Childhood idiopathic nephrotic syndrome in Turkey

BACKGROUND: It has been reported that there are racial and regional differences in peak incidence age, histopathological features and response to steroid therapy in childhood idiopathic nephrotic syndrome. METHODS: One hundred and thirty-eight patients with a diagnosis of idiopathic nephrotic syndrome, followed up in 1994-2000, were assessed retrospectively. The aim of this study was to assess the patients' response pattern to steroid therapy, to determine whether the duration of the initial steroid therapy alters the steroid response pattern of the disease and to assess renal biopsy results. RESULTS: One hundred and fourteen patients who initially received only steroid therapy and were followed up regularly were classified according to response pattern. Of the 114 patients, 30 children had an initial response, 25 children had infrequent relapse, 19 had frequent relapse, 25 had steroid dependence and 15 children had steroid resistance. The 99 patients with steroid responsive nephrotic syndrome were divided into two groups with respect to duration of the initial steroid therapy. There was no statistically significant difference between standard and short therapy groups with respect to the steroid response patterns. Percutaneous renal biopsy was performed in 43 of the 138 patients. Mesengioproliferative glomerulonephritis was the most common histopathological lesion, followed by membranoproliferative glomerulonephritis. The proportions of membranous glomerulonephritis, focal segmental glomerulosclerosis and minimal change nephrotic syndrome were low in our group. CONCLUSIONS: Our study group is similar to one reported from Saudi Arabia with respect to the steroid response pattern and to Saudi Arabian and Nigerian reports with respect to the histopathology. Although it has been reported that short initial steroid therapy was followed by a higher rate of relapses, there was no statistically significant difference between standard and short therapy groups with respect to the relapse rate in our study group.