Author/Editor		Kersnik-Levart, Tanja; Kenda, Rajko B; Avguštin-Čavić, Mojca; Ferluga, Dušan; Hvala, Anastazija; Vizjak, Alenka
Title		C1Q nephropathy in children
Type		članek
Source		Pediatr Nephrol
Vol. and No.		Letnik 20
Publication year		2005
Volume		str. 1756-61
Language		eng
Abstract		C1q nephropathy (C1qNP) is a peculiar form of glomerulonephritis characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence of systemic lupus erythematosus. We describe the incidence, manifestation, histopathologic findings, follow-up, treatment and outcome of C1qNP. Twelve C1qNP patients were identified among 131 children who had undergone renal biopsy, accounting for a 9.16% incidence of C1qNP. Light microscopy examination showed focal segmental glomerulosclerosis (FSGS) with or without diffuse mesangial proliferation (n=6), minimal change disease (MCD) (n=4) or focal glomerulonephritis (n=2). C1q deposits were found in all, while electron microscopy revealed visible deposits in nine cases. Eight children presented with nephrotic syndrome, while one had nephrotic proteinuria and renal insufficiency that progressed to end-stage renal failure. The remaining three patients presented with nonnephrotic proteinuria associated with microhematuria, hypertension or renal insufficiency. Only one nephrotic syndrome patient responded excellently to corticosteroids, while four became corticosteroid dependent, and three were corticosteroid resistant, showing a very poor response to other immunosuppressive therapy as well. Patients with nonnephrotic proteinuria demonstrated fixed laboratory findings. Most C1qNP patients had FSGS or MCD, the majority of them presenting with corticosteroid-dependent or corticosteroid-resistant nephrotic syndrome. The latter showed a vety poor response to any imtnunosuppressive therapy and high risk for progressive renal insufficiency.
Descriptors		GLOMERULONEPHRITIS COMPLEMENT 1Q GLOMERULAR MESANGIUM IMMUNOSUPPRESSIVE AGENTS NEPHROTIC SYNDROME SEX FACTORS TIME FACTORS RETROSPECTIVE STUDIES SLOVENIA RECURRENCE COHORT STUDIES BIOPSY FOLLOW-UP STUDIES FLUORESCENT ANTIBODY TECHNIQUE, DIRECT HYPERTENSION PROTEINURIA KIDNEY FAILURE