Date : 2022-06-01 DOI:10.4274/bmj.galenos.2022.2021.12-9
Mahmut Aslan, S. Güngör, Y. Tabel, Bilge Özgör, S. Kırık
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Abstract

Objective: Juvenile idiopathic inflammatory myopathies are systemic autoimmune disorders that are characterized by chronic skeletal muscle inflammation, skin rashes and other systemic involvements. We analyzed the clinical findings, laboratory values at admission and treatment protocols and treatment responses of patients who were followed up with a diagnosis of juvenile dermatomyositis (JDM) in a department of pediatric neurology and rheumatology clinics. Methods: Fifteen patients who were referred to the department of pediatric neurology and pediatric rheumatology clinics, diagnosed with JDM between 2010 and 2017 were evaluated retrospectively via their medical records. Results: Of the study sample, 12 (80%) of the patients were female and 3 (20%) were male, and their mean age was 9.26±3.21 years. The mean time between complaint and diagnosis was 7.8±6 months, and the patients were followed up for 24.93±15.28 months after their diagnosis. The mean creatine kinase levels of the patients were 1.354±840 U/L. Fifteen (100%) of the patients had muscle weakness, 14 (93.3%) had Gottron’s papules and 12 (80%) patients had a heliotrope rash. Ten (66.6%) underwent muscle biopsy, 9 (60%) underwent electromyography and 5 (33.3%) patients underwent muscle magnetic resonance imaging. All the patients were treated with corticosteroids and immunosuppressive agents. Conclusion: JDM is a rare inflammatory myopathy observed during childhood. Better responses can be achieved by early diagnosis, intensive immunosuppressive therapy and effective physical therapy.
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诊断为皮肌炎的儿童的回顾性评价:一项单中心研究
目的:青少年特发性炎症性肌病是一种全身性自身免疫性疾病,以慢性骨骼肌炎症、皮疹和其他全身性受累为特征。我们分析了在儿科神经科和风湿病诊所诊断为青少年皮肌炎(JDM)的患者的临床表现、入院时的实验室值、治疗方案和治疗反应。方法:通过病历对2010年至2017年间转诊至儿童神经内科和儿童风湿病诊所的15名诊断为JDM的患者进行回顾性评估。结果:在研究样本中,12名(80%)患者为女性,3名(20%)为男性,平均年龄为9.26±3.21岁。从主诉到诊断的平均时间为7.8±6个月,患者在诊断后随访24.93±15.28个月。患者的平均肌酸激酶水平为1.354±840U/L。15名(100%)患者出现肌无力,14名(93.3%)患者出现戈特龙丘疹,12名(80%)患者出现日光疹。10例(66.6%)接受了肌肉活检,9例(60%)接受了肌电图检查,5例(33.3%)患者接受了肌肉磁共振成像检查。所有患者均接受皮质类固醇和免疫抑制剂治疗。结论:JDM是一种罕见的儿童炎症性肌病。早期诊断、强化免疫抑制治疗和有效的物理治疗可以获得更好的反应。
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来源期刊
Medical Journal of Bakirkoy
Medical Journal of Bakirkoy MEDICINE, GENERAL & INTERNAL-
CiteScore
0.20
自引率
0.00%
发文量
58
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