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Korean J Pediatr 2010 July;53(7) :766-769.
doi:https://doi.org/10.3345/kjp.2010.53.7.766
A case of regression of atypical dense deposit disease without C3 deposition in a child
Min Sun Kim (Kim MS)1, Pyoung Han Hwang (Hwang PH)1, Mung Jae Kang (Kang MJ)2, Dae-Yeol Lee (Lee DY)1
1Departments of Pediatrics, Institute of Clinical Medicine, Chonbuk National, University Medical School, Jeonju, Korea
2Departments of Pathology, and Research Institute of Clinical Medicine, Chonbuk National University Medical School, Jeonju, Korea
Corresponding Author: Dae-Yeol Lee ,Email: leedy@chonbuk.ac.kr
Copyright © 2010 by The Korean Pediatric Society
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.
Keywords: Child | Glomerulonephritis | Membranoproliferative | Remission induction
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