Abstract
The quadriceps femoris muscle and the muscularis mucosae of the rectum from two children with mitochondrial myopathic syndrome associated with lactic acidosis were studied by electron microscopy. Striking morphological abnormalities of mitochondria were noted not only in the skeletal but also in the smooth muscle cells. Endothelial cells of blood capillaries distributed in these affected muscles were so greatly swollen that the capillary lumen was almost occluded. In contrast, surface epithelial and glandular epithelial cells of the rectum contained normal mitochondria, and fenestrated capillaries in the propria mucosae remained intact. Long-term ischemia resulting from occlusive changes of the capillary wall may be responsible for the mitochondrial alterations of muscle cells.
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Kobayashi, Y., Miyabayashi, S., Takada, G. et al. Ultrastructural study of the childhood mitochondrial myopathic syndrome associated with lactic acidosis. Eur J Pediatr 139, 25–30 (1982). https://doi.org/10.1007/BF00442074
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DOI: https://doi.org/10.1007/BF00442074