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用阿那白滞素治疗的Muckle-Wells综合征患者的耳聋恢复情况。

Recovery from deafness in a patient with Muckle-Wells syndrome treated with anakinra.

作者信息

Mirault Tristan, Launay David, Cuisset Laurence, Hachulla Eric, Lambert Marc, Queyrel Viviane, Quemeneur Thomas, Morell-Dubois Sandrine, Hatron Pierre-Yves

机构信息

Claude-Huriez Hospital, Department of Internal Medicine, National Center for Vascular Manifestations of Scleroderma, Lille 2 University, Lille, France.

出版信息

Arthritis Rheum. 2006 May;54(5):1697-700. doi: 10.1002/art.21807.

Abstract

Muckle-Wells syndrome (MWS) is a dominantly inherited autoinflammatory disease characterized by rashes, fever, arthralgia, sensorineural deafness, and the possible development of systemic AA amyloidosis. We used anakinra to treat a 22-year-old patient with MWS who had deafness and a high serum level of C-reactive protein (CRP). Following treatment with anakinra, the patient's CRP level normalized, and she recovered from deafness. The fact that this occurrence has never been previously reported strengthens the role of anakinra in MWS but also raises new questions about the physiopathology of such deafness.

摘要

穆克-韦尔斯综合征(MWS)是一种常染色体显性遗传的自身炎症性疾病,其特征为皮疹、发热、关节痛、感觉神经性耳聋以及可能发展为系统性AA型淀粉样变性。我们使用阿那白滞素治疗一名患有MWS的22岁患者,该患者有耳聋症状且血清C反应蛋白(CRP)水平较高。使用阿那白滞素治疗后,患者的CRP水平恢复正常,且耳聋症状也有所缓解。此前从未有过此类病例报告,这一事实既凸显了阿那白滞素在MWS治疗中的作用,也引发了关于此类耳聋发病机制的新问题。

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