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原发性淀粉样变性中的骨骼肌假性肥大。

Skeletal muscle pseudohypertrophy in primary amyloidosis.

作者信息

Whitaker J N, Hashimoto K, Quinones M

出版信息

Neurology. 1977 Jan;27(1):47-54. doi: 10.1212/wnl.27.1.47.

Abstract

Macroglossia and skeletal muscle enlargement with weakness appeared in a 38-year-old male who was shown to have amyloidosis and light chain multiple myeloma. Free lambda light chains were present in serum and urine. Skeletal muscle contained large amounts of amyloid and other amorphous material infiltrating blood vessel walls and connective tissue. Muscle fibers, particularly the type II variety, were diminished in size. Amyloid and similar amorphous material also were present in skin. This patient represents an unusual, but somewhat uniform, type of involvement of the neuromuscular apparatus in nonfamilial primary amyloidosis.

摘要

一名38岁男性出现巨舌症和骨骼肌增大伴肌无力,检查发现其患有淀粉样变性和轻链多发性骨髓瘤。血清和尿液中存在游离λ轻链。骨骼肌含有大量淀粉样蛋白和其他无定形物质,这些物质浸润血管壁和结缔组织。肌纤维,尤其是II型肌纤维,尺寸减小。皮肤中也存在淀粉样蛋白和类似的无定形物质。该患者代表了非家族性原发性淀粉样变性中神经肌肉装置受累的一种不寻常但较为一致的类型。

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