Cyclosporin A Therapy in Childhood Nephrotic Syndrome
Cyclosporin A Therapy in Childhood Nephrotic Syndrome
Nephrotic syndrome (NS) is among the most common pediatric kidney diseases and is defined as
 massive proteinuria (>40 mg/m2/h or urine protein to creatinine ratio >2 g/g) leading to
 hypoalbuminemia (<2.5 g/dL), edema, and hyperlipidemia. 60-70 % of patients present prior to
 age of 6 years
. Most children with NS are treated initially with oral corticosteroids, and they can be
 clinically classified based on their ability to achieve remission (i.e., complete
 normalization of proteinuria). Approximately 85 % of children under the age of 6 years are
 steroid-sensitive, whereas the remainder have steroid-resistant disease. Older children are
 more likely to have steroid-resistant NS. Children with steroid-resistant disease may have an
 underlying genetic cause for NS, and providers should consider genetic testing in this
 population, depending on the age of the child . While inherited causes of NS are often
 resistant to all therapies, there are reports of complete or partial remission in some
 children .

 For those children who respond to steroids, the majority will have one or more relapses and
 half will have frequently relapsing (≥4 relapses/year) or steroid-dependent (two consecutive
 relapses during steroid therapy or within 14 days of stopping steroids). NS Children with
 frequently relapsing NS and steroid-dependent NS may have significant side effects from
 cumulative corticosteroid therapy so treatment with other agents is often required .

 Cyclosporine and tacrolimus are calcineurin inhibitors that are commonly used as
 immunosuppressive agents in solid organ transplantation. CNIs are recommended as first-line
 therapy for children with steroid-resistant NS and as steroid-sparing agents for children
 with frequently relapsing or steroid-dependent NS .Calcineurin inhibitors (CNIs) inhibit
 T-cell activation and may be exerting their effect in nephrotic syndrome through this
 mechanism.

 Alternately, cyclosporine has been shown to directly target the podocyte and stabilize the
 actin cytoskeleton responsible for maintaining cell shape(5) .. Although the majority of
 studies in nephrotic syndrome have been performed with cyclosporine, tacrolimus appears to be
 equally efficacious.

 Cyclosporin A therapy is well recognised regarding its steroid sparing effect in steroid
 dependant patients and is responsible for maintaining remission in more than 75% of patients
 with Steroid dependent nephrotic syndrome even after discontinuation of steroids Furthermore,
 it has been shown to be effective in inducing remission in steroid resistant nephrotic
 syndrome. However ,Cyclosporin A is associated with a plethora of side effects such as
 hypertension, nephrotoxicity hypertrichosis, gum hyperplasia, gastrointestinal disturbances
 and tremor.
Inclusion Criteria:

 -  The study will include all children (on cyclosporine therapy for treatment of Steroid
 dependent or Steroid resistant nephrotic syndrome ) who present to Nephrology Unit ,
 and clinic at Assiut University Children Hospital during one year duration.

 Exclusion Criteria:

 -  children with adequate response to steroid therapy and without relapses or resistance
 to steroid therapy