Abstract
BACKGROUND: To clarify the predictive factors for poor outcome in pediatric C3 glomerulonephritis (C3GN), we retrospectively evaluated the relationship between the clinico-pathological findings and prognosis in cases of pediatric C3GN. METHODS: We enrolled 18 patients diagnosed with C3GN. These patients were divided into two groups, four patients in the end-stage renal disease (ESRD) group and 14 patients in non-ESRD group, based on clinical status at the last examination. Patients in the non-ESRD group were further divided into Subgroup A, consisting of 6 treatment responders, and Subgroup B, consisting of 8 non- responders. The clinical and laboratory findings, as well as the histological findings were investigated for each group. RESULTS: The frequency of nephrotic syndrome at onset in the ESRD group was higher than that in the non-ESRD group. Before treatment and at 2 years after treatment, urinary protein excretion levels and serum creatinine levels in the ESRD group were higher than those in the non-ESRD group. The mean serum C3 and CH50 levels at 2 years after treatment in the ESRD group were lower than those in the non-ESRD group. The degree of renal injury, level of mesangial deposits and degree of alpha SMA staining at the time of the first renal biopsy in the ESRD group were all higher than those in the non-ESRD group. CONCLUSIONS: Our results suggest that the severity of C3GN at onset and persistent complements activity are associated with poor prognosis in C3GN.