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重症肌无力中的血浆灌注

Plasma perfusion in myasthenia gravis.

作者信息

Passalacqua S, Splendiani G, Sturniolo A, Costanzi S, Barbera G, D'Agostini G, Scoppetta C, Adorno D, DiGiulio S, Casciani C

机构信息

C.I. Columbus-Rome, Italy.

出版信息

ASAIO Trans. 1988 Jul-Sep;34(3):602-5.

PMID:3196571
Abstract

Seven patients with myasthenia gravis (MG) unresponsive to thymectomy and steroid treatment (Osserman group III) underwent plasma perfusion (PP). All patients showed palpebral ptosis, diplopia, dysphonia, dysphagia, and muscle weakness; five of them had impaired ventilatory function. Separated plasma was perfused onto a column to adsorb anti-AChR-Ab. Each patient received a treatment cycle of six PP sessions. Clinical conditions were assessed before and after the treatment, with evaluation of muscular strength, ventilatory function, and electromyographic testing (RSS). Immunologic markers were tested before and after each PP. Clinical improvement in bulbar symptoms and respiratory function was noted in all patients after one to three PP. Limb muscle strength began to improve later. Serum concentration decreased (mean % +/- SD) after each PP:anti-AChR-Ab 36.47 +/- 17.43; IgA 20.44 +/- 11.26; IgG 21.24 +/- 32.56; IgM 23.22 +/- 11.40; C3 36.78 +/- 10.15; C4 42.69 +/- 14.82. In five of seven patients the improvement continues (follow-up 1 to 10 months). In one patient it lasted only 1 month, and in another a relapse occurred after 10 months of benefit, but was successfully reversed by retreatment.

摘要

7例对胸腺切除术和类固醇治疗无反应的重症肌无力(MG)患者(奥斯勒曼Ⅲ型)接受了血浆置换(PP)。所有患者均表现为上睑下垂、复视、发音困难、吞咽困难和肌无力;其中5例通气功能受损。分离的血浆被灌注到柱上以吸附抗乙酰胆碱受体抗体(anti-AChR-Ab)。每位患者接受一个包含6次PP治疗的疗程。在治疗前后评估临床状况,包括肌肉力量、通气功能和肌电图测试(RSS)。每次PP前后检测免疫标志物。所有患者在1至3次PP后球部症状和呼吸功能均有临床改善。肢体肌肉力量随后开始改善。每次PP后血清浓度下降(平均值±标准差):anti-AChR-Ab为36.47±17.43;IgA为20.44±11.26;IgG为21.24±32.56;IgM为23.22±11.40;C3为36.78±10.15;C4为42.69±14.82。7例患者中有5例改善持续(随访1至10个月)。1例患者改善仅持续1个月,另1例患者在获益10个月后复发,但再次治疗成功逆转。

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