Abstract
In surveying the AP in children, 7 CN with normal serum C3 but low-normal or low-Factor B were studied. Heavy proteinuria was found in all, impaired renal function in 2. Two received daily and 2 alternate day prednisone. Renal biopsy revealed minimal changes in 2, mesangial nephritis in 2; focal, post-strep and congenital nephritis in one each. C3, C4, C5, B, properdin (P), and B1H were measured by radial immunodiffusion. C function was evaluated by lysis of EA (CH50) or EAC142 in the presence of EDTA (C3--9), lysis of glutathione-treated human E (APLys) and C3 and B conversion on immunoelectrophoresis in fresh serum and following 37°C incubation with buffer or inulin (C3-B/C). All data were compared to age-matched normal children.
B correlated with APLys, not so well with P, and not at all with B1H or C3-B/C. B was found in concentrated urine specimens of 2 with low-normal serum B. Classical C activation was present only in post-strep nephritis. Low B levels cannot be explained simply by urinary loss or consumptive depletion, and may reflect depressed synthesis. The relationship of normal C3 to low B remains to be explored, but our data clearly emphasize the importance of age-matched controls in evaluating the AP in children.
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Norman, M., Taylor, A., Nilsson, U. et al. ALTERNATIVE COMPLEMENT PATHWAY (AP) IN CHILDREN WITH NEPHROSIS (CN). Pediatr Res 11, 555 (1977). https://doi.org/10.1203/00006450-197704000-01111
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DOI: https://doi.org/10.1203/00006450-197704000-01111
