Monday 14 June 2010

renal pearls 2: getting neurotic with nephritic syndrome

first, a reminder of what a normal glomeruli looks like:



there are 2 types of GN: nephritic and nephrotic. it cannot be both at the same time, but nephritic can progress to nephrotic.

NEPHRITIC SYDROME
  • hematuria (active damage to glomeruli)
  • rbc casts 
  • protein spill but less than 3.5g in 24hrs (mild to moderate proteinurea)
  • oliguria (because you have an inflammation of the glomerulus = decrease GFR)
  • HT (because of the decreased GFR, there is no filtering of Na and a build up of it)
there are FOUR diseases that can cause this syndrome:
  1. proliferative GN:

    • diffused inflammation of all glomeruli plus active proliferation of cells
  2. post strep GN:

    • had strep throat/ scarlet fever week(s) prior and then comes in with puffy face (edema) and cola coloured urine (hematuria).
    • IC deposits in subepithelial space
    • treat with corticosteroids


    hypercellularity of post-infectious glomerulonephritis is due to increased numbers of epithelial, endothelial, and mesangial cells as well as neutrophils in and around the glomerular capillary loops. this disease may follow several weeks after infection with certain strains of group A beta hemolytic streptococci. patients who have had a strep infection typically have an elevated anti-streptolysin O (ASO) titer.

  3. lupus GN

    • almost always young woman (~30-35 y.o) with serum ANAs.
    • six types but most important = diffuse proliferative GN

      • lots of nuclei
      • wire loops
    • subendothelial deposits


      extensive immune complex deposition in the thickened glomerular capillary loops, giving a so-called wire loop appearance

  4. crescentic GN

    • also known as Rapidly Progressing GN because of its fast progression to acute RF 
    • RPGN is a description not a specific disease. the diseases that can causeit: SLE, post strep GN, goodpasture's syndrome, vasculitis (polyarteritis nodosa, wegner's granulomatosa) or it could even be idiopathic
    • the glomerulus is surrounded by proliferating cells that are epithelial cell. the glomeruli damage is so severe, fibrinogen leaks into the bowman's space inducing epithelial proliferation.



     
    anti IgG ab deposits as seen in Goodpasture's: linear appearance on immunoflorescence


    crescents made up of proliferating epithelial cells


    to be continued...

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