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Steroid Therapy and Cardiac Function in Duchenne Muscular Dystrophy

机译：杜氏肌营养不良症的类固醇治疗和心脏功能

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摘要

Duchenne muscular dystrophy leads to progressive deterioration in skeletal and cardiac muscle function. Steroids prolong ambulation and improve respiratory muscle strength. The authors hypothesized that steroid treatment would stabilize cardiac muscle function. Echocardiograms performed from 1997 to 2004 for 111 subjects 21 years of age or younger with Duchenne muscular dystrophy were restrospectively reviewed. The medical record was reviewed for steroid treatment. Untreated and steroids-treated subjects did not differ in age, height, weight, body mass index, systolic and diastolic blood pressure, or left ventricular mass. The shortening fraction was lower in the untreated group. Of those treated, 29 received prednisone and 19 received deflazacort. There was no difference in the shortening fraction between the two treated subgroups. Treated subjects not receiving steroids still had a normal shortening fraction, which was no different from the shortening fraction of those still receiving treatment. As compared with the treated subjects, the untreated subjects 10 years of age or younger were 4.4 times more likely to have a shortening fraction less than< 28% (p = 0.03), and the untreated subjects older than 10 years were 15.2 times more likely to have a shortening fraction less than< 28% (p < 0.01). This retrospective study suggests that the progressive decline in cardiac function of patients with Duchenne muscular dystrophy can be altered by steroid treatment. The effect appears to be sustained beyond the duration of treatment and independent of steroid type.
机译：杜氏肌营养不良症会导致骨骼和心肌功能的进行性恶化。类固醇可延长下床活动并改善呼吸肌力量。作者假设类固醇治疗可以稳定心肌功能。回顾性分析了1997年至2004年对21岁以下21岁以下Duchenne肌营养不良症患者的超声心动图。对病历进行了类固醇治疗的审查。未经治疗和接受类固醇治疗的受试者的年龄，身高，体重，体重指数，收缩压和舒张压或左心室质量无差异。未治疗组的缩短率较低。在接受治疗的患者中，有29人接受了泼尼松治疗，而19人接受了去黄酮治疗。在两个处理的亚组之间的缩短分数没有差异。未接受类固醇治疗的受试者仍具有正常的缩短率，这与仍接受治疗的受试者的缩短率没有区别。与接受治疗的受试者相比，年龄小于或等于10岁的未经治疗的受试者的短缩率低于<28％的可能性是4.4倍（p = 0.03），而年龄大于10岁的未经治疗的受试者的起酥分数小于15.2倍。缩短率小于<28％（p <0.01）。这项回顾性研究表明，类固醇治疗可以改变患有杜兴氏肌营养不良症患者心脏功能的逐步下降。该作用似乎在治疗持续时间内得以维持，并且与类固醇类型无关。

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  • 来源
    《Pediatric Cardiology》 |2005年第6期|768-771|共4页
  • 作者

    L.W. Markham; R.L. Spicer; P.R. Khoury; B.L. Wong; K.D. Mathews; L.H. Cripe;

  • 作者单位

    Department of Pediatrics Division of Pediatric Cardiology Cincinnati Children’s Hospital Medical Center;

    Department of Pediatrics Division of Pediatric Cardiology Cincinnati Children’s Hospital Medical Center;

    Department of Pediatrics Division of Pediatric Cardiology Cincinnati Children’s Hospital Medical Center;

    Department of Neurology Cincinnati Children’ Hospital Medical Center;

    Department of Pediatrics and Neurology University of Iowa;

    Department of Pediatrics Division of Pediatric Cardiology Cincinnati Children’s Hospital Medical Center;

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  • 原文格式 PDF
  • 正文语种 eng
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  • 关键词

    Cardiomyopathy; Duchenne muscular dystrophy; Steroids;

    机译：心肌病;杜氏肌营养不良症;类固醇;

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