Korean Journal of Nephrology 2000;19(3):518-522.

증례 : 특발성 원섬유성 사구체신염 (Fibrillary Glomerulonephritis) 1예 (A Case of Idiopathic Fibrillary Glomerulonephritis)

김지훈(Ji Hoon Kim),정해혁(Hae Hyuk Jung),박정식(Jung Sik Park),이상구(Sang Koo Lee),유은실(Eun Sil Yu),장상필(Sang Pil Chang),이준승(Joon Seung Lee),정재걸(Jae Gul Chung)

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 para-proteinemia, 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 stain-ing was negative. Granular IgG and C3 deposits were found along the glomerular capillary wall and mesangium by immunofluorescence microscope. Ultrastruc-turally, abundant subendothelial and mesangial fibril- lary deposits were found associated with thickening and wrinkling of GBM. These fibrils, measured about 20-30nm in diameter, were nonbranching and ran- domly 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.