KOBAYASHI, Y., SUZUKI, H., IINUMA, K., TADA, K. and YAMAMOTO T.Y.
Endothelial Alterations of Skeletal Muscle Capillaries in Childhood Myopathies. Tohoku J. exp. Med., 1983,
140, (4) 381-389 - Intramuscular blood capillaries in thirteen biopsied specimens obtained from eleven patients with various childhood myopathies were investigated ultrastructurally. The patients were classified into 4 different groups according to the percent distribution of various stages of endothelial alteration; normal endothelial type (E
0 Type), slightly abnormal type (E
1 Type), moderately abnormal type (E
2 Type) and markedly abnormal type (E
3 Type). Endothelial types in Duchenne dystrophy progressed from E
0 to E
1 and further to E
2 Type, which kept pace with muscle degeneration. The endothelial types in Werdnig-Hoffmann disease and central core disease were E
0 and those in multicore disease and Fukuyama type congenital muscular dystrophy were E
1. Two cases of mitochondrial myopathy showed E
3 Type, which was characterized by markedly swollen endothelial cells and narrowed lumens. The endothelium in these two cases showed more marked changes than in other childhood myopathies.
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