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Clinical course and outcome of idiopathic membranous nephropathy in Japanese children
H Tsukahara1, Y Takahashi, M Yoshimoto
1Department of Paediatrics, Fukui Medical School, Japan.
Insights
Idiopathic membranous nephropathy (IMN) in Japanese children shows a better prognosis compared to non-Japanese children. Early steroid therapy may improve remission rates and renal function preservation in pediatric IMN.
Area of Science:
- Pediatric Nephrology
- Immunology
- Glomerular Diseases
Background:
- Idiopathic membranous nephropathy (IMN) is a primary glomerular disease.
- Outcomes in pediatric IMN can vary significantly across different ethnic groups.
- Understanding ethnic differences in IMN is crucial for tailored treatment strategies.
Purpose of the Study:
- To investigate the clinical characteristics and outcomes of idiopathic membranous nephropathy in Japanese children.
- To compare the course and prognosis of pediatric IMN between Japanese and non-Japanese children.
- To evaluate the effectiveness of steroid therapy in Japanese pediatric patients with IMN.
Main Methods:
- Retrospective study of 12 Japanese children diagnosed with IMN.
- Analysis of clinical data including age at onset, urinalysis findings, nephrotic syndrome (NS) presence, and electron microscopy stages.
- Review of treatment regimens, primarily steroids alone or with cyclophosphamide.
- Comparative analysis with previously reported non-Japanese pediatric IMN cases.
Main Results:
- Proteinuria was present in all 12 Japanese children; 3 had nephrotic syndrome at onset.
- Complete remission of proteinuria was achieved in 8 patients within a mean of 0.6 years.
- After a mean follow-up of 5.9 years, all patients were in remission or had mild proteinuria, with no hypertension or impaired renal function.
- Comparative analysis suggested a better outcome in Japanese children, potentially linked to more effective steroid therapy.
Conclusions:
- Idiopathic membranous nephropathy in Japanese children appears to have a favorable course and outcome.
- Steroid therapy may be particularly effective in inducing remission and preserving renal function in this population.
- Further research is warranted to confirm these ethnic differences and optimize treatment protocols for pediatric IMN.
Abstract:
We retrospectively studied 12 Japanese children (8 boys, 4 girls) with idiopathic membranous nephropathy (IMN), aged 2.9-15.8 (mean 7.7) years at onset. All patients were identified through either screening or a routine urinalysis; proteinuria was present in all, haematuria, which was macroscopic in 4, in 11. Three had nephrotic syndrome (NS) at or soon after onset. Stages on electron microscopy, performed in 10 patients, were I in 3, II in 5 and III in 2. Steroids alone or with cyclophosphamide were administered to 5 patients, including the 3 patients showing NS. Complete remission of proteinuria occurred in 8 patients 0.3-1.6 (mean 0.6) years after onset, and proteinuria did not recur. After a follow-up of 1.6-11.6 (mean 5.9) years, these 8 patients were in complete remission and the remaining 4 had only mild proteinuria; none had hypertension or impaired renal function. Thus, we infer that IMN in Japanese children may have a better course and outcome than IMN in non-Japanese children. Based on a comparative study of Japanese (previously reported cases added to ours) and non-Japanese (mostly Caucasian) children with IMN, this was confirmed; it is possible that steroid therapy in Japanese patients is more effective in inducing remission of NS and preserving renal function.