Risky Vitria Prasetyo
Division of Nephrology, Department of Child Health, Medical Faculty of Airlangga UniversityDrSoetomo Hospital Surabaya
Nephrotic syndrome is the most common glomerular disorder of childhood and an important chronic disease in children. Its main character is massive leakage of protein from the blood into the urine through damaged glomeruli. Management of children with nephrotic syndrome is challenging.More than two thirds children with idiopathic nephrotic syndrome show remission of proteinuria following treatment with corticosteroids and are classified as steroid sensitive. However, most patients experience multiple episodes of relapse with the consequence of being at risk for steroid toxicity, systemic infections, and other complications. These risks also occur in a small proportion of patients who are resistant to steroids, with long term effect in renal insufficiency.
Immune dysregulation has been invoked as the major cause of steroid sensitive nephrotic syndrome with the concept of damaged podocyte as a secondary event in a primarily immune disease. The overall mortality of idiopathic nephrotic syndrome has decreased dramatically from over 50% to approximately 2-5%. Nevertheless, despite the improvement in survival, the episodes of relapse may continue beyond adolescence into adulthood that require additional steroid-sparing medications in customized approach based on clinical condition of each child.
Overall, the prognosis of nephrotic syndrome is excellent, with less than 5% rapidly progressing to end-stage renal disease. Most pediatricians would encounter patients with nephrotic syndrome in their practice. Therefore they should be familiar with management of these patients and be aware of situations in which referral to a pediatric nephrologist is required. Long-term management of these patients should thereafter be a joint effort between the pediatrician and the pediatric nephrologist.
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