We investigated the risk factors for relapse in 48 children with steroid-sensitive nephrotic syndrome using univariate and multivariate proportional hazard analysis. All patients were treated with the same corticosteroid regimen. A low serum level of total protein and young age at onset increased the relapse rate. Recurrence risk was not associated with the patient's sex, the percentage body weight gain, blood urea nitrogen level, serum level of creatinine, the hematocrit, or the administration of human albumin. We conclude that young age and a low serum level of total protein at onset were independent risk factors for relapse of childhood nephrotic syndrome.

In order to elucidate long-term effects of immunosuppressants, we studied 60 children with steroid-dependent nephrotic syndrome who were treated with three immunosuppressants: cyclophosphamide (n=34), chlorambucil (n=11), and cyclosporin A (n=15). Each relapse before and after the administration of immunosuppressants was evaluated longitudinally in terms of the relapse-free period and the maintenance dose of prednisolone required. The median follow-up period after immunosuppressants was 5.2 years (range 0.5-20.3 years). The relapse-free period was significantly longer in all groups after the initiation of immunosuppressants. However, the relapse-free period after subsequent relapses as compared with the previous relapse was longer in the cyclophosphamide group, similar in the chlorambucil group, and shorter in the cyclosporin A group. The prednisolone dosage at relapse was reduced in subsequent relapses after cyclophosphamide and chlorambucil treatment, but tended to be higher in later relapses after the initiation of cyclosporin A. These findings suggest that the effects of cyclophosphamide are long lasting, while those of chlorambucil and cyclosporin A are of short duration. Children who relapse after cyclosporin A treatment may experience a worse relapsing course.

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A female infant presented with renal insufficiency at age 4 weeks. She had bilateral multiple renal cysts but no other malformations of the urinary tract and no family history of renal disease. The kidney, liver, and spleen were not enlarged. Pathology examination of her left kidney when she was 6 years old revealed numerous cortical cysts with a dilatated Bowman's space and small glomerular tufts, and immature metanephric cells which often formed primitive ducts, suggesting a diagnosis of dysplastic glomerulocystic kidney. Glomerulocystic kidney is a rare type of congenital renal cystic disease with various clinical features and etiologies. It must be considered in the differential diagnosis of cystic diseases in infancy.