Korean J Nephrol.  2000 May;19(3):518-522.

A Case of Idiopathic Fibrillary Glomerulonephritis

Affiliations
  • 1Department of Internal Medicine, College of Medicine, University of Ulsan, Seoul, Korea.
  • 2Department of Pathology, College of Medicine, University of Ulsan, Seoul, Korea.

Abstract

A 17-year-old girl was admitted to our hospital due to mild generalized edema. Laboratory tests revealed a serum creatinine was 0.7mg/dL, protein/albumin 6.7/3.5g/dL, cholesterol 190mg/dL, hemoglobin 10.0g/dL, and 24 hour urinary protein 4,40mg/day. Chest X-ray and renal ultrasound were normal. There were no clinical or serologic evidences of paraproteinemia, cryoglobulinemia, light chain disease or systemic lupus erythematosus. Renal biopsy showed membranoproliferative glomerulonephritis-like pattern with lobular accentuation, hypercellularity and diffuse GBM thickening by light microscope. Congo red staining was negative. Granular IgG and C3 deposits were found along the glomerular capillary wall and mesangium by immunofluorescence microscope. Ultrastructurally, abundant subendothelial and mesangial fibrillary deposits were found associated with thickening and wrinkling of GBM. These fibrils, measured about 20-30nm in diameter, were nonbranching and randomly arranged without either periodicity or an organized structure. These findings were compatible with those of fibrillary glomerulonephritis. Thus we report a case of idiopathic fibrillary glomerulonephritis, which is a rare cause of nephrotic syndrome.

Keyword

Fibrillary glomerulonephritis; Neph-rotic syndrome

MeSH Terms

Adolescent
Biopsy
Capillaries
Cholesterol
Congo Red
Creatinine
Cryoglobulinemia
Edema
Female
Fluorescent Antibody Technique
Glomerulonephritis*
Humans
Immunoglobulin G
Lupus Erythematosus, Systemic
Nephrotic Syndrome
Paraproteinemias
Periodicity
Thorax
Ultrasonography
Cholesterol
Congo Red
Creatinine
Immunoglobulin G
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