Schadt Kimberly A, Friedman Lisa S, Regner Sean R, Mark George E, Lynch David R, Lin Kimberly Y
Department of Pediatrics, Division of Neurology, Children's Hospital of Philadelphia, PA 19104, USA.
J Child Neurol. 2012 Sep;27(9):1187-92. doi: 10.1177/0883073812448461. Epub 2012 Jun 29.
Electrocardiographic (ECG) findings in Friedreich ataxia and their relation to disease characteristics have not been well described. In this retrospective cross-sectional study, the authors reviewed baseline ECGs from 239 children and adults with Friedreich ataxia. ECG abnormalities--assessed in relation to participant age, sex, shorter guanine-adenine-adenine triplet repeat length, age of disease onset, and functional disability score--were found in 90% of subjects, including nonspecific ST-T wave changes (53%), right axis deviation (32%), left ventricular hypertrophy (19%), and right ventricular hypertrophy (13%). Female sex and shorter guanine-adenine-adenine repeat lengths were associated with a normal ECG (P = .004 and P = .003). Males and those of younger age were more likely to show ventricular hypertrophy (P = .006 and P = .026 for left ventricular hypertrophy and P < .001 and P = .001 for right). Neurologic status as measured by the functional disability score did not predict ECG abnormalities.
弗里德赖希共济失调的心电图（ECG）表现及其与疾病特征的关系尚未得到充分描述。在这项回顾性横断面研究中，作者回顾了239例弗里德赖希共济失调儿童和成人的基线心电图。在90%的受试者中发现了心电图异常，这些异常是根据参与者的年龄、性别、较短的鸟嘌呤-腺嘌呤-腺嘌呤三联体重复长度、疾病发病年龄和功能残疾评分进行评估的，包括非特异性ST-T波改变（53%）、电轴右偏（32%）、左心室肥厚（19%）和右心室肥厚（13%）。女性和较短的鸟嘌呤-腺嘌呤-腺嘌呤重复长度与正常心电图相关（P = 0.004和P = 0.003）。男性和年龄较小者更有可能出现心室肥厚（左心室肥厚的P = 0.006和P = 0.026，右心室肥厚的P < 0.001和P = 0.001）。通过功能残疾评分衡量的神经功能状态并不能预测心电图异常。
