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We reported two cases of nephrotic syndrome associated with hydronephrosis. A forty four year old male patient who suddenly complained of nephrotic syndrome, showed left hydronephrosis caused by lower ureteral stenosis. Renal biopsy specimen obtained from the right kidney revealed minor glomerular abnormalities. A sixteen year old female patient had a long history of proteinuria before the onset of nephrotic syndrome. She suffered from bilateral hydronephrosis of which the etiology was unclear. Renal biopsy specimen obtained from the left kidney revealed membranous nephropathy. Because in both cases urine samples collected from each ureter showed the presence of a massive protein, the other kidney was thought to be involved with the same disease. The association of nephrotic syndrome with hydronephrosis is extremely rare. The significance of this unusual combination was discussed with respect to the literature.
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