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.
Department of Paediatrics and Child Health, Kurume University School of Medicine, Japan.
M
M
Kuwano
Y
Y
Ito
Y
Y
Amamoto
K
K
Aida
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