PROGNOSIS OF GLOMERULAR FOCAL SCLEROSIS IN CHILDREN

Abstract

It is generally stated that focal sclerotic lesions of the glomeruli are associated with a poor prognosis. Nodistinction is usually mode between focal global sclerosis (complete hyalinization of affected glomeruli) and focal segmental sclerosis (hyalinization of only one area of the affected glomeruli). Seventeen children with focal sclerosis were surveyed to determine whether the type of lesion has prognostic significance. Four children, 3 male 1 female, presenting with nephrotic syndrome (NS) at the age of 13 to 19 months, were found to have focal global sclerosis. They all responded to prednisone therapy, relapsed frequently, and have normal renal function 9 to 14 years later. The 13 children with focal segmental sclerosis ranged in age from 1½ to 13 years; 7 were male and 6 female. Eleven presented with NS. Of these, 2 have died, 2 have received renal transplants, and 5 have mild to moderate decreases in renal function and show progression on subsequent biopsies. It is suggested that: 1) Focal global sclerosis is a disorder different from focal segmental sclerosis, presenting at an earlier age, responding to steroid therapy, and carrying a good prognosis. 2) Although focal segmental sclerosis is usually associated with NS, it can also present with only proteinuria and hematuria. Patients with this lesion do not respond to steroid therapy. Despite the fact that some patients maintain near-normal renal function over several years, all show histological deterioration.