THE ROLE OF I AND B IN PERITONITIS ASSOCIATED WITH THE NEPHROTIC SYNDROME OF CHILDHOOD

Children with nephrotic syndrome (NS) are susceptible to bacterial infections, including primary bacterial peritonitis. Immunologic abnormalities associated with NS include low serum levels of the complement proteins I and B of the alternative complement pathway. We developed a novel and highly sensitive enzyme immunoassay using murine MAb to I and B to quantitate urinary concentrations of these proteins. We studied 22 patients with minimal change NS, including seven with a history of peritonitis (1.6 +/- 0.3 episodes, mean +/- SEM) and 15 without such a history. The two groups did not differ significantly in age, sex, race, age at onset of disease, or duration of disease. Children with minimal change NS complicated by peritonitis had 1) increased urinary excretion of both I (p < 0.05) and B (p < 0.05) in relapse versus remission, 2) greater excretion of I in both relapse (p < 0.01) and remission (p < 0.05) compared with patients without peritonitis, 3) greater excretion of B in relapse compared with patients without peritonitis (p < 0.05), and 4) decreased plasma levels of I compared with patients without peritonitis and controls (p < 0.01) and decreased plasma levels of B compared with controls. Increased urinary excretion of I correlated with decreased plasma levels of I (r = 0.88, p < 0.001). These data support our initial hypothesis that depressed plasma concentrations of these proteins of the alternative complement pathway may predispose patients with minimal change NS to peritonitis and that urinary loss of these proteins is a tenable mechanism.