Nephrotic Syndrome in Children Quiz
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Questions and Answers

What is the primary cause of nephrotic syndrome in children aged 1-7 years old?

  • Focal Segmental Glomerulosclerosis
  • Minimal-Change Disease (correct)
  • Heroin nephropathy
  • HIV infection
  • Which condition is characterized by an injury to podocytes leading to nephrotic syndrome?

  • Focal Segmental Glomerulosclerosis (correct)
  • Hypertension
  • Minimal-Change Disease
  • Oliguria
  • Which of the following is not a clinical feature related to nephritic syndrome?

  • Fluid retention
  • Low creatinine levels (correct)
  • Oliguria
  • Elevated blood urea nitrogen
  • What is a common feature of Focal Segmental Glomerulosclerosis?

    <p>Involves only parts of affected glomeruli (B)</p> Signup and view all the answers

    What percentage of nephrotic syndrome cases does Focal Segmental Glomerulosclerosis account for?

    <p>20%-30% (C)</p> Signup and view all the answers

    What mechanism primarily causes azotemia in nephritic syndrome?

    <p>Reduced glomerular filtration rate (D)</p> Signup and view all the answers

    Which of the following is true about the renal function in cases of Minimal-Change Disease?

    <p>It is preserved in most cases (D)</p> Signup and view all the answers

    What is the primary treatment method for Minimal-Change Disease?

    <p>Corticosteroids (A)</p> Signup and view all the answers

    What are the main functions of the kidneys?

    <p>Excretion of waste products and regulation of body water (D)</p> Signup and view all the answers

    What are the two main clinical manifestations of glomerular diseases?

    <p>Nephrotic syndrome and nephritic syndrome (C)</p> Signup and view all the answers

    Which component of the glomerulus is responsible for forming a fenestrated endothelium?

    <p>Endothelial cells (D)</p> Signup and view all the answers

    Which of the following is NOT a characteristic of nephrotic syndrome?

    <p>Hematuria (A)</p> Signup and view all the answers

    What causes the increased permeability to plasma proteins seen in nephrotic syndrome?

    <p>Abnormalities in the podocyte structure (A)</p> Signup and view all the answers

    Which of the following indicates the presence of nephritic syndrome?

    <p>Proteinuria with less severity (C)</p> Signup and view all the answers

    What do podocytes in the glomerulus do?

    <p>Wrap around capillaries and create filtration slits (C)</p> Signup and view all the answers

    Which condition is associated with an increase in lipids in the blood?

    <p>Nephrotic syndrome (D)</p> Signup and view all the answers

    What is the most common type of kidney stone, accounting for 80% of cases?

    <p>Calcium oxalate stones (A)</p> Signup and view all the answers

    Which condition increases the risk of uric acid stone formation?

    <p>Gout disease (C)</p> Signup and view all the answers

    What percentage of individuals with urolithiasis may develop stones without any symptoms?

    <p>50% (B)</p> Signup and view all the answers

    What urine pH level is typically associated with the formation of uric acid stones?

    <p>pH &lt; 5.5 (C)</p> Signup and view all the answers

    What factor does NOT contribute to the development of struvite stones?

    <p>Acidic urine (A)</p> Signup and view all the answers

    What is the primary characteristic of Membranous Nephropathy as observed under a light microscope?

    <p>Diffused thickening of the GBM (A)</p> Signup and view all the answers

    Which percentage of Membranous Nephropathy cases are classified as primary?

    <p>80% (D)</p> Signup and view all the answers

    What is the typical initial event associated with hematuria in the context described?

    <p>Upper respiratory tract infection (C)</p> Signup and view all the answers

    What is a notable clinical feature of Poststreptococcal Glomerulonephritis (PSGN)?

    <p>Hematuria with cola-colored urine (C)</p> Signup and view all the answers

    What percentage of cases may progress to end-stage renal disease within 20 years?

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

    What triggers the podocyte injury in Membranous Nephropathy?

