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Revista del Nacional (Itauguá)

Print version ISSN 2072-8174


BASABE OCHOA, Aura Mearlyn; TROCHE HERMOSILLA, Avelina Victoria  and  MARTINEZ PICO, Marlene. C1q nephropathy: unusual diagnosis of nephrotic syndrome in childhood. Case report. Rev. Nac. (Itauguá) [online]. 2020, vol.12, n.2, pp.124-129. ISSN 2072-8174.

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

Keywords : C1q nephropathy; nephrotic syndrome; proteinuria; hematuria.

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