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C4 Dense-Deposit Disease
Letter/Communication

C4 Dense-Deposit Disease

Richard J.H Smith, Anne Sullivan and Sanjeev Sethi
The New England journal of medicine, Vol.370(8), pp.784-786
02/20/2014
DOI: 10.1056/NEJMc1309449
PMID: 24552345

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Abstract

To the Editor: Dense-deposit disease is a complement-mediated disorder characterized by a proliferative glomerulonephritis, bright capillary-wall C3 staining on immunofluorescence microscopy, and large intramembranous osmiophilic dense deposits that markedly thicken the glomerular capillary walls. 1 , 2 The underlying pathophysiology — fluid-phase dysregulation of the alternative pathway — leads to accumulation of C3 breakdown products in the glomerular basement membrane. C4 is conspicuously absent. 3 Proteinuria was detected in a 12-year-old Hispanic girl at a well-child visit. The blood urea nitrogen and serum creatinine levels were normal, urinalysis showed trace blood with a urinary protein-to-creatinine ratio of 1.5, and renal ultrasonography showed normal-size . . .

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