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儿科急性中枢神经系统炎症性脱髓鞘疾病患者的治疗性血液净化。

Therapeutic apheresis in pediatric patients with acute CNS inflammatory demyelinating disease.

机构信息

Department of Nephrology and Rheumatology, Georg August University Göttingen, Göttingen, Germany.

出版信息

Blood Purif. 2013;36(2):92-7. doi: 10.1159/000354077. Epub 2013 Sep 7.

DOI:10.1159/000354077
PMID:24021839
Abstract

BACKGROUND/AIMS: In adults, plasma exchange (PE) has been shown to be an efficient treatment for severe relapses of acute inflammatory CNS demyelinating diseases. The aim of this study was to evaluate the safety and efficacy of this treatment in pediatric patients.

METHODS

We retrospectively analyzed a single-center cohort of pediatric patients with inflammatory CNS demyelinating disorders who underwent apheresis between 2007 and 2011.

RESULTS

Ten patients (mean age: 11.6 ± 3.4 years) with an acute relapse of multiple sclerosis (n = 5), neuromyelitis optica (n = 2) or acute disseminated encephalomyelitis were included. All received methylprednisolone prior to treatment with either PE (n = 5) or immunoadsorption (n = 5). Apheresis-related side effects were either self-limiting or easily managed. EDSS (Expanded Disability Status Scale) improved in 7 of 8 patients during apheresis and in all patients within 30 days from a median of 7.5 to 1 (p < 0.01). The visual acuity initially worsened during the procedure in 3 of 7 affected eyes (mean 0.09), but improved in all at follow-up (mean: 0.5; p = 0.008).

CONCLUSIONS

Apheresis was well tolerated and associated with a favorable outcome in all pediatric patients similar to reports in adults.

摘要

背景/目的:在成人中,血浆置换(PE)已被证明是治疗急性炎症性中枢神经系统脱髓鞘疾病严重复发的有效方法。本研究的目的是评估该治疗方法在儿科患者中的安全性和疗效。

方法

我们回顾性分析了 2007 年至 2011 年间在单中心接受过血浆分离术的炎症性中枢神经系统脱髓鞘疾病儿科患者的队列。

结果

10 例患者(平均年龄:11.6 ± 3.4 岁)患有急性多发性硬化症(n = 5)、视神经脊髓炎(n = 2)或急性播散性脑脊髓炎的复发。所有患者在接受 PE(n = 5)或免疫吸附(n = 5)治疗前均接受了甲基强的松龙治疗。与治疗相关的副作用是自限性的或易于管理的。EDSS(扩展残疾状况量表)在 8 例患者中有 7 例在接受血浆分离术期间和所有患者在 30 天内从中位数 7.5 改善至 1(p < 0.01)。7 只受影响眼中有 3 只(平均 0.09)在手术过程中最初视力恶化,但在随访时所有视力均有所改善(平均:0.5;p = 0.008)。

结论

与成人报告相似,所有儿科患者均耐受良好,且与良好的结局相关。

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