血浆置换在儿童危重自身免疫性疾病中的应用

Clinical effects of plasma exchange therapy on critical autoimmune diseases in children： report of 8 cases

目的观察血浆置换技术对儿童危重自身免疫性疾病的疗效及安全性。方法经股静脉或锁骨下静脉建立血管通路，对8例危重自身免疫性疾病患儿进行17次血浆置换治疗，病种分别为Guillain—Barre二氏综合征、类脊髓灰质炎综合征、系统性红斑狼疮、巨噬细胞活化综合征、Steven—Johnson二氏综合征和过敏性紫癜，男孩6例，女孩2例，年龄（10．2±3．7）岁（2岁3月-14岁），接受17次经股静脉或锁骨下静脉建立血管通路的血浆置换治疗，每次置换血浆量50～100ml／kg，每隔1～3日1次，共置换1-3次。1例系统性红斑狼疮和1例巨噬细胞活化综合征患儿同时联合连续性静脉静脉血液滤过治疗，8例患儿均联合糖皮质激素、免疫球蛋白或免疫抑制剂治疗。观察治疗前后临床症状、体征、血生化及自身抗体等变化。结果经血浆置换及综合治疗，7例患儿临床症状及生化指标明显好转，1例过敏性紫癜患儿治疗后无明显好转；有效率87．5％，8例患儿均存活。17次血浆置换治疗均顺利完成，治疗过程中1例次出现一过性荨麻疹，未见休克、严重出血倾向及感染等严重并发症。结论血浆置换联合其他综合治疗可迅速改善危重自身免疫性疾病患儿病情，明显提高生存率。

Objective To explore the clinical effects and safety of plasma exchange （PE） therapy on critical autoimmune diseases in children. Methods Eight children with critical autoimmune diseases, including Guillain- Barre syndrome, poliomyelitis-like syndrome, systemic lupus erythematosus, macrophage activation syndrome, Steven-Johnson syndrome, and Henoch-Schonlein purpura, received 17 sessions of underwent PE through lumen catheter inserted into femoral or subclavian veins, with the plasma volume of 50-100 ml/kg, in addition to administration of steroid, immunosuppressor and/or intravenous immunoglobin,. 1-3 sessions of PE were performed to each patient and 17 sessions were performed in total. The clinical symptoms, blood biochemical parameters and auto-antibodies were examined before and after therapy. Results The 8 patients all survived, and 7 patients＇ clinical symptoms and blood biochemical parameters were improved. The effective rate was 87.5%.No effect was seen only in 1 patient with Henoch-Schonlein purpura. The 17 sessions of PE were all completed smoothly. Only one patient experienced transient urticaria without any severe complications such as shock, serious bleeding tendency or infections. Conclusion PE therapy combined with other complex treatment alleviates the symptoms of critical autoimmune diseases in pediatric patients rapidly and improve their survival rate.