年龄发病是否与弗里德赖希共济失调中左心室肥厚的发生有关？
May age onset be relevant in the occurrence of left ventricular hypertrophy in Friedreich's ataxia?
作者信息
Maione S, Giunta A, Filla A, De Michele G, Spinelli L, Liucci G A, Campanella G, Condorelli M
机构信息
Department of Internal Medicine, Federico II University of Naples, School of Medicine, Italy.
出版信息
Clin Cardiol. 1997 Feb;20(2):141-5. doi: 10.1002/clc.4960200211.
BACKGROUND
Although heart involvement has been widely reported in Friedreich's ataxia (FA), which is the most prevalent of the spino-cerebellar degenerative diseases, the reason that cardiac abnormalities develop has not been yet established.
HYPOTHESIS
The investigation was undertaken to study the prevalence and characteristics of cardiac abnormalities in patients with FA and to evaluate whether the presence of left ventricular hypertrophy could be predicted.
METHODS
In all, 75 patients with FA and 16 patients with late onset FA (LOFA) disease were investigated for cardiac abnormalities using noninvasive methods.
RESULTS
A significant (p < 0.01) difference in the age onset (9.8 +/- 3.9 years) was found in 31 of the 75 patients with FA (41%) who showed left ventricular hypertrophy (LVH) at echocardiographic examination compared with the remaining 44 patients with FA without LVH (12.6 +/- 4.3 years). Moreover, none of the 16 patients with LOFA (age onset 26.5 +/- 4.2 years) showed abnormalities at echocardiographic examination. A significant (p < 0.01) concordance in the familial distribution of hypertrophy was also found.
CONCLUSION
These data suggest that the earlier the disease develops the more frequently LVH occurs.
背景
尽管心脏受累在弗里德赖希共济失调（FA）中已有广泛报道，FA是最常见的脊髓小脑退行性疾病，但心脏异常发生的原因尚未明确。
假说
本研究旨在探讨FA患者心脏异常的患病率及特征，并评估是否可以预测左心室肥厚的存在。
方法
总共对75例FA患者和16例晚发型FA（LOFA）患者采用非侵入性方法进行心脏异常检查。
结果
75例FA患者中，31例（41%）在超声心动图检查时显示左心室肥厚（LVH），其发病年龄（9.8±3.9岁）与其余44例无LVH的FA患者（12.6±4.3岁）相比有显著差异（p<0.01）。此外，16例LOFA患者（发病年龄26.5±4.2岁）在超声心动图检查中均未显示异常。在肥厚的家族分布方面也发现了显著的一致性（p<0.01）。
结论
这些数据表明疾病发病越早，LVH发生越频繁。
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