Pathology of the Heart in Friedreich's Ataxia: Review of the Literature and Report of One Case
Pathology of the Heart in Friedreich's Ataxia: Review of the Literature and Report of One Case
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弗里德赖希共济失调的心脏病理学：文献复习及一例报道
DOI：
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发表时间：
1976
期刊：
影响因子：
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通讯作者：
A. Barbeau
中科院分区：
文献类型：
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作者：
G. Sanchez‐Casis;M. Côté;A. Barbeau
摘要：对一例典型的Friedreich共济失调患者的心脏改变进行了分析，并与文献中的结果进行了比较。肉眼可见心脏增大，有不同程度的心室肥厚，可能有轻微的耳廓扩张。更重要和持续的组织学改变是心肌纤维化和心肌细胞变性。肌细胞内可见钙盐和铁的颗粒状沉积。肥厚型和偶有梗阻的心肌病似乎是Friedreich共济失调的组成部分。
SUMMARY: A single case of typical Friedreich's ataxia was analyzed for cardiac changes and compared to the findings from the literature. Macroscopically, there was a cardiomegaly with some degree of ventricular hypertrophy and probable mild dilatation of the auricles. The more important and constant histologic changes were myocardial fibrosis and degeneration of the cardiac muscle cells. Granular deposits of calcium salts and iron were found in the muscle cells. A cardiomyopathy hypertrophic in type and occasionally obstructive appears to be an integral part of Friedreich's ataxia.