NCLEX Review Guide: Nephrotic Syndrome in Children

Definition and Mechanism

• Nephrotic syndrome is characterized by increased glomerular permeability resulting in massive proteinuria, hypoalbuminemia, edema, and hyperlipidemia. The primary defect occurs in the glomerular filtration barrier, specifically in the podocytes that maintain the selective permeability of the glomerular membrane.
• In children, minimal change disease (MCD) accounts for approximately 85% of cases, with focal segmental glomerulosclerosis (FSGS) and membranoproliferative glomerulonephritis (MPGN) being less common etiologies.

Pathophysiological Changes

• The loss of albumin in urine leads to decreased plasma oncotic pressure, resulting in fluid shifting from intravascular to interstitial spaces, causing edema. The kidneys respond by retaining sodium and water, further exacerbating the edema.
• Hyperlipidemia occurs due to increased hepatic synthesis of lipoproteins and decreased lipid catabolism, which is a response to hypoalbuminemia.

Primary Symptoms

• Edema is typically the first noticeable sign, beginning periorbital (especially in the morning) and progressing to dependent areas like the lower extremities, genitalia, and abdomen (ascites). The edema is pitting and may become generalized in severe cases.
• Children often present with weight gain, lethargy, decreased appetite, and irritability. Urine may appear foamy or frothy due to high protein content.

Complications

• Children with nephrotic syndrome are at increased risk for infections, particularly pneumococcal infections, due to urinary loss of immunoglobulins and complement factors. Thromboembolism is another serious complication resulting from urinary loss of anticoagulant proteins and increased platelet aggregation.
• Acute kidney injury may develop in some cases, especially with severe hypovolemia or interstitial nephritis from medication side effects.

Laboratory Findings

• Urinalysis reveals significant proteinuria (3+ to 4+ on dipstick), with urine protein:creatinine ratio >2.0 mg/mg. Microscopic hematuria may be present in some cases, but gross hematuria is uncommon and suggests a different diagnosis.
• Blood tests show hypoalbuminemia (<3 g/dL), hyperlipidemia (elevated cholesterol and triglycerides), and possible elevated BUN and creatinine if kidney function is affected.

Diagnostic Procedures

• Renal biopsy is not routinely performed in children with typical presentation and response to steroids. It is reserved for steroid-resistant cases, atypical presentations, or when secondary causes are suspected.
• Complement levels (C3, C4) are typically normal in minimal change disease but may be decreased in other forms of nephrotic syndrome.

Pharmacological Management

• Corticosteroids (prednisone or prednisolone) are the first-line treatment, typically started at 2 mg/kg/day (max 60 mg/day) for 4-6 weeks, followed by alternate-day therapy for 2-5 months with gradual tapering. Most children with minimal change disease respond within 1-2 weeks.
• For steroid-dependent or frequently relapsing cases, immunosuppressive agents such as cyclophosphamide, cyclosporine, tacrolimus, or mycophenolate mofetil may be used as steroid-sparing agents.

1. Begin prednisone at 2 mg/kg/day (max 60 mg/day) for 4-6 weeks
2. Transition to alternate-day therapy at 1.5 mg/kg/dose
3. Gradually taper over 2-5 months
4. Monitor for remission (urine protein negative or trace for 3 consecutive days)
5. For relapse, restart daily steroid therapy until remission, then return to alternate-day regimen

Supportive Management

• Sodium restriction (1-2 g/day) and fluid restriction may be necessary during periods of edema. Diuretics, particularly loop diuretics like furosemide, may be used cautiously to manage severe edema, but should be used with caution due to risk of hypovolemia.
• Albumin infusions (25%, 1 g/kg) may be given for severe edema, hypovolemia, or to prepare for diuretic administration. These are typically followed by furosemide to prevent fluid overload.

Preventive Measures

• Pneumococcal vaccination is recommended due to increased risk of infection. Some children may require prophylactic antibiotics (penicillin) during periods of significant edema or relapse.
• Monitoring for complications such as thromboembolism is essential, especially in children with severe disease or prolonged immobility.

Assessment

• Perform daily weight measurements at the same time, using the same scale, to monitor fluid status. Weight gain or loss of 0.5-1 kg can represent approximately 500-1000 mL of fluid.
• Monitor strict intake and output, assess for edema distribution and severity, and evaluate respiratory status for signs of pulmonary edema or pleural effusions.

Nursing Interventions

• Administer medications as prescribed, ensuring steroids are given with food to reduce gastric irritation. Monitor for side effects of steroid therapy, including mood changes, increased appetite, cushingoid features, hypertension, and hyperglycemia.
• Provide meticulous skin care, especially in edematous areas, to prevent skin breakdown. Elevate edematous extremities when possible and reposition frequently.

Patient and Family Education

• Teach families about the importance of medication adherence, especially the steroid tapering schedule. Abrupt discontinuation can lead to relapse or adrenal crisis.
• Educate about dietary modifications, including sodium restriction during edematous phases and adequate protein intake (but not excessive) to replace urinary losses.

Nephrotic vs. Nephritic Syndrome

Primary vs. Secondary Nephrotic Syndrome

• Primary (idiopathic) nephrotic syndrome is not caused by another disease and includes minimal change disease, focal segmental glomerulosclerosis, and membranous nephropathy. In children, minimal change disease accounts for approximately 85% of cases.
• Secondary nephrotic syndrome results from systemic diseases such as lupus nephritis, diabetes mellitus, amyloidosis, or medication reactions. These are less common in children but more prevalent in older children and adolescents.

Key NCLEX Focus Areas

• Focus on recognizing the classic tetrad of nephrotic syndrome: massive proteinuria, hypoalbuminemia, edema, and hyperlipidemia. Questions often ask about assessment findings, complications, and nursing interventions.
• Understand the rationale behind treatments, especially corticosteroid therapy, sodium restriction, and albumin infusions. Be prepared to identify appropriate nursing interventions for managing edema and preventing complications.

Summary of Nursing Priorities

• Monitor for complications, especially infections and thromboembolism. Assess vital signs, edema distribution, and urine output regularly.
• Administer medications correctly and monitor for side effects. Provide comprehensive education to the child and family about the disease process, medication regimen, and when to seek medical attention.