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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.