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Pathology · Renal: membranoproliferative glomerulonephritis
Sample itemA 14-year-old girl has haematuria, proteinuria and persistently low serum C3 with normal C4. Light microscopy shows lobulated glomeruli with a tram-track basement membrane, and electron microscopy shows ribbon-like intramembranous dense deposits. What is responsible for the low complement?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. C3 nephritic factor, an autoantibody that stabilises alternative pathway C3 convertase
Dense deposit disease (MPGN type II, now C3 glomerulopathy) is due to uncontrolled alternative pathway activation, often because of C3 nephritic factor. This causes persistent hypocomplementaemia with a normal C4. Cryoglobulinaemic MPGN lowers C4 through classical pathway activation.
Option by option
A. C3 nephritic factor, an autoantibody that stabilises alternative pathway C3 convertase (correct)
Correct. C3 nephritic factor stabilises C3 convertase and consumes C3.
B. Cryoglobulins with hepatitis C
Cryoglobulins activate the classical pathway, so C4 would also be low.
C. Streptococcal antigen planted in the subepithelium
Streptococcal antigens cause subepithelial humps with transient low C3 rather than persistent intramembranous deposits.
D. Anti-glomerular basement membrane antibody
Anti-GBM antibodies produce linear IgG deposition and crescentic disease with normal complement.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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