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猪身上被视为所谓“脂肪性肌营养不良”的贝克尔型肌营养不良样肌病:一例报告及其诊断方法

Becker muscular dystrophy-like myopathy regarded as so-called "fatty muscular dystrophy" in a pig: a case report and its diagnostic method.

作者信息

Horiuchi Noriyuki, Aihara Naoyuki, Mizutani Hiroshi, Kousaka Shinichi, Nagafuchi Tsuneyuki, Ochiai Mariko, Ochiai Kazuhiko, Kobayashi Yoshiyasu, Furuoka Hidefumi, Asai Tetsuo, Oishi Koji

机构信息

Department of Basic Veterinary Medicine, Obihiro University of Agriculture and Veterinary Medicine, Inada, Obihiro, Hokkaido 080-8555, Japan.

出版信息

J Vet Med Sci. 2014 Mar 1;76(2):243-8. doi: 10.1292/jvms.13-0336. Epub 2013 Oct 28.

Abstract

We describe a case of human Becker muscular dystrophy (BMD)-like myopathy that was characterized by the declined stainability of dystrophin at sarcolemma in a pig and the immunostaining for dystrophin on the formalin-fixed, paraffin-embedded (FFPE) tissue. The present case was found in a meat inspection center. The pig looked appeared healthy at the ante-mortem inspection. Muscular abnormalities were detected after carcass dressing as pale, discolored skeletal muscles with prominent fat infiltrations and considered so-called "fatty muscular dystrophy". Microscopic examination revealed following characteristics: diffused fat infiltration into the skeletal muscle and degeneration and regeneration of the remaining skeletal muscle fibers. Any lesions that were suspected of neurogenic atrophy, traumatic muscular degeneration, glycogen storage disease or other porcine muscular disorders were not observed. The immunostaining for dystrophin was conducted and confirmed to be applicable on FFPE porcine muscular tissues and revealed diminished stainability of dystrophin at the sarcolemma in the present case. Based on the histological observations and immunostaining results, the present case was diagnosed with BMD-like myopathy associated with dystrophin abnormality in a pig. Although the genetic properties were not clear, the present BMD-like myopathy implied the occurrence of dystrophinopathy in pigs. To the best of our knowledge, this is the first report of a natural case of myopathy associated with dystrophin abnormalities in a pig.

摘要

我们描述了一例猪的贝氏肌营养不良症(BMD)样肌病病例,其特征为猪肌膜上抗肌萎缩蛋白的染色性下降,以及在福尔马林固定、石蜡包埋(FFPE)组织上进行的抗肌萎缩蛋白免疫染色。该病例在一个肉类检验中心被发现。这头猪在宰前检查时看起来健康。胴体修整后发现肌肉异常,表现为骨骼肌苍白、变色,有明显的脂肪浸润,被认为是所谓的“脂肪性肌营养不良”。显微镜检查显示出以下特征:脂肪弥漫性浸润到骨骼肌中,剩余的骨骼肌纤维发生变性和再生。未观察到任何疑似神经源性萎缩、外伤性肌肉变性、糖原贮积病或其他猪肌肉疾病的病变。进行了抗肌萎缩蛋白的免疫染色,证实其适用于FFPE猪肌肉组织,并显示在本病例中肌膜上抗肌萎缩蛋白的染色性减弱。基于组织学观察和免疫染色结果,本病例被诊断为猪的与抗肌萎缩蛋白异常相关的BMD样肌病。尽管遗传特性尚不清楚,但目前的BMD样肌病提示猪中发生了抗肌萎缩蛋白病。据我们所知,这是首例关于猪中与抗肌萎缩蛋白异常相关的肌病自然病例的报告。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4c7d/3982806/24e6a4ead99b/jvms-76-243-g001.jpg

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