Clinical and Pathological Analysis of Complement 1q Nephropathy in 10 Cases of Children

Objective To explore the relationship between clinical and pathological changes of complement 1q(C1q) nephropathy. Methods Clinical manifestation, pathologic features including glomerulus change, renal tubule - interstitial change and im-munopathology were compared between 10 cases of C1q nephropathy in children, who were diagnosed by renal biopsy. Results Presentation included idiopathic nephritic syndrome(6 cases), simple hematuria(2 cases), nephritic syndrome(1 case), rapidly progressive glomerulonephritis( 1 case); Renal biopsy revealed focal segmental glomerulosclerosis( FSGS) in 5, minimal-change disease( MCD) and mesangial proliferative glomerulonephritis (MsPGN) respectively in two and crescentic glomerulonephritis in one. In addition, there were renal - tubule interstitial changes with 3 cases of grade I and grade II each other, 2 of grade III , 1 of grade IV . The prominent immunofluorescent features was the presence of bright mesangial deposition of C1q. The average follow - up time was 25.7 months. Six cases presenting nephrotic syndrome were resistant to steroid, but 5 were released after immunosuppressive therapy, the other had progressive renal insufficiency. Conclusions C1q nephropathy falls with the clinical - pathologic spectrum of FSGS generally. It is also presented as steroid - resistant nephritic syndrome. Moreover, the prognosis of C1q nephropathy is related to renal tubulointerstitial pathologic lesions not to C1q deposition.