Artacho Pérula E, Roldán Villalobos R, Vaamonde Lemos R
Department of Cell Biology, School of Medicine, University of Córdoba, Spain.
Histol Histopathol. 1989 Jul;4(3):265-70.
We report a case of the Werdnig-Hoffmann disease in a 4-month-old male infant. The morphological study revealed perimysial fibrosis, variability in the size of muscle fibers, absence of target fibers, few central nuclei and normality in vessels, nerves and neuromuscular junctions. The morphometrical examination showed the existence of normal-sized and atrophic fibers in both fibrillar types, as well as in hypertrophic type I fibers. The percentage of fibrillar types and the data obtained from the form factor are normal. Random distribution of type I and II muscle fibers were observed.
我们报告一例4个月大男婴的韦尔尼克-霍夫曼病。形态学研究显示肌束膜纤维化、肌纤维大小不一、无靶纤维、中央核较少以及血管、神经和神经肌肉接头正常。形态测量检查显示，在两种纤维类型以及肥大的I型纤维中均存在正常大小和萎缩的纤维。纤维类型的百分比以及从形态因子获得的数据均正常。观察到I型和II型肌纤维随机分布。
