Options of care and management of children with SSNS | AAP CPG 2009 [25] Moderate-quality CPG (Domain 3: 40–69%) | High-quality CPG (Domain 3: ≥ 70%) | KDIGO CPG 2012 [29] High-quality CPG (Domain 3: ≥ 70%) |
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Case definition | |||
▪ Nephrotic syndrome | A urine protein/creatinine ratio (Up/c) of ≥2 and a serum albumin level of ≤2.5 mg/dL | Severe proteinuria (≥40 mg/m2/h in pooled night urine) or early morning urine protein/creatinine ratio ≥2.0 g/gCr and hypoalbuminemia (serum albumin level ≤2.5 g/dL) | Presence of the following: ▪ Edema ▪ uPCR ≥2000 mg/g (≥200 mg/mmol) or ≥300 mg/dL or 3+ protein on urine dipstick ▪ Hypoalbuminemia ≤2.5 g/dl (≤25 g/L) |
▪ Remission | Up/c < 0.2 or Albustix-negative (Albustix, Miles, Inc, Diagnostics Division, Elkhart, IN) or trace for 3 days | • Complete Negative protein on dipstick testing of early morning urine for 3 consecutive days or early morning urine protein/creatinine ratio < 0.2 g/gCr for 3 consecutive days • Incomplete ≥ 1+ protein on dipstick testing of early morning urine or early morning urine protein creatinine ratio ≥ 0.2 g/gCr and serum albumin > 2.5 g/dL | • Complete remission: uPCR < 200 mg/g (< 20 mg/mmol) or < 1+ of protein on urine dipstick for 3 consecutive days • Partial remission: Proteinuria reduction ≥ 50% from the presenting value and absolute uPCR between 200 and 2000 mg/g (20–200 mg/mmol) |
▪ Relapse | After remission, an increase in the first morning Up/c to ≥ 2 or Albustix reading of ≥ 2 for 3 of 5 consecutive days | ≥ 3+ protein on dipstick testing of early morning urine for 3 consecutive days | uPCR ≥ 2000 mg/g (≥ 200 mg/mmol) or ≥ 3+ protein on urine dipstick for 3 consecutive days |
â–ª FRNS | Two or more relapses within 6 months after initial therapy or four or more relapses in any 12-month period | Two or more relapses within 6 months after initial remission or four or more relapses within any 12 consecutive months | Two or more relapses within 6 months of initial response or four or more relapses in any 12-month period |
â–ª SDNS | Relapse during taper or within 2 weeks of discontinuation of steroid therapy. | Two consecutive relapses during prednisolone tapering or within 14 days after discontinuation of prednisolone | Two consecutive relapses during corticosteroid therapy or within 14 days of therapy discontinuation |
â–ª SRNS | Inability to induce a remission with 4 weeks of daily steroid therapy | Absence of complete remission after at least 4 weeks of daily prednisolone therapy | No remission after a minimum of 8 weeks treatment with corticosteroids |
Genetic testing | Not mentioned | • Useful in genetic illnesses (type of testing not mentioned) | Not mentioned |