From the Guidelines
Nephrotic syndrome should be treated with corticosteroids as the initial treatment, as recommended by the KDIGO practice guideline 1. This approach is based on the guideline's suggestion that corticosteroids be given for initial treatment of nephrotic syndrome (1C). The treatment typically includes corticosteroids like prednisone, starting at 1 mg/kg/day (maximum 80 mg) for 4-6 weeks, followed by a gradual taper over several months. Some key points to consider in the management of nephrotic syndrome include:
- Supportive care with diuretics such as furosemide 20-80 mg daily to manage edema
- ACE inhibitors like enalapril 5-20 mg daily to reduce proteinuria
- Statins for hyperlipidemia
- Dietary modifications including moderate sodium restriction (2-3 g/day) and adequate protein intake (0.8-1 g/kg/day)
- Immunosuppressive medications like cyclophosphamide, cyclosporine, or rituximab may be needed for steroid-resistant cases, as discussed in the context of FSGS treatment 1 Regular monitoring of kidney function, protein levels, and medication side effects is essential, and identifying and treating the underlying cause is crucial for effective management.
From the FDA Drug Label
Published studies provide evidence of efficacy and safety in pediatric patients for the treatment of nephrotic syndrome (patients >2 years of age) The treatment of nephrotic syndrome with prednisone is supported by published studies in pediatric patients over 2 years of age 2.
- The efficacy and safety of corticosteroids in pediatric patients are based on well-established courses of effect similar to adult populations.
- Pediatric patients should be carefully observed with frequent measurements of blood pressure, weight, height, intraocular pressure, and clinical evaluation for the presence of infection, among other factors.
- The adverse effects of corticosteroids in pediatric patients are similar to those in adults.
From the Research
Definition and Treatment of Nephrotic Syndrome
- Nephrotic syndrome is a kidney disorder characterized by excessive excretion of protein in the urine, leading to complications such as edema, hyperlipidemia, and thrombosis 3.
- The most common cause of nephrotic syndrome in school-aged children is minimal change disease, which is often treated with corticosteroids as first-line therapy 3.
Corticosteroid Therapy
- Corticosteroids, such as prednisone, are effective in inducing remission in most cases of minimal change nephrotic syndrome (MCNS), with response rates of 93% in children and 81% in adults 4.
- The standard dose of prednisone is 60 mg/m2/day (maximum 80 mg/day) for 4 weeks, followed by 40 mg/m2 on alternate days for several weeks 4.
- However, recent studies suggest that lower doses of prednisone (1-1.5 mg/kg/day) may be equally effective in achieving remission, with a significantly lower cumulative dose 5.
- In adults, treatment with low-dose prednisone plus enteric-coated mycophenolate sodium was not superior to a standard high-dose prednisone regimen in inducing complete remission of MCNS 6.
Alternative Therapies
- For patients with steroid-resistant or steroid-dependent nephrotic syndrome, alternative therapies such as cyclophosphamide, chlorambucil, and cyclosporine may be effective in inducing remission 4.
- However, these therapies can have significant adverse effects, including bone marrow depression, hemorrhagic cystitis, and gonadal toxicity 4.
- Levamisole may be helpful in steroid-dependent cases, but data on its efficacy are conflicting 4.
Complications and Management
- Patients with nephrotic syndrome are at risk of developing complications such as dyslipidemia, infection, and thrombosis, which require prompt recognition and management 3.
- Corticosteroid therapy can also have adverse effects, including opportunistic infections, osteoporosis, and aseptic necrosis of the femoral head, which require preventive measures and urgent treatment 7.