Cardiac involvement in glycogen storage disease type III
Cardiac involvement in glycogen storage disease type III
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III 型糖原累积病心脏受累
DOI：
10.1007/bf00443106
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发表时间：
1989
影响因子：
3.6
通讯作者：
M. Frydman
中科院分区：
文献类型：
--
作者：
S. Moses;K. Wanderman;A. Myroz;M. Frydman
20例3-30岁的经酶证实的糖原累积病III型（GSD III）患者接受了心脏病学评价。17例患者在ECG上显示出心室肥大形式的心脏受累亚临床证据。在16例进行心电图检查的患者中，13例有异常超声心动图特征。仅2例X线表现为心脏扩大。详细描述了其中1例患者（25岁女性）的心脏检查结果，该患者临床表现为明显的心肌病。鉴于我们的研究结果，确诊为GSD III的患者不仅应检查其肌肉受累情况，还应详细评估其心脏状况。
Twenty patients with enzymatically proven glycogen storage disease type III (GSD III) aged 3–30 years underwent cardiological evaluation. Seventeen showed subclinical evidence of cardiac involvement in form of ventricular hypertrophy on ECG. Of 16 patients in whom an ECG examination was performed, 13 had abnormal echocardiographic features. Only 2 patients had cardiomegaly on X-ray. The cardiac findings in 1 of the patients, a 25-year-old female with clinically evident cardiomyopathy are described in detail. In view of our findings, patients with established GSD III, should not only be investigated regarding their muscular involvement, but should also undergo a detailed evaluation of their cardiac status.