    <p>Activation of complement on the surface of podocytes (D)</p> Signup and view all the answers

    Which symptom is commonly associated with acute pyelonephritis?

    <p>Pain at the costovertebral angle (D)</p> Signup and view all the answers

    Which demographic is most commonly affected by IgA Nephropathy?

    <p>Children and young adults (D)</p> Signup and view all the answers

    What is a common characteristic of simple cysts in the kidney?

    <p>Usually confined to the cortex (D)</p> Signup and view all the answers

    What is the typical age by which Polycystic Kidney Disease (PKD) usually produces symptoms?

    <p>40 years (A)</p> Signup and view all the answers

    What is the underlying issue leading to immune complex formation in IgA Nephropathy?

    <p>Abnormally glycosylated IgA1 (C)</p> Signup and view all the answers

    Which of the following clinical features is NOT typically associated with Membranous Nephropathy?

    <p>Acute nephritic syndrome (A)</p> Signup and view all the answers

    What is the most common type of renal stones formed in the urinary system?

    <p>Calcium oxalate stones (C)</p> Signup and view all the answers

    Which of the following is a risk factor for acute pyelonephritis?

    <p>Catheterization (C)</p> Signup and view all the answers

    What is the primary type of immune complex involved in the pathogenesis of PSGN?

    <p>Host antibodies and bacterial antigens (B)</p> Signup and view all the answers

    What is a significant outcome of Polycystic Kidney Disease?

    <p>Renal failure (A)</p> Signup and view all the answers

    Flashcards

    What is a nephron?

    The functional and structural unit of the kidney, responsible for filtering waste products from the blood and producing urine.

    What is a glomerulus?

    A network of capillaries surrounded by two layers of epithelium in the kidney, responsible for filtering blood.

    What are podocytes?

    Cells that form the visceral layer of the glomerular capsule, responsible for filtering blood.

    What is nephrotic syndrome?

    A syndrome characterized by proteinuria, hypoalbuminemia, edema, hyperlipidemia, and lipiduria, indicating a problem with glomerular filtration.

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    What is nephritic syndrome?

    A syndrome characterized by red blood cells in the urine and varying levels of proteinuria, indicating inflammation or damage to the glomeruli.

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    What is glomerulonephritis?

    A condition involving the kidneys that primarily affects the glomeruli, leading to increased permeability to proteins.

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    What is chronic kidney disease?

    A condition in which the glomerular filtration rate (GFR) is significantly reduced, leading to waste buildup and fluid retention.

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    What is kidney failure?

    A condition in which the kidneys fail to function properly, leading to the need for dialysis or a kidney transplant.

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    Azotemia

    An increase in blood urea nitrogen and creatinine levels, indicating impaired kidney function.

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    Oliguria

    A low volume of urine output, often a sign of decreased kidney function.

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    Nephritic Syndrome

    Inflammatory lesions of the glomeruli, causing damage to capillary walls and reduced filtration rate.

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    Primary Glomerular Disease

    A condition where the kidney is the primary organ affected, with no systemic involvement.

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    Secondary Glomerular Disease

    A condition where the glomeruli are damaged as a consequence of other systemic diseases.

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    Minimal-Change Disease (MCD)

    A common cause of nephrotic syndrome, particularly in children, characterized by normal glomerular appearance under a light microscope, but with podocyte foot process effacement under an electron microscope.

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    Focal Segmental Glomerulosclerosis (FSGS)

    A condition characterized by scarring of some glomeruli, involving only a part of each affected glomerulus. It is a common cause of nephrotic syndrome in adults.

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    Primary FSGS

    FSGS that occurs without a known underlying cause.

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    Membranous Nephropathy (MN)

    A kidney disease characterized by subepithelial deposits of immune complexes along the glomerular basement membrane (GBM), causing nephrotic syndrome.

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    Spike and dome pattern

    A pattern seen under an electron microscope in MN, where subepithelial deposits are separated from each other by spike-like protrusions of GBM.

