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Lancet. 2018 Jul 7;392(10141):61-74. doi: 10.1016/S0140-6736(18)30536-1. Epub 2018 Jun 14.

Idiopathic nephrotic syndrome in children.

Author information

1
Department of Paediatrics, University of Toronto, Toronto, ON, Canada; Division of Nephrology, The Hospital for Sick Children, Toronto, ON, Canada.
2
Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan.
3
Department of Paediatrics, University of Toronto, Toronto, ON, Canada; Division of Nephrology, The Hospital for Sick Children, Toronto, ON, Canada; Child Health Evaluative Sciences, Research Institute, Hospital for Sick Children, Toronto, ON, Canada; University Health Network, Toronto, ON, Canada; Dalla Lana School of Public Health, and Health Policy, Management and Evaluation, University of Toronto, Toronto, ON, Canada. Electronic address: rulan.parekh@sickkids.ca.

Erratum in

Abstract

The incidence of idiopathic nephrotic syndrome (NS) is 1·15-16·9 per 100 000 children, varying by ethnicity and region. The cause remains unknown but the pathogenesis of idiopathic NS is thought to involve immune dysregulation, systemic circulating factors, or inherited structural abnormalities of the podocyte. Genetic risk is more commonly described among children with steroid-resistant disease. The mainstay of therapy is prednisone for the vast majority of patients who are steroid responsive; however, the disease can run a frequently relapsing course, necessitating the need for alternative immunosuppressive agents. Infection and venous thromboembolism are the main complications of NS with also increased risk of acute kidney injury. Prognosis in terms of long-term kidney outcome overall is excellent for steroid-responsive disease, and steroid resistance is an important determinant of future risk of chronic or end-stage kidney disease.

PMID:
29910038
DOI:
10.1016/S0140-6736(18)30536-1
[Indexed for MEDLINE]

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