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Pathology of Kidney Disorders

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Today is the First Day, of Rest of Your Life...! Pathology of Glomerulonephritis Dr. Venkatesh Murthy Shashidhar Senior Lecturer in Pathology Normal Kidney: Normal Glomerulus (PAS) Anatomy: Cap. loops J.G.App. DCT Afferent.A DCT Efferent.A Filtration Unit: 1. Fenestrated Endothelium 2. Lamina Rara Interna 3. Lamina Rara Densa 4. Lamina Rara Externa 5. Podocytes + Slit membrane Capillary Lumen Glomerular Filtration: BLOOD Proteins 3.6nm/70,000MW Glomerular Capillary Lumen L.R.I L.D. L.R.E GBM Plasma Proteins FILTRATE Foot Process Podocyte (Visceral epithelium) Bowman’s Capsule Space Pathogenesis:  Immune mechanisms – Most common  Autoimmune  Planted Antigen  Immune complex.  Toxins  Metabolic Glomerular diseases: Primary GN: • Acute Diffuse Prol. Poststrept & other Inf. • Crescentic (Rapidly Progressive) • Membranous GN. • Lipoid / Minimal Change . • Focal segmental G.sclerosis. • Membranoproliferative GN. • IgA nephropathy. • Chronic Idiopathic GN. Associated with Systemic Diseases: • SLE, DiabetesMellitus. • Goodpasture's Syndrome. • Polyarteritis Nodosa. • Wegener's Granulomatosis. • Henoch-Schonlein Purpura. • Bacterial Endocarditis. • Amyloidosis • Hereditary Disorders: • Alport's Syndrome. • Fabry's Disease Pathogenesis of Immune GN: 1. Ab, Ag/Ab or Immune complex deposition. 2. Immune reaction 3. Inflammation Activation of complement 4. destruction of glomerular structure 5. Renal dysfunction, Proteinuria, Hematuria Immune Glomerulonephritis: Glomerular damage - patterns: Immune Glomerulonephritis:  In-Situ immune complex formation:  Tissue antigens - Goodpasture anti GBM Ag  Planted antigens - infections, toxins, drugs.  Circulating immune complex deposition.  Endogenous - DNA as in SLE  Exogenous - infections.  Cell mediated Immune injury Immune Glomerulonephritis: C.Immune Complex ANTI-GBM HEYMANN Clinical Syndromes:  Nephritic syndrome.  Oliguria, Haematuria, Proteinuria, Oedema.  Nephrotic syndrome.  Gross proteinuria, hyperlipidemia,  Acute renal failure (RPGN).  Oliguria, loss of Kidney function - within weeks  Chronic renal failure.  Over months and years - Uremia Nephritic Syndromes :  Diffuse Proliferative GN  Post Streptococcal.  Rapidly Progressive GN (or Crescentic)  Post Streptococcal, Goodpasture’s,  Focal Glomerulonephritis  Primary: Bergers disease (IgA Nephritis)  Secondary IgA nephritis, Henoch Schonlein purpura, SBE, Coeliac Disease etc. Post Streptococcal GN (Prol.GN):  1-4 weeks following streptococcal infection (nephritogenic strains)  Immune mediated (time for Ab formation)  Granular deposits of IgG,IgM & C3 in GBM, (subepithelial location common)  Humps in GBM on EM or IF Microscopy Pathogenesis of Diffuse PGN:  Streptococcal infection - Immune complex deposition, inflammation & proliferation.  Glomerular capillary obstruction:  J.G.A stimulation – Renin – high blood pressure  Reduced filtration – raised blood urea  Fluid retention – Oedema  Damage to GBM:  Unselective proteinuria (form Pr. casts in tubule)  Haematuria (form RBC casts in tubule) Progression of DPGN: Poststreptococcal DPGN Complete Healing Focal segmental glomerulo sclerosis CGN Tubulo – Interstitial Damage Clinical Features: G.Nephritis •Hypertension •Skin Infections •Congestive Cardiac Failure Laboratory Features: G.Nephritis •Inflammation •Decreased filtration •Damage to filtration unit Diffuse Proliferative GN:  Hyperplasia of epithelium & endothelium.  Cell Swelling.  Inflammatory cells.  Obstruction to flow.  Enlarged hypercellular glomeruli. •Normal •Proliferative •Post strepto IF- Diffuse Proliferative GN Complications: Glomerular diseases: Chronic Glomerulonephritis: Urine Microscopy :  Cells Casts Crystals.  Casts are formed within nephron.  Casts Suggest Kidney pathology.  Casts can be made up of Protein, lipid, cells or mixed.  Crystals suggest high concentration or altered solubility. Formation of Casts: Red cell Casts in Urine: The greatest test of courage is to bear defeat without losing heart…! Robert G. Ingersoll Glomerular diseases: Minimal Change/Lipoid : Minimal Change Disease: Loss of Foot processes Membranous GN : Crescentic GN - (RPGN) Crescentic GN - (Trichrome Stain) Goodpasture Syndrome: Membranous GN : Focal Segmental Gl. Sclerosis: Mesangiocapillary GN: (MPGN)
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