Kesici Selman, Tanyıldız Murat, Yetimakman Filiz, Bayrakci Benan
1 Pediatric Intensive Care Unit, Dr. Sami Ulus Education and Research Hospital, Ankara, Turkey.
4 Pediatric Intensive Care Unit, Hacettepe University İhsan Doğramacı Children's Hospital, Ankara, Turkey.
J Child Neurol. 2019 Apr;34(5):277-283. doi: 10.1177/0883073819826225. Epub 2019 Jan 29.
Intravenous immunoglobulin and plasma exchange are proven treatments for Guillain-Barré syndrome. Despite these treatments, the prognosis for severe Guillain-Barré syndrome is still not satisfactory. This article seeks for a logical timing for plasma exchange-intravenous immunoglobulin synergy, which may improve outcome in severe Guillain-Barré syndrome requiring mechanical ventilation.
This study is an open-label study. Nine pediatric severe Guillain-Barré syndrome patients requiring mechanical ventilation were treated with novel treatment strategy named as "zipper method." In this method, following diagnosis of Guillain-Barré syndrome, plasma exchange was started immediately. In the first session of plasma exchange, one and a half volume of patients' plasma was removed by using 5% albumin as replacement solution. At the end of the plasma exchange session, 0.4 g/kg intravenous immunoglobulin infusion was started immediately. Second plasma exchange session was applied with one volume change after 24 hours from the end of the intravenous immunoglobulin infusion. Each plasma exchange session was followed by intravenous immunoglobulin infusions. This plasma exchange-intravenous immunoglobulin cycle was repeated for 5 times.
Among the 9 patients, the mean mechanical ventilation duration was 7 (5-14) days and the mean hospital stay was 18 (10-30) days. Medical Research Council sum score was increased in all patients, especially after the third session. All patients survived and all patients were able to walk unaided on the 28th day of admission.
The zipper method as a novel treatment modality seems to reduce mortality, speed up weaning from mechanical ventilation, and shorten hospital stay, with excellent outcome in severe Guillain-Barré syndrome patients, who require intensive care. This technique stands as a promising immunomodulation strategy for various scenarios.
静脉注射免疫球蛋白和血浆置换是治疗吉兰-巴雷综合征的有效方法。尽管有这些治疗方法，但严重吉兰-巴雷综合征的预后仍不尽人意。本文旨在寻找血浆置换-静脉注射免疫球蛋白协同作用的合理时机，这可能改善需要机械通气的严重吉兰-巴雷综合征的预后。
本研究为开放标签研究。9例需要机械通气的儿童严重吉兰-巴雷综合征患者采用名为“拉链法”的新治疗策略进行治疗。在这种方法中，吉兰-巴雷综合征诊断后立即开始血浆置换。在第一次血浆置换中，使用5%白蛋白作为置换液去除患者一半半体积的血浆。在血浆置换结束时，立即开始静脉注射0.4 g/kg免疫球蛋白。在静脉注射免疫球蛋白结束24小时后进行第二次血浆置换，置换量为一个血浆体积。每次血浆置换后均进行静脉注射免疫球蛋白。这个血浆置换-静脉注射免疫球蛋白周期重复5次。
9例患者中，平均机械通气时间为7（5-14）天，平均住院时间为18（10-30）天。所有患者的医学研究委员会总分均增加，尤其是在第三次治疗后。所有患者均存活，所有患者在入院第28天能够独立行走。
作为一种新的治疗方式，“拉链法”似乎可以降低死亡率，加快机械通气脱机速度，缩短住院时间，对于需要重症监护的严重吉兰-巴雷综合征患者有良好的预后。这项技术是一种有前景的免疫调节策略，适用于各种情况。
