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karyomegalic tubulointerstitial Nephritis

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

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  1. And Review of Acute nephritis Syndromes karyomegalic tubulointerstitial Nephritis

  2. KaryomegalicTubulointerstitial Nephritis • Symptoms: • Recurrent Pneumonias • Renal failure leading invariably to ESRD • Hypertension • Hematuria • Typically patients present in third decade of life.

  3. 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

  4. 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

  5. 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.

  6. 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.

  7. 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,

  8. 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

  9. 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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