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KMID : 1130320100530070766
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Korean Journal of Pediatrics
2010 Volume.53 No. 7 p.766 ~ p.769
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A case of regression of atypical dense deposit disease without C3 deposition in a child
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Kim Min-Sun
Hwang Pyoung-Han
Kang Myoung-Jae
Lee Dae-Yeol
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Abstract
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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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KEYWORD
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Child, Glomerulonephritis, Membranoproliferative, Remission induction
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