Characteristics of repeated renal biopsy-proven primary focal segmental glomerulosclerosis in children / 中华儿科杂志

<p><b>OBJECTIVE</b>To analyze the characteristics of repeated renal biopsy-proven primary focal segmental glomerulosclerosis (PFSGS) in 8 children, and to reveal the relationship between clinical features and pathology, between the two times of renal biopsy pathology, and the indications for repeated renal biopsy.</p><p><b>METHOD</b>The records of cases who ever experienced renal biopsy in this hospital were reviewed, of whom 8 cases of repeated renal biopsy-proven PFSGS were enrolled. The clinical manifestations, the reason why they had renal biopsy again, the difference in renal pathological findings, between the two biopsies and their therapeutic response. The classification of focal segmental glomerulosclerosis (FSGS) was based on the new criteria suggested by D'Agati in 2004.</p><p><b>RESULT</b>Of the 8 cases, age of onset ranged from 1 to 12 years, all were diagnosed as nephrotic syndrome (NS), the age of first biopsy ranged from 1.1 to 15.0 years, and the follow-up period was 10 months to 14 years. The reason for repeated biopsy was poor therapeutic response, continuous heavy proteinuria, or the progressive renal dysfunction. Four cases had the both biopsies in this hospital, and the first renal pathology showed minimal change disease (MCD), mesangial proliferation, FSGS CELL type and FSGS GTL type. After the second biopsy, they were additionally treated with immunosuppressive agents or switched to another one, 2 cases with FSGS COLL type presented renal dysfunction or end stage renal disease (ESRD), 1 case who developed the disease at 1.4 years of age, presented renal dysfunction at 10 months follow-up. The remaining 5 cases acquired complete remission.</p><p><b>CONCLUSION</b>FSGS is a clinicopathological syndrome, NS predominates clinically. It often indicates pathologic transformation when the patients show poor therapeutic response or continuous heavy proteinuria without remission. Mesangial proliferation can convert into FSGS, and the subtype of FSGS can shift. FSGS COLL type and onset at young age may suggest poor prognosis.</p>

Characteristics of pediatric C3 glomerulopathy with decreased factor H in 3 cases / 中华儿科杂志

<p><b>OBJECTIVE</b>To study the characteristics of clinicopathology and prognosis of 3 pediatric cases diagnosed as C3 glomerulopathy, and to improve the understanding of C3 glomerulopathy in children.</p><p><b>METHOD</b>The medical record, plasma complement C3, Factor H (FH) and its autoantibody, and therapeutic response of the 3 cases were analyzed, and their prognosis were followed up.</p><p><b>RESULT</b>Of the 3 cases, 2 were male and 1 was female, the age of onset was 9 years, 12 years, 5 years 4 months, the duration from onset to renal biopsy was 3 months, 7 months and 20 days, and the follow-up period were 2.6 years, 8 months and 1.5 years respectively.</p><p><b>CLINICAL MANIFESTATIONS</b>All the 3 cases showed microscopic hematuria, with or without gross hematuria and proteinuria. Two showed persistently decreased plasma complement C3, in the other one C3 was in normal lower limit, all presented with decreased FH concertration, in 1 case anti-FH antibody was positive. Their clinical diagnosis was post-streptococcal glomerulonephritis, nephrotic syndrome (NS) nephritis type, and mesangial proliferative glomerulonephritis respectively.</p><p><b>PATHOLOGICAL FINDINGS</b>All showed evident deposition of C3 on glomerular basement membrance (GBM) and mesangial region by immunofluorescence (IF) and electron dense deposit in GBM, mesangial region or para-mesangial region by Electron microscopic (EM) examination Treatment and prognosis: The case with NS showed no response to steroid, so steroid was gradually stopped after renal biopsy and replaced by angiotensin converting enzyme inhibitors (ACEI) and angiotensin receptor antagonist (ARB). The other two cases were treated with ACEI and renal protective treatment. Of the 3 cases, one gradually showed elevated serum creatinine (Scr) and decreased creatinine clearance rate (Ccr), the other two were normal, but slightly increased indications for early kidney injury.</p><p><b>CONCLUSION</b>C3 glomerulopathy is characterized by evident C3 deposition under IF. Its clinical and pathological manifestations vary a lot. The decreased plasma C3 and FH suggest that the abnormal regulation of complement system play an importment role in its pathogenesis.</p>