Crescentic glomerulonephritis in a patient with heterozygous Fabry's disease.
A 58-year-old woman who suffered from a heterozygous Fabry's disease and immune complex crescentic glomerulonephritis (GN) is reviewed. The diagnosis was made on the basis of the pathologic findings and peripheral leukocyte alpha-galactosidase activity. Light microscopy revealed a vacuolization of epithelial cells and electron microscopy showed myelin figures in the cytoplasm of visceral epithelial cells typical of Fabry's disease at the first renal biopsy. During the following 4 months she developed progressive renal failure and a second renal biopsy disclosed the formation of cellular crescents in 7 of 11 glomeruli observed. A rare case of combined Fabry's disease and crescentic glomerulonephritis is discussed.
Shimazu
Kanako
K
Department of Internal Medicine, Saga Medical School, Nabeshima 5-1-1, Saga 849-8501, Japan.
Tomiyoshi
Yoshiyuki
Y
Aoki
Shigehisa
S
Sakemi
Takanobu
T
Sugihara
Hajime
H
D000936:Antigen-Antibody Complex / Q000378:metabolism; D000795:Fabry Disease / Q000150:complications* / Q000235:genetics; D005260:Female; D005921:Glomerulonephritis / Q000150:complications* / Q000276:immunology / Q000473:pathology; D006579:Heterozygote; D006801:Humans; D007668:Kidney / Q000648:ultrastructure; D008854:Microscopy, Electron; D008875:Middle Aged
D000936:Antigen-Antibody Complex