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Low-dose methylprednisolone pulse therapy in Chinese children with steroid resistant focal segmental
Jei-Wen Chang1, Ling-Yu Yang, Hsin-Hui Wang
1Department of Pediatrics, Taipei Veterans General Hospital, Taipei, Taiwan.
Insights
Low-dose pulse methylprednisolone therapy (PMT) effectively reduced proteinuria in children with steroid-resistant focal segmental glomerulosclerosis (FSGS). This treatment offers a promising alternative with fewer adverse effects for pediatric kidney disease.
Area of Science:
- Pediatric Nephrology
- Glomerular Diseases
- Immunosuppressive Therapy
Background:
- Focal segmental glomerulosclerosis (FSGS) is a leading cause of pediatric renal failure.
- High-dose pulse methylprednisolone therapy (PMT) is effective but has significant adverse effects.
- Parental concerns regarding adverse effects often lead to treatment hesitation.
Purpose of the Study:
- To evaluate the efficacy of a low-dose PMT protocol in treating steroid-resistant FSGS in children.
- To assess the potential of low-dose PMT to induce proteinuria remission and slow renal insufficiency progression.
- To determine the safety and tolerability profile of low-dose PMT in pediatric FSGS.
Main Methods:
- Retrospective study of eight children with steroid-resistant FSGS.
- Treatment involved intravenous methylprednisolone pulse (10 mg/kg/day for 3 consecutive days weekly for 8 weeks).
- Partial responders received additional immunosuppressants (chlorambucil or cyclosporine) and high-dose PMT pulses.
Main Results:
- Six of eight patients achieved complete remission initially.
- Median 24-hour urinary protein excretion decreased significantly from 4.25 to 0.39 g (P=0.012).
- Serum albumin levels increased significantly (median 3.35 to 4.1 mg/dL, P=0.018); two patients with partial remission progressed.
Conclusions:
- Low-dose PMT significantly reduced proteinuria in Chinese children with steroid-resistant FSGS.
- The low-dose protocol demonstrated a low frequency of intolerance.
- This approach may offer a safer alternative for managing pediatric steroid-resistant FSGS.
Background:
Focal segmental glomerulosclerosis (FSGS) is a primary glomerular disease that usually progresses to renal failure. Although high-dose pulse methylprednisolone therapy (PMT) has been shown to be effective in the treatment of steroid-resistant FSGS, adverse effects have caused parents to hesitate in approving the treatment. The aim of this study is to investigate whether low-dose PMT based protocol for treatment of young children with steroid resistant FSGS would effectively induce remission of proteinuria and prevent the progression of renal insufficiency.
Methods:
This is a retrospective study. The authors treated eight children with steroid-resistant FSGS with intravenous methylprednisolone pulse 10 mg/kg per day for three consecutive days weekly for 8 weeks. Partial responders were treated with the addition of chlorambucil or cyclosporine (CsA) and four fortnightly and eight monthly pulses of high-dose PMT (30 mg/kg per day).
Results:
Of the eight patients, six attained complete remission initially. The median urinary protein excretion in 24 h decreased from 4.25 to 0.39 g following 8 weeks of low dose (P = 0.012). Marked decrease in urinary protein-creatinine ratio was noted soon after treatment (P = 0.012). There was a significant increase in serum albumin level after treatment compared to the pretreatment condition (median, 3.35 vs 4.1 mg/dL, P = 0.018). Five of the eight patients remained in complete remission, and one of the eight patients relapsed during follow up. Relapse responded to repeated treatments of PMT and cyclosporine. The two patients with partial remission initially progressed to renal insufficiency in one patient and end-stage renal disease in the other patient.
Conclusions:
Low-dose PMT caused a significant decrease in the proteinuria of Chinese children with steroid-resistant FSGS with a low frequency of intolerance.
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