13. Renal Pathology F - Cox

• alport syndrome clinical finding

  • hematuria = nephritis
  • nerve deafness
  • eye disorders
• Alport is type 4 collagen mutation
• alport syndrome - interstitial cells have foamy appearance due to

  • accumulation of neutral fats and mucopolysaccharides (foam cells)
• Alport - why basketweave appearance

  • GBM develops thickening - pronounced splitting and lamination (arranged in thin layer) of lamina densa
• *** renal scleroderma is diffuse disease
• ** thin membrane disease = < 250 nm

  • mild proteinuria
  • renal function maintained
  • genetic mutation
• Scleroderma renal crisis - clinical

  • result in thrombotic microangiopathic process leading to renal ischemia
• Scleroderma renal crisis - clinical

  • acute HTN
  • acute decline renal function
  • increase creatinine
  • proteinuria or hematuria
• Scleroderma renal crisis vascular findings

  • acute
  • intimal edema
  • thrombi
• Scleroderma renal crisis vascular findings

  • chronic
  • concentric intimal proliferation
  • lumen narrowing
  • fibrointimal sclerosis
• Fibrillary GN - deposition of IgG and infiltrative fibrils

  • present with proteinuria
• immunotactoid glomerulopathy - extracellular glomerular deposition of nonamyloid fibrils

  • pts have monoclonal immunoglobulin deposition in glomeruli
• Immunotactoid Glomerulopathy

  • have circulating paraproteins or myeloma protein
  • present with nephrotic syndrome
• chronic glomerulonephritis - late stages: hyaline masses accumulate

  • transforming glomeruli into acellular eosinophilic mass
• long term dialysis lead to changes to kidney

  • arterial intimal thickening
  • calcification - due to deposition of calcium oxalate