Initial Treatment for Nephrotic Syndrome in Children

Diagnostic Criteria and Initial Evaluation

Children younger than 1 year are more likely to have a genetically definable cause for nephrotic syndrome and should be managed differently 1

The American Academy of Pediatrics recommends beginning with oral prednisone 60 mg/m²/day or 2 mg/kg/day (maximum 60 mg/day) as a single daily dose 1, 4, 3
Continue daily dosing for 4-6 weeks 1, 2, 3
After the daily phase, transition to alternate-day prednisone at 40 mg/m² or 1.5 mg/kg (maximum 40 mg on alternate days) 1, 4, 5
Continue alternate-day dosing for 2-5 months with gradual tapering 1, 2
Total treatment duration should be at least 12 weeks to reduce relapse risk 3

Remission is defined as urine protein <1+ on dipstick for 3 consecutive days or uPCR <200 mg/g (<20 mg/mmol) 4, 5

Relapse is defined as ≥3+ protein on urine dipstick for 3 consecutive days or uPCR ≥2000 mg/g (≥200 mg/mmol) 4, 5
For infrequent relapses, treat with prednisone 60 mg/m²/day (maximum 60 mg/day) until remission for at least 3 days 1, 4
After achieving remission, switch to alternate-day prednisone (40 mg/m² or 1.5 mg/kg) for at least 4 weeks 4
For frequent relapses or steroid-dependent nephrotic syndrome, consider steroid-sparing agents 3

During episodes of upper respiratory tract infections or other infections in children with frequent relapses, daily prednisone at 0.5 mg/kg/day for 5-7 days may reduce relapse risk 3
For steroid-dependent or frequently relapsing nephrotic syndrome, steroid-sparing agents should be considered, including Rituximab 3

Avoid treating children under 1 year of age with the standard regimen without further evaluation, as they often have genetic forms of nephrotic syndrome 1, 2
Do not discontinue steroids too rapidly, as this increases relapse risk 4, 5
Monitor for steroid side effects and consider steroid-sparing agents in children with frequent relapses or steroid dependence 3
Approximately 80% of children will experience at least one relapse, and 50% will have frequent relapses or become steroid-dependent 1, 2