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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.

Clinical Patterns and Renal Survival of Nephrotic Syndrome in Childhood: A Single-Center Study (1980-2006)~!2009-12-01~!2010-05-17~!2010-06-23~!