Glomerular Disease Overview
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Glomerular Disease Overview

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Questions and Answers

What is the primary function of the glomerular basement membrane (GBM)?

  • Produce podocytes
  • Act as a filtration barrier for large molecules (correct)
  • Regulate blood flow in the glomerulus
  • Support mesangial cells

    • Glomerulopathy is specifically defined as the inflammation of the glomerulus.

    False

    Which type of nephropathy is characterized by a fusion of podocytes?

    Minimal change disease

    _________ is the condition where there is an increase in type IV collagen synthesis due to diabetes mellitus.

    <p>Membranous nephropathy</p> Signup and view all the answers

    Match the type of glomerular disease with its corresponding characteristic:

    <p>Focal = &lt; 50% of abnormal glomeruli Diffuse = &gt; 50% of abnormal glomeruli Segmental = Only a segment affected Global = Whole glomerulus affected</p> Signup and view all the answers

    Which cells provide support to glomerular capillaries?

    <p>Mesangial cells</p> Signup and view all the answers

    Crescentic glomerulonephritis is characterized by a global effect on the glomerulus.

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

    What is the role of immunofluorescence in renal biopsy studies?

    <p>Identify protein deposition</p> Signup and view all the answers

    The main cells responsible for producing the glomerular basement membrane are the __________.

    <p>Visceral epithelial cells</p> Signup and view all the answers

    In IgA nephropathy, what leads to mesangial expansion?

    <p>Inflammatory mediator release</p> Signup and view all the answers

    What is the most common cause of nephrotoxic acute kidney injury (AKI)?

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

    Ischemic acute kidney injury can be caused by hypovolaemia due to shock?

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

    What is a reversible lesion that indicates acute tubular necrosis (ATN)?

    <p>necrosis with intact tubular basement membrane</p> Signup and view all the answers

    Acute tubular necrosis leads to ______ of tubular epithelial cells.

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

    Match the following conditions with their corresponding characteristics:

    <p>Acute Tubular Necrosis = Reversible lesion with intact basement membrane Ischemic AKI = Caused by hypovolaemia and shock Nephrotoxic AKI = Resulting from drugs like aminoglycosides Obstructive Nephropathy = Includes renal stone (urolithiasis)</p> Signup and view all the answers

    Which of the following is NOT a cause of obstructive nephropathy?

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

    Acute flank pain is a symptom of obstructive nephropathy.

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

    Oxidative injury from paracetamol is caused by a toxic ______.

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

    What does the recovery from acute tubular necrosis depend on?

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

    Which type of nephropathy is typically asymptomatic and may result from renal stones?

    <p>Obstructive nephropathy</p> Signup and view all the answers

    What is a primary feature of acute tubular injury?

    <p>Desquamation of epithelial cells</p> Signup and view all the answers

    Impaired renal function is a defining characteristic of chronic kidney disease.

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

    What type of rejection involves the immune response against the transplanted kidney?

    <p>Transplant rejection</p> Signup and view all the answers

    Which gene is associated with Adult Polycystic Kidney Disease (ADPKD)?

    <p>PKD1</p> Signup and view all the answers

    Hypertensive nephrosclerosis leads to ________ arteriolosclerosis of renal arterioles.

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

    Match the following conditions with their causes:

    <p>Atherosclerosis = Large arterial wall thickening Hypertensive nephrosclerosis = Chronic hypertension Acute Tubular Injury = Injury to renal tubules Thrombotic thrombocytopenic purpura = Severe hemolytic symptoms</p> Signup and view all the answers

    Cystic renal dysplasia is characterized by functional abnormalities of the kidneys.

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

    What is the consequence of severe hypertension on the kidneys?

    <p>Tubular atrophy and glomerular sclerosis</p> Signup and view all the answers

    What type of genetic inheritance is associated with Juvenile Polycystic Kidney Disease (ARPKD)?

    <p>Autosomal recessive</p> Signup and view all the answers

    Cellular and humoral immunity play no role in transplant rejection.

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

    Adult Polycystic Kidney Disease involves mutations in the ______ gene leading to ciliopathy.

    <p>PKD1</p> Signup and view all the answers

    What is indicated by the presence of swollen kidneys and inflammation?

