Description
Nephrotic syndrome in children is a common pediatric kidney disorder characterized by heavy proteinuria, hypoalbuminemia, edema, and hyperlipidemia. Early recognition is crucial, as children often present with puffiness around the eyes, swelling of the face and limbs, frothy urine, and unexplained weight gain due to fluid retention. Parents and clinicians should also be alert to associated symptoms such as fatigue, reduced urine output, or recurrent infections. Prompt diagnosis through urine analysis and clinical evaluation helps initiate timely treatment, most commonly with corticosteroids. Early management and regular follow-up are essential to prevent complications and ensure better long-term kidney outcomes in affected children.
Summary Listen
- Nephrotic syndrome in children is characterized by nephrotic-range proteinuria (over 40 mg/m2/hr, 100 mg/m2/day, or a protein/creatinine ratio > 2), hypoalbuminemia (albumin < 3 g/dL), and generalized edema. Hyperlipidemia, previously part of the definition, is no longer included. In children, the most common cause is idiopathic, with minimal change disease accounting for around 80% of cases. Secondary causes, seen in about 10% of cases, include systemic diseases like SLE, infections (hepatitis, malaria), and drug-induced nephrotic syndrome.
- Minimal change disease is the most prevalent variety, with normal light microscopy findings, effacement of podocyte foot processes on electron microscopy, and minimal immune complex deposition. Focal segmental glomerulosclerosis (FSGS) comprises 10-15% of steroid-resistant cases, demonstrating sclerosis in specific glomeruli segments. The exact classification of nephrotic syndrome is vital as it guides management.
- Nephrotic syndrome is categorized based on steroid responsiveness: steroid-sensitive (complete remission within six weeks of treatment), initially steroid-resistant (no remission after six weeks), and later steroid-resistant (initially sensitive, then resistant). Remission is defined by trace proteinuria or a protein/creatinine ratio below 0.2 for three consecutive early morning specimens. A partial remission is diagnosed when there is reduction in proteinuria (1+ or 2+) but doesn't disappear and the albumin is >3.0. A relapse is the reappearance of proteinuria above specified levels for three consecutive mornings.
- A frequent relapse is defined as two or more relapses in the first six months after initial therapy or three relapses in any six-month period. Steroid dependence involves two consecutive relapses within 14 days of discontinuing steroids. Stable remission is sustained remission during immunosuppressive therapy. A complicated relapse is a relapse with hypervolemia, severe infections, or thrombosis. Difficult-to-treat steroid-sensitive nephrotic syndrome includes frequent relapses, significant steroid toxicity, or failure of multiple steroid-sparing agents.
- Clinically, nephrotic syndrome typically presents insidiously with edema around the eyes and lower body. Most children appear well, although sick appearance suggests a hidden infection. Blood pressure is usually normal. Differentiation from post-streptococcal glomerulonephritis and acute post-streptococcal glomerulonephritis is vital, particularly considering hematuria, hypertension, and life-threatening complications like acute kidney injury.
- Initial investigations for nephrotic syndrome include a complete blood count (CBC), urine examination, serum protein, lipid profile, electrolytes, and a tuberculin test. Additional evaluations involve chest X-rays (for respiratory infections), renal ultrasounds (for gross hematuria), and serum electrolytes (particularly after diuretic therapy). A kidney biopsy is rarely needed in children but is indicated in persistent macroscopic hematuria, acute kidney injury, systemic features, or steroid resistance.
- Prednisolone (60 mg/m2/day or 2 mg/kg/day, max 60 mg) is the cornerstone of initial nephrotic syndrome treatment, followed by 40 mg/m2 on alternate days for six weeks, for a total of 12 weeks. In frequent relapses or steroid dependency, prednisone is combined with steroid-sparing agents like levamisole, cyclophosphamide, cyclosporine, tacrolimus, or rituximab. Prednisone is usually administered at .5 to .7 mg on alternate days for 6-12 months. Close monitoring for steroid toxicity is crucial.
- Steroid toxicity can manifest as hyperglycemia, obesity, short stature, increased intraocular pressure, cataracts, myopathy, osteonecrosis, and psychosis. Edema management in nephrotic syndrome starts with ruling out hypovolemia. Mild edema is managed with salt restriction, whereas severe edema warrants diuretics (furosemide, spironolactone) and potentially albumin infusions followed by furosemide.
- Infections are a significant threat in nephrotic syndrome. Suspect peritonitis in patients presenting with fever or abdominal pain. Prophylactic antibiotics (ceftriaxone, cefotaxime) are given based on the diagnosis. Varicella infection requires immediate treatment with acyclovir.
- Vaccination guidelines are important for immunocompromised patients with nephrotic syndrome. Live vaccines are generally avoided during high-dose steroid therapy. Specific vaccines, like pneumococcal and influenza vaccines, are recommended. Varicella exposure needs prompt post-exposure prophylaxis with varicella-zoster immune globulin (VZIG) and/or acyclovir.
- Parental counseling is vital to ensure treatment adherence and recognize potential complications. Parents need to understand the disease process, the importance of steroid therapy, the potential side effects, and the need for regular monitoring. Maintaining a nephrotic syndrome diary aids in tracking symptoms and treatment response.
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About the Speakers
Dr. Baldev Prajapati
Senior Consultant Pediatrician, Akanksha Children Hospital, Ahmedabad
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