title: Crescentic glomerulonephritis in a patient with heterozygous Fabry's disease.
abstract: 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.
authors.affiliation: Department of Internal Medicine, Saga Medical School, Nabeshima 5-1-1, Saga 849-8501, Japan.
authors.forename: Kanako
authors.initials: K
authors.lastname: Shimazu
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Tomiyoshi
authors.forename: Shigehisa
authors.initials: S
authors.lastname: Aoki
authors.forename: Takanobu
authors.initials: T
authors.lastname: Sakemi
authors.forename: Hajime
authors.initials: H
authors.lastname: Sugihara
mesh_terms: 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
chemical_list: D000936:Antigen-Antibody Complex