    <p>Impaired renal function</p> Signup and view all the answers

    Match the following conditions with their characteristics:

    <p>Cystic renal dysplasia = Abnormal kidney development with non-functionality Adult polycystic kidney disease = AD mutation in PKD1 gene Juvenile polycystic kidney disease = AR mutation in PKHD1 gene Acquired cystic disease = 50% occurrence in long-term dialysis patients</p> Signup and view all the answers

    Which type of toxin is responsible for thrombotic microangiopathy related to E. Coli?

    <p>Shiga toxin</p> Signup and view all the answers

    Which complication is increased in patients with acquired cystic disease?

    <p>Renal cell carcinoma (RCC)</p> Signup and view all the answers

    Tissue inflammation and infiltration of the interstitium are features of ________ rejection.

    <p>acute cellular</p> Signup and view all the answers

    Polycystin is a protein involved in the regulation of ion secretion in kidneys.

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

    ANCA vasculitis is characterized by neutrophil infiltration of vessel walls due to autoantibodies.

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

    Match the following types of rejection with their mechanisms:

    <p>Cell-mediated rejection = T-cell involvement Antibody-mediated rejection = Antibody production against donor tissue Acute rejection = Immediate immune response Chronic rejection = Progressive fibrosis</p> Signup and view all the answers

    Which of the following conditions is a complication of chronic hypertension?

    <p>Hypertensive nephrosclerosis</p> Signup and view all the answers

    In Juvenile Polycystic Kidney Disease, the gene responsible for the condition is ______.

    <p>PKHD1</p> Signup and view all the answers

    What is the primary consequence of microthrombi obstructing blood flow?

    <p>Ischemia in various organs</p> Signup and view all the answers

    Simple cysts in the kidneys generally arise from defective fluid-filled sacs due to obstruction in the tubular _____ matrix.

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

    Reversible lesions can occur as a result of high-dose immunosuppressants during kidney transplantation.

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

    What is indicated by the term 'atypical cellular features' in renal pathology?

    <p>Regenerative atypia</p> Signup and view all the answers

    Which of the following conditions is primarily associated with Thrombotic Thrombocytopenic Purpura (TTP)?

    <p>Malignant hypertension</p> Signup and view all the answers

    In acute renal failure, elevated levels of ________ reflect impaired kidney function.

    <p>BUN and creatinine</p> Signup and view all the answers

    Match the following renal diseases with their corresponding characteristics:

    <p>Simple cyst = Benign, fluid-filled sacs due to intratubular obstruction HUS = Hemolytic uremic syndrome associated with Shiga toxin ANCA vasculitis = Autoimmune disease causing vessel wall inflammation TTP = Thrombotic thrombocytopenic purpura leading to platelet fragmentation</p> Signup and view all the answers

    What kind of kidney injury is regarded as prerenal?

    <p>Hemodynamic changes</p> Signup and view all the answers

    Cystic diseases typically result in malignant progression in renal tissues.

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

    What form of necrosis is associated with the renal cortex in ANCA vasculitis?

    <p>Fibrinoid necrosis</p> Signup and view all the answers

    Study Notes

    Glomerular Disease

    • Glomerular disease is a broader term than glomerulonephritis (GN) because inflammation must be present in GN.
    • Glomerular filtration barrier is made up of:
      • Endothelial cells with fenestrations (60-80nm)
      • Glomerular basement membrane (GBM) with type IV collagen and heparan sulfate.
        • GBM is negatively charged and acts as the main filtration barrier to filter large molecules of negative charge, e.g., albumin.
      • Visceral epithelial cells (podocytes)
      • Mesangial cells
      • Parietal epithelial cells
    • Glomerular disease terminology:
      • The percentage of glomeruli affected:
        • Focal: <50%
        • Diffuse: >50%
      • The part of the glomerulus affected:
        • Segmental: only a segment is affected
        • Global: the whole glomerulus is affected
      • Cell details:
        • Hypercellularity
        • Crescent formation
        • Tubular changes
    • Studies on renal biopsy specimens:
      • Technique:
        • Light microscopy (H&E, PAS, PASM) - classifies the type of glomerular disease
        • Immunofluorescence - identifies protein deposition (e.g., anti-IgA Ab)

    Acute Tubular Necrosis (ATN)

    • Causes of ATN:
      • Ischemic - hypovolemia caused by shock, MI, etc.
      • Nephrotoxic - aminoglycosides (most common)
        • Paracetamol causes oxidative injury through a toxic metabolite.
    • Pathology:
      • Death of tubular epithelial cells leading to cast formation.
      • Tubular basement membrane remains intact.
      • Proximal tubules are mainly affected due to oxidative stress and accumulation.
    • Recovery depends on the severity.

