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Table 3 Summary comparison between the three included clinical practice guidelines for management of steroid-sensitive nephrotic syndrome in children): Case definition

From: AGREEing on clinical practice guidelines for idiopathic steroid-sensitive nephrotic syndrome in children

Options of care and management of children with SSNS AAP CPG 2009 [25]
Moderate-quality CPG (Domain 3: 40–69%)
JSPN CPG 2014 [26,27,28]
High-quality CPG (Domain 3: ≥ 70%)
KDIGO CPG 2012 [29]
High-quality CPG (Domain 3: ≥ 70%)
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
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
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
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
  1. AAP American Academy of Pediatrics; CPGs clinical practice guidelines; CNI calcineurin inhibitor; CPG ID short identity or acronym; JSPN Japanese Society of Paediatric Nephrology; CNIs KDIGO, Kidney Disease: Improving Global Outcomes; AAP 2009 CPG Management of childhood onset nephrotic syndrome; JPNS 2014 CPG evidence-based clinical practice guidelines for nephrotic syndrome; KDIGO 2012 CPG clinical practice guideline for glomerulonephritis—Chapter 3; ISKDC International Study of Kidney Disease in Children; MCNS minimal change nephrotic syndrome; MMF mycophenolate mofetil; FRNS Frequently relapsing nephrotic syndrome; SSNS steroid-sensitive nephrotic syndrome; SDNS steroid-dependent nephrotic syndrome; SRNS: steroid resistant nephrotic syndrome