Abstract
The authors are relating 4 cases of renal amyloid6sis in children between 2-14 years of age, with nephrotic syndrome. Clinically, the oedema were variably developed. The most characteristic laboratory data indicated: proteinuria 18-4,5 g%, hypoproteinemia, inconstant and moderate hypercholesterolemia, alpha-2 fraction increased, up to 33%. In one case, was associated mild hematuria and hypertension. The diagnosis of amyloidosis was relied on renal biopsy(3 cases) and gingival biopsy (1 case). The diagnosis of primary amyloidosis was based on clinical criteria: absence of chronic suppurative infections(tuberculosis, Bronchiectasis, osteomyelitis) accompanying amyloid nephrosis. In one child, the amyloidosis was associated with a pulmonary tumor (bronchogenie dysembryoma) The site and extent of depots of amyloid in renal tissue was variable: initially, in glomerular and tubular basement membrane, subsequently arterio-arteriolar, finally affecting all the glomeruli. The evolution of all cases was progressively rapid(8-l4 months) to renal failure. The corticoids were inefficient and the chlorambucil deteriorated the clinical and biological evolution of the illness.
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Goldis, G., Gane, R. & Arneil, G. Renal Amyloidosis in Children. Pediatr Res 8, 902 (1974). https://doi.org/10.1203/00006450-197411000-00042
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DOI: https://doi.org/10.1203/00006450-197411000-00042