What is the initial treatment for nephrotic syndrome in children?

Initial Treatment for Nephrotic Syndrome in Children

The initial treatment for nephrotic syndrome in children is prednisone or prednisolone at a daily single dose of 60 mg/m²/day (maximum 60 mg) for 4-6 weeks, followed by 40 mg/m²/day (maximum 40 mg) on alternate days for 4-6 weeks, with a total treatment duration of 8-12 weeks. 1

Diagnostic Approach Before Treatment

Before initiating treatment, consider the following:

• Confirm nephrotic syndrome based on:

  • Proteinuria
  • Hypoalbuminemia
  • Edema
  • Hyperlipidemia

• For children under 12 years without atypical features:

  • Initial empiric treatment with glucocorticoids without biopsy is recommended 1

• For children aged 12 years or older or with atypical features:

  • Kidney biopsy and/or genetic testing with referral to a specialty center 1

• Obtain renal ultrasound to assess kidney size, echogenicity, and rule out structural abnormalities 1

Corticosteroid Treatment Protocol

First-Line Therapy

1. Initial Phase:

   • Prednisone/prednisolone 60 mg/m²/day (maximum 60 mg) as a single daily dose for 4-6 weeks 1

2. Tapering Phase:

   • Follow with 40 mg/m²/day (maximum 40 mg) on alternate days for 4-6 weeks 1
   • Total treatment duration: 8-12 weeks

3. After Remission:

   • After achieving complete remission, steroids should be tapered slowly over a period of 6 months 1
   • Rapid steroid tapering should be avoided to reduce relapse risk

Important Administration Considerations

• Administer prednisone as a single morning dose rather than divided doses 2
• Ensure adequate fluid intake during treatment to maintain good hydration

Management of Steroid-Resistant Cases

For children who fail to respond adequately to corticosteroid therapy:

Second-Line Options

• Cyclophosphamide:

  • FDA-approved for biopsy-proven minimal change nephrotic syndrome in pediatric patients who failed to respond to or cannot tolerate corticosteroids 3
  • Dosage: 2 mg/kg orally once daily for 8-12 weeks (maximum cumulative dose 168 mg/kg) 1, 3
  • Important: Treatment beyond 90 days increases the probability of sterility in males 3

• Other Steroid-Sparing Agents:

  • Calcineurin inhibitors (cyclosporine: 3-4 mg/kg/day) 1
  • Levamisole: 2.5 mg/kg on alternate days for 12-24 months 1
  • Mycophenolate mofetil: 600-1200 mg/m²/day divided into 2 doses for 6-24 months 1

Monitoring and Complication Prevention

Regular Monitoring

• Proteinuria
• Renal function
• Blood pressure
• Weight and height
• Steroid-related adverse effects

Complication Prevention

• Infections:

  • Pneumococcal and annual influenza vaccination
  • Defer live vaccines until prednisone dose <1 mg/kg/day 1
  • Prompt treatment of infections

• Conservative Measures:

  • Blood pressure control with ACE inhibitors or ARBs
  • Sodium restriction
  • Diuretics for edema management
  • Statins for hyperlipidemia 1

Common Pitfalls to Avoid

• Inadequate initial steroid duration: Ensure minimum 4 weeks of high-dose treatment and total treatment course of 8-12 weeks 1
• Rapid steroid tapering: Taper slowly over 6 months after remission to reduce relapse risk 1
• Delayed introduction of steroid-sparing agents in frequently relapsing cases
• Overlooking infections during immunosuppressive therapy
• Inadequate monitoring of drug toxicity and complications

Expected Outcomes

• Most children with minimal change disease will respond to initial corticosteroid therapy within 2 weeks
• The goal of treatment is to achieve reduction in proteinuria by at least 25% by 3 months and 50% by 6 months 1
• Approximately 60-70% of children will experience relapses requiring repeated courses of treatment