PDF(Pubmed)
Abstract：
Juvenile myasthenia gravis (MG) is a rare autoimmune neuromuscular disease, often treated with anticholinesterases, corticosteroids, and immunosuppressants. However, optimal treatment durations remain unclear. This study investigated the clinical characteristics and treatment of juvenile MG, including medication duration. The administration period for all drugs, immunosuppressants, and prednisolone at doses greater than 0.35 mg/kg daily was extracted retrospectively from medical records. Nineteen participants (8 boys, 11 girls) aged 8 months to 14 years (median, 2.5 years) at onset were identified. Fourteen patients (73.7%) had ocular MG and five (26.3%) had generalized MG. Drug treatment was conducted in 18 cases; however, 7 patients did not complete the treatment. Among the patients who completed drug treatment, the duration of treatment ranged from 11 to 100 months (median, 47 months). In the six patients treated with continuous administration of prednisolone or immunosuppressants, the treatment duration ranged from 33 to 99 months (median, 56 months). No severe adverse effects requiring hospitalization were reported. The patients treated with prednisolone or immunosuppressants required at least 33 months of treatment. These results will help develop protocols for juvenile MG treatment.
摘要：
青少年重症肌无力（MG）是一种罕见的自身免疫性神经肌肉疾病，经常用抗胆碱酯酶治疗，皮质类固醇,和免疫抑制剂.然而,最佳治疗持续时间仍不清楚。本研究探讨了青少年MG的临床特点和治疗方法。包括用药时间。所有药物的给药期限，免疫抑制剂,和泼尼松龙剂量大于0.35mg/kg/日,我们从医疗记录中回顾性提取.19名参与者(8名男孩，11名女孩)年龄8个月至14岁(中位数，发病2.5年）。14例（73.7％）患有眼部MG，5例（26.3％）患有全身性MG。18例进行了药物治疗；然而，7例患者未完成治疗。在完成药物治疗的患者中，治疗持续时间为11至100个月(中位数，47个月）。在连续使用泼尼松龙或免疫抑制剂治疗的六名患者中，治疗持续时间为33至99个月(中位数，56个月）。没有报告需要住院治疗的严重不良反应。使用泼尼松龙或免疫抑制剂治疗的患者需要至少33个月的治疗。这些结果将有助于开发青少年MG治疗方案。
