Proteinuria in a child with sialidosis: case report and histological studies

A 9-year-old body with sialidosis had nephrotic-range proteinuria. Histological studies demonstrated massive distension of renal cells, particularly glomerular visceral epithelial cells, by cytoplasmic vesicles which contained material reactive with concanavalin A and wheat-germ agglutinin. In addit...

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Bibliographic Details
Published in:Pediatric nephrology (Berlin, West) West), 1989-04, Vol.3 (2), p.166-174
Main Authors: KASHTAN, C. E, NEVINS, T. E, POSALAKY, Z, VERNIER, R. L, FISH, A. J
Format: Article
Language:English
Subjects:
Biological and medical sciences
Child
Errors of metabolism
Fluorescent Antibody Technique
Humans
Kidney - pathology
Kidney - ultrastructure
Lectins
Lipids (lysosomal enzyme disorders, storage diseases)
Male
Medical sciences
Metabolic diseases
Microscopy, Electron
Mucolipidoses - complications
Mucolipidoses - pathology
Proteinuria - complications
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Summary:A 9-year-old body with sialidosis had nephrotic-range proteinuria. Histological studies demonstrated massive distension of renal cells, particularly glomerular visceral epithelial cells, by cytoplasmic vesicles which contained material reactive with concanavalin A and wheat-germ agglutinin. In addition, some glomeruli exhibited segmental mesangial thickening or glomerulosclerosis. Ultrastructurally, focal detachment of visceral epithelial cells from the underlying glomerular basement membrane was observed. We postulate that glomerular visceral epithelial cell dysfunction may underlie the proteinuria and focal glomerulosclerosis exhibited by this patient. Hyperfiltration, as suggested by the child's elevated creatinine clearances, may be a contributing factor.
ISSN:0931-041X
1432-198X
DOI:10.1007/BF00852901