Natural history of cardiac involvement in myotonic dystrophy: Correlation with CTG repeats
Natural history of cardiac involvement in myotonic dystrophy: Correlation with CTG repeats
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DOI：
10.1212/wnl.55.8.1207
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发表时间：
2000-10-24
期刊：
影响因子：
9.9
通讯作者：
Gennarelli, M
中科院分区：
文献类型：
--
作者：
Antonini, G;Giubilei, F;Gennarelli, M
作者前瞻性地研究了 50 名强直性肌营养不良患者心脏受累的自然病程及其与胞嘧啶-胸腺嘧啶-鸟嘌呤 (CTG) 扩张的关系，这些患者在中位随访 56 个月期间接受了定期心血管 EKG 和 EKG-Holter 监测。十九名患者 (38%) 出现了严重的心电图变化。 CTG 长度与 EKG 异常频率不相关，但与 EKG 异常发病年龄呈负相关 (p < 0.0001)。 CTG 长度影响强直性肌营养不良中心脏并发症的发生时间。
The authors prospectively studied the natural course of cardiac involvement and its relationship to cytosine-thymine-guanine (CTG) expansion in 50 patients with myotonic dystrophy who were submitted to periodic cardiovascular EKG and EKG-Holter monitoring during a median follow-up of 56 months. Nineteen patients (38%) developed major EKG changes. CTG length was not correlated with the frequency of EKG abnormalities, but was inversely correlated with the age at onset of EKG abnormalities (p < 0.0001). CTG length influences the timing of cardiac complications in myotonic dystrophy.