Dense deposit disease in a 6-year-old girl

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A 4-year-old girl presented with proteinuria, gross hematuria and renal insufficiency. Laboratory studies revealed normal serum titers of complement and absences of anti-neutrophil cytoplasmic autoantibody (ANCA) and anti- glomerular basement membrane (GBM) antibody. A renal biopsy showed crescentic glomerulonephritis with diffuse tubule-interstitial injury and C3 deposition along the capillary walls of the glomeruli alone in immunofluorescence (IF), so she was diagnosed with idiopathic crescentic glomerulonephritis. Although she required a temporary hemodialysis, urinary findings and renal function gradually improved by intravenously with 9 pulses of methylprednisolone, followed by combinations of prednisolone (PSL), mizoribine (MZB), dipyridamole and warfarin. At 6 years old, the second renal biopsy showed no active lesions in the glomeruli and prednisolone was discontinued. Four months after that, however, hematuria and proteinuria recurred with decreased C3 levels. They do not improve with conservative therapy. As with the first, the third renal biopsy showed the image of severe segmental necrotizing glomerulonephritis, also found Hump. Since electron-dense deposits have been observed in the basement membrane on electron microscopy of the first renal biopsy, it was diagnosed with dense deposit disease (DDD). As during initial, steroid pulse therapy was very effective. Currently, she was treated with PSL and MZB as prevention of recurrence, and angiotensin converting enzyme inhibitor (ACEI)/ angiotensin receptor blocker (ARB) as renal protective effect.

Dense deposit disease in a 6-year-old girl

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