A patient report with focal segmental glomelular sclerosis and severe interstitial fibrosis 8 years after the onset of hemolyticuremic syndrome

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  • 溶血性尿毒症症候群発症８年後より高度蛋白尿を呈し，糸球体硬化および著明な間質の線維化を認めた１例

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Abstract

 Hemolytic-uremic syndrome (HUS) patients often need treatment with dialysis temporally at the acute phase of HUS and rarely develop chronic renal failure after years of apparent recovery. We report a 14-year-old girl who developed HUS at 3 years of age. She showed acute renal failure and treated with peritoneal dialysis for about a month. She also showed seizure and disturbed consciousness on acute phase. Although her kidney function normalized, and her protenuria was disappeared, high urine β2MG level was persisted. Therefore we performed kidney biopsy, and showed a portion of scarring tissue which findings may derived from the cortical necrosis on acute phase. Because of these findings, we started to treat with ACE inhibitor. After the induction of this treatment, her urine β2MG level was normalized. Eight years after the onset of HUS, she began to showed severe proteinuria and we performed repeated kidney biopsy. It showed focal segmental glomerular sclerosis and severe fibrosis of intestitiums and predicted to have bad renal prognosis. We should follow up HUS patients carefully and need long period observation after the onset of HUS in patients with prolonged renal failure on early phase.

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