PDF(1254 KB)
环磷酰胺序贯治疗儿童抗N-甲基-D-天冬氨酸受体脑炎的疗效及安全性分析
朱蔚文, 廖卫平, 易咏红, 宋兴旺
中国当代儿科杂志 ›› 2017, Vol. 19 ›› Issue (6) : 668-671.
PDF(1254 KB)
PDF(1254 KB)
环磷酰胺序贯治疗儿童抗N-甲基-D-天冬氨酸受体脑炎的疗效及安全性分析
Efficacy and safety of cyclophosphamide as a sequential immunotherapy drug for anti-N-methyl-D-aspartate receptor encephalitis in children
目的 分析环磷酰胺作为二线药物治疗儿童抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎的疗效及安全性。方法 6例激素和丙种球蛋白冲击治疗效果欠佳的抗NMDAR脑炎患者序贯给予环磷酰胺治疗，进行疗效观察及安全性随访。结果 6例患儿经一线免疫治疗1~4周后，在精神症状、癫癎发作、不自主运动方面症状减轻；3例意识水平有所改善，能进行简单对答；所有患儿均仍有明显的反应迟钝，失语、失读、失写、失算、失用、运动障碍等高级皮层功能受损症状。予环磷酰胺序贯治疗，7 d内6例患儿均能进行简单对答；2~3周后3名学龄期患儿能进行简单计算，读写能力有较大改善，生活自理能力基本恢复；2~3个月后，6例患儿认知功能均基本恢复至病前水平，生活能力恢复正常。无不良反应发生及血常规、肝肾功能异常。结论 儿童抗NMDAR脑炎确诊后应及早给予免疫治疗，环磷酰胺序贯治疗效果良好，不良反应少，安全性高。
Objective To evaluate the efficacy and safety of cyclophosphamide as a second-line drug in the treatment of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Methods Six children with anti-NMDAR encephalitis, who showed poor response to steroids and intravenous immunoglobulin, were given cyclophosphamide as a second-line immunotherapy. Follow-up was performed to evaluate the efficacy and safety of cyclophosphamide. Results After first-line immunotherapy for 1-4 weeks, the six patients had reduced psychiatric symptoms, seizures, and involuntary movements; three patients had an improved level of consciousness and were able to make simple conversations. However, all the patients still showed slow response, as well as cortical dysfunction symptoms such as aphasia, alexia, agraphia, acalculia, apraxia, and movement disorders. The six patients continued to receive cyclophosphamide as a sequential therapy. They were able to answer simple questions 7 days after treatment. Three school-aged patients were able to make simple calculation, had greatly improved reading and writing ability, and almost recovered self-care ability 2-3 weeks later. The cognitive function of the six patients was almost restored to the level before the onset of disease, and their living ability returned to normal 2-3 months later. During the treatment period, there were no adverse reactions or abnormal results of routine blood test and liver and kidney function tests. Conclusions Children with anti-NMDAR encephalitis should be given appropriate immunotherapy as soon as possible. Cyclophosphamide as a sequential therapy has good efficacy and safety.
抗N-甲基-D-天冬氨酸受体脑炎 / 环磷酰胺 / 二线免疫治疗 / 儿童
Anti-N-methyl-D-aspartate receptor encephalitis / Cyclophosphamide / Immunotherapy / Child
[1] Penny DJ, Vick GW 3rd. Ventricular septal defect[J]. Lancet, 2011, 377(9771):1103-1112.
[2] 王一斌, 华益民, 刘瀚旻, 等. 室间隔缺损经导管关闭术后传导阻滞的相关因素分析[J]. 临床儿科杂志, 2007, 25(12):1002-1004.
[3] Ghaderian M, Merajie M, Mortezaeian H, et al. Efficacy and safety of using amplatzer ductal occluder for transcatheter closure of perimembranous ventricular septal defect in pediatrics[J]. Iran J Pediatr, 2015, 25(2):e386.
[4] Lee SM, Song JY, Choi JY, et al. Transcatheter closure of perimembranous ventricular septal defect using Amplatzer ductal occluder[J]. Catheter Cardiovasc Interv, 2013, 82(7):1141-1146.
[5] Du RZ, Qian J, Wu J, et al. Cardiac resynchronization therapy for heart failure induced by left bundle branch block after transcatheter closure of ventricular septal defect[J]. J Geriatr Cardiol, 2014, 11(4):357-362.
[6] Maréchaux S, Guiot A, Castel AL, et al. Relationship between two-dimensional speckle-tracking septal strain and response to cardiac resynchronization therapy in patients with left ventricular dysfunction and left bundle branch block:a prospective pilot study[J]. J Am Soc Echocardiogr, 2014, 27(5):501-511.
[7] Fukuda T, Suzuki T, Kashima I, et al. Shallow stitching close to the rim of the ventricular septal defect eliminates injury to the right bundle branch[J]. Ann Thorac Surg, 2002, 74(2):550-555.
[8] Yip WC, Zimmerman F, Hijazi ZM, et al. Heart block and empirical therapy after transcatheler closure of perimembranous ventricular septal defect[J]. Catheter Cardiovasc Interv, 2005, 66(3):436-441.
[9] 戴辰程, 魏淑萍, 郭保静, 等. 室间隔缺损封堵术后持续性完全性左束支传导阻滞外科治疗缓解一例[J]. 中国介入心脏病学杂志, 2010, 18(6):358-359.
[10] Bhardwaj R. Etiology and left ventricular functions in left bundle branch block-a prospective observational study[J]. J Assoc Physicians India, 2016, 64(9):36-38.
[11] Deniz A, Özmen Ç, Akta? H, et al. Electrocardiographic markers of left ventricular systolic dysfunction in patients with left bundle branch block[J]. Kardiol Pol, 2016, 74(1):25-31.
[12] Ozdemir K, Altunkeser BB, Dani? G, et al. Effect of the isolated left bundle branch block on systolic and diastolic functions of left ventricle[J]. J Am Soc Echocardiogr, 2001, 14(11):1075-1079.
[13] Zanco P, Desideri A, Mobilia G, et al. Effects of left bundle branch block on myocardial FDG PET in patients without significant coronary artery stenoses[J]. J Nucl Med, 2000, 41(6):973-977.
[14] Mozos I, Serban C. The relation between QT interval and T-wave variables in hypertensive patients[J]. J Pharm Bioallied Sci, 2011, 3(3):339-344.
[15] Ciobanu A, Gheorghe GS, Ababei M, et al. Dispersion of ventricular repolarization in relation to cardiovascular risk factors in hypertension[J]. J Med Life, 2014, 7(4):545-550.
[16] Rahimi Darabad B, Vatandust J, Pourmousavi Khoshknab MM, el al. Survey of the effect of streptokinase on ventricular repolarization by examining the QT dispersion in patients with acute myocardial infraction in Seyed-Al-Shohada Hospital, Urmia[J]. Glob J Health Sci, 2014, 6(7):74-82.
[17] Bol Raap G, Meijboom FJ, Kappetein AP, et al. Long-term follow-up and quality of life after closure of ventricular septal defect in adults[J]. Eur J Cardiothorac Surg, 2007, 32(2):215-219.
