And review of acute nephritis syndromes
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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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And review of acute nephritis syndromes

And Review of Acute nephritis Syndromes

karyomegalic tubulointerstitial Nephritis


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


References

References

  • Harrisons internal medicine

  • Baba et al, karyomegalic tubulointerstital nephritis – a case report, Pathology, research and practice, 202 2006 555-559.


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