Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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A case of regression of atypical dense deposit disease without C3 deposition in a child

  • Kim, Min-Sun (Department of Pediatrics, and Research Institute of Clinical Medicine, Chonbuk National University Medical School) ;
  • Hwang, Pyoung-Han (Department of Pediatrics, and Research Institute of Clinical Medicine, Chonbuk National University Medical School) ;
  • Kang, Mung-Jae (Department of Pathology, and Research Institute of Clinical Medicine, Chonbuk National University Medical School) ;
  • Lee, Dae-Yeol (Department of Pediatrics, and Research Institute of Clinical Medicine, Chonbuk National University Medical School)
  • Received : 2010.03.11
  • Accepted : 2010.05.18
  • Published : 2010.07.15
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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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References

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