Management Plan for a 7-Year-Old with Nephrotic Syndrome in the Pediatric ER
A 7-year-old presenting to the Pediatric ER with nephrotic syndrome requires immediate referral to a specialized pediatric nephrology unit due to the complexity of the condition and need for specialized management.
Initial Assessment and Stabilization
Clinical Evaluation
- Vital signs: Monitor blood pressure every 1-2 hours initially
- Physical examination: Assess for:
- Degree of edema (periorbital, peripheral, ascites, pleural effusions)
- Signs of hypovolemia (tachycardia, prolonged capillary refill, hypotension)
- Respiratory distress from pleural effusions
- Abdominal distension from ascites
Immediate Laboratory Tests
- Complete blood count
- Renal function tests (BUN, creatinine)
- Serum electrolytes, albumin, total protein
- Lipid profile (cholesterol, triglycerides)
- Urinalysis with protein/creatinine ratio
- Coagulation profile
Imaging
- Renal ultrasound to assess kidney size and echogenicity
- Chest X-ray if respiratory symptoms present
Acute Management
Fluid Management
- Avoid intravenous fluids and saline unless clinically indicated for hypovolemia 1
- Restrict fluid intake if significant edema present
- Monitor daily weight and abdominal girth
Albumin Administration
- Administer albumin infusions (0.5-1 g/kg) only for:
- Clinical signs of hypovolemia (oliguria, acute kidney injury, prolonged capillary refill)
- Severe symptomatic edema
- Not based on serum albumin levels alone 1
Diuretic Therapy
- Furosemide (0.5-2 mg/kg/dose) following albumin infusion if significant edema present
- Avoid high doses of furosemide (>6 mg/kg/day) for periods longer than 1 week 1
- Consider amiloride over spironolactone when potassium-sparing diuretics are needed 2, 1
Corticosteroid Therapy
- Initiate prednisone 60 mg/m²/day or 2 mg/kg/day (maximum 60 mg/day) as soon as diagnosis is confirmed 3
- Continue daily dosing for 4-6 weeks, followed by alternate-day therapy
- Monitor for side effects including hypertension, hyperglycemia, and mood changes 3, 4
Prevention of Complications
Infection Prevention
- Assess for signs of infection (fever, elevated WBC)
- Consider prophylactic antibiotics if severely immunocompromised
- Defer live vaccines while on high-dose corticosteroids 3
Thromboembolism Prevention
- Monitor for thrombocytosis (>750,000/ml) 1
- Consider prophylactic anticoagulation if:
- Central venous access is required
- Severe hypoalbuminemia (<2 g/dL)
- History of thrombosis
- Immobility 5
Nutritional Management
- Low salt diet
- High protein diet (2-3 g/kg/day) 1
- Adequate caloric intake
- Calcium and vitamin D supplementation
Discharge Planning and Follow-up
Education
- Teach parents about:
- Daily urine dipstick monitoring
- Daily weight monitoring
- Medication administration
- Signs of relapse (edema, proteinuria)
- When to seek medical attention
Follow-up
- Arrange follow-up with pediatric nephrology within 1 week
- Regular monitoring of:
- Growth and development
- Blood pressure
- Renal function
- Electrolytes
- Lipid profile
Special Considerations
Steroid Response Assessment
- Evaluate response to steroids after 4 weeks of therapy
- Steroid-responsiveness is of greater prognostic value than renal histology 6
- Consider renal biopsy if no response to steroids after 4-6 weeks
Second-line Therapy Considerations
- For steroid-dependent or frequently relapsing cases, consider:
- Calcineurin inhibitors (cyclosporine, tacrolimus)
- Mycophenolate mofetil
- Cyclophosphamide
- Rituximab 7
Common Pitfalls to Avoid
- Overuse of IV fluids worsening edema
- Administering albumin based solely on serum albumin levels
- Delaying steroid initiation
- Inadequate monitoring for thromboembolism
- Overlooking nutritional needs
- Failing to educate family about home monitoring
Remember that nephrotic syndrome is a chronic relapsing disease for most steroid-responsive patients 6, requiring long-term management strategies to minimize complications and optimize quality of life.