Successful management of a patient with a C3 Glomerulonephritis and crescentic pattern: a case report.
BACKGROUND
Crescentic glomerulonephritis is a rare condition in children and is typically associated with renal insufficiency. Dysfunction of the alternative complement pathway is an unusual aetiology with an unknown mechanism.


METHODS
We report a case of a previously healthy 12-year-old Caucasian girl who was examined on emergency owing to an asymptomatic gross haematuria. An active urinary sediment and nephrotic-range proteinuria were identified, and serologic examination showed a decreased serum C3 concentration not associated with any immunologic or infectious cause. Oedema, hypertension, and renal insufficiency were not observed. A renal biopsy was performed, and crescentic glomerulonephritis associated with C3 glomerulonephritis was diagnosed. Prompt treatment with intravenous steroids resulted in complete resolution of the gross haematuria. Further examination did not detect any underlying acquired cause. A combination of oral steroids and cyclophosphamide, followed by mycophenolate mofetil, was maintained and resulted in clinical remission during an 8-month follow-up.


CONCLUSIONS
The presence of severe injury such as crescentic glomerulonephritis secondary to C3 glomerulonephritis is extremely unusual in children. This is the first known case of paediatric crescentic glomerulonephritis secondary to C3 glomerulonephritis that presented with gross haematuria and was treated early and effectively with immunosuppressive therapy based on its severe histologic features.
Department of Nephrology, Centro Hospitalar Algarve, Faro Hospital, Leão Penedo, 8000-386 Faro, Portugal. ana.pinho82@gmail.com.
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D002648:Child; D003176:Complement C3 / Q000378:metabolism*; D005260:Female; D005921:Glomerulonephritis / Q000150:complications / Q000188:drug therapy*; D006417:Hematuria / Q000150:complications; D006801:Humans; D007668:Kidney / Q000002:abnormalities* / Q000473:pathology; D011507:Proteinuria / Q000150:complications; D044465:White People
D003176:Complement C3