What is the treatment approach for a 7-year-old child with frequent relapsing nephrotic syndrome (nephrotic syndrome), low albumin (hypoalbuminemia), and no prior renal biopsy?

Treatment for a 7-Year-Old Child with Frequent Relapsing Nephrotic Syndrome

Immediate Management

For this 7-year-old child with frequent relapsing nephrotic syndrome (8th relapse) and severe hypoalbuminemia (albumin 1.5), rituximab should be administered as a single intravenous infusion of 375 mg/m² with appropriate premedication. 1

• First treat the current relapse with oral prednisone at 60 mg/m² per day until remission, then decrease to 40 mg/m² per day on alternate days for 1 week, followed by tapering by 10 mg/m² per week to complete a total of 4 weeks 1
• Consider albumin infusion only if there are clinical indicators of hypovolemia (such as oliguria, acute kidney injury, prolonged capillary refill time, tachycardia, hypotension, or abdominal discomfort), not solely based on the low albumin level of 1.5 2, 1
• If albumin infusion is necessary, administer furosemide at the end of the infusion to enhance diuresis 1

Rituximab Administration Protocol

• Administer rituximab as a single intravenous infusion at a dose of 375 mg/m² 1, 3
• Provide premedication with acetaminophen and an antihistamine to prevent infusion reactions 1
• Monitor closely during administration for potential infusion reactions 1
• Consider a second dose after 1-3 months if B-cell recovery occurs with early signs of relapse 1

Monitoring After Rituximab

• Check CD19/CD20 B-cell counts every 1-3 months to monitor for B-cell recovery and potential need for a second dose 1
• Monitor for proteinuria regularly to detect early signs of relapse 4
• Implement fluid and sodium restriction in combination with the pharmacological management to help control edema 5

Need for Kidney Biopsy

• A kidney biopsy should be strongly considered given the frequent relapses (8th relapse), severe hypoalbuminemia (1.5), and the absence of any previous biopsy 1
• Histological confirmation is important to rule out other glomerular pathologies and guide further therapy, especially after multiple relapses 1, 4

Alternative Therapies if Rituximab Fails or is Unavailable

• If rituximab is not available or contraindicated, consider alternative therapies in the following order of preference 1:
  1. Calcineurin inhibitors (cyclosporine or tacrolimus)
  2. Mycophenolate mofetil
  3. Cyclophosphamide
  4. Levamisole

Risk Factors and Considerations

• Several factors may contribute to the frequent relapses in this case, including 6:

  • Young age (7 years)
  • Severe hypoalbuminemia (albumin 1.5)
  • Potentially delayed response to steroid therapy in previous episodes

• The absence of a previous biopsy after 8 relapses is concerning and should be addressed to ensure appropriate management 1

Long-term Management

• After achieving remission, implement strategies to minimize treatment-related side effects while preventing relapses 4
• Consider ambulatory management when the child is stable to increase quality of life and decrease the risk of nosocomial infections 2
• Monitor for and prevent long-term complications such as metabolic bone disease, micronutrient deficiencies, and hyperlipidemia 5