A case of regression of atypical dense deposit disease without C3 deposition in a child.
10.3345/kjp.2010.53.7.766
- Author:
Min Sun KIM
1
;
Pyoung Han HWANG
;
Mung Jae KANG
;
Dae Yeol LEE
Author Information
1. Department of Pediatrics and Research Institute of Clinical Medicine, Chonbuk National University Medical School, Jeonju, Korea. leedy@chonbuk.ac.kr
- Publication Type:Case Report
- Keywords:
Child;
Glomerulonephritis;
Membranoproliferative;
Remission induction
- MeSH:
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
- From:Korean Journal of Pediatrics
2010;53(7):766-769
- CountryRepublic of Korea
- Language:English
-
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.