What is the management of nephrotic syndrome?

Management of Nephrotic Syndrome

The management of nephrotic syndrome requires a comprehensive therapeutic approach focused on maintaining intravascular euvolemia, adequate nutrition, preventing complications, and preserving kidney function while addressing the underlying cause when possible. 1

Initial Diagnostic Workup

Before initiating treatment, establish the diagnosis and assess disease severity through targeted testing:

• Obtain blood biochemistry including complete blood count, sodium, chloride, albumin, magnesium, creatinine, urea, protein, cholesterol, triglycerides, and glucose 1
• Measure thyroid function (TSH and free T4) and serum IgG levels 1
• Assess calcium-phosphate metabolism including ionized calcium, phosphate, alkaline phosphatase, PTH, and vitamin D 1
• Perform abdominal ultrasound to evaluate kidney echogenicity, size, and detect ascites or effusions 1
• Conduct cardiac ultrasound to check for effusions and left ventricular mass 1
• Consider genetic testing as a first-line diagnostic measure, especially in congenital nephrotic syndrome 1

Fluid and Edema Management

First-Line Interventions

• Restrict dietary sodium intake as the primary intervention for edema management 1
• Restrict fluid intake when feasible, particularly in cases of hyponatremia and severe edema 1

Diuretic Therapy

• Administer furosemide (0.5-2 mg/kg per dose up to six times daily) for patients with intravascular fluid overload and preserved kidney function 1
• Give furosemide at the end of albumin infusions unless the patient has marked hypovolemia or hyponatremia 1
• Avoid diuretics entirely in patients with evidence of intravascular hypovolemia 1

Critical Pitfall: Doses >6 mg/kg per day should not be given for periods longer than 1 week due to risk of intravascular volume depletion 1

Albumin Infusions

• Administer intravenous albumin only when clinically indicated or as part of regular albumin infusion protocols 1
• Understand that the purpose is to support intravascular volume and reduce edema, not to normalize serum albumin levels 1
• Consider home administration by trained parents/caregivers to improve quality of life and reduce hospitalization 1

Immunosuppressive Therapy

Idiopathic Nephrotic Syndrome

• Corticosteroids (prednisone) are first-line therapy for idiopathic nephrotic syndrome to induce remission of proteinuria 2, 3
• For steroid-resistant cases, use calcineurin inhibitors (cyclosporine or tacrolimus) 4
• Consider alternative agents including mycophenolate mofetil, rituximab, or cyclophosphamide depending on the underlying cause 4

Genetic Forms

Do not use immunosuppressive agents for genetic forms of congenital nephrotic syndrome as they are unlikely to respond 1, 4

Prevention and Management of Complications

Infection Prevention

• Implement infection prophylaxis measures, especially in children with congenital nephrotic syndrome 1
• Consider ambulatory management when possible to reduce risk of nosocomial infections 1

Thromboembolism Prevention

• Consider prophylactic anticoagulation when central venous access is required 1
• Monitor for thromboembolism, especially with significant hypoalbuminemia 4

Nutritional Support

• Ensure adequate nutrition to prevent malnutrition and support growth, particularly in children 1
• Consider growth hormone substitution in children with growth impairment 1

Special Considerations for Congenital Nephrotic Syndrome

• Refer patients to specialized teams in tertiary pediatric nephrology centers 1
• Manage with a multidisciplinary team including neonatologists, pediatric nephrologists, nurses, dieticians, surgeons, psychologists, and social workers 1
• Use a stepwise approach with prolonged conservative management; avoid routine early nephrectomies 1
• Consider nephrectomy only in patients with persistent hypovolemia, thrombosis, and failure to thrive despite optimal conservative management 1
• Consider nephrectomy before transplantation in patients with persisting nephrotic syndrome and/or WT1-dominant pathogenic variant 1

Critical Pitfalls to Avoid

• Never routinely perform early nephrectomies in congenital nephrotic syndrome 1
• Do not use excessive diuretic doses (>6 mg/kg per day for >1 week) 1
• Avoid immunosuppression in genetic forms as it provides no benefit 1, 4
• Do not use prophylactic antibiotics or anticoagulation routinely in adults with nephrotic syndrome 3
• Monitor pediatric patients on corticosteroids closely for growth suppression, which may occur even without HPA axis suppression 2