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      Focal Glomerulosclerosis Associated with Charcot-Marie-Tooth Disease

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          Abstract

          This report describes a new case of the rare association of focal glomerulosclerosis with a peroneal muscular atrophy of the Charcot-Marie-Tooth type. The young patient was admitted 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 a review of the literature and to further information concerning 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.

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          Author and article information

          Journal
          NEF
          Nephron
          10.1159/issn.1660-8151
          Nephron
          S. Karger AG
          1660-8151
          2235-3186
          1985
          1985
          04 December 2008
          : 40
          : 3
          : 357-361
          Affiliations
          aDepartment of Pathology, Henri Mondor Hospital, Créteil, and Department of Histology, Faculté de médecine, Créteil; bClinique des maladies du système nerveux (Prof. Castaigne), Salpetriere Hospital, Paris; cDepartment of Nephrology, Henri Mondor Hospital (INSERM U139), Créteil, France
          Article
          183493 Nephron 1985;40:357–361
          10.1159/000183493
          4010852
          © 1985 S. Karger AG, Basel

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          Page count
          Pages: 5
          Categories
          Original Paper

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