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一只魏玛犬患肌营养不良蛋白缺乏性肌营养不良症。

Dystrophin-deficient muscular dystrophy in a Weimaraner.

作者信息

Baltzer Wendy Irene, Calise David V, Levine Jonathan M, Shelton G D, Edwards John F, Steiner Joerg M

机构信息

Department of Clinical Sciences, College of Veterinary Medicine, Oregon State University, Corvallis, Oregon 97331, USA.

出版信息

J Am Anim Hosp Assoc. 2007 Jul-Aug;43(4):227-32. doi: 10.5326/0430227.

DOI:10.5326/0430227
PMID:17615404
Abstract

A 2-year-old, male Weimaraner with muscular dystrophy was presented with generalized muscle atrophy of the limbs; hypertrophy of the neck, infraspinatus, and lingual muscles; dysphagia; and regurgitation. Unilateral cryptorchidism, unilateral renal agenesis, and hiatal hernia were also detected. Spontaneous muscle activity was identified on myography. Serum creatine kinase was markedly elevated. Immunohistochemical staining for dystrophin was restricted to suspected revertant (characteristics of immaturity) fibers. Histologically, skeletal myofiber degeneration, endomysial fibrosis, and mineralization were present. Following euthanasia, necropsy revealed hypertrophy of the diaphragm and cardiac muscle fibrosis. This case of muscular dystrophy represents a slowly progressive form with organ agenesis.

摘要

一只患有肌肉萎缩症的2岁雄性魏玛犬出现四肢广泛性肌肉萎缩;颈部、冈下肌和舌肌肥大;吞咽困难和反流。还检测到单侧隐睾、单侧肾发育不全和食管裂孔疝。肌电图显示有自发肌肉活动。血清肌酸激酶明显升高。抗肌萎缩蛋白的免疫组织化学染色仅限于疑似回复(不成熟特征)纤维。组织学检查发现骨骼肌纤维变性、肌内膜纤维化和矿化。安乐死后,尸检显示膈肌肥大和心肌纤维化。这例肌肉萎缩症代表了一种伴有器官发育不全的缓慢进展型。

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