Korean J Pediatr.  2010 Jul;53(7):766-769. 10.3345/kjp.2010.53.7.766.

A case of regression of atypical dense deposit disease without C3 deposition in a child

• Kim MS
• Hwang PH
• Kang MJ
• Lee DY

• KMID: 2073608
• DOI: http://doi.org/10.3345/kjp.2010.53.7.766

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.

Keyword

Child; Glomerulonephritis; Membranoproliferative; Remission induction

MeSH Terms

Biopsy
Child
Creatinine
Dichlorodiphenyldichloroethane
Edema
Follow-Up Studies
Glomerular Basement Membrane
Glomerulonephritis
Glomerulonephritis, Membranoproliferative
Hematuria
Humans
Kidney
Kidney Failure, Chronic
Nephrotic Syndrome
Proteinuria
Remission Induction
Creatinine
Dichlorodiphenyldichloroethane