Table 1.

Definitions on the basis of references (9,10,64) and on the authors’ clinical experience

Nephrotic Syndrome
 Edema
 Massive proteinuria (>40 mg/m2 per h in children, >3.5 g/d in adults)
 Hypoalbuminemia (<2.5 g/dl)
Remission
 Resolution of edema
 Normalization of serum albumin (≥3.5 g/dl)
 Marked reduction in proteinuria
 Complete remission (<4 mg/m2 per h or negative dipstick in children, <0.3 g/d in adults)
 Partial remission (<2 g/1.73 m2 per d, decreased by 50% and serum albumin ≥2.5 g/dl in children, <3.5 g/d and decreased by 50% in adults)
Relapse
 Recurrence of massive proteinuria (>40 mg/m2 per h in children, >3.5 g/d in adults)
 Positive urine dipstick (≥3+ for 3 d or positive for 7 d, usually applicable to children)
 ±Edema
Steroid-Sensitive Nephrotic Syndrome
 Response to PDN 60 mg/m2 per d within 4–6 wk ±MPD boluses in children
 Response to PDN 1 mg/kg per d or 2 mg/kg every other d, within 16 wk in adults
Nonrelapsing Nephrotic Syndrome
 No relapses for >2 yr after the end of therapy for the first episode of nephrotic syndrome (applicable to children, not yet defined in adults)
Infrequently Relapsing Nephrotic Syndrome
 <2 relapses per 6 mo (or <4 relapses per 12 mo)
Frequently Relapsing Nephrotic Syndrome
 ≥2 relapses per 6 mo (or ≥4 relapses per 12 mo)
Steroid-Dependent Nephrotic Syndrome
 Relapse during steroid therapy or within 15 d of discontinuation
Steroid-Resistant Nephrotic Syndrome
 No response to PDN 60 mg/m2 per d within 4 wk ±MPD boluses in children
 No response to PDN 1 mg/kg per d or 2 mg/kg every other d, within 16 wk in adults
Multidrug-Resistant Nephrotic Syndrome
 Poorly defined as absence of partial remission after 6 mo OR absence of complete remission after 2 yr
 Treatment often consists of MPD boluses + oral prednisone for 6 mo + CsA and, in some cases, rituximab. Other protocols are also used
  • PDN, prednisone; MPD, methylprednisolone; CsA, cyclosporine A.