Nephrotic Syndrome: Relapsing and Steroid-Dependent Disease
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Questions and Answers

What is the definition of Steroid-Dependent Nephrotic Syndrome (SDNS)?

  • SSNS with 2 or more consecutive relapses during tapering or within 14 days of stopping steroids (correct)
  • NS return immediately after limiting or stopping steroid
  • SSNS with 2 or more relapses within 6 months or three times or more within 1 year period
  • FRNS with 2 or more relapses within 3 months
  • What is the dosage of prednisone prescribed for FRNS and SDNS?

  • 1.5 mg/kg/d
  • 3 mg/kg/d
  • 2 mg/kg/d (correct)
  • 4 mg/kg/d
  • What is the recommended treatment for FSGS and SRNS?

  • Prednisone
  • Cyclosporin A (correct)
  • Alkylating agents
  • Cyclophosphamide
  • What is the duration of Cyclophosphamide (CYP) treatment?

    <p>8-12 weeks</p> Signup and view all the answers

    What is the indication for performing a diagnostic renal biopsy?

    <p>After 8 weeks of steroid therapy</p> Signup and view all the answers

    What is the definition of Frequently Relapsing Nephrotic Syndrome (FRNS)?

    <p>SSNS with 2 or more relapses within 6 months or three times or more within 1 year period</p> Signup and view all the answers

    What is the reduced dosage of prednisone after remission of proteinuria?

    <p>1.5 mg/kg</p> Signup and view all the answers

    What is the side effect of Cyclophosphamide (CYP) treatment?

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

    What is the primary indication for considering steroid treatment prior to kidney biopsy?

    <p>Absence of systemic disease symptoms</p> Signup and view all the answers

    What is the maximum daily dose of oral prednisone for induction therapy?

    <p>60 mg</p> Signup and view all the answers

    What is the purpose of maintenance therapy after induction therapy?

    <p>To reduce the frequency of relapses</p> Signup and view all the answers

    What is the dosage of prednisone for treatment of relapses?

    <p>2 mg/kg/d</p> Signup and view all the answers

    What is the reason for giving prednisone as a single dose on alternate days?

    <p>To reduce the side effects of the drug</p> Signup and view all the answers

    What is the duration of maintenance therapy after induction therapy?

    <p>6 weeks</p> Signup and view all the answers

    What is the significance of normal complement levels in patients being considered for steroid treatment?

    <p>It excludes APSGN</p> Signup and view all the answers

    What is the age criterion for considering patients for steroid treatment prior to kidney biopsy?

    <p>1-8 years</p> Signup and view all the answers

    What is a potential complication of administering 25% albumin to a patient with anasarca and signs of intravascular volume depletion?

    <p>Shock due to decreased volume</p> Signup and view all the answers

    Which of the following vaccinations is contraindicated in patients with nephrotic syndrome?

    <p>Live-attenuating vaccines</p> Signup and view all the answers

    What is the definition of remission in nephrotic syndrome?

    <p>Urinary protein excretion &lt; 4 mg/m2/h or dipstick neg/trace for 3 consecutive days</p> Signup and view all the answers

    What is the term for two or more relapses within 6 months of initial response or 3 or more in 12 months in nephrotic syndrome?

    <p>Frequent relapses</p> Signup and view all the answers

    What is the minimum duration of steroid therapy required to achieve a response in nephrotic syndrome?

    <p>8 weeks</p> Signup and view all the answers

    What is the definition of nephrotic syndrome?

    <p>Edema, plasma albumin &lt; 2.5 gm/dl, proteinuria &gt; 40 mg/m2/h</p> Signup and view all the answers

    Study Notes

    Nephrotic Syndrome

    • Nephrotic syndrome is characterized by edema, plasma albumin < 2.5 gm/dl, and proteinuria > 40 mg/m2/h
    • Remission is defined as urinary protein excretion < 4 mg/m2/h or dipstick neg/trace for 3 consecutive days
    • Steroid response is achieved when remission is achieved with steroid therapy alone
    • Relapse occurs when urinary protein excretion > 40 mg/m2/h or dipstick 2+ for 3 consecutive days having previously been in remission
    • Frequent relapses are defined as two or more relapses within 6 months of initial response or 3 or more in 12 months
    • Steroid dependence is characterized by two consecutive relapses occurring during corticosteroid treatment or within 14 days of cessation
    • Steroid resistance is defined as failure to achieve response in spite of 8 weeks of prednisolone 60 mg/m2/day

    Treatment of Nephrotic Syndrome

    • For frequently relapsing and steroid-dependent disease, prednisone is prescribed at 2 mg/kg/d (60 mg/m2/d) as a single morning dose until the patient has been free of proteinuria for at least 3 days
    • Following remission of proteinuria, prednisone is reduced to 1.5 mg/kg (40 mg/m2) given as a single dose on alternate days and tapered over 3 or more months
    • A steroid-sparing agent, such as cyclophosphamide or cyclosporin A, may be considered once proteinuria is in remission
    • For steroid-resistant disease and focal segmental glomerulosclerosis (FSGS), cyclosporin A is the most frequently recommended treatment
    • Alkylating agents, such as cyclophosphamide, may be used in steroid-resistant disease

    Corticosteroid Therapy

    • Oral prednisone is prescribed at 60 mg/m2/d (2 mg/kg/d) for 6 weeks
    • Maintenance therapy involves oral prednisone at 40 mg/m2 (or 1.5 mg/kg) given as a single dose on alternate days for 6 weeks
    • Longer duration of alternate-day steroid treatment may further reduce the number of children with subsequent relapses

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    Description

    Learn about frequently relapsing and steroid-dependent nephrotic syndrome, including definitions and treatment options. Understand the differences between these two types of nephrotic syndrome.

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