Nephrotic Syndrome (NS) is a common renal disease seen
in children. Children who go into complete remission
following treatment with corticosteroids are classified as
having steroid sensitive NS. In developed countries over
80% of children with idiopathic NS have steroid sensitive
disease. The exact pathogenesis of this condition remains
elusive. Podocyte injury and proteinuria are the two main
issues in the pathogenesis. Recent studies suggest release of
cytokines by T-cells as well as a strong contribution of Bcell
immunity. Genetic studies have reported human
leucocyte antigen (HLA) class II antigens DR and DQ
associations linked to steroid sensitive NS. Most children
with steroid sensitive NS have multiple relapses and a
significant percentage also develop steroid dependent NS.
Diuretic- resistant edema also a clinical problem to manage
these patients. These children receive multiple courses of
steroids and are at high risk of developing steroid toxicity.
Patient with frequent relapses who develop steroid
dependency thus require alternative treatment. Steroid
resistant NS considers when failure to response within 8
weeks of steroid therapy. Steroids sparing agents used
include levamisole, cyclophosphamide, mycophenolate
mofetil (MMF), calcineurin inhibitors (cyclosporine and
tacrolimus), rituximab and vincristine; these agents have
greatly reduced the adverse effects seen with long-term use
of steroids; so therapy needs to be individualized to achieve
optimal care of each child.
Keywords: Nephrotic Syndrome( NS),Relapse ,Steroid
sensitive, Steroid dependency, Steroid sparing agents.
(J Banagladesh Coll Phys Surg 2016; 34: 26-32)