血浆置换治疗儿童危重自身免疫性疾病临床分析

目的 观察血浆置换(plasma exchange,PE)技术对儿童危重自身免疫性疾病的疗效并探讨其机制.方法 用PE治疗3例系统性红斑狼疮,3例血栓性血小板减少性紫癜,5例吉兰-巴雷综合征,1例溶血尿毒综合征患儿.12例危重自身免疫性疾病患儿体重10～35 kg,平均(26.25±6.85) kg,每次置换量50～70 ml/kg,每日或隔日1次,共置换3～5次.检测PE治疗前后血生化、自身抗体及临床症状、体征等变化.结果 2例系统性红斑狼疮患儿在第1次PE治疗后神经系统症状明显减轻,血清补体升高,ANA和抗ds-DNA抗体滴度下降;3例血栓性血小板减少性紫癜患儿神志改善,于PE治疗后第5天血小板恢复正常;4例吉兰-巴雷综合征患儿均未发生呼吸衰竭,于PE疗程结束后双下肢肌力逐渐恢复.PE治疗前后血电解质和CO2CP保持稳定.1例溶血尿毒综合征和1例系统性红斑狼疮患儿死于肺出血,余10例好转出院,有效率83%.结论 PE联合其他综合治疗可明显提高危重自身免疫性疾病患儿的生存率,值得进一步研究和应用.

Plasma exchange in the treatment of pediatric antoimmune diseases

Objective To investigate the efficacy of plasma exchange (PE) and to explore its mechanism in the treatment of pediatric antoimmune diseases.Methods The 12 children with antoimmune diseases,including 3 systemic lupus erythematosus (SLE),3 thrombotic thrombocytopenic purpura (TTP),5 Guillain-Barre syndrome (GBS),1 hemolytic uremic syndrome(HUS),weighted 10～35 kg,were treated with PE.The PE volume was 50～70 ml/kg(1/4 Hydroxyethyl Starch,3/4 fresh frozen plasma)every time.The ANA,anti-dsDNA,complements and clinic symptoms were examined before and after PE.Results The neurological and psychiatric symptoms in 2 SLE childrern were ameliorated after the first PE.The levels of complement were increased,while the levels of ANA and anti-dsDNA were decreased after PE.The clinical symptoms of the 3 TTP were resolved immediately after the therapy,and their thrombocyte returned to normal in the fifth day after PE.The 4 GBS had not acute respiratory failure.Myodynamia of the lower limbs had improved increasedly after PE.Two children in 12 patiens died of pulmonary hemorrhage,10 had been discharged from the hospital,the cure rate was 83%.Conclusion PE was effective in treatment of pediatric antoimmune diseases and improved survival rate of patients.