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良性假肥大型肌营养不良症患者肺功能的表现特点与康复对策 被引量:1

Pulmonary Function Characteristics and Rehabilitation Strategy for Becker Muscular Dystrophy
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摘要 目的:探讨良性假肥大型肌营养不良症(BMD)患者的肺功能表现特点与康复对策。方法:测定12例BMD患者的肺通气功能并将数据进行统计学处理,分析其临床表现。结果:12例患者的肺通气功能主要指标均值均在正常范围,但1 s用力呼气容积与用力肺活量之比值(FEV1/FVC)较正常人群明显增高。结论:FEV1/FVC比例不协调是BMD患者肺通气功能的主要特点,其原因可能与呼吸肌尤其是主要吸气肌膈肌和主要辅助呼气肌腹肌肌力较弱有关,康复锻炼对改善FEV1/FVC比例的不协调有帮助,应列为BMD患者必做的康复训练。 Objective: To investigate the pulmonary function characteristics and rehabilitation strategy for Becker muscular dystrophy (BMD). Methods: Pulmonary ventilation function of 12 patients with BMD was measured and analyzed. Results: The values of pulmonary ventilation function of the 12 cases were basically normal except for FEV1/FVC,Conclusion: FEV1/FVC in the patients with DMD is significantly higher than that of healthy population, while other pulmonary ventilation indices were in normal range. The elevated FEV1/FVC is probably due to the weakness of diaphragmatic muscle and the accessory respiratory muscles, Rehabilitation exercise aiming at the respiratory muscles may be helpful for improving the pulmonary function of BMD patients.
出处 《中国康复》 2006年第3期159-160,共2页 Chinese Journal of Rehabilitation
关键词 良性假肥大型肌营养不良症 肺功能 康复 becker muscular dystrophy pulmonary function rehabilitation
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  • 1陈灏珠.实用内科学[M](第10版)[M].北京:人民卫生出版社,1997.835-838.
  • 2RIDEAU Y. Treatment of Duchenne's Myopathy with Early Physiotherapy. Critical Analysis[J]. Arch Fr Pediatr(S0004-1254),1985,42(1):17-21.
  • 3ALEXANDER MA, JOHNSON EW, PETTY J, et al.Mechanical Ventilation of Patients with Late Stage Duchenne Muscular Dystrophy: Management in Home[J].Arch Phys Med Rehabil (S0003 - 9993 ), 1979,60:289-292.
  • 4EMERY AE.X-linked Muscular Dystrophy with Early Contractures and Cardiomyopathy(Emery-Dreifuss Type)[J]. Clin Genet(S0002-9297) ,1987,32(5) :360-367.
  • 5孟凤芹,刘学梅,任秀红.慢性阻塞性肺疾病病人呼吸训练方法的对比观察[J].护理学杂志(综合版),2003,18(3):171-172. 被引量:66

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  • 1刘焯霖,梁秀龄,张成神经遗传病学.3版.北京:人民卫生出版社,2011:99-101.
  • 2Mostacciuolo ML, Lombardi A, Cambissa V, Danieli GA, Angelini C. Population data on benign and severe forms of X- linked muscular dystrophy. Hum Genet, 1987, 75:217-220.
  • 3张成杜兴式肌营养不良基因结构与膜缺陷.广州:广东科技出版社,1993:410-427.
  • 4Fayssoil A, Nardi O, Orlikowski D, Annane D. Cardiomyopathy in Duchenne muscular dystrophy: pathogenesis and therapeutics. Heart Fail Rev, 2010, 15:103-107.
  • 5Finsterer J, Stllberger C. The heart in human dystrophinopathies. Cardiology, 2003, 99:1-19.
  • 6Oldfors A, Eriksson BO, Kytlerman M, Martinsson T, Wahlstrfim J. Dilated cardiomyopathy and the dystrophin gene: an illustrated review. Br Heart J, 1994, 72:344-348.
  • 7Juan- Mateu J, Paradas C, Oliv M, Verdura E, Rivas E, Gonz61ez- Quereda L, Rodriguez M J, Baiget M, Gallano P. Isolated cardiomyopathy caused by a DMD nonsense mutation in somatic mosaicism: genetic normalization in skeletal muscle. Clin Genet, 2012, $2:574-578.
  • 8Jefferies JL, Eidem BW, Belmont JW, Craigen WJ, Ware SM, Fernbach SD, Neish SR, Smith EO, Towbin JA. Genetic predictors and remodeling of dilated cardiomyopathy in muscular dystrophy. Circulation, 2005, 112:2799-2804.
  • 9Neri M, Valli E, Alfano G, Bovolenta M, Spitali P, Rapezzi C, Muntoni F, Banfi S, Perini G, Gualandi F, Ferlini A. The absence of dystrophin brain isoform expression in healthy human heart ventricles explains the pathogenesis of 5" X-linked dilated cardiomyopathy. BMC Med Genet, 2012, 13:20.
  • 10Bastianutto C, De Visser M, Muntoni F, Klamut H J, Patarnello T. A novel muscle-specific enhancer identified within the deletion overlap region of two XLDC patients lacking muscle exon 1 of the human dystrophin gene. Genomics, 2002, 80:614-620.

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