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伴有小梁状肌纤维的成人杆状体肌病

Adult nemaline myopathy with trabecular muscle fibers.

作者信息

Irodenko Viktoriya S, Lee Han S, de Armond Stephen J, Layzer Robert B

机构信息

Department of Neurology, University of California, School of Medicine, 505 Parnassus Avenue, / San Francisco, California 94143-0114, USA.

出版信息

Muscle Nerve. 2009 Jun;39(6):871-5. doi: 10.1002/mus.21190.

DOI:10.1002/mus.21190
PMID:19229965
Abstract

The term "trabecular myopathy" has been used to designate a syndrome resembling limb-girdle muscular dystrophy in which the predominant pathological feature is an abundance of lobulated or trabecular muscle fibers. However, the validity of this nosological entity has not been verified. Herein we describe a 63-year-old man with a severe, progressive myopathy who exhibited the typical pathological features of both trabecular myopathy and nemaline myopathy in association with a biclonal gammopathy. In this case, adult-onset nemaline myopathy was probably the primary disease process. The diagnostic significance of trabecular muscle fibers remains uncertain.

摘要

术语“小梁状肌病”已被用于指代一种类似于肢带型肌营养不良的综合征,其主要病理特征是存在大量分叶状或小梁状肌纤维。然而,这个疾病实体的有效性尚未得到证实。在此,我们描述一名63岁患有严重进行性肌病的男性,其表现出小梁状肌病和杆状体肌病的典型病理特征,并伴有双克隆丙种球蛋白病。在这个病例中,成人起病的杆状体肌病可能是主要的疾病过程。小梁状肌纤维的诊断意义仍不确定。

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1
Adult nemaline myopathy with trabecular muscle fibers.伴有小梁状肌纤维的成人杆状体肌病
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2
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引用本文的文献

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Inflammatory features in sporadic late-onset nemaline myopathy are independent from monoclonal gammopathy.散发性晚发性杆状体肌病的炎症特征与单克隆丙种球蛋白无关。
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2
Chemotherapy with stem cell transplantation is more effective than immunotherapy in sporadic late onset nemaline myopathy with monoclonal gammopathy.对于伴有单克隆丙种球蛋白病的散发性晚发型杆状体肌病,化疗联合干细胞移植比免疫疗法更有效。
Bone Marrow Transplant. 2018 Jul;53(7):895-899. doi: 10.1038/s41409-017-0080-6. Epub 2018 Jan 24.
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Sporadic late-onset nemaline myopathy: clinical, pathology and imaging findings in a single center cohort.
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Sporadic late-onset nemaline myopathy: clinico-pathological characteristics and review of 76 cases.散发性迟发性杆状体肌病:76例临床病理特征及文献复习
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[Treatability of sporadic late onset nemaline myopathy].
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Muscle phenotypic variability in limb girdle muscular dystrophy 2 G.肢带型肌营养不良 2G 中的肌肉表型变异性。
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