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杜兴氏肌营养不良症导致支气管阻塞

[Duchenne Muscle Dystrophy Caused Bronchial Obstruction].

作者信息

Fujiwara Aki, Iwashiro Nozomu, Ohara Masanori

机构信息

Division of Surgery, Hakodate National Hospital, Hakodate, Japan.

出版信息

Kyobu Geka. 2017 Apr;70(4):313-315.

Abstract

Duchenne muscle dystrophy (DMD), X-linked recessive genetic disorder, causes a variety of complications including scoliosis. We report a case of bronchial obstruction and hemorrhage caused by scoliosis with DMD. A man in his forties having been hospitalized due to DMD since the age of 6, produced bloody sputum. A chest X-ray showed atelectasis in his right lower lung. A computed tomography and bronchoscopy indicated that scoliosis and thoracic deformity due to muscle dystrophy caused compression of a right main bronchus by the vertebra, leading to bronchial obstruction and bleeding. We decided to follow closely without therapy because the bleeding had stopped as of the bronchoscopy. A high-risk surgery or bronchial stenting may be needed if he presents with severe bleeding or infection.

摘要

杜氏肌营养不良症(DMD)是一种X连锁隐性遗传病,会引发包括脊柱侧弯在内的多种并发症。我们报告了一例由DMD合并脊柱侧弯导致支气管阻塞和出血的病例。一名40多岁的男性自6岁起因DMD住院,出现了咯血症状。胸部X线显示其右下肺肺不张。计算机断层扫描和支气管镜检查表明,肌肉营养不良导致的脊柱侧弯和胸廓畸形使椎骨压迫右主支气管,进而导致支气管阻塞和出血。由于在支气管镜检查时出血已停止,我们决定密切观察而不进行治疗。如果他出现严重出血或感染,可能需要进行高风险手术或支气管支架置入术。

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