Clinical and pathological features of dense deposit disease in children

Objectives To discuss the clinical,pathological features and treatment in 5 dense deposit disease(DDD) patients.Methods The data of 5 biopsy-confirmed DDD patients were analyzed and related literatures were reviewed.Results Five patients(3 males and 2 females) were diagnosed with DDD and it accounted for 0.3% of renal biopsy performed in the same period.Two cases manifested as nephrotic syndrome,2 cases as hematuria and proteinuria and 1 case as nephritic syndrome.Serum C3 was reduced in 3 cases.Four cases had mesangial proliferative glomerulonephritis and only 1 case had membrane proliferative glomerulonephritis.Immunofluorescence showed predominant C3 deposition in all cases and 3 patients also had immunoglobulin deposits.All 5 cases had typical ribbon-like dense deposits in glomerular basement menbrane(GBM) under electron microscopy and some cases had mesangial,endothelial and subepithelial deposition.Conclusions The clinical and microscopic pathological manifestations of DDD are variable and non-specific,and may include minor proteinuria and even normal level of serum C3.The diagnosis of DDD still relies on the examination by electron microscopy.