Objective

To introduce and evaluate a modified version of the “zipper method”—a treatment strategy alternating intravenous immunoglobulin (IVIG) and plasma exchange (PLEX) first reported for 9 pediatric cases of Guillain-Barré syndrome in 2018—for treatment of severe immune-mediated neurologic disorders in children.

Methods

The modified zipper method comprised longer intervals between PLEX-IVIG cycles (48 hours instead of 24 hours), more cycles (7-10 instead of 5), a consistent plasma volume exchange (instead of the original multistep approach), and variable infusion times for IVIGs (4-8 hours). The modified zipper method was applied as an individual treatment approach once standard therapy failed. The follow-up ranged from 6 months to 2 years. Cases were analyzed retrospectively. Disease severity was mainly quantified by the Guillain-Barré syndrome disability score.

Results

Four children (9-15 years) with (1) Miller-Fisher syndrome, (2) Bickerstaff brainstem encephalitis, (3) common Guillain-Barré syndrome, and (4) severe acute disseminated encephalomyelitis were treated by the modified zipper method. Results for duration of mechanical ventilation (median of 12 days, interquartile range [IQR] 8-16), hospital stay (median of 23 days, IQR 22-24), and time to unaided walking (median of 22 days, IQR 21-37) outperformed previous studies with IVIG/PLEX alone or IVIG + PLEX combinations unlike the zipper method.

Conclusion

The modified zipper method is associated with a low mortality, a short mechanical ventilation time, a short hospital stay, and an excellent outcome in children with severe Guillain-Barré syndrome or acute disseminated encephalomyelitis. Our regimen is streamlined for applicability. Results emphasize its robust effectiveness as an option for therapy escalation in severe neuroimmunologic diseases. Now, multicenter trials are needed to evaluate this novel treatment strategy.

中文翻译：

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改良拉链法，一种有希望的治疗严重儿科免疫介导神经系统疾病的选择

客观的

引入和评估改良版“拉链法”——一种交替静脉免疫球蛋白 (IVIG) 和血浆置换 (PLEX) 的治疗策略，该策略于 2018 年首次报道 9 例格林-巴利综合征儿科病例——用于治疗严重的免疫介导儿童神经系统疾病。

方法

改进的拉链方法包括更长的 PLEX-IVIG 循环之间的间隔（48 小时而不是 24 小时）、更多的循环（7-10 次而不是 5 次）、一致的血浆容量交换（而不是原来的多步方法）和可变的输注时间对于 IVIG（4-8 小时）。一旦标准治疗失败，改进的拉链方法被用作个体治疗方法。随访时间为 6 个月至 2 年。对病例进行回顾性分析。疾病严重程度主要通过格林-巴利综合征残疾评分来量化。

结果

采用改良拉链法治疗了 4 名（1）Miller-Fisher 综合征、（2）Bickerstaff 脑干脑炎、（3）常见 Guillain-Barré 综合征和（4）严重急性播散性脑脊髓炎的儿童（9-15 岁）。机械通气持续时间（中位数 12 天，四分位距 [IQR] 8-16）、住院时间（中位数 23 天，IQR 22-24）和独立行走时间（中位数 22 天，IQR 21- 37) 优于以前单独使用 IVIG/PLEX 或 IVIG + PLEX 组合的研究，这与拉链方法不同。

结论

改良的拉链方法与重度格林-巴利综合征或急性播散性脑脊髓炎患儿的死亡率低、机械通气时间短、住院时间短和预后良好有关。我们的方案针对适用性进行了精简。结果强调了其作为严重神经免疫疾病治疗升级的一种选择的强大有效性。现在，需要多中心试验来评估这种新的治疗策略。