Clinical Patterns and Renal Survival of Nephrotic Syndrome in Childhood: A Single-Center Study (1980-2006)

Abstract

To investigate changes in the diagnostic patterns, disease profiles courses and therapeutic strategies for severe forms of childhood nephrotic syndrome (NS), the clinical features of 1 349 children treated during two consecutive time periods, 1980-2000 (n=1 162) and 2001-2006 (n=187), were retrospectively reviewed. The significant increase in initial renal impairment, NS with hypertension, and NS with hypertension and hematuria was observed (27.7% vs 51.3%, 1.0% vs 5.3% and 16.4% vs 21.9%, respectively). The rate of both secondary steroid resistance (SR) and Focal Segmental Glomerulosclerosis increased significantly, (1.8% vs 5.6%, p=0.032, and 14.9% vs 29.0%, p=0.034, respectively). The initial renal insufficiency and hypertension were highly predictive of the development of stage 3 of chronic kidney disease (CKD3) among SR patients in a multivariate Cox regression (p=0.001) for years 1980-2000. A higher hazard of CKD3 in male SR patients from three to six years old was observed in 2001-2006. Kaplan-Meier survival curves revealed a shift in the cumulative probability of CKD3, indicating a slower decline of the renal function for SR NS in the years 2001-2006 (p=0.008): the estimated five-year CKD3 risk was 39.7% vs 27.7%. Achievements in inducing remission and retarding the development of CKD3 in combination with increased severity of NS indicate the effectiveness of domestic strategies of NS management.