A case of congenital nephrotic syndrome associated with positive C1q immunofluorescence.
We present a 1-month-old girl with a congenital nephrotic syndrome and unusual histological findings. Immunofluorescence microscopy demonstrated granular mesangial deposition of C1q and electron microscopy revealed electron-dense mesangial deposits. Her heavy proteinuria gradually decreased and the steroid therapy did not have a significant effect. Her renal function was normal throughout the entire period of observation. The clinical evidence and histopathological features of this patient were compatible with C1q nephropathy.
Kuwano
M
M
Department of Paediatrics and Child Health, Kurume University School of Medicine, Japan.
Ito
Y
Y
Amamoto
Y
Y
Aida
K
K
D015922:Complement C1q / Q000032:analysis*; D005260:Female; D005455:Fluorescent Antibody Technique; D005920:Glomerular Mesangium / Q000276:immunology* / Q000648:ultrastructure; D006801:Humans; D007223:Infant; D009404:Nephrotic Syndrome / Q000151:congenital* / Q000276:immunology* / Q000473:pathology; D011507:Proteinuria / Q000473:pathology
D015922:Complement C1q