Nephron Loss and Glomerular Injury
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Questions and Answers

What is the characteristic histological feature of Focal Segmental Glomerulosclerosis?

  • Sclerosis affecting only the cortical glomeruli
  • Sclerosis affecting some but not all glomeruli (correct)
  • Sclerosis affecting only the juxtamedullary glomeruli
  • Sclerosis affecting all glomeruli
  • What is the term used to describe the involvement of only segments of each affected glomerulus?

  • Focal
  • Diffuse
  • Global
  • Segmental (correct)
  • What is often seen in affected glomeruli using immunofluorescence microscopy?

  • Trapping of C3 only
  • Nonspecific trapping of immunoglobulins, usually IgM, and complement (correct)
  • Absence of immunoglobulins
  • Specific trapping of IgG
  • What is deposited in the affected glomeruli?

    <p>Hyaline masses and lipid droplets</p> Signup and view all the answers

    What is the term used to describe the combination of focal and segmental involvement?

    <p>Focal and segmental</p> Signup and view all the answers

    What is expressed by podocytes in Focal Segmental Glomerulosclerosis?

    <p>Complement</p> Signup and view all the answers

    What is the consequence of cell injury or apoptosis in the context of the complement system?

    <p>Activation of the alternative and mannose-binding lectin pathways</p> Signup and view all the answers

    What is the suspected role of antibodies against glomerular cell antigens in certain disorders?

    <p>To directly damage glomerular cells or slit diaphragms</p> Signup and view all the answers

    What is the primary mechanism of tissue injury in poststreptococcal GN?

    <p>Activation of the classical pathway</p> Signup and view all the answers

    What is the characteristic feature of membranous nephropathy?

    <p>Granular subepithelial deposits</p> Signup and view all the answers

    What is the consequence of a 30-50% reduction in GFR due to renal disease?

    <p>Progression to end-stage kidney disease</p> Signup and view all the answers

    What is the underlying cause of dense deposit disease?

    <p>Complement dysregulation</p> Signup and view all the answers

    What is the characteristic of kidneys in end-stage kidney disease?

    <p>Widespread glomerulosclerosis</p> Signup and view all the answers

    What is the prognosis of dense deposit disease?

    <p>Often progresses to end-stage renal disease</p> Signup and view all the answers

    What is the role of adaptive changes in the remaining glomeruli in progressive sclerosis?

    <p>To initiate and perpetuate progressive sclerosis</p> Signup and view all the answers

    What is the consequence of hypertrophy in remaining glomeruli?

    <p>Increased glomerular permeability</p> Signup and view all the answers

    What is the target of the autoimmune response in membranous nephropathy?

    <p>Phospholipase A2 receptor</p> Signup and view all the answers

    What is the role of streptococcal exotoxin B in poststreptococcal GN?

    <p>Activates the alternative complement pathway</p> Signup and view all the answers

    What is the role of sensitized T cells in experimental glomerulonephritis?

    <p>To cause experimental glomerular injury</p> Signup and view all the answers

    What is the characteristic feature of MPGN?

    <p>Granular subendothelial deposits</p> Signup and view all the answers

    What is the final consequence of the alterations that occur in the remaining glomeruli?

    <p>Further endothelial and podocyte injury</p> Signup and view all the answers

    What is the difference between MPGN and dense deposit disease?

    <p>MPGN is caused by immune complex deposition, while dense deposit disease is caused by complement dysregulation</p> Signup and view all the answers

    What is the typical age range for the onset of hereditary nephritis?

    <p>5-20 years</p> Signup and view all the answers

    What is the most common reason for gross hematuria in patients with IgA nephropathy?

    <p>Respiratory tract infection</p> Signup and view all the answers

    What is the estimated proportion of patients with IgA nephropathy who develop chronic renal failure?

    <p>25-50%</p> Signup and view all the answers

    What is the typical duration of hematuria in patients with IgA nephropathy?

    <p>Several days</p> Signup and view all the answers

    What is the characteristic feature of the nephritic syndrome?

    <p>Hematuria with proteinuria and oliguria</p> Signup and view all the answers

    What is the usual outcome for female carriers of X-linked Alport syndrome?

    <p>Benign clinical course with persistent hematuria</p> Signup and view all the answers

    What is the histological feature that may indicate a worse prognosis in IgA nephropathy?

