IMMUNOFLUORESCENT AND MORPHOLOGICAL STUDIES IN CONGENITAL NEPHROTIC SYNDROME1

Two kidneys from patients with congenital nephrotic syndrome obtained at nephrectomy at the age of 3 and 23 months, were investigated for possible immunopathological mechanisms for the kidney injury. Immuno-fluorescent staining with anti IgG, IgM, IgA and /SIC-globulins did not show attachment of immunolglobulins or complement to the glomeruli. Staining was found in some tubular casts and occasionally in tubular epithelium, as well. Electron microscopic studies of the glomeruli corroborated the idea that immunocomplexes are not conglomerated in the glomeruli because of the absence of typical deposits, which are usually found in association with positive immunofluorescent staining in various glomerulonephritides. Kidneys were also eluted with acidic buffer in order to remove antibodies from immunocomplexes. The eluates were analyzed for their immunoglobulin content and used for indirect immunofluorescent staining. The content of IgG of the kidney eluates was low and no difference of eluted IgG between the nephrotic kidneys and the control was apparent. No binding of immunoglobulin with structures of normal kidney could be seen in the indirect immunofluorescent study. Our results corroborate the previous results (10) that immunopathogenesis is not essential in the congenital nephrotic syndrome.