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面肩肱型肌营养不良的的肺和呼吸功能

发表者:吴士文 人已读

 

     研究表明,在面肩肱型肌营养不良(FSHD)病程中,很少累及膈肌,虽然疾病可造成轻度全呼吸肌的力弱,其中吸气肌比呼气肌更易受累及。但对于尚能独立行走,且无呼吸症状的FSHD,不需要监测其呼吸功能。武警总医院神经内科吴士文

1: Muscle Nerve. 2009 Mar 19. [Epub ahead of print]Click here to read Lung and respiratory muscle function in facioscapulohumeral muscular dystrophy.

    Stübgen JP, Schultz C.

    Department of Neurology, University of Pretoria, Pretoria, South Africa.

    Pulmonary dysfunction is not a well-recognized feature of facioscapulohumeral muscular dystrophy (FSHD). The aim of this study was to establish the prevalence and type of pulmonary and respiratory muscle dysfunction in FSHD. Sixteen patients with moderately advanced FSHD and 16 healthy controls were evaluated. Standard lung and respiratory muscle function tests were performed. Diaphragm muscle inspiratory action was evaluated with transdiaphragmatic pressure measurements. Lung function tests showed an increased residual volume in five patients. There was a significant difference in global respiratory muscle function in patients versus controls; weakness was mild, and it affected expiratory more than inspiratory muscles. There was no significant difference in the diaphragm inspiratory action of patients versus controls. The dystrophic process that underlies FSHD did not significantly involve the muscles of the diaphragm, but it caused mild global respiratory muscle weakness that affected expiratory more than inspiratory muscles. It is probably not necessary to routinely monitor respiratory muscle function in ambulant FSHD patients who lack symptoms or signs of respiratory impairment.

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发表于:2009-03-22 16:52

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