Nephrotic Syndrome: Treatment and Management

• This text discusses nephrotic syndrome, its treatment, and potential complications.

General Information

• Initial treatment includes 60mg of medication or 2mg/kg for 1 month
• Followed by either 60mg or 2mg/kg every 2 days for 2 months, then a gradual decrease of 0.5mg/kg every 15 days.
• The total duration of corticoid treatment is 4.5 months
• Anti-pneumococcal vaccination is recommended.
• Antibiotics should only be used if an infection is present.
• Advise parents about the risk of varicella (chickenpox) transmission.

Specific Treatments

• Vitamin D supplementation, 400-800 units daily, is advised.
• Calcium supplementation may be necessary depending on dietary intake.
• A calcium-rich diet (dairy products) is generally recommended.
• Calcium carbonate supplements can be administered at 30-50 mg/kg daily.
• A potassium-rich diet (bananas, dried fruits, chocolate) or potassium chloride supplementation may be necessary.
• Sodium restriction may be warranted

Symptomatic treatments

• Bed rest should be avoided due to the risk of thrombosis.
• Maintain a normal protein intake through diet.
• Restrict fluids if hyponatremia (low sodium) is present, specifically if it's below 125mmol/L.
• Diuretics should be used cautiously due to the risk of complications like collapse and thromboembolism.
• Only use diuretics in cases of significant and poorly tolerated edema.
• Albumin infusions are indicated in cases of collapse or hypovolemia.
• 20% human albumin should be administered by electric syringe over 1-2 hours.
• Closely monitor arterial pressure during infusion.

Thrombosis prevention

• Mobilize patients and discourage bed rest.
• Correct hypovolemia and hemoconcentration.
• Avoid deep artery and vein punctures/perfusion.
• Avoid central catheters and unnecessary intravenous infusions.

Complications

• The main complication is infections, often bacterial (peritonitis, meningitis, pneumonia, UTI).
• Common pathogens involved are pneumococcus and streptococcus.
• Abdominal pain could indicate peritonitis, ulcer due to corticotherapy, pancreatitis, or renal vein thrombosis
• Collapses can be due to hypovolemia during severe episodes, sodium depletion from diuretics, etc.
• Acute renal failure is generally reversible.
• Thromboembolic complications are secondary.
• Complications from corticotherapy include impact on growth, obesity and osteoporosis.

Paraclinical Assessment

• Assess the intensity and risks of complications.
• Check for lipid disorders
• Assess coagulation factors for hypercoagulability
• Assess kidney function, which is usually normal but can have functional renal impairment
• Assess hydro-electrolyte balance
• Serum calcium can be normal or decreased due to hypoalbuminemia.
• Potassium levels in urine are normal unless there is significant oliguria

Paraclinical Assessment

• Assess hematologic parameters
• Patients may have thrombocytosis, leukocytosis, and mild microcytic anemia.
• Total complement fraction levels are usually normal.
• Kidney biopsy may be necessary in specific cases (<1 year or >10 years old).

Nephrotic Syndrome Definition

• Idiopathic or lipoid nephrosis is a pure & primitive
• Hematuria and high blood pressure is absent
• AAN counts are negative, HBS and HCV serology with ASLO
• Common at 2-12 years old
• More common in males (2:1)

Pathophysiology

• Increased glomerular permeability
• Loss of albumin
• Low blood volume stimulates aldosterone