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目的总结2例儿童噬血细胞综合征合并可逆性后部脑病综合征(PRES)的临床特点,提高国内儿科医生对该病的认识。方法回顾分析2013-04-03和2013-02-27中山大学附属第一医院东院儿科收治的2例噬血细胞综合征合并PRES患儿的临床表现及诊治过程,并结合国内外相关文献分析病例特点。结果 2例诊断为噬血细胞综合征,在使用环孢素A+地塞米松+鬼臼乙叉甙治疗过程中出现神经系统异常表现,伴高血压,综合分析诊断为PRES,经过及时降压、环孢素A减量或停药等对症治疗后完全恢复,随访至今病情稳定。结论 PRES是一种短期内可以完全恢复的神经系统疾病,在噬血细胞综合征患者中预后良好,但须与其他神经系统疾病鉴别,尤其是噬血细胞综合征神经系统病变。提高对PRES认识可减少误诊率。
Objective To summarize the clinical features of 2 cases of hemophagocytic syndrome in children with reversible posterior encephalopathy syndrome (PRES) and to raise the awareness of domestic pediatricians on the disease. Methods The clinical manifestations and diagnosis and treatment of two hemophagocytic syndrome patients with PRES admitted to the Eastern Hospital Affiliated to the First Affiliated Hospital of Sun Yat-sen University from April 2013 to March 2013 were retrospectively analyzed. The cases were analyzed according to relevant literature both at home and abroad Features. Results Two cases were diagnosed as hemophagocytic syndrome. Neurologic abnormalities were observed during treatment with cyclosporin A + dexamethasone + etoposide. Hypertension was diagnosed by comprehensive analysis, PRES was diagnosed by comprehensive analysis, Sporin A reduction or withdrawal and other symptomatic treatment after complete recovery, follow-up has been stable. Conclusions PRES is a neurological disorder that can be completely recovered within a short period of time and has a good prognosis in patients with hemophagocytic syndrome but must be distinguished from other neurological diseases, especially hemophagocytic neuropathy. Increasing the awareness of PRES can reduce the misdiagnosis rate.