Abstract

Lipoprotein glomerulopathy (LPG) is a rare inherited kidney disease in which lipoprotein thrombi are fulfilled in the glomerular capillaries. Most of these patients are found in Japan and East Asian countries. The presenting symptoms include proteinuria, an abnormal plasma lipoprotein profile that resembles type III hyperlipoproteinemia, and a marked increase in serum apolipoprotein E (ApoE) concentration. Previous studies have suggested that LPG might be related to ApoE gene (APOE) mutation. No definite therapeutic regimen has been established for LPG. We report a case of biopsy-proven LPG who presented with nephrotic syndrome and dyslipidemia. There was significant improvement in the clinical parameters and resolution of symptoms after the introduction of fenofibrate. Further studies will be needed to clarify the role of ApoE mutation in the pathogenesis of LPG.

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