C1q nephropathy: unusual diagnosis of nephrotic syndrome in childhood. Case report
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Abstract
C1q nephropathy is a poorly understood and underdiagnosed glomerulopathy. It is defined by a
dominant or codominant immunfluorescence pattern of C1q positivity, with electrodense deposits in
the mesangium, in the absence of serology and symptoms of systemic lupus erythematosus. Clinically,
it manifests with severe proteinuria or nephrotic range, occasionally hematuria and arterial
hypertension. Histologically it presents varied morphology. It usually manifests as a corticodependent
or cortico-resistant nephrotic syndrome with poor response to immunosuppressive treatment and
evolution to chronicity. We present the clinical case of a child diagnosed with C1q nephropathy, who
underwent a renal biopsy due to corticosteroid-resistant nephrotic syndrome, with negative serology
and absence of clinical data for systemic lupus erythematosus. Timely intervention and early
management slow down its progression to chronicity
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