Idiopathic nephrotic syndrome in children
Damien G Noone1, Kazumoto Iijima2, Rulan Parekh3
1Department of Paediatrics, University of Toronto, Toronto, ON, Canada; Division of Nephrology, The Hospital for Sick Children, Toronto, ON, Canada.
Lancet (London, England)
|June 19, 2018
Summary
Idiopathic nephrotic syndrome (NS) in children has varying incidence and unknown causes, often involving immune issues or genetic factors. While steroid-responsive NS has an excellent prognosis, steroid resistance indicates a higher risk of kidney disease.
Area of Science:
- Pediatric Nephrology
- Immunology
- Genetics
Background:
- Idiopathic nephrotic syndrome (NS) affects 1.15-16.9 per 100,000 children, with incidence varying by ethnicity and region.
- Pathogenesis is complex, potentially involving immune dysregulation, circulating factors, or podocyte abnormalities.
- Genetic factors are more implicated in steroid-resistant NS.
Purpose of the Study:
- To summarize the current understanding of idiopathic nephrotic syndrome in children.
- To highlight key aspects of its pathogenesis, treatment, complications, and prognosis.
Main Methods:
- Review of existing literature on idiopathic nephrotic syndrome.
- Analysis of epidemiological data, pathogenic mechanisms, therapeutic approaches, and prognostic factors.
Main Results:
- The primary treatment is prednisone for steroid-responsive cases.
- Frequent relapses may necessitate alternative immunosuppressive agents.
- Key complications include infection, venous thromboembolism, and acute kidney injury.
Conclusions:
- Steroid-responsive NS generally has an excellent long-term kidney outcome.
- Steroid resistance is a critical determinant for the future risk of chronic or end-stage kidney disease.
- Further research into pathogenesis and treatment is warranted for steroid-resistant cases.
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