The systemic disorder of connective tissue metabolism and hypoplasia of the nephron elements in hereditary nephritis in children

: Clinical biochemistry and morphological methods were employed to examine 25 children aged 3 to 15 years with hereditary nephritis. Measurements were made of morphological alterations in renal biopsy specimens, excretion with urine of connective tissue metabolites (hydroxyproline, hydroxylysine glycosides, glycosaminoglycans), the level of the same metabolites and characteristic features of the cellular growth of skin fibroblasts in culture. The early stages of nephritis development were marked by hypoplasia of nephron elements, followed by dystrophy and destruction of its ultrastructural elements including collagen of the glomerular basal membranes. The status of the skin fibroblast cell culture corresponded with the changes seen in renal cells of mesenchymal origin. The conclusion is made that in children with hereditary nephritis, nephron cells and skin fibroblasts reflect systemic metabolic defect of the connective tissue.