Nephrotic Syndrome: Current Management.

Nephrotic syndrome (NS) is one of the most common kidney diseases in children characterised by heavy proteinuria, edema, and hypoalbuminemia. Most cases in children over one year are idiopathic with higher incidence in Asian and Black populations. The hallmark of idiopathic NS is podocyte injury. However, the pathogenesis is not completely understood and is possibly due to a complex interplay of immune dysfunction, circulating factors, podocyte factors and genetics. NS often follows a relapsing-remitting course with a subset continuing to experience relapse in adulthood. Corticosteroids are the primary treatment, and further management is guided by steroid responsiveness and relapse pattern. Children with frequently relapsing course need steroid sparing immunosuppressive medications to sustain remission and prevent steroid related toxicity. Management of steroid resistant NS remains a clinical challenge with limited therapeutic options. Those with frequently relapsing or steroid resistant NS are at risk of complications such as severe edema, infections, acute kidney injury and thromboembolism as well as medication related adverse effects. These patients require monitoring and long term follow up and should be co-managed with subspecialist. Patient and family education including training in home monitoring of proteinuria and maintaining medication records forms an integral part of management strategy. While overall long-term kidney outcome is good for steroid sensitive NS, children with steroid resistant NS who are also multidrug resistant are at high risk of disease related morbidity and progression to end stage kidney disease.