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    Primary Membranous Nephropathy

    The most common form of MN, where the cause is unknown and believed to be due to autoantibodies attacking podocyte antigens.

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    Secondary Membranous Nephropathy

    A form of MN that develops as a result of other conditions such as infections or malignancies.

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    Poststreptococcal Glomerulonephritis (PSGN)

    A type of glomerulonephritis occurring after a strep infection, affecting mainly children, characterized by immune complexes deposited in the GBM.

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    Abnormally glycosylated IgA1

    A protein in the blood that is abnormally glycosylated, playing a central role in the pathogenesis of IgA nephropathy.

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    IgA Nephropathy

    A kidney disease characterized by the deposition of IgA-anti IgA immune complexes in the mesangium.

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    Recurrent hematuria

    A common clinical feature of IgA nephropathy, affecting children and young adults.

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    What is Urolithiasis?

    The formation of stones in the urinary tract, typically in the kidneys.

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    What is the main cause of kidney stones?

    Most kidney stones form when the concentration of certain substances in the urine becomes too high, causing them to precipitate out.

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    What is hypercalciuria?

    A condition characterized by increased levels of calcium in the urine, a common factor in the formation of calcium-based kidney stones.

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    What are Struvite stones?

    A type of kidney stone that forms in individuals with persistently alkaline urine, often caused by urinary tract infections.

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    What are Uric Acid stones?

    A type of kidney stone that forms in individuals with persistently acidic urine, often linked to gout.

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    Acute Pyelonephritis

    Inflammation of the kidney and renal pelvis, usually caused by bacterial infection. It can occur through the lower urinary tract, ascending to the kidneys, or via the bloodstream.

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    Simple Kidney Cysts

    Fluid-filled sacs that grow in the kidneys, common after age 40. Most are harmless, but some can cause symptoms.

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    Adult Polycystic Kidney Disease (PKD)

    An inherited disorder causing multiple cysts in both kidneys, leading to kidney enlargement and eventual loss of function.

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    Renal Stones (Urolithiasis)

    Kidney stones, typically formed in the kidneys. More common in men than women.

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    Calcium Oxalate Kidney Stone

    A type of kidney stone primarily composed of a calcium-containing mineral.

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    Urinary Outflow Obstruction

    Any blockage in the urinary tract that prevents urine from flowing out of the kidneys normally.

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    Kidney Failure

    A condition where the kidneys fail to function properly, often leading to the need for dialysis or a kidney transplant.

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    Study Notes

    Kidney Pathology

    • Kidneys are bean-shaped organs with multiple functions
    • Key functions include: waste excretion (urea, uric acid, bilirubin), water and salt regulation (normal range PH 7.35-7.45), acid balance maintenance, and hormone secretion
    • Nephrons are the structural and functional units of the kidney
    • Kidney diseases are categorized based on their effect on different components: glomeruli, tubules, interstitium, and blood vessels

    Glomerular Diseases

    • Glomeruli are the filtering units of the kidneys
    • The glomerulus has two layers of epithelium, namely visceral and parietal epithelium
    • The GBM, endothelial cells, and podocytes are key components of the glomerulus
    • Two well-defined glomerular disease syndromes are nephrotic and nephritic syndromes

    Nephrotic Syndrome

    • Characterized by proteinuria, hypoalbuminemia, generalized edema, hyperlipidemia, and lipiduria
    • Caused by abnormal capillary walls in glomeruli, leading to increased permeability to plasma proteins
    • Common causes include Minimal change disease, Focal segmental glomerulosclerosis, and systemic diseases like diabetes mellitus

    Nephritic Syndrome

    • Characterized by hematuria, less severe proteinuria than nephrotic syndrome (with or without edema), azotemia, oliguria, and hypertension
    • Caused by inflammatory lesions in glomeruli
    • Inflammation causes injury to capillary walls, allowing red blood cells to pass into the urine and changes in hemodynamics that result in oliguria, azotemia, fluid retention, and hypertension

