The nephrotic syndrome in early infancy: a report of three cases

The nephrotic syndrome is characterized by generalized oedema, hypoproteinaemia with diminu-tion or reversal of the albumin/globulin ratio, hyper-lipaemia and gross albuminuria. Haematuria is not prominent; hypertension and azotaemia, except in the terminal stages, are slight and transient and often do not occur at all. Recognition of this syndrome presents no difficulty but in many cases, particularly in childhood, its cause remains obscure. Occasionally certain drugs such as troxidone or mercury, or certain diseases such as amyloidosis, pyelonephritis, diabetes, disseminated lupus erythe-matosus or renal vein thrombosis, can be in-criminated. In cases which develop renal failure and come to necropsy the lesions of glomerulo-nephritis (Type I or Type II of Ellis, 1942) may be found. Other patients, especially children, may never show any unequivocal features of glomerulo-nephritis and appear ultimately to make a complete recovery; such cases belong to the group usually classified as lipoid nephrosis, in which the lesions are held to involve predominantly the renal tubules, glomerular damage being slight or absent. How-ever, this condition is relatively uncommon and, by definition, not of itself fatal; descriptions of the morbid anatomy must therefore be based on some-what limited observation, and the primary cause of the disorder and its relationship to glomerulo-nephritis are still uncertain. It is exceedingly rare for the nephrotic syndrome to appear during the first few weeks of life but when this does happen it is reasonable to suggest that the basic disorder may have originated in utero. The study of the small group of cases of 'congenital nephrosis ' is therefore of particular interest. In the three examples now reported there is, moreover, * Cases 1 and 2 were presented at a meeting of the Pathologica