Treatment of dystrophinopathic cardiomyopathy: review of the literature and personal results
Treatment of dystrophinopathic cardiomyopathy: review of the literature and personal results
复制标题
肌营养不良性心肌病的治疗：文献回顾和个人结果
作者：
L. Politano;G. Nigro
心肌病在患有Duchenne肌营养不良症(DMD)的男孩中几乎是普遍存在的。由于缺乏dystrophin，心肌的变化包括细胞膜降解、间质炎症、脂肪替代和纤维化。营养不良型心肌病通常始于临床前或中期，发展到晚期，特征是脑室增大，但也有心力衰竭的症状和体征(呼吸困难、外周水肿和肝脏增大)。然而，在少数患者中，心脏扩张可能是心脏受累的第一个表现。发现显性心肌病的能力随着年龄的增长而增加，因此超过80%的18岁以上的男孩会有异常的收缩功能。已经使用了几种药物来对比心肌病向严重充血性心力衰竭阶段的演变。报告和讨论了DMD心脏治疗的回顾和个人经验。
Cardiomyopathy is an almost universal finding in boys affected by Duchenne muscular dystrophy (DMD). Myocardial changes, as a result of the lack of dystrophin, consist of cell membrane degradation, interstitial inflammation, fatty replacement and fibrosis. Dystrophinopathic cardiomyopathy generally starts as a preclinical or intermediate stage, with evolution toward advanced stages characterized by ventricle enlargement but also by symptoms and signs of heart failure (dyspnoea, peripheral edema and liver enlargement). However in few patients the dilation could be the first manifestation of the heart involvement. The ability to detect overt cardiomyopathy increases with age, such that more than 80% of boys older than 18 years will have abnormal systolic function. Several drugs have been employed with the aim to contrast the evolution of cardiomyopathy toward stages of severe congestive heart failure. A review of cardiac treatment in DMD and personal experience are reported and discussed.
影响因子：
37.8
作者：
T. Ryoke;Yusu Gu;L. Mao;M. Hongo;R. Clark;Kirk L. Peterson;J. Ross
通讯作者：
T. Ryoke;Yusu Gu;L. Mao;M. Hongo;R. Clark;Kirk L. Peterson;J. Ross
DOI：
10.1073/pnas.90.8.3710
发表时间：
1993-04-15
影响因子：
11.1
作者：
PETROF, BJ;SHRAGER, JB;SWEENEY, HL
通讯作者：
SWEENEY, HL