FOCAL GLOMERULOSCLEROSIS ASSOCIATED WITH CHARCOT-MARIE-TOOTH DISEASE
FOCAL GLOMERULOSCLEROSIS ASSOCIATED WITH CHARCOT-MARIE-TOOTH DISEASE
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DOI：
10.1159/000183493
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发表时间：
1985-01-01
期刊：
影响因子：
2.5
通讯作者：
LAGRUE, G
中科院分区：
文献类型：
--
作者：
GHERARDI, R;BELGHITIDEPREZ, D;LAGRUE, G
一个罕见的局灶性肾小球硬化症与腓骨肌萎缩的Charcot-Marie-Tooth型的情况下描述。患者[14岁男性]因肾病综合征入住[法国Creteili的Henri Mondor医院]。晚期肾衰竭迅速发展。患者家族中有许多慢性肾病。肾活检的超微结构研究显示上皮足突局灶性融合，系膜基底层增厚和皱褶，足细胞空泡化，核内可见小的清晰包涵体。沙可-玛丽-图斯病属于肥厚型。根据文献对先前报道的5例病例的随访，肾病的预后在几年内进展为终末期肾脏疾病时受到保护。本病例是第6例局灶性肾小球硬化与腓骨肌萎缩症相关的报告病例。
A case of the rare association of focal glomerulosclerosis with a peroneal muscular atrophy of the Charcot-Marie-Tooth type is described. The patient [14 yr old male] was admitted [to the Henri Mondor Hospital, [Creteili, France]] with a nephrotic syndrome. A terminal renal failure rapidly developed. Numerous chronic nephropathies were known in the patient''s family. The ultrastructural study of the renal biopsy revealed a focal fusion of the epithelial foot processes, thickened and pleated mesangial basal laminae, vacuolated podocytes and small intranuclear clear inclusions. The Charcot-Marie-Tooth disease was of the hypertrophic type. According to the literature in the follow-up of the 5 previously reported cases, the prognosis of the nephropathy is guarded with progression to end-stage renal disease in a few years. This case represents the 6th reported case of focal glomerulosclerosis associated with Charcot-Marie-Tooth disease.