Nephrotic syndrome in Children

1. Nephrotic Syndrome

2. • Idiopathic nephrotic syndrome (INS), characterized by massive proteinuria,
hypoalbuminemia, and/or concomitant edema is the most frequent
glomerular disease in children.
• Its incidence ranges from 1.15 to 16.9 per 100,000 children and varies by
ethnicity and region.
• Majority of affected children (85%) show complete remission of proteinuria
within 4–6 weeks with daily prednisolone/prednisone (PDN) and have
steroid-sensitive NS (SSNS).
• About 70–80% of patients will experience at least one relapse during
follow-up.
• About 50% of patients have frequent relapses or are steroid-dependent.

3. • Childhood onset SSNS may resolve spontaneously following puberty;
• however, 10–30% continue to have a relapsing course into young
adulthood.
• most common diagnoses are minimal change disease (MCD) showing
either minimal changes, i.e., podocyte foot process efacement, or mild
mesangial proliferation with IgM deposition, or less commonly focal-
segmental glomerulosclerosis (FSGS)

4. • Nephrotic-range proteinuriaa
Urinary protein creatinine ratio (UPCR)≥200 mg/mmol (2 mg/mg) in a
spot urine, or proteinuria≥1000 mg/m2 per day in a 24-h urine sample
corresponding to 3+(300–1000 mg/ dL) or 4+(≥1000 mg/dL) by urine
dipstick
• Nephrotic syndrome Nephrotic-range proteinuria and either
hypoalbuminemia (serum albumin<30 g/L) or edema
when serum albumin is not available

5. Complete remission
• UPCR (based on frst morning void or 24 h urine sample)≤20
mg/mmol (0.2 mg/mg) or<100 mg/m2 per day, respectively, or
negative or trace dipstick on three or more consecutive days
Partial remission
• UPCR (based on frst morning void or 24 h urine sample)>20 but<200
mg/mmol (>0.2 mg/mg but<2 mg/mg) and serum albumin≥30 g/L

6. Steroid-sensitive nephrotic syndrome (SSNS)
• Complete remission within 4 weeks of PDN at standard dose (60
mg/m2/day or 2 mg/kg/day, maximum 60 mg/day)
Steroid-resistant nephrotic syndrome (SRNS)
• Lack of complete remission within 4 weeks of treatment with PDN at
standard dose
• A patient not achieving complete remission by 6 weeks, although
partial remission was achieved at 4 weeks, is defned as SRNS

7. • Relapse
Urine dipstick≥3+(≥300 mg/dl) or UPCR≥200 mg/mmol (≥2 mg/mg) on
a spot urine sample on 3 consecutive days, with or without reappearance
of edema in a child who had previously achieved complete remission
• Infrequently relapsing nephrotic syndrome
<2 relapses in the 6 months following remission of the initial episode or
fewer than 3 relapses in any subsequent 12-month period

8. • Frequently relapsing nephrotic syndrome (FRNS)
≥2 relapses in the frst 6-months following remission of the initial episode
or≥3 relapses in any 12 months
• Steroid-dependent nephrotic syndrome (SDNS)
A patient with SSNS who experiences 2 consecutive relapses during
recommended PDN therapy for frst presentation or relapse or within 14 days
of its discontinuation

9. Initial work-up for a child with nephrotic
syndrome

16. • Recommended in patients with atypical features including macroscopic
hematuria, low C3 levels, AKI not related to hypovolemia, sustained
hypertension, arthritis and/or rash
• Consider in patients with infantile onset NS if genetic screening is not
available (age 3–12 months)
• Consider in patients>12 years of age on a case-by-case basis
• Consider in patients with persistent microscopic hematuria in specifc
populations with a high incidence of glomerular diseases such as IgA
nephropathy in East Asia (grade C, weak recommendation)
• Recommended in patients diagnosed with SRNS

17. Genetic testing
• Recommended in patients with congenital NS, extrarenal features
and/or family history suggesting syndromic/hereditary SRNS
• Consider in patients with infantile onset NS (age 3–12 months)
• Recommended in patients diagnosed with SRNS