What are the KDIGO treatment guidelines for steroid-sensitive pediatric nephrotic syndrome?

Updated: Mar 04, 2020
  • Author: Jerome C Lane, MD; Chief Editor: Craig B Langman, MD  more...
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Answer

The Kidney Disease: Improving Global Outcomes (KDIGO) group released guidelines that address management of steroid-sensitive nephrotic syndrome in children aged 1-18 years. [63]

Highlights of these guidelines include the following [63] :

  • Definition of nephrotic syndrome: Edema, urine protein:creatinine ratio ≥2 mg/mg; urine protein ≥300 mg/dL, dipstick urine protein 3+, hypoalbuminemia ≤2.5 mg/L.

  • Initial treatment: Oral prednisone, starting as a daily dose of 60 mg/m2/day or 2 mg/kg/day (maximum, 60 mg/day) for 4-6 weeks. After 4-6 weeks, switch to 40 mg/m2 or 1.5 mg/kg (maximum, 40 mg) on alternate days for 2-5 months with tapering, with a minimum total duration of treatment of 12 weeks.

  • Treatment of infrequent relapse (1 relapse in 6 months or 1-3 relapses in 12 months): Administer initial treatment dose (60 mg/m2/day or 2 mg/kg/day) until urinary protein is negative for 3 days; after urine is negative for protein for 3 days, change prednisone to 40 mg/m2 or 1.5 mg/kg (maximum, 40 mg) on alternate days for 4 weeks, then stop or taper dose.

  • Treatment of frequent relapse (2 relapses in 6 months or ≥4 relapses in 12 months): Continue infrequent relapse treatment for 3 months at the lowest dose to maintain remission or use corticosteroid-sparing agents, including alkylating agents, levamisole, calcineurin inhibitors, and mycophenolate mofetil.

The treatment of steroid-sensitive INS, steroid-dependent and frequently relapsing INS, steroid-resistant nephrotic syndrome (SRNS), and FSGS are discussed in detail below. The treatment of membranoproliferative glomerulonephritis (MPGN), membranous nephropathy (MN), congenital nephrotic syndrome, and secondary nephrotic syndrome (eg, lupus nephritis and vasculitis) are beyond the scope of this article.


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