The significance of focal global sclerosis in idiopathic nephrotic syndrome. Long-term clinical observations.

The clinical course of five pediatric patients with idiopathic nephrotic syndrome associated with focal global sclerosis in renal biopsy was analyzed retrospectively. The condition was defined as complete obsolescence of more than 15% of glomeruli associated with tubular atrophy and interstitial fibrosis. At onset, all children had a steroid-sensitive relapsing nephrotic syndrome. Two became steroid-dependent and one steroid-resistant. Cyclophosphamide or chlorambucil treatment was initially followed by remission in all patients; a second course was successful in one of two patients. After 12 to 18 years of observation, four patients are in remission for periods of 2 to 12 years. Glomerular filtration rate is slightly reduced in only one patient. This study suggests that the clinical course of children with idiopathic nephrotic syndrome associated with focal global sclerosis is similar to that observed in patients with minimal glomerular changes but different from that in patients with focal segmental sclerosis.