Nephrotic Syndrome

Definitions Related to Nephrotic Syndrome

• Nephrotic syndrome Nephrotic range proteinuria (40 mg/m2 /hr or >1000 mg/m^2/day; spot Up/Uc>2 mg/mg; 3-4+ by dipstick); hypoalbuminemia (albumin <3.0 g/dL); and edema;
• Steroid sensitive nephrotic syndrome Complete remission within 6-weeks’ treatment with prednisolone at a dose of 60 mg/m2/ day (2 mg/kg/day; maximum 60 mg/day)
• Initial steroid-resistance Failure to achieve complete remission after 6-weeks initial therapy with prednisolone (as defined above)
• Late (secondary) steroid-resistance Initially steroid-sensitive; steroid resistance in a subsequent relapse
• Complete remission Urine protein nil-trace by dipstick for 3 consecutive days, Up/Uc <0.2, or 24-hr protein <100 mg/m2/day
• Partial remission Urine protein 1+/2+ (dipstick), Up/Uc between 0.2-2, or 24-hr urine protein 100-1000 mg/m2/day; serum albumin ≥3.0 g/dL; and absence of edema
• Non-response Urine protein 3+/4+ (dipstick), Up/Uc >2, or 24-hr urine protein >1000 mg/m2/day; albumin <3.0 g/dL or edema
• Relapse Urine albumin 3+/4+ for 3 consecutive days, Up/Uc>2, or 24-hr protein >1000 mg/m2/day, in a patient previously in partial or complete remission
• Monogenic disease Pathogenic or likely pathogenic variation, defined by American College of Medical Genetics and Genomics, in a gene associated with nephrotic syndrome
• CNI-resistant disease Non-response to cyclosporine or tacrolimus, given in adequate doses and titrated to blood levels, for 6-months
• Allograft recurrence of nephrotic syndrome Persistent proteinuria (Up/Uc >1) if previouslyanuric; or increase of Up/Uc >1 if proteinuria at time of transplant (in absence of other apparent causes)