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包括免疫吸附在内的多模式方法治疗重症肌无力危象的长期效果。

Long-term effects of a multimodal approach including immunoadsorption for the treatment of myasthenic crisis.

作者信息

Zeitler Heike, Ulrich-Merzenich Gudrun, Hoffmann Lars, Kornblum Cornelia, Schmidt Stephan, Vetter Hans, Walger Peter

机构信息

Medical Policlinic, University of Bonn, Bonn, Germany.

出版信息

Artif Organs. 2006 Aug;30(8):597-605. doi: 10.1111/j.1525-1594.2006.00268.x.

Abstract

A multimodal treatment protocol with immunoadsorption (IA) as the central element was used in the treatment of myasthenic crisis (MC). Fifteen patients with MC were treated in repeated, uninterrupted 7-day cycles until mobilization with: (i) large-volume IA using an antihuman-IgG adsorber, days 1-5; (ii) intravenous immunoglobulin substitution (0.3-0.5 g/kg body weight [BW]/day), days 5-7; and (iii) immunosuppression with cyclophosphamide (1-2 mg/kg BW/day) and prednisolone (0.5-1 mg/kg BW/day), until remission. Patients required a median of 8 days of mechanical ventilation, 12 days in the intensive care unit, and 35 days of hospitalization. Functional improvement compared to their precrisis condition was attained by 14 of 15 patients. MG severity score improved by a mean of 10 points, quality of life score by 9.8 points, and Karnofsky index by 29 points in 14 of 15 patients. Improvements remained stable and no further crises occurred during long-term follow-up, which averaged 4.4 years. No fatalities due to MC occurred. The results demonstrate that our protocol is a potent therapeutic approach in the treatment of MC.

摘要

采用以免疫吸附(IA)为核心要素的多模式治疗方案治疗重症肌无力危象(MC)。15例MC患者接受为期7天的重复、不间断治疗周期,直至病情缓解,治疗方法包括:(i)第1 - 5天使用抗人IgG吸附柱进行大容量IA;(ii)第5 - 7天静脉注射免疫球蛋白替代治疗(0.3 - 0.5 g/kg体重[BW]/天);(iii)使用环磷酰胺(1 - 2 mg/kg BW/天)和泼尼松龙(0.5 - 1 mg/kg BW/天)进行免疫抑制治疗。患者机械通气的中位时间为8天,在重症监护病房的时间为12天,住院时间为35天。15例患者中有14例相较于危象前状况获得了功能改善。15例患者中有14例重症肌无力严重程度评分平均提高了10分,生活质量评分提高了9.8分,卡氏指数提高了29分。改善情况保持稳定,在平均4.4年的长期随访期间未再发生危象。未发生因MC导致的死亡。结果表明,我们的方案是治疗MC的一种有效治疗方法。

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