Immunoconglutinin and complement studies in congenital nephrotic syndrome and nephritis of Henoch-Schönlein purpura in children.

Several renal diseases are believed to have an immune pathogenesis; the evidence includes depression of serum complement and the demonstration of the glomerular deposition of complement components and immunoglobulin by immunofluorescence. However, the congenital nephrotic syndrome and the nephritis associated with HenochSchonlein purpura (HSP) are two diseases in which the role of immunopathogenetic mechanisms is unclear. The congenital nephrotic syndrome is invariably resistant to therapy and virtually always fatal (Hallman et al., 1970; Kouvalainen, 1963). An immune pathogenesis has been suggested (Lange et al., 1962, 1963; Kouvalainen, 1963), but the evidence is far from secure and the histological and laboratory data suggest considerable heterogeneity. Some features suggest antigen-antibody complex deposition in Henoch-Schonlein purpura. However, serum C3 levels have consistently been normal (Ayoub and Hoyer, 1969), though mesangial deposits of C3, immunoglobulin, and fibrinogen have been shown by immunofluorescence (Urizar et al., 1968).