Predictors of Steroid Response in Childhood Nephrotic Syndrome

Nephrotic syndrome is the most common glomerular disease affecting children worldwide ,Nephrotic syndrome is the combination of nephrotic-range proteinuria with a low serum albumin level and edema. Nephrotic-range proteinuria is the loss of 3 grams or more per day of protein into the urine or, on a single spot urine collection, the presence of 2 g of protein per gram of urine creatinine. Minimal change disease (MCD) or minimal change glomerulonephritis and focal segmental glomerulosclerosis (FSGS) are the two major causes of nephrotic syndrome in children and young adults, Idiopathic, hereditary, and secondary forms are due to underlying increased protein leakiness across the glomerular capillary wall, as a result of immune and non-immune insults affecting the podocyte.

The incidence worldwide varies widely between 1.2 and 16.9 cases per 100,000 children with the highest incidence observed on the Indian subcontinent compared to 2-3 cases per 100,000 children in most other regions . Males appear to be more affected than females at a ratio of 2:1 at a younger age, but this predominance fails to persist in adolescence.

treatment of nephrotic syndrome:- Corticosteroids have been used to treat childhood nephrotic syndrome since 1950 when large doses of adrenocorticotrophic hormone (ACTH) and cortisone given for two to three weeks were found to induce diuresis with loss of oedema and proteinuria. The response to treatment with steroids has been shown to vary by ethnicity, likely due to environmental and genetic factors. Corticosteroid usage has reduced the mortality rate in childhood nephrotic syndrome to around 3%, with infection remaining the most important cause of death. Of children who present with their first episode of nephrotic syndrome, approximately 80% will achieve remission with corticosteroid Prednisolone is the mainstay treatment, whose response is often presumed to determine the long-term risk of disease progression and is a better prognostic indicator . Although patients with steroid-sensitive nephrotic syndrome generally have good outcomes, more than half will have a frequently relapsing course and steroid dependence. Up to 95% of patients with minimal change nephrotic syndrome (MCNS) attain complete remission after an 8-week course of high dose steroids there are some factors affecting response to treatment with the steroids such as age of onset (the older the age of onset the better the response) ,gender (the females respond more to treatment) and type of nephrotic syndrome( minimal change glomerulonephritis responds more than focal segmental glomerulosclerosis to steroids ).