RT期刊文章SR电子T1预后指标的改进与血浆置换治疗儿科脱髓鞘摩根富林明神经病学神经学乔FD Lippincott Williams &威尔金斯SP e2065 OP e2073 10.1212 / WNL。首页93签证官0000000000008551 22 A1 Andrea Savransky A1 Adrian Rubstein A1滨Huaman里奥斯A1西尔瓦娜l . Vergel A1梅布尔卡斯特罗委拉斯开兹A1萨拉·佩雷斯·塞拉A1加芙Marcarian A1的大猩猩Alba A1安娜·m·Pugliese A1西尔维亚Tenembaum年2019 UL //www.ez-admanager.com/content/93/22/e2065.abstract AB目标来确定治疗的安全性和临床效益血首页浆置换(TPE)救援疗法在儿童急性炎性脱髓鞘中枢神经系统症状和识别基线治疗预后指标的改善。连续方法这单中心回顾性儿科人群包括所有我们住院的病人在此期间从2003年到2017年由于steroid-refractory急性中枢神经系统事件被认为是炎症需要TPE。功能状态评估,以确定改进包括以下性能类别尺度:视觉结果,膀胱控制,步态,并扩大残疾状态量表(eds)。这些评估每个病人之前和之后进行TPE。结果78年六十五名儿童需要TPE治疗CNS袭击包括进行分析。在TPE年龄中位数为10.5年(-18 - 1.9年);45%是女生。血清阳性(aquaporin-4水channel-immunoglobulin G(免疫球蛋白)或髓少突细胞glycoprotein-IgG)被发现在20 42例(48%)患者。攻击表型导致TPE视神经炎(上)42%,纵向广泛的横向脊髓炎(LETM)的31%,在15%,+ LETM等合并症状11%。总的来说,中度到标志着72%的儿童观察神经功能改善的TPE, 88.5%在6个月的随访。 Lower baseline scores on the EDSS, visual outcome, and gait scales were found to be independent prognostic indicators of treatment benefit. Sex, age at onset and at TPE, attack phenotype, disease duration, and time from attack onset to TPE initiation were not significantly associated with treatment outcome. Adverse events were observed in 31 of 524 (5.9%) procedures, being severe in 4.Conclusions TPE was an effective rescue therapy associated with functional improvement. No therapeutic window for TPE initiation was identified in this pediatric cohort. Overall frequency of adverse events was low; however, serious events should be anticipated and avoided.Classification of evidence This study provides Class IV evidence that for children with acute inflammatory demyelinating CNS syndromes, TPE leads to functional improvement.ADEM=acute disseminated encephalomyelitis; AQP4=aquaporin-4 water channel; BCS=bladder control scale; CI=confidence interval; CVC=central vein catheter; EDSS=Expanded Disability Status Scale; GS=gait scale; HDIC=high-dose IV corticosteroids; IgG=immunoglobulin G; LETM=longitudinally extensive transverse myelitis; MOG=myelin oligodendrocyte glycoprotein; MS=multiple sclerosis; NMOSD=neuromyelitis optica spectrum disorder; ON=optic neuritis; OR=odds ratio; TPE=therapeutic plasma exchange; VOS=visual outcome scale