Đặc điểm lâm sàng, xét nghiệm và kết quả điều trị của bệnh nhược cơ thể toàn thân ở trẻ em

Abstract

Myasthenia Gravis is a neuromuscular synaptic transmission disorder characterized by muscle weakness and fatigue of skeletal muscles after strenuous activity due to impaired neuromuscular transmission. In Vietnam, there have not been many studies on clinical characteristics, laboratory indices, treatment response and factors related to acute attacks of autoimmune myasthenia gravis in children. Therefore, our study was conducted with the aim of describing the clinical characteristics, laboratory tests and outcomes of myasthenia gravis in children at the National Children's Hospital from 2023 - 2024. There were 17 patients with generalized myasthenia frepresenting 20.7% of myasthenia gravis. The Male/female ratio was 1/7.5. The earliest age of onset was 1 year old, the latest was 14 years old, the median was 7.35 years old. The most common clinical symptoms were ptosis (76%), dysphagia (64.7%), followed by pneumonia and weakness in walking (35.3%) which were triggered by cough and fever. The median AchR antibody level was 7.2 nmol/L (0.57 - 11.4 nmol/L). Patients responded well to treatment during the acute crisis and initial diagnosis: 100% of patients had no more difficulty breathing, no more dysphagia, no more weakness in walking, and had relief of ptosis symptoms. Pneumonia is a factor that increases respiratory failure in children with generalized myasthenia gravis. Recurrent acute crisis occurred in 58.8%.