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Nephrotic Syndrome in Children - Child Health Nursing

Definition

• Massive proteinuria: >40 mg/m²/h or urine protein:creatinine ratio >2.0 mg/mg (in children; >3.5 g/24h in adults)
• Hypoalbuminemia: serum albumin <3 g/dL
• Generalized edema (anasarca)
• Hyperlipidemia and lipiduria

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Epidemiology

• Most common glomerular disease in children
• Peak incidence: preschool age, median 2.5 years
• Male:female ratio ~2:1 in young children
• Minimal Change Nephrotic Syndrome (MCNS) accounts for ~90% of cases before age 10 years

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Classification

Histological subtypes (Primary NS):

• MCNS - most common in children; excellent prognosis; steroid responsive (~90%)
• FSGS - majority of steroid-resistant cases; higher incidence in African-American, Hispanic, Asian children
• Membranous nephropathy - rare in childhood; secondary causes more common
• Mesangial proliferation

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Pathophysiology

1. Glomerular injury (podocyte/GBM damage) → increased permeability of the glomerular capillary wall
2. Massive proteinuria → loss of albumin beyond hepatic synthetic capacity
3. Hypoalbuminemia → reduced plasma oncotic pressure
4. Fluid shift into interstitial spaces → underfill edema
5. Compensatory responses (RAAS activation, aldosterone, sympathetic activation, reduced ANP) → sodium and water retention → overfill edema
6. Hyperlipidemia → increased hepatic lipoprotein synthesis + reduced catabolism due to low oncotic pressure

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Clinical Features

Hallmark Presentation

• Periorbital edema - often the first sign, especially in the morning; parents may initially think it is an allergic reaction
• Dependent edema - ankles/feet when ambulatory; sacral when supine
• Ascites - abdominal distension
• Pleural effusion - respiratory distress in severe cases
• Scrotal/labial edema
• Pallor, lethargy, decreased urine output (frothy/foamy urine)
• Weight gain (from fluid retention)

Typically ABSENT in MCNS:

• Significant hypertension (hypertension is NOT typical of MCNS)
• Gross hematuria (microscopic hematuria seen in ~20%)

Atypical features requiring biopsy before treatment:

• Age <1 year at presentation
• Macroscopic (gross) hematuria
• Hypertension
• Low complement levels
• Extrarenal symptoms (rash, arthritis)

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Complications (Nursing Alert Points)

1. Infection (PRIORITY CONCERN)

• Loss of immunoglobulins (IgG) in urine + depressed T-cell function
• Most dangerous: peritonitis (spontaneous bacterial peritonitis - Streptococcus pneumoniae commonest)
• Also vulnerable to staphylococcal infections, cellulitis, sepsis
• Corticosteroid therapy further immunosuppresses

2. Thromboembolism

• Loss of antithrombin III, protein C, protein S in urine
• Increased procoagulant factors (fibrinogen) + platelet hyperactivity
• Risk of: renal vein thrombosis, DVT, pulmonary embolism
• Most dangerous in hypoalbuminemia <2 g/dL

3. Hypovolemia (Intravascular Depletion)

• Despite generalized edema, intravascular volume may be contracted (underfill)
• Signs: abdominal pain, tachycardia, cold extremities, oliguria
• Dangerous if diuretics given without assessing volume status

4. Acute Kidney Injury

• A 2024 systematic review (PMID: 39759881) found significant incidence of AKI in children with NS - key risk factor requiring monitoring

5. Cardiovascular

• Hyperlipidemia (↑ LDL, cholesterol, triglycerides, ↓ HDL)
• A 2025 meta-analysis showed increased carotid intima-media thickness in pediatric NS, indicating early atherosclerosis risk (PMID: 40963441)

6. Steroid Side Effects (with prolonged therapy)

• Growth retardation, Cushingoid features, hypertension, osteoporosis, behavioral changes, cataracts

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Diagnostic Investigations

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Medical Management

First-line: Corticosteroids

• Prednisone 60 mg/m²/day or 2 mg/kg/day (max 80 mg/day) for 4-6 weeks
• Then alternate-day dosing, tapered over 6 weeks or longer
• Remission = urine protein-free for 3 consecutive days

Relapse Patterns:

• Frequent relapse (FRNS): ≥2 relapses within 6 months OR ≥4 within 12 months
• Steroid-dependent (SDNS): relapses during or immediately after steroid taper
• Steroid-resistant (SRNS): no remission after 6-8 weeks full-dose prednisone (15-20% of children)

Steroid-Sparing Agents (for FRNS/SDNS/SRNS):

