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成人起病的杆状体肌病表现为呼吸功能不全。

Adult onset of nemaline myopathy presenting as respiratory insufficiency.

作者信息

Falgà-Tirado C, Pérez-Pemán P, Ordi-Ros J, Bofill J M, Balcells E

机构信息

Vall D'Hebrón Hospital, Barcelona, Spain.

出版信息

Respiration. 1995;62(6):353-4. doi: 10.1159/000196479.

DOI:10.1159/000196479
PMID:8552868
Abstract

A 49-year-old woman was admitted to the hospital for hypercapnia. Pulmonary function testing showed small lung volumes without parenchymal lung disease. Muscle enzyme levels were normal and the EMG was nonspecific. Finally, muscle biopsy revealed abundant nemaline bodies characteristic of nemaline myopathy. Nasal intermittent pressure ventilation was started with a preset pressure ventilator during sleeping hours with a good response.

摘要

一名49岁女性因高碳酸血症入院。肺功能测试显示肺容积小,无实质性肺部疾病。肌肉酶水平正常,肌电图无特异性。最后,肌肉活检显示有大量符合杆状体肌病特征的杆状体。在睡眠时间使用预设压力呼吸机开始鼻间歇正压通气,反应良好。

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Adult onset of nemaline myopathy presenting as respiratory insufficiency.成人起病的杆状体肌病表现为呼吸功能不全。
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