    <p>All of the above</p> Signup and view all the answers

    What is the genetic defect associated with hereditary nephritis?

    <p>Defects in the genes encoding α3 or α4 type IV collagen</p> Signup and view all the answers

    What is the characteristic appearance of glomeruli in advanced cases of membranous nephropathy?

    <p>Diffuse thickening of the capillary wall</p> Signup and view all the answers

    What is the primary cause of membranous nephropathy in approximately 85% of cases?

    <p>Autoantibodies that cross-react with antigens</p> Signup and view all the answers

    What is the characteristic finding on immunofluorescence microscopy in membranous nephropathy?

    <p>Granular deposits of immunoglobulins and complement</p> Signup and view all the answers

    What percentage of cases of idiopathic nephrotic syndrome in children and adults is accounted for by membranoproliferative glomerulonephritis?

    <p>5% to 10%</p> Signup and view all the answers

    What is the characteristic histological feature of membranoproliferative glomerulonephritis?

    <p>Proliferation of glomerular cells</p> Signup and view all the answers

    What is the primary location of immunoglobulins and complement deposits in membranous nephropathy?

    <p>Glomerular basement membrane</p> Signup and view all the answers

    What is the eventual outcome of the glomeruli in advanced cases of membranous nephropathy?

    <p>Sclerosis</p> Signup and view all the answers

    What is the characteristic feature of membranous nephropathy on light microscopy?

    <p>Normal appearance</p> Signup and view all the answers

    Study Notes

    Nephron Loss and Glomerular Injury

    • Renal disease, glomerular or otherwise, can destroy sufficient nephrons to reduce the GFR to 30% to 50% of normal, leading to end-stage kidney disease.
    • Antibodies against glomerular cell antigens can directly damage glomerular cells or slit diaphragms, potentially contributing to certain disorders.
    • Monocytes and macrophages can infiltrate the glomerulus and release diverse mediators, causing glomerular damage.
    • Sensitized T cells can cause experimental glomerular injury.

    Focal Segmental Glomerulosclerosis

    • Focal segmental glomerulosclerosis (FSGS) is characterized by sclerosis affecting some but not all glomeruli (focal involvement) and involving only segments of each affected glomerulus (segmental involvement).
    • FSGS often presents with proteinuria, and the kidneys show widespread glomerulosclerosis.
    • Progression to end-stage kidney disease is driven by adaptive changes in the remaining glomeruli, leading to hypertrophy, hemodynamic changes, and increased glomerular permeability to proteins.

    Membranous Nephropathy

    • Membranous nephropathy is caused by an autoimmune response, often directed against the phospholipase A2 receptor on podocytes.
    • The disease is characterized by granular subepithelial deposits of antibodies with GBM thickening and loss of foot processes, but little or no inflammation.
    • Membranous nephropathy is often resistant to steroid therapy.

    Membranoproliferative Glomerulonephritis and Dense Deposit Disease

    • Membranoproliferative GN (MPGN) is characterized by alterations in the GBM and mesangium, and proliferation of glomerular cells.
    • Dense deposit disease carries a poor prognosis and tends to recur more frequently in renal transplants.
    • MPGN is caused by immune complex deposition, whereas dense deposit disease is a consequence of complement dysregulation.

    Pathogenesis

    • Poststreptococcal GN is an immune complex disease, primarily caused by complement activation by the classical pathway.
    • The relevant antigens are streptococcal proteins, including streptococcal exotoxin B (Spe B) and streptococcal GAPDH.

    Clinical Course

    • IgA nephropathy often affects children and young adults, with more than half presenting with gross hematuria after an infection of the respiratory or urinary tract.
    • The subsequent course is highly variable, with many patients maintaining normal renal function for decades, while slow progression to chronic renal failure occurs in 25% to 50% of cases over a period of 20 years.

    The Nephritic Syndrome

    • The nephritic syndrome is characterized by hematuria, oliguria with azotemia, proteinuria, and hypertension.
    • The disease is often caused by immune complex deposition, complement activation, and inflammation.

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    Description

    This quiz covers the mechanisms of nephron loss and glomerular injury, including the role of renal disease, antibodies, and immune cells in causing glomerular damage and end-stage kidney disease.

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