    Mechanisms of Glomerular Injury and Disease

    • Primary glomerular diseases affect the kidney only or predominantly
    • Secondary glomerular diseases are secondary to systemic diseases, where glomerular injury occurs
    • Most primary and secondary glomerular diseases are immune-mediated

    Minimal-Change Disease (MCD)

    • One of the most common causes of nephrotic syndrome in children (1-7 years old)
    • Light microscopy shows normal glomeruli
    • Electron microscopy reveals diffuse effacement or fusion of podocyte foot processes
    • Pathogenesis is unknown
    • Usually responsive to corticosteroid treatment

    Focal Segmental Glomerulosclerosis (FSGS)

    • Characterized by sclerosis of some glomeruli, affecting only parts of each glomerulus
    • A common cause of nephrotic syndrome, especially in adults
    • Pathogenesis is due to podocyte injury, but the exact cause is unknown
    • Poorly responsive to corticosteroid treatment, and end-stage renal disease in 50% of cases within 10 years

    Membranous Nephropathy (MN)

    • Also known as membranous glomerulopathy
    • Causes nephrotic syndrome
    • Characterized by subepithelial deposits of immune complexes along the GBM
    • Morphology shows diffuse thickening of the GBM, podocyte foot process effacement, and subepithelial deposits separated by spike-like projections (spike and dome pattern)
    • Usually affects adults (30–60 years) and not responsive to corticosteroid treatment

    Poststreptococcal Glomerulonephritis (PSGN)

    • Develops in children (or rarely in adults) 2–4 weeks after recovery from infection with certain group A streptococci strains
    • An immune complex disease where immune complexes deposit in the glomerular basement membrane (GBM)
    • Deposition of immune complexes causes acute injury to the glomeruli; leading to an inflammatory reaction
    • Symptoms of acute nephritic syndrome include edema, hypertension, mild to moderate azotemia, hematuria (cola-colored urine), and potential chronic renal disease

    IgA Nephropathy

    • One of the most common causes of recurrent gross or microscopic hematuria
    • Caused by IgA immune complexes in the mesangium
    • Pathogenesis involves abnormally glycosylated IgA1 eliciting an immune response and forming complexes that deposit in the mesangium inducing inflammation, injuring the glomerulus and leading to the release of red blood cells into the urine
    • Symptoms usually start with an episode of hematuria that often recurs after a respiratory infection

    Acute Pyelonephritis

    • A common suppurative inflammation of the kidney and renal pelvis caused primarily by bacterial infections (typically enteric gram-negative bacilli)
    • Bacteria reach kidneys through the urinary tract (ascending infection) or bloodstream (hematogenous infection)
    • Symptoms include pain in the costovertebral angle, chills, fever, nausea, malaise, dysuria, frequency, urgency, and turbid urine

    Cystic Diseases of the Kidney

    • Simple cysts are fluid-filled sacs, commonly found in the kidney cortex, usually asymptomatic, occurring in 25% of adults over 40
    • Adults Polycystic Kidneys Disease (PKD) is characterized by multiple expanding cysts in both kidneys, progressive enlargement, and loss of kidney function; often asymptomatic until the fourth decade; common cause of chronic kidney disease.

    Urinary Outflow Obstruction

    • Includes various conditions that block urine flow
    • Renal stones (Urolithiasis) can be caused by multiple factors (supersaturation of urine constituents, predisposing conditions). These stone types vary including calcium oxalate, struvite, uric acid, and cysteine. Symptoms include severe pain (renal colic) and hematuria

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    Pathology of the Kidney PDF

    Description

    Test your knowledge on nephrotic syndrome, particularly focusing on its causes, clinical features, and treatment in children aged 1-7 years. This quiz covers essential topics like Minimal-Change Disease and Focal Segmental Glomerulosclerosis. Perfect for students and healthcare professionals looking to deepen their understanding of pediatric nephrology.

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