Systemic non-amyloidotic fibril deposition disease: a probable variant form of fibrillary glomerulonephritis.
Clin Nephrol
; 75(1): 74-9, 2011 Jan.
Article
em En
| MEDLINE
| ID: mdl-21176754
ABSTRACT
Fibrillary glomerulonephritis (FGN) is characterized by deposition of non-amyloidotic fibrillary material in glomeruli, and most patients with the disease show heavy proteinuria and hematuria, and progress into end-stage renal failure. We report a 62-year-old woman with FGN who showed mild proteinuria without hematuria and developed rapidly progressive renal failure requiring hemodialysis. Renal biopsy showed severe tubulointerstitial injury associated with non-amyloidotic fibrillary deposits in the tubular basement membrane, interstitium and vessel walls, in addition to glomeruli. The patient died from liver abscess 1 year after the introduction of hemodialysis. Postmortem examination showed the presence of non-amyloidotic fibrillary deposits arranged in tightly packed electron-dense and bundle-shaped structures in many organs. These findings suggest systemic non-amyloidotic fibril deposition in FGN.
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Base de dados:
MEDLINE
Assunto principal:
Glomerulonefrite
/
Rim
Idioma:
En
Ano de publicação:
2011
Tipo de documento:
Article