Nephrotic syndrome is characterized by protein loss in the urine, hypoalbuminemia, hyperlipidemia and edema. Several diseases cause a nephrotic syndrome, as they damage the glomerular podocytes. These specialized epithelial cells, together with endothelial cells of the glomerular capillaries and the basal membrane, form a filter that retains plasma proteins in the circulation. A disturbance of this filter causes proteinuria. The three most common primary glomerular diseases are minimal change, membranous glomerulonephritis, and the primary focal segmental glomerulosclerosis. The familiar forms are rare; however, the identification of the relevant gene defects has greatly advanced our understanding of podocyte function as well as the pathogenesis of nephrotic syndrome.
机构:
Chungnam Natl Univ Hosp, Dept Pediat Nephrol, Daejeon 35015, South KoreaUniv Hamburg Eppendorf, Dept Pediat, D-20246 Hamburg, Germany
Lee, Jiwon M.
Shin, Jae Il
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机构:
Yonsei Univ, Coll Med, Dept Pediat, Seoul 03722, South Korea
Severance Childrens Hosp, Div Pediat Nephrol, Seoul 03722, South Korea
Yonsei Univ, Coll Med, Inst Kidney Dis Res, Seoul 03722, South KoreaUniv Hamburg Eppendorf, Dept Pediat, D-20246 Hamburg, Germany