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首页> 外文期刊>The Canadian Journal of Neurological Sciences: le Journal Canadien des Sciences Neurologiques >Microvascular pathology of Friedreich cardiomyopathy
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Microvascular pathology of Friedreich cardiomyopathy

机译：Friedreich心肌病的微血管病理学

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Nikolaus Friedreich (1877) was aware of heart disease in his patients but thought it was unrelated to the neurological disorder. In 1946, Dorothy Russell considered cardiomyopathy an integral part of Friedreich ataxia (FA). In addition to sparse inflammatory infiltration, sections show fibrosis and capillary hyperplasia. We examined the left ventricular walls of 41 homozygous FA patients aged 10–87 and 21 controls aged 2–69. An antibody to CD34 enabled quantitative capillary profile counts for a comparison with cardiomyocyte counts in the same field. Mean capillary counts in normals were 1926±341/mm2, and the median ratio of capillaries to cardiomyocytes was 1.0 (interquartile range [IQR]: 0.9-1.2). In FA, however, the number of cardiomyocytes/mm2 was less, and the median ratio of capillaries to heart fibers was 2.0 (IQR 1.4-2.4). There was a significant correlation of the higher guanine-adenine-adenine trinucleotide length (shorter allele, GAA1) with the younger age of onset, shorter disease duration, and lower cardiomyocyte counts. The ratio of capillaries to heart fibers was higher in patients with long GAA1 repeat expansions (e.g., 3.31 in GAA1 of 1200). Double-label immunofluorescence for CD34 and S100A4 revealed co-expression in endothelial cells, supporting endothelial-to-mesenchymal transition in the pathogenesis of cardiac fibrosis (supported by Friedreich’s Ataxia Research Alliance).
机译：Nikolaus Friedreich（1877）意识到他的患者的心脏病，但思想与神经疾病无关。 1946年，Dorothy Russell被认为是Friedreich Ataxia（FA）的一个组成部分的心肌病。除了稀疏炎症浸润外，部分还显示纤维化和毛细血管增生。我们检查了10-87岁和21岁的41岁的纯合子FA患者的左心室壁。对CD34的抗体使定量毛细血管分布计数计数与相同领域的心肌细胞计数进行比较。法线中的平均毛细管计数为1926±341 / mm2，毛细血管与心肌细胞的中值比为1.0（侧链范围[IQR]：0.9-1.2）。然而，在FA中，心肌细胞/ mm2的数量较小，并且毛细管与心脏纤维的中值比为2.0（IQR 1.4-2.4）。具有较小的发病寿命，较短的疾病持续时间，较短的疾病持续时间和较低的心肌细胞计数较高的鸟嘌呤 - 腺嘌呤 - 腺嘌呤三核苷酸长度（较短的等位基因，GAA1）具有显着的相关性。长GaA1重复膨胀患者的毛细纤维与心脏纤维的比例更高（例如，在GaA1的GaA1中的3.31）。 CD34和S100A4的双标菌免疫荧光揭示了内皮细胞中的共表达，支持心脏纤维化的发病机制中的内皮 - 间充质转变（由Friedreich的Ataxia Research Alliance的支持）。

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