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诊断性肌病病理学中的特殊技术。

Special techniques in diagnostic myopathology.

作者信息

Goebel H H, Husmann G, Voit T, Calişkan M

机构信息

Division of Neuropathology, University of Mainz, Germany.

出版信息

Turk J Pediatr. 1994 Jul-Sep;36(3):223-37.

PMID:7974813
Abstract

While muscle biopsy has been standardized since the introduction of electron microscopy, histochemistry, and morphometry into diagnostic myopathology, additional diagnostic techniques have recently been added. Immunomorphologic methods-firmly established in oncology long ago-are beginning to be applied in diagnostic myopathology. These methods concern the evidence of intermediate filaments in regenerating and denervated fibers as well as in certain myopathies marked by intermediate filament-related inclusions such as cytoplasmic bodies, spheroid bodies, or Mallory body-like inclusions. The connotation of the immunomorphologic approach is further emphasized by the discovery of dystrophin, a normal protein within muscle fibers which is deficient in Duchenne's and Becker's muscular dystrophies resulting in the now-standard examination of dystrophin in muscle biopsy specimens. It is to be expected that immunomorphologic techniques will rapidly develop further, depending on the isolation of muscle-and disease-specific proteins or defective proteins and the availability of antibodies against these proteins. It is conceivable that immunohistological diagnostic methods in myopathology may, one day, render currently established techniques obsolete.

摘要

自从电子显微镜、组织化学和形态测量学被引入诊断性肌病学以来,肌肉活检已经标准化,最近又增加了其他诊断技术。免疫形态学方法——很久以前就在肿瘤学中牢固确立——开始应用于诊断性肌病学。这些方法涉及再生和失神经纤维以及某些以中间丝相关包涵体如胞质体、球形体或马洛里小体样包涵体为特征的肌病中中间丝的证据。肌营养不良蛋白的发现进一步强调了免疫形态学方法的内涵,肌营养不良蛋白是肌肉纤维中的一种正常蛋白质,在杜兴氏和贝克氏肌营养不良症中缺乏,这导致了现在对肌肉活检标本中肌营养不良蛋白的标准检测。可以预期,免疫形态学技术将根据肌肉和疾病特异性蛋白质或缺陷蛋白质的分离以及针对这些蛋白质的抗体的可用性而迅速进一步发展。可以想象,有一天,肌病学中的免疫组织学诊断方法可能会使目前既定的技术过时。

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