Left ventricular hypertrophy (LVH) is a frequent finding in Friedreich’s ataxia (FRDA). In previous studies treatment with idebenone, a synthetic analogue of coenzyme Q10, has been associated with a substantial decrease in myocardial hypertrophy, despite great variability in cardiac responsiveness among patients. Here we present the results of a retrospective analysis of a cohort of 35 patients (20 with LVH, 15 without LVH) with confirmed molecular diagnosis of FRDA, treated with idebenone 5 mg/kg/day for up to five years. At the end of the study, we found an increase of interventricular septum and posterior wall thickness in the group without LVH before treatment and no change in the group with LVH before treatment. The neurological picture of the disease significantly deteriorated with time in both groups.
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机译：弗里德里希共济失调（FRDA）经常发现左心室肥大（LVH）。在先前的研究中，尽管艾迪苯醌是辅酶Q10的合成类似物，但尽管患者的心脏反应性差异很大，但它与心肌肥大的显着降低有关。在此，我们对35例经确诊为FRDA的分子确诊为FRDA的35例患者（20例行LVH，15例不行LVH）进行回顾性分析，结果用艾地苯醌5 mg / kg /天治疗长达五年。在研究结束时，我们发现治疗前无LVH的组室间隔和后壁厚度增加，治疗前无LVH的组无变化。在两组中，随着时间的流逝，该疾病的神经学图像均明显恶化。
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