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α1-抗胰蛋白酶替代疗法可控制2例PI ZZ型α1-抗胰蛋白酶缺乏症患者的纤维肌痛症状。

Alpha1-antitrypsin replacement therapy controls fibromyalgia symptoms in 2 patients with PI ZZ alpha1-antitrypsin deficiency.

作者信息

Blanco Ignacio, Canto Hortensia, de Serres Frederick Joseph, Fernandez-Bustillo Enrique, Rodríguez Maria Carmen

机构信息

Department of Internal Medicine, Respiratory Diseases Branch, Hospital Valle del Nalón, Asturias, Spain.

出版信息

J Rheumatol. 2004 Oct;31(10):2082-5.

Abstract

Two Spanish sisters with alpha1-antitrypsin (AAT) deficiency and fibromyalgia (FM) started AAT replacement therapy with commercial alpha1-antitrypsin infusions in 1992. They both experienced a rapid, progressive, and constant control of their FM symptoms during the next 6 years (1992-98). However, in 1998, treatment of both patients was affected by the worldwide commercial shortage of AAT replacement therapy; replacement therapy infusions were halted for about 4-6 consecutive months every year for 5 years. As a result, we observed a striking recurrence of FM symptoms. Equally striking was the total disappearance of these symptoms when AAT replacement therapy infusions were resumed.

摘要

1992年,两名患有α1抗胰蛋白酶(AAT)缺乏症和纤维肌痛(FM)的西班牙姐妹开始接受市售α1抗胰蛋白酶输注进行AAT替代疗法。在接下来的6年(1992 - 1998年)里,她们的FM症状都迅速、渐进且持续得到了控制。然而,1998年,两名患者的治疗受到全球AAT替代疗法商业短缺的影响;在5年的时间里,替代疗法输注每年都会连续中断约4 - 6个月。结果,我们观察到FM症状显著复发。同样显著的是,当恢复AAT替代疗法输注时,这些症状完全消失了。

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