Podcast
Questions and Answers
What is the characteristic histological feature of Focal Segmental Glomerulosclerosis?
What is the characteristic histological feature of Focal Segmental Glomerulosclerosis?
What is the term used to describe the involvement of only segments of each affected glomerulus?
What is the term used to describe the involvement of only segments of each affected glomerulus?
What is often seen in affected glomeruli using immunofluorescence microscopy?
What is often seen in affected glomeruli using immunofluorescence microscopy?
What is deposited in the affected glomeruli?
What is deposited in the affected glomeruli?
Signup and view all the answers
What is the term used to describe the combination of focal and segmental involvement?
What is the term used to describe the combination of focal and segmental involvement?
Signup and view all the answers
What is expressed by podocytes in Focal Segmental Glomerulosclerosis?
What is expressed by podocytes in Focal Segmental Glomerulosclerosis?
Signup and view all the answers
What is the consequence of cell injury or apoptosis in the context of the complement system?
What is the consequence of cell injury or apoptosis in the context of the complement system?
Signup and view all the answers
What is the suspected role of antibodies against glomerular cell antigens in certain disorders?
What is the suspected role of antibodies against glomerular cell antigens in certain disorders?
Signup and view all the answers
What is the primary mechanism of tissue injury in poststreptococcal GN?
What is the primary mechanism of tissue injury in poststreptococcal GN?
Signup and view all the answers
What is the characteristic feature of membranous nephropathy?
What is the characteristic feature of membranous nephropathy?
Signup and view all the answers
What is the consequence of a 30-50% reduction in GFR due to renal disease?
What is the consequence of a 30-50% reduction in GFR due to renal disease?
Signup and view all the answers
What is the underlying cause of dense deposit disease?
What is the underlying cause of dense deposit disease?
Signup and view all the answers
What is the characteristic of kidneys in end-stage kidney disease?
What is the characteristic of kidneys in end-stage kidney disease?
Signup and view all the answers
What is the prognosis of dense deposit disease?
What is the prognosis of dense deposit disease?
Signup and view all the answers
What is the role of adaptive changes in the remaining glomeruli in progressive sclerosis?
What is the role of adaptive changes in the remaining glomeruli in progressive sclerosis?
Signup and view all the answers
What is the consequence of hypertrophy in remaining glomeruli?
What is the consequence of hypertrophy in remaining glomeruli?
Signup and view all the answers
What is the target of the autoimmune response in membranous nephropathy?
What is the target of the autoimmune response in membranous nephropathy?
Signup and view all the answers
What is the role of streptococcal exotoxin B in poststreptococcal GN?
What is the role of streptococcal exotoxin B in poststreptococcal GN?
Signup and view all the answers
What is the role of sensitized T cells in experimental glomerulonephritis?
What is the role of sensitized T cells in experimental glomerulonephritis?
Signup and view all the answers
What is the characteristic feature of MPGN?
What is the characteristic feature of MPGN?
Signup and view all the answers
What is the final consequence of the alterations that occur in the remaining glomeruli?
What is the final consequence of the alterations that occur in the remaining glomeruli?
Signup and view all the answers
What is the difference between MPGN and dense deposit disease?
What is the difference between MPGN and dense deposit disease?
Signup and view all the answers
What is the typical age range for the onset of hereditary nephritis?
What is the typical age range for the onset of hereditary nephritis?
Signup and view all the answers
What is the most common reason for gross hematuria in patients with IgA nephropathy?
What is the most common reason for gross hematuria in patients with IgA nephropathy?
Signup and view all the answers
What is the estimated proportion of patients with IgA nephropathy who develop chronic renal failure?
What is the estimated proportion of patients with IgA nephropathy who develop chronic renal failure?
Signup and view all the answers
What is the typical duration of hematuria in patients with IgA nephropathy?
What is the typical duration of hematuria in patients with IgA nephropathy?
Signup and view all the answers
What is the characteristic feature of the nephritic syndrome?
What is the characteristic feature of the nephritic syndrome?
Signup and view all the answers
What is the usual outcome for female carriers of X-linked Alport syndrome?
What is the usual outcome for female carriers of X-linked Alport syndrome?
Signup and view all the answers
What is the histological feature that may indicate a worse prognosis in IgA nephropathy?
What is the histological feature that may indicate a worse prognosis in IgA nephropathy?
Signup and view all the answers
What is the genetic defect associated with hereditary nephritis?
What is the genetic defect associated with hereditary nephritis?
Signup and view all the answers
What is the characteristic appearance of glomeruli in advanced cases of membranous nephropathy?
What is the characteristic appearance of glomeruli in advanced cases of membranous nephropathy?
Signup and view all the answers
What is the primary cause of membranous nephropathy in approximately 85% of cases?
What is the primary cause of membranous nephropathy in approximately 85% of cases?
Signup and view all the answers
What is the characteristic finding on immunofluorescence microscopy in membranous nephropathy?
What is the characteristic finding on immunofluorescence microscopy in membranous nephropathy?
Signup and view all the answers
What percentage of cases of idiopathic nephrotic syndrome in children and adults is accounted for by membranoproliferative glomerulonephritis?
What percentage of cases of idiopathic nephrotic syndrome in children and adults is accounted for by membranoproliferative glomerulonephritis?
Signup and view all the answers
What is the characteristic histological feature of membranoproliferative glomerulonephritis?
What is the characteristic histological feature of membranoproliferative glomerulonephritis?
Signup and view all the answers
What is the primary location of immunoglobulins and complement deposits in membranous nephropathy?
What is the primary location of immunoglobulins and complement deposits in membranous nephropathy?
Signup and view all the answers
What is the eventual outcome of the glomeruli in advanced cases of membranous nephropathy?
What is the eventual outcome of the glomeruli in advanced cases of membranous nephropathy?
Signup and view all the answers
What is the characteristic feature of membranous nephropathy on light microscopy?
What is the characteristic feature of membranous nephropathy on light microscopy?
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.
Studying That Suits You
Use AI to generate personalized quizzes and flashcards to suit your learning preferences.
Related Documents
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.