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治疗性血浆置换在儿童中枢神经系统急性脱髓鞘综合征患者中的应用:单中心经验。

Therapeutic plasma exchange in pediatric patients with acute demyelinating syndromes of the central nervous system: A single-center experience.

机构信息

Pediatric Intensivist, Pediatric Intensive Care, University of Health Sciences Turkey, Bakirkoy Dr. Sadi Konuk Training and Research Hospital, Istanbul, Turkey.

Pediatric Neurology, University of Health Sciences Turkey, Bakirkoy Dr. Sadi Konuk Training and Research Hospital, Istanbul, Turkey.

出版信息

Transfus Apher Sci. 2022 Aug;61(4):103421. doi: 10.1016/j.transci.2022.103421. Epub 2022 Mar 14.

Abstract

BACKGROUND

Therapeutic plasma exchange (TPE) is an extracorporeal treatment that can be used in adult and pediatric patients with acute demyelinating syndromes of the central nervous system. In this study, the efficacy and safety of TPE was evaluated in 10 pediatric patients who underwent TPE that were unresponsive to corticosteroid treatment.

METHODS

Records of 10 pediatric patients who underwent TPE in our pediatric intensive care unit (PICU) between May 2017 and June 2020 were used. Expanded Disability Status Scale (EDSS), Gait Scale (GS), and Visual Outcome Scale (VOS) were applied to the patients before and after TPE.

RESULTS

Of the 10 patients who underwent TPE, five were diagnosed with multiple sclerosis (MS), three with transverse myelitis (TM), and two with acute disseminated encephalomyelitis (ADEM). The median age of the patients was 13.3 years (IQR 8-15), and the median day from symptom onset to onset of TPE was 12.5 days (IQR 7-28). A total of 104 TPE sessions were performed successfully. While no complications were encountered in three patients during the sessions, the most common complication was hypofibrinogenemia. The decrease in EDSS and GS scores was found to be consistent with the clinical response of the patients. There was no statistically significant decrease in the VOS.

CONCLUSIONS

With this study, we can say that TPE is a feasible, effective, and safe treatment modality in children with acute demyelinating syndromes of the central nervous system.

摘要

背景

治疗性血浆置换(TPE)是一种体外治疗方法,可用于治疗成人和儿科中枢神经系统急性脱髓鞘综合征患者。在这项研究中,评估了 TPE 在 10 名对皮质类固醇治疗无反应的儿科患者中的疗效和安全性。

方法

使用了 2017 年 5 月至 2020 年 6 月期间在我们儿科重症监护病房(PICU)接受 TPE 的 10 名儿科患者的记录。在 TPE 前后应用扩展残疾状况量表(EDSS)、步态量表(GS)和视觉结果量表(VOS)对患者进行评估。

结果

在接受 TPE 的 10 名患者中,5 名被诊断为多发性硬化症(MS),3 名被诊断为横贯性脊髓炎(TM),2 名被诊断为急性播散性脑脊髓炎(ADEM)。患者的中位年龄为 13.3 岁(IQR 8-15),从症状出现到开始 TPE 的中位时间为 12.5 天(IQR 7-28)。总共成功进行了 104 次 TPE 治疗。虽然在治疗过程中没有 3 名患者出现并发症,但最常见的并发症是低纤维蛋白原血症。EDSS 和 GS 评分的降低与患者的临床反应一致。VOS 没有统计学上的显著降低。

结论

通过这项研究,我们可以说 TPE 是治疗儿童中枢神经系统急性脱髓鞘综合征的一种可行、有效且安全的治疗方法。

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