Nephrotic syndrome

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  • 1. By: Hamza AlGhamdi
  • 2. NORMAL KIDNEY’S PHYSIOLOGY AND HISTOLOGY
  • 3. Capillary Space Endothelium Urinary Space GBM Podocyte
  • 4. NORMAL KIDNEY HISTOLOGY
  • 5. Parietal Epithelium Visceral Epithelium Capillary Lumen Mesangial Cell Basement Membrane (GBM) Urinary Space RBC Endothelial Cell
  • 6. NEPHROTIC SYNDROME IS NOT A DISEASE
  • 7.
    • Fluid retention -> abdominal distention , ascites , edema , puffy eyelids ,scrotal swelling , weight gain , shortness of breath
    • Anorexia
    • Hypertension
    • Oliguria
    • Orthostatic hypertension
    • Skin striae
    • Foamy urine
  • 8. Periorbital edema Pitting edema of lower limbs
  • 9. INVESTIGATIONS
    • Urine sample shows proteinuria (>3.5/day )
    • It is also examined for urinary casts
    • Comprehensive metabolic panel (CMP) shows Hypoalbuminemia
    • High levels of cholesterol (hypercholesterolemia), specifically elevated LDL
    • Electrolytes, urea and creatinine (EUCs): to evaluate renal function
    • Biopsy of kidney
    • Auto-immune markers
  • 10.
    • Excessive permeability of plasma proteins - >> heavy proteinuria
    • Depletion of plasma proteins , mainly albumin - hypoalbuminaemia
  • 11.
    • Liver compensates but not successful - >> reversed A:G ratio
    • Reduced albumin -> decreased colloid oncotic pressure of the blood -> oedema
  • 12.
    • ADH is stimulated - >> oedema
    • Increased Lipoprotein synthesis and decreased catabolism by liver ->> Hyperlipidaemia (mainly VLDL and /or LDL)
    • HDL is also lost in urine when heavy proteinuria occurs
  • 13.
    • Loss of body proteins (immunoglobulins /complement) -> frequent infection
    • Loss of anticoagulants antithrombin III , antiplasmin - > thrombotic and embolic phenomenon
  • 14.  
  • 15.  
  • 16.
    • Major cause of NS in adults
    • Characterised by presence of electron-dense immune deposits along the epithelial side of GBM (subepithelial )
    • Idiopathic in 85% of patients
    • The 15% remaining GN is associated with malignant tumors , SLE , drugs , infections or metabolic disorders
  • 17.  
  • 18. Membranous GN Sub-epithelial immune Complex deposits Thickened GBM
  • 19. Membranous GN H&E
  • 20. Membranous GN IF
  • 21. Capillary Lumen GBM GBM Membranous GN EM
  • 22. MINIMAL CHANGE DISEASE
    • the major cause of nephritic syndrome in Children
    • normal glomeruli on light Microscopy
    • uniform and diffuse effacement of the foot Processes of visceral epithelial cells on electron microscopy
    • Immunofluorescence shows no immune deposits
    • The cause and pathogenesis are unknown
    • dramatic response to corticosteroid therapy.
    • long-term prognosis is excellent.
  • 23.  
  • 24. MINIMAL CHANGE DISEASE Foot Process Fusion
  • 25.  
  • 26.  
  • 27.
    • sclerosis of some, but not all, glomeruli (thus, it is focal)
    • FSG can be.
    • * Idiopathic.
    • * A secondary evernt, refecting glomerular scarring, consequent to another primary glomerular disease (e.g., IgA nephropathy).
    • * associated to other known disorders (e.g. heroin abuse, HIV infection, obesity).
    • * The result of inherited mutations of proteins present in podocytes (podocin, a –actinin) or in the slit diaphragm between podocytes (nephrin).
  • 28.
    • 1- Light Microscopy:
    • Normal and diseased glomeruli (Focal)
    • Segmental tuft sclerosis in diseased glomeruli
    • 2- Immunofluorescence
    • No deposits
    • Deposits of IgM and C3
    • 3- EM:
    • Focal fusion of foot processes
    • Glomerular fibrosis
  • 29. FSGS H&E
  • 30. MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS
    • Thickness of capillary loops and glomerular cell proliferation
    • Glomeruli have a lobular appearance because of mesangial proliferation
    • Capillary walls often have a double-counter or tram-track appearance
  • 31. MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS
    • Type 1 has subendothelial electron-dense deposits of immunoglobulins and complements
    • It occurs in patients with SLE , hepatitis B and other diseases
    • Type 2 has electron-dense GBM deposition in a confluent ribbon-like fashion
    • Type 2 is due to activation of alternative pathway of complement activation
  • 32. Figure 8. Pathology of membranoproliferative glomerulonephritis type I. (a) Light microscopy shows a hypercellular glomerulus with accentuated lobular architecture and a small cellular crescent (methenamine silver).
  • 33. IGA NEPHROPATHY (BERGER DISEASE)
    • the most common type of glomerulonephritis worldwide
    • major cause of recurrent glomerular hematuria
    • By light microscope, the glomeruli can appear nearly normal, showing only subtle mesangial hyprcellularity, or can reveal focal proliferative or sclerotic lesions
    • The pathogenesis is nuclear
    • although a genetic or acquired defect in immune regulation leading to increased mucosal IgA secretion. There isalso decreased clearance of IgA complexes by the liver.
  • 34.
    • Similar IgA deposits are seen in Henoch-Schönlein purpura in children
    • hematuria typically lasts for several days
    • chronic renal failure develops in 50% over a period of 20 years
  • 35. THANK YOU Powerpoint presentation