Kobayashi Y, Tazawa Y, Nakagawa M, Suzuki H, Konno T, Yamamoto T Y
Tohoku J Exp Med. 1984 Mar;142(3):337-46. doi: 10.1620/tjem.142.337.
The skeletal muscle from a patient with familial intrahepatic cholestasis associated with vitamin E deficiency was studied by electron microscopy. The muscle fibers showed a variety of pathologic features including degenerative, necrotic and regenerative changes. Granule-like inclusions found in our biopsy specimens were similar in structure to those observed in vitamin E deficient animals. These inclusions were noted not only in the skeletal muscle fibers but also in endothelial cells of the intramuscular capillaries, Schwann cells and perineural cells. Disruption and disappearance of the plasma membrane, separation, disruption and pleats formation of the external lamina, and multilayered external laminae were observed in muscle fibers most frequently. The nerves among muscle fibers also showed degenerative features. These severely degenerative alterations of the muscle fiber have not so far been reported in vitamin E deficient patients. We discuss the process of cell damages caused by vitamin E deficiency.
通过电子显微镜对一名患有与维生素E缺乏相关的家族性肝内胆汁淤积症患者的骨骼肌进行了研究。肌纤维呈现出多种病理特征，包括退行性、坏死性和再生性改变。我们活检标本中发现的颗粒样包涵体在结构上与维生素E缺乏动物中观察到的相似。这些包涵体不仅见于骨骼肌纤维，还见于肌内毛细血管的内皮细胞、施万细胞和神经周细胞。在肌纤维中最常观察到质膜的破坏和消失、外板的分离、破坏和褶皱形成以及多层外板。肌纤维之间的神经也表现出退行性特征。迄今为止，维生素E缺乏患者中尚未报道过如此严重的肌纤维退行性改变。我们讨论了维生素E缺乏引起的细胞损伤过程。
