What is the initial approach to treating a pediatric patient with nephrotic syndrome?

Initial Treatment of Pediatric Nephrotic Syndrome

Begin oral corticosteroids immediately as first-line therapy for all children presenting with idiopathic nephrotic syndrome, as steroid response is the single most important prognostic factor and over 85% of children will achieve complete remission. 1

Immediate Corticosteroid Therapy

• Start daily oral prednisone for 4-6 weeks as the cornerstone of initial treatment 1, 2
• Steroid-responsiveness determines prognosis more reliably than renal histology 3
• Most children (>85%) between ages 1-12 years will show complete remission of proteinuria with daily corticosteroid treatment 2
• The FDA approves prednisone for nephrotic syndrome in children >2 years of age 4

Supportive Management During Initial Presentation

Fluid and Edema Management

• Avoid intravenous fluids and saline - concentrate oral fluid intake to prevent marked edema 5, 6
• Use diuretics cautiously and only when intravascular fluid overload is present (evidenced by good peripheral perfusion and high blood pressure) 6
• Start furosemide at 0.5-2 mg/kg per dose (up to 6 times daily, maximum 10 mg/kg/day) for severe edema 6
• Limit high-dose furosemide (>6 mg/kg/day) to less than 1 week to avoid hearing loss 6

Albumin Infusion Criteria

• Administer albumin (0.5-1 g/kg over 1-4 hours) only for clinical signs of hypovolemia, not based on serum albumin levels alone 5, 6
• Clinical indicators requiring albumin include: oliguria, acute kidney injury, prolonged capillary refill time, tachycardia, hypotension, or abdominal discomfort 5, 6
• Consider furosemide bolus (0.5-2 mg/kg) at the end of albumin infusions if no marked hypovolemia exists 6

Thromboprophylaxis Assessment

• Consider prophylactic anticoagulation when serum albumin drops below 20-25 g/L AND any additional risk factor is present (proteinuria >10 g/d, BMI >35, heart failure, recent surgery, prolonged immobilization) 6
• Use low molecular-weight heparin or unfractionated heparin 5000 U subcutaneously twice daily for prophylaxis 6
• Thromboembolism is one of the most severe and potentially fatal complications of nephrotic syndrome 3
• If central venous access is required for repeated albumin infusions, administer prophylactic anticoagulation for the entire duration the line is in place 5, 6

Infection Prevention

• Bacterial infections represent the most severe and potentially fatal complication alongside thromboembolism 3
• Administer pneumococcal and influenza vaccines 6
• Monitor closely for peritonitis, sepsis, cellulitis, and other infections 7
• Patients on corticosteroid therapy may exhibit diminished response to vaccines, so routine vaccination should ideally be deferred until corticosteroid therapy is discontinued when possible 4

Monitoring During Initial Treatment

• Measure blood pressure, weight, height, and intraocular pressure frequently 4
• Evaluate for infection, psychosocial disturbances, thromboembolism, peptic ulcers, cataracts, and osteoporosis 4
• Monitor electrolytes (particularly potassium and sodium), kidney function, and fluid status during diuretic therapy 6
• Growth velocity may be the most sensitive indicator of systemic corticosteroid exposure in children 4

Defining Treatment Response

• Children who do not achieve remission after 4 weeks of daily prednisolone are classified as steroid-resistant nephrotic syndrome (SRNS) 2
• Steroid-sensitive patients have favorable long-term outcomes, while most children with refractory SRNS ultimately develop end-stage renal disease 3
• For SRNS, calcineurin inhibitors (cyclosporine or tacrolimus) become the standard second-line therapy, achieving complete or partial remission in approximately 70% of patients 2

Critical Pitfalls to Avoid

• Do not confuse nephrotic syndrome with nephritic syndrome - nephritic syndrome requires only supportive care with fluid/sodium restriction and blood pressure management, with no role for corticosteroids 8
• Never administer albumin infusions based solely on low serum albumin levels without clinical signs of hypovolemia 5, 6
• Avoid central venous lines when possible due to extremely high thrombosis risk 5, 6
• Do not use factor Xa inhibitors for anticoagulation, as they have not been systematically studied in nephrotic syndrome 6

Specialized Referral

• Rapidly refer all children with nephrotic syndrome to a specialized pediatric nephrology unit due to the complexity of disease and fluid management 5, 6
• Early referral to a transplant center is warranted for patients showing signs of progressive disease 6