Abstract
A woman in her 40s with kidney failure secondary to biopsy-proven dense deposit disease (DDD) with positive C3 nephritic factor (C3Nef) and no complement gene defect received a kidney transplant from a deceased donor. Eighteen years after transplantation, laboratory workup revealed new-onset microscopic hematuria, with 547 red blood cells/μL (normal <25), and proteinuria, with a urine protein-to-creatinine ratio of 1.0 g/g creatinine (normal <0.150), increasing to 7.5 g/g within a few months. Glomerular filtration rate, estimated from the CKD-EPI equation, was normal (88 mL/min/1.73m2). Serum C3 level was very low (0.09 g/L, N 0.90 -1.80), while serum C4 level was normal. Screening for monoclonal gammopathy, BK viremia and circulating donor-specific antibodies, was negative.
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