Kobayashi Y, Tazawa Y, Nakagawa M, Higashi O, Yamamoto T Y
Department of Pediatrics, Akita University School of Medicine, Japan.
Tohoku J Exp Med. 1988 Mar;154(3):285-96. doi: 10.1620/tjem.154.285.
We investigated the effects of long-term oral and intramuscular vitamin E repletion in children with chronic cholestasis. Clinical improvement or suppression of neuromuscular involvement after adequate vitamin E repletion was demonstrated. Light and electron microscopic abnormalities of the skeletal muscle, however, did not completely disappeared despite the correction of the biochemical abnormalities for more than 3 years. The muscle fibers showed less variety of pathologic features than before vitamin E repletion. Inclusions observed in the skeletal muscle fibers before vitamin E treatment were still observed in subsarcolemmal cytoplasm and the perinuclear regions. They were more irregularly curved and consisted of various substances. Similar inclusions were also observed in Schwann cells, perineural cells, fibroblasts, pericytes, endothelial cells and smooth muscle cells of intramuscular vessels. Although the external lamina was not disrupted, separation of the external lamina from the plasma membrane and multilayered external lamina were often observed. The nerves among muscle fibers still showed degenerative features. Morphological changes of the skeletal muscle during vitamin E therapy have not so far been reported in cases of chronic cholestasis. We discuss the relationship of these findings to vitamin E replacement in children with chronic cholestasis.
我们研究了长期口服和肌肉注射维生素E对慢性胆汁淤积症患儿的影响。结果表明，充足的维生素E补充后临床症状改善或神经肌肉受累情况得到抑制。然而，尽管生化异常已得到纠正超过3年，骨骼肌的光镜和电镜异常并未完全消失。与维生素E补充前相比，肌纤维的病理特征种类减少。维生素E治疗前在骨骼肌纤维中观察到的包涵体仍可见于肌膜下细胞质和核周区域。它们弯曲更不规则，由多种物质组成。在肌内血管的施万细胞、神经周细胞、成纤维细胞、周细胞、内皮细胞和平滑肌细胞中也观察到类似的包涵体。虽然外板未被破坏，但常观察到外板与质膜分离以及多层外板。肌纤维间的神经仍表现出退行性特征。迄今为止，慢性胆汁淤积症病例中维生素E治疗期间骨骼肌的形态学变化尚未见报道。我们讨论了这些发现与慢性胆汁淤积症患儿维生素E替代治疗的关系。
