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Idiopathic membranoproliferative glomerulonephritis in Japanese children
1Department of Paediatrics, Kitasato University School of Medicine, Kanagawa, Japan.
Insights
Early detection and treatment of idiopathic membranoproliferative glomerulonephritis (MPGN) in Japanese children led to favorable outcomes. High-dose alternate-day prednisolone showed the highest remission rates for urinary abnormalities.
Area of Science:
- Pediatric Nephrology
- Glomerular Diseases
- Renal Pathology
Background:
- Idiopathic membranoproliferative glomerulonephritis (MPGN) is a rare kidney disease.
- Early detection is crucial for managing MPGN in pediatric populations.
- School urinary screening plays a vital role in identifying asymptomatic cases.
Purpose of the Study:
- To report the long-term course of idiopathic MPGN in Japanese children.
- To evaluate the effectiveness of different treatment regimens.
- To assess histological and clinical outcomes.
Main Methods:
- Retrospective analysis of 41 Japanese children with MPGN.
- Follow-up period averaged 8 years and 9 months.
- Treatment groups included multiple low-dose cocktail therapy (MLD), MLD followed by alternate-day prednisolone (ALD), and ALD alone.
Main Results:
- 71% of MPGN cases were detected through school urinary screening.
- High-dose ALD prednisolone resulted in the highest remission rates for urinary abnormalities.
- 33% of patients showed histological improvement after rebiopsy, with few side effects observed.
Conclusions:
- Early detection and prompt treatment contribute to better outcomes in pediatric MPGN.
- High-dose alternate-day prednisolone appears effective in achieving remission.
- MPGN management in children requires a multidisciplinary approach.
Abstract:
The course of idiopathic membranoproliferative glomerulonephritis (MPGN) in 41 Japanese children (21 boys, 20 girls) is reported. The mean follow-up period was 8 years, 9 months; 29 children with MPGN (71%) were identified by school urinary screening; 32 patients had type I MPGN, 2 type II and 7 type III; 10 patients were treated with multiple low-dose cocktail therapy (MLD), 8 with MLD followed by high-dose alternate-day (ALD) prednisolone and 21 with high-dose ALD prednisolone alone. In 1 patient, MPGN progressed to end-stage renal failure. The serum creatinine level in all of the remaining 40 patients was < or = 1.3 mg/dl at the last follow-up. Urinalysis was normal in 24 (59%). Of the 17 patients who still had urinary abnormalities, 4 had nephrotic syndrome. The incidence of remission of urinary abnormalities was highest in the patients treated with high-dose ALD prednisolone. Rebiopsy was performed in 33 patients, and revealed slight histological improvement in 11 (33%) patients, moderate improvement in 8 (24%), marked improvement in 5 (15%) and deterioration or no improvement in 9 patients (27%). Relatively few side effects of treatment were observed. The superior outcome of the MPGN patients in this compared with other studies may be the result of earlier detection and treatment.