The Congenital Nephrotic Syndrome: An Immune Reaction?

A number of infants of both sexes with a nephrotic syndrome noted at birth or shortly thereafter have been reported in the literature. 1-9 The most striking histologic finding of the kidneys from biopsy material or at autopsy and especially on microdissection 10 was relatively small cystlike dilatations of the tubuli. For this reason the term "microcystic disease" has been suggested by other investigators in contrast to the congenital polycystic kidney with grossly visible cysts and an entirely different histologic appearance. These small cystlike dilatations of the tubules can not account either for the massive proteinuria and the presence of many erythrocytes and granular casts found in the urine of the majority of infants with the congenital nephrotic syndrome or for the histologic findings of endothelial proliferation, hyalinization, and fibrosis of many glomeruli especially in the more advanced cases. We recently observed a newborn infant with congenital nephrotic syndrome. Sections