    Obstructive Nephropathy

    • Causes:
      • Intraluminal obstruction - renal stone (urolithiasis)
      • Mural changes - in the wall of the urinary track (e.g., bladder stone)
    • Pathology:
      • Acute pain caused by acute obstruction.
      • Impaired renal function.
      • Dilated ureter.
      • Cellular changes in tubules (e.g., cell size increase)
      • Acute tubular injury

    Immunologic Transplant Rejection (Kidney Transplantation)

    • Types:
      • Cellular-mediated rejection
      • Antibody-mediated rejection (humoral)
    • Pathology:
      • Swollen kidney with inflammation and edema.
      • Immunosuppressant treatment.
      • Interstitial infiltrate (inflammation of the interstitium)
      • Tubulitis (inflammation of renal tubules)
    • Lesions:
      • Reversible with acute cellular rejection.
      • Chronic insult (progressive fibrosis) with chronic rejection.

    Vascular Diseases:

    • Atherosclerosis:
      • Large arterial wall thickening and loss of elasticity.
    • Hypertensive nephrosclerosis:
      • Cause: benign hypertension
      • Pathology:
        • Hyaline arteriolosclerosis of renal arterioles - protein and lipid deposition.
        • Tubular atrophy, glomerular sclerosis (focal segmental)
        • Small kidneys with a finely granular surface.
    • Thrombotic Microangiopathy:
      • Caused by malignant hypertension, HUS/TTP, etc.
      • Pathology:
        • Microthrombi obstruct blood flow, causing ischemia in various organs.
        • Endothelial cell degeneration.
        • Thrombosis
    • ANCA Vasculitis:
      • Autoimmune disease
      • Pathology:
        • ANCAs (anti-neutrophil cytoplasmic antibodies) stimulate neutrophil infiltration of the vessel wall.
        • Endothelial cell degeneration leading to cortical infarcts.
        • Fibrinoid necrosis.
        • Inflammatory cell infiltration.
        • Leakage of serum protein and coagulation factors.
        • Deposition of fibrin-like protein in small vessels.

    Cystic Diseases:

    • Simple cyst:
      • Most common adult renal cyst; generally benign.
      • Pathogenesis:
        • Intratubular obstruction.
        • Defect in tubular BM matrix causing altered cell growth.
        • Defective fluid secretion resulting in fluid-filled sacs.### Cystic Renal Dysplasia
    • Abnormality in fetal kidney development, characterized by abnormal structures like cartilage.
    • The kidneys are non-functional and often associated with urinary tract obstruction.
    • This condition can be caused by mutations in the genes responsible for cilia, sensory organelles that regulate cell function.

    Adult Polycystic Kidney Disease (ADPKD)

    • An autosomal dominant condition caused by mutation in the PKD1 gene on chromosome 16.
    • The mutation affects polycystin, a protein involved in cell signaling pathways.
    • This leads to abnormal cell proliferation and imbalances in ion secretion.
    • Cysts can form in various organs, including the kidneys, liver, and pancreas.

    Juvenile Polycystic Kidney Disease (ARPKD)

    • An autosomal recessive disease caused by mutations in the PKHD1 gene on chromosome 6.
    • The mutation affects fibrocystin, a protein that regulates the function of polycystin-2.
    • This can result in stillbirth or rapid progression to end-stage renal disease.

    Dialysis-Acquired Cystic Disease (DACD)

    • Occurs in approximately 50% of individuals on long-term dialysis.
    • Tubular obstruction develops due to interstitial fibrosis, oxalate crystal deposits, and other factors.
    • Increases the risk of renal cell carcinoma (RCC).

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    Description

    Explore the essential aspects of glomerular disease, including its pathology and classification. Learn about the components of the glomerular filtration barrier and the significance of renal biopsy studies. This quiz will enhance your understanding of the terminology and conditions associated with glomerular diseases.

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