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Korean J Pediatr. 2010 Jul;53(7):766-769. English. Case Report.
Kim MS , Hwang PH , Kang MJ , Lee DY .
Department of Pediatrics and Research Institute of Clinical Medicine, Chonbuk National University Medical School, Jeonju, Korea. leedy@chonbuk.ac.kr
Department of Pathology and Research Institute of Clinical Medicine, Chonbuk National University Medical School, Jeonju, Korea.
Abstract

Dense deposit disease (DDD) is a rare disorder characterized by the deposition of abnormal electron-dense material within the glomerular basement membrane of the kidneys. The diagnosis is made in most patients between 5 and 15 years of age, and within 10 years, approximately half of the affected patients progress to end-stage renal disease. We report a rare case of regressive DDD without C3 deposition after steroid therapy in an 11-year-old boy. The patient presented with edema, gross hematuria, and nephrotic-range proteinuria. Laboratory testing revealed a serum creatinine level of 1.17 mg/dL, albumin level of 2.3 g/dL, and serum C3 level of 125 mg/dL (range 90-180 mg/dL). The results of the renal biopsy were consistent with DDD without C3 deposition. After 6 weeks of steroid therapy, the nephrotic syndrome completely resolved. The follow-up renal biopsy showed a significant reduction in mesangial proliferation and disappearance of electron-dense deposits in the GBM.

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