GIDEON ROBERT UNIVERSITY Paediatrics: Nephrotic Syndrome Quiz
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Questions and Answers

What is the primary characteristic of Nephrotic Syndrome?

  • Low cholesterol levels
  • High blood pressure
  • Heavy proteinuria (correct)
  • Low white blood cell count
  • Which type of Nephrotic Syndrome has no known cause?

  • Systemic NS
  • Infection-induced NS
  • Primary (Idiopathic) NS (correct)
  • Secondary NS
  • Which of the following is a secondary cause of Nephrotic Syndrome?

  • Goodpasture syndrome
  • Lymphoma (correct)
  • Diabetes mellitus
  • Heavy proteinuria
  • What is the incidence rate of Nephrotic Syndrome in children per year?

    <p>2-3/100,000 children</p> Signup and view all the answers

    Which disease is NOT associated with causing Nephrotic Syndrome?

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

    Which of the following drugs is a known secondary cause of Nephrotic Syndrome?

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

    Which disease is characterized by hematuria, hypertension, edema, and renal insufficiency?

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

    What is the classic example of nephritic syndrome?

    <p>Acute poststreptococcal glomerulonephritis</p> Signup and view all the answers

    What mediates the pathogenesis of glomerulonephritis in nephritic syndrome?

    <p>Immune complexes</p> Signup and view all the answers

    When does renal involvement in nephritic syndrome typically present?

    <p>1-2 weeks after an antecedent streptococcal infection</p> Signup and view all the answers

    What may range from asymptomatic hematuria to acute renal failure in nephritic syndrome?

    <p>Renal involvement</p> Signup and view all the answers

    What causes lumpy-bump deposits on basement membranes in nephritic syndrome?

    <p>Immune complexes</p> Signup and view all the answers

    What defines a patient as steroid-dependent?

    <p>Relapsing within 28 days of stopping prednisone therapy</p> Signup and view all the answers

    What is a potential side effect of cyclophosphamide in nephrotic syndrome treatment?

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

    Which medication may help maintain remission in children with steroid-dependent or frequently relapsing nephrotic syndrome?

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

    What is a potential complication of cyclosporine and tacrolimus therapy in children?

    <p>Gingival hyperplasia</p> Signup and view all the answers

    When should cyclophosphamide be withheld in nephrotic syndrome treatment?

    <p>If WBC falls below 5,000/mm3</p> Signup and view all the answers

    What is the primary characteristic of steroid-resistant patients?

    <p>Failure to respond to initial daily prednisolone therapy</p> Signup and view all the answers

    What is the main reason for the development of edema in patients with the described condition?

    <p>Salt and water retention</p> Signup and view all the answers

    Which symptoms are considered non-specific in patients with the described condition?

    <p>Malaise and lethargy</p> Signup and view all the answers

    What type of cells are commonly found in the urinalysis of patients with the described condition?

    <p>Polymorphonuclear leukocytes</p> Signup and view all the answers

    What is a common presentation in the blood work of patients with the described condition?

    <p>Reduced Serum C3 level</p> Signup and view all the answers

    When should a renal biopsy be considered in patients with the described condition?

    <p>Development of acute renal failure</p> Signup and view all the answers

    Which histopathological type of primary NS is characterized by lesions occurring in some tufts within a glomerulus and sparing of others?

    <p>Focal segmental glomerulosclerosis (FSGS)</p> Signup and view all the answers

    Which histopathological type of primary NS is associated with proximal convoluted tubules laden with protein droplets and lipids?

    <p>Minimal change disease (MCD)</p> Signup and view all the answers

    What is the defining abnormality observed in the glomeruli under electron microscope in cases of FSGS?

    <p>Effacement of foot processes</p> Signup and view all the answers

    At what age range does the onset of Nephrotic Syndrome usually occur?

    <p>Between 2-6 years</p> Signup and view all the answers

    'Anasarca' refers to generalized oedema that includes swelling in which areas?

    <p>Genital area and abdomen</p> Signup and view all the answers

    What is the term for the older name used for Minimal Change Disease?

