Clinicopathological study of children with idiopathic steroid insensitive nephrotic syndrome

This study was carried out on 100 children with idiopathic steroid insensitive nephrotic syndrome aged 3 to 16 years. They were 61 males and 39 females. Cases with the secondary forms of nephrotic syndrome and those who were steroid sensitive were excluded from the study. After a thorough history and clinical examination, all cases were subjected to laboratory investigations including complete urine analysis, 24 hr urinary protein estimation, serum creatinine. Plasma proteins, serum albumin, total serum cholesterol, C3, C4, and ultrasound - guided renal biopsy where kidney tissues were examined using light and immunofluorescence microscopy. Our results showed the presence of the minimal change nephrotic syndrome in 62 percent of cases [36 patients with nil disease and 26 patients with other minimal change variants], focal segmental glomerulosclerosis [FSGS] in 25 percent, diffuse mesangial proliferation in 5 percent, membranous nephropathy in 4 percent and membranoproliferative glomerulonephritis in 4 percent of cases. Patients with MCNS had significantly younger age of onset, less frequent hypertension, and lower level of serum creatinine compared to those with other renal histopathology [P < 0.01, < 0.05 and < 0.01, respectively]. Steroid resistance was significantly more frequent than steroid dependence among children with renal histopathology other than MCNS. Patients with steroid resistant nephrotic syndrome had significantly older age of onset, significantly higher frequency of hypertension, significantly higher serum creatinine, and significantly more frequent heavy proteinuria when compared with those having frequently relapsing steroid dependent nephrotic syndrome [P < 0.05, < 0.01, < 0.01, and < 0.05, respectively]