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Difficult-to-treat nephrotic syndrome: management and outcome
Achra Sumboonnanonda1, Nawarat Chongchate, Vibul Suntornpoch
1Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand. siasb@mahidol.ac.th
Summary
Cyclophosphamide is recommended as a first-line treatment for difficult-to-treat nephrotic syndrome, showing better remission rates. Enalapril may offer benefits, while thrombocytosis is linked to poorer treatment responses.
Area of Science:
- Pediatric Nephrology
- Immunology
Background:
- Idiopathic nephrotic syndrome (INS) presents challenges in management, particularly steroid-dependent, steroid-resistant, and frequent relapse subtypes.
- Management strategies for difficult-to-treat INS require ongoing investigation to optimize patient outcomes.
Purpose of the Study:
- To evaluate the efficacy of cyclophosphamide and enalapril in patients with difficult-to-treat idiopathic nephrotic syndrome.
- To compare treatment outcomes across different therapeutic regimens and identify factors influencing remission.
Main Methods:
- A retrospective study of 68 pediatric patients with INS treated between 1996 and 2004.
- Analysis of treatment regimens including cyclophosphamide, enalapril, and combination therapy, with comparison of remission rates using chi-square test.
Main Results:
- Cyclophosphamide monotherapy demonstrated significantly better remission rates (p = 0.014) compared to other regimens.
- Remission at one year was achieved in 70% of patients receiving cyclophosphamide, with prednisolone discontinuation in 52%.
- Complications included hypertension (44%), cataracts (40%), and recurrent infections (69%).
Conclusions:
- Cyclophosphamide is suggested as a first-line agent for difficult-to-treat INS.
- Enalapril may provide benefits in select cases, and thrombocytosis appears to correlate with a poor treatment response.
- Long-term follow-up is crucial for monitoring complications associated with the disease and its treatment.