GLOMERULAR-BASEMENT-MEMBRANE ABNORMALITIES IN INFANTS WITH HEAVY PROTEINURIA

Two Indian male children with infantile-onset heavy proteinuria (with nephrotic syndrome in 1) had thickening of the glomerular basement mem brane with splitting and basket-weave appearance of lamina densa on el ectron microscopic evaluation of kidney tissue (like Alport's syndrome ), with normal light microscopic findings and negative immunofluoresce nce. The proteinuria was non-familial and was not associated with micr ohaematuria in patient 1; transient microhaematuria, perhaps associate d with urinary tract infection, was noted in patient 2. There was no n eurosensory deafness in the patients or their parents. The nephrotic s yndrome remitted totally in one patient over a 7-month period. The pro teinuria, as well as the renal disease, was non-progressive in the sec ond patient over a 27-month period. The significance of these basement membrane abnormalities (classically described in Alport's syndrome) i n early-onset nephrotic syndrome/heavy proteinuria that is non-familia l and non-progressive needs to be evaluated.