• Cyclophosphamide (oral alkylating agent)
• Mycophenolate mofetil (MMF)
• Calcineurin inhibitors: cyclosporine, tacrolimus
• Rituximab (B-cell depletion)

Congenital NS:

• No steroids (nonresponsive)
• Conservative: sodium/fluid restriction, IV albumin + loop diuretics, hypercaloric diet, thyroid hormone replacement

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Nursing Management (Core Focus)

1. Assessment

• Daily weight (same time, same scale, same clothing) - best indicator of fluid balance
• Strict intake and output monitoring
• Measure abdominal girth daily
• Assess edema: location, pitting grade, degree
• Blood pressure monitoring (4-hourly during acute phase)
• Urine testing for protein (dipstick) - teach parents for home monitoring
• Monitor for signs of infection (fever, abdominal pain, peritonitis)
• Monitor for hypovolemia (tachycardia, cold peripheries, postural hypotension)

2. Fluid and Electrolyte Management

• Fluid restriction during severe edema
• Sodium restriction - low-sodium diet (no added salt)
• Administer furosemide as prescribed; monitor potassium levels
• IV albumin (25%) before diuretics in severe hypoalbuminemia with symptomatic edema
• Monitor for electrolyte imbalances (hyponatremia, hypokalemia with diuretics)

3. Skin Care

• Edematous skin is fragile and vulnerable to breakdown
• Change position every 2 hours
• Use pressure-relieving mattress
• Keep skin clean and dry; avoid friction
• Elevate edematous limbs (scrotal support for boys)
• Inspect skinfolds, perianal area, periorbital skin regularly

4. Infection Prevention

• Strict hand hygiene by all staff and visitors
• Isolate from children with known infections (especially chickenpox)
• Monitor temperature regularly
• Avoid live vaccines while on steroids
• Pneumococcal vaccine (Pneumovax) recommended in all children with NS
• Any febrile episode must be urgently evaluated (risk of peritonitis/sepsis)

5. Nutrition

• High protein diet - to compensate protein losses (only when proteinuria is present; high protein actually increases urinary protein losses, so this is debated - low-salt, normal-to-high protein is pragmatic guidance)
• Low sodium - reduces edema
• Low fat - to manage hyperlipidemia
• Adequate calories for growth
• Avoid concentrated sweets (steroid-induced hyperglycemia risk)

6. Medication Administration and Teaching

• Administer corticosteroids with food (reduces GI irritation)
• Never stop steroids abruptly (adrenal suppression risk)
• Monitor for steroid side effects: blood glucose, BP, mood, weight
• Educate parents on urine dipstick testing at home
• Teach signs of relapse (periorbital puffiness, weight gain, frothy urine)
• Teach signs that require emergency care (fever, abdominal pain, decreased urine output)

7. Psychosocial Care

• Body image concerns (Cushingoid features, edema)
• School attendance disruption - liaise with school
• Parental anxiety and fatigue - provide emotional support and clear education
• Emphasize that MCNS has an excellent long-term prognosis in most children
• Support groups for families with frequently-relapsing NS

8. Discharge Teaching / Parent Education

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Nursing Diagnoses (NANDA-based)

1. Excess Fluid Volume r/t decreased oncotic pressure, sodium retention
2. Risk for Impaired Skin Integrity r/t edema and immobility
3. Risk for Infection r/t immunosuppression (disease + steroids)
4. Imbalanced Nutrition: Less than body requirements r/t anorexia, dietary restrictions
5. Deficient Knowledge r/t disease process, medication management (parents)
6. Anxiety (parental) r/t child's chronic illness and relapsing course
7. Risk for Disturbed Body Image r/t edema, steroid-induced changes

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Prognosis

• MCNS: ~90% achieve remission with steroids; up to 85% achieve long-lasting remission. Frequently relapsing course in a subset, but most excellent long-term prognosis
• FSGS: <25% respond to steroids; higher risk of progression to chronic kidney disease
• Congenital NS: Poor prognosis without aggressive management; may need kidney transplantation
• A 2025 systematic review (PMID: 40600975) confirmed that kidney function outcomes and mortality risk vary significantly by underlying histology

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• Campbell-Walsh-Wein Urology, p. 459-460 (Pediatric Nephrotic Syndrome section)
• Robbins, Cotran & Kumar Pathologic Basis of Disease, p. 844 (Nephrotic Syndrome pathophysiology)
• Brenner & Rector's The Kidney (Edema mechanisms, diuretic therapy)
• Symptom to Diagnosis: An Evidence-Based Guide, 4th Ed. (Clinical consequences)