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脂质沉积性肌病 被引量:2

Lipid storage myopathy Clinical and pathological study in 2cases, electron microscopic changes before and after treatment, and 10-year follow-up.
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摘要 两例因四肢无力 ,生活不能自理入院 ,经实验室检查 ,肌活检发现肌原纤维间及肌膜下有成堆或成串的脂肪空泡 ,线粒体结构改变而诊为脂质沉积性肌病。经激素、核黄素及低脂饮食治疗后好转。再次肌活检发现一例脂质沉积已明显减轻 ,另一例病理改变完全消失。对患者进行为期近 10年的随访 ,患者情况一直稳定 ,无复发。两例为国内跟踪观察最长的病人 。 Two patients showed weakness of extremities and difficulties to take care of themselves. Laboratory study and muscle biopsy revealed numerous lipid droplets accumulated between myofibrils and under sarcokmma in clusters or in aprallel rows. They had a good response to treatment of prednisone, riboflavin and low fat diet. Later, another muscle biopsy was done. Only scarce fat droplets were seen in one case, and normal in the other one. Two cases had undergone nearly 10years follow up study, both presenting in healthy state and no relapse. We suggest that lipid storage myopathy may be benign under adequate treatment.
作者 陈伟贤 陈诒
出处 《脑与神经疾病杂志》 2001年第6期342-344,共3页 Journal of Brain and Nervous Diseases
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