PowerPoint-presentasjon

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Fabry Disease Renal Biopsy Findings
Leif Bostad Dept. of Pathology Haukeland
University Hospital
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Renal Involvement in Fabry Disease

• Proteinuria the most frequently reported
  sign
• of kidney disease

• Renal failure Primary cause of death in males

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Early Renal Changes

• Glycolipid accumulation in glomerular cells,
• distal tubules and vascular cells
• Segmental effacement of foot processes
• Glomerular hyalinosis
• FSGS
• Tubulointerstitial damage

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PROGRESSION OF RENAL INVOLVEMENT IN FABRY DISEASE
Glomerular injury
Proteinuria
Injury of epithelial cells proximal tub
?
Injury of epithelial cells distal tub
AGAL
Tubulointerstitial inflammation
?
Scarring GL Sclerosis
Vascular injury/arteriosclerosis Peritubular Capil
lary volume