    <p>'Lipoid nephrosis'</p> Signup and view all the answers

    Study Notes

    Definition and Introduction to Nephrotic Syndrome

    • Nephrotic Syndrome (NS) is characterized by:
      • Heavy proteinuria (> 3.5 g/day in adults or 40 mg/m2/hr in children)
      • Hypoproteinaemia (< 2.5 g/dL)
      • Oedema
      • Hyperlipidaemia
    • NS is 15 times more common in children, with an incidence of 2-3 cases per 100,000 children per year
    • There are two types of NS:
      • Primary (Idiopathic) NS (90%): no known cause
      • Secondary NS (10%): due to either systemic or glomerular causes

    Secondary Causes of Nephrotic Syndrome

    • Infections:
      • Hepatitis B, C
      • HIV-1
      • Malaria (Plasmodium malariae)
      • Syphilis
      • Toxoplasmosis
    • Drugs:
      • Gold
      • Penicillamine
      • NSAIDs
      • Pamidronate
      • Interferon
      • Mercury
      • Heroin
      • Lithium
    • Systemic diseases:
      • Diabetes mellitus
      • Sickle cell disease (SCD)
      • Goodpasture syndrome
      • Amyloidosis
      • Systemic lupus erythematosus (SLE)
      • Henoch-Schönlein purpura (HSP) nephritis
      • Alport syndrome
    • Associated with Malignant Disease:
      • Lymphoma
      • Leukemia
      • Melanoma
    • Miscellaneous:
      • Bee sting allergy
      • Metabolic disorders:
        • α1-Antitrypsin deficiency
        • Fabry disease
        • Glutaric acidemia
        • Glycogen storage disease
      • Congenital syndromes

    Clinical Features and Investigations

    • Non-specific symptoms:
      • Malaise
      • Lethargy
      • Abdominal or flank pain
      • Fever
    • Urinalysis:
      • RBCs with RBC casts and proteinuria
      • Polymorphonuclear leukocytes are common
    • Mild normochromic anemia may be present due to hemodilution and low-grade hemolysis
    • Serum C3 level is usually reduced
    • High ASOT titers
    • Renal biopsy is only done if there is:
      • Development of acute renal failure
      • Absence of evidence for streptococcal infection
      • Absence of hypocomplementemia
      • Persistence of marked hematuria or proteinuria
      • Low C3 level for more than 3 months after onset

    Treatment and Complications

    • Strict input/output and daily weight monitoring
    • Salt restriction and monitor BP
    • Oral penicillin for 10 days
    • Antihypertensive medications:
      • Diuretics
      • Angiotensin-converting enzyme inhibitors
    • Complications:
      • Volume overload
      • Heart failure
      • Hypertension with hypertensive encephalopathy
      • Hyperkalemia
      • Hyperphosphatemia
      • Hypocalcemia

    Nephrotic Vs Nephritic Syndrome

    • Nephrotic Syndrome:
      • Characterized by hematuria, hypertension, edema, and renal insufficiency
      • Classic example: acute poststreptococcal glomerulonephritis

    Steroid-Dependent and Steroid-Resistant Patients

    • Steroid-dependent patients:
      • Relapse while on alternate-day steroid therapy or within 28 days of stopping prednisone therapy
    • Steroid-resistant patients:
      • Failure to respond to initial daily prednisolone therapy
    • Alternative agents for steroid-dependent and steroid-resistant patients:
      • Levamisole
      • Cyclophosphamide
      • Cyclosporine
      • Tacrolimus
      • Mycophenolate

    Histopathological Subtypes of Idiopathic NS

    • Minimal change disease (MCD): 85-95% respond to steroid therapy
    • Focal segmental glomerulosclerosis (FSGS): 10-20% respond to steroid
    • Membranous Nephropathy (MN)
    • Membranoproliferative glomerulonephritis (MPGN): 50% respond to steroid

    Complications of NS

    • Denys-Drash syndrome
    • Nail Patella syndrome

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    Description

    Test your knowledge on Nephrotic Syndrome (NS) in paediatrics with this quiz from GIDEON ROBERT UNIVERSITY. Learn about the definition, characteristics, and incidence of Nephrotic Syndrome in children.

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