and review of acute nephritis syndromes n.
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And Review of Acute nephritis Syndromes. karyomegalic tubulointerstitial Nephritis. Karyomegalic Tubulointerstitial Nephritis. Symptoms: Recurrent Pneumonias Renal failure leading invariably to ESRD Hypertension Hematuria Typically patients present in third decade of life.

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karyomegalic tubulointerstitial nephritis
KaryomegalicTubulointerstitial Nephritis
  • Symptoms:
    • Recurrent Pneumonias
    • Renal failure leading invariably to ESRD
    • Hypertension
    • Hematuria
    • Typically patients present in third decade of life.
karyomegalic tubulointerstitial nephritis1
KaryomegalicTubulointerstitial Nephritis
  • Clinical features:
    • Negative seriologies for autoimmune and viral causes
    • Pathology shows:
      • Interstitial fibrosis
      • Large nuclei in proximal tubular cells with odd shape and focal intra-nuclear clearing
      • Negative for immunoflorescence
      • Irregular nuclear membranes on electron microscopy
karyomegalic tubulointerstitial nephritis2
KaryomegalicTubulointerstitial Nephritis
  • Etiology: believed to be secondary to a mitotic block
  • Associated with HLA A9/B35 genotype
  • Viral Etiologies have mostly been debunked due to negative seriologies
  • Chemical exposure is not a consistent trademark
acute nephritis syndromes
Acute Nephritis Syndromes
  • Post Strep GN:
    • Happens after skin or throat infection with Beta hemolytic Strep particulary type M, 1-3 weeks after throat or 2-6 weeks after skin infection
    • Acute endocapillaryproliferatie GN
  • Dx: symptoms of hematuria, edema, nephrotic range proteinuria, headache, hypertension, oliguria
    • Antistrep antibodies positive
    • Renal biopsy showing PMNs, glomerular immune deposits of IGG, IGM, C3, C4, and C5-9 (humps)
  • Rx: supportive.
acute nephritis syndromes1
Acute Nephritis Syndromes
  • Lupus nephritis:
    • Symptoms: hematuria, hypertension, renal failure
    • WHO classifications:
      • I: normal
      • II: mesanigal immune complexes with mesangial proliferation
      • III- IV: focal to global diffuse proliferative disease
        • III: steroids, IV: steroids mycofenalate or cyclophosphamide.
      • V: bubendothelial immune deposits (membranous pattern,
        • -steroids
  • Remember: full house: of IG’s and positive Anti-ds DNA antibodies.
acute nephritis syndromes2
Acute Nephritis Syndromes
  • Anti Basement membrane disease
    • Hemorrhage and glomerulonephritis, oliguria
      • Associated with pulmonary hemorrhage (goodpastures disease)
    • DX: Anti-GMB antibodies (IgG rarely IgA)
      • Biopsy with smooth glomerular staining.
      • ANCAs may be positive with goodpastures syndrome (myeloperoxidase
    • Rx: Plasmapheresis, steroids,
acute nephritis syndromes3
Acute Nephritis Syndromes
  • IgA Nephropathy
    • Dx: <20 years old typically, preceding infection, hematuria, Deposition of IgA in mesangium.
      • One of most common GN worldwide
      • Common in asians and souther europeans (30 and 20% prevalance respectively)
    • Rx: ACE inhibitors, tonsilectomy, steroids,
      • If RPGN: steroids, cytotoxic agents, plasmapheresis
  • Harrisons internal medicine
  • Baba et al, karyomegalic tubulointerstital nephritis – a case report, Pathology, research and practice, 202 2006 555-559.