Rapid Response to @entity205 and Favorable Renal Outcome in Nongenetic Versus Genetic @entity479 -Resistant XXXX .

@entity3441 @entity1 @entity9472 @entity644 @entity454 @entity6333 @entity8587 @entity21188 @entity205 @entity74 @entity1985 @entity479
BACKGROUND AND OBJECTIVES: Treatment of @entity3441 ( @entity3441 ) and @entity479 -resistant @entity1985 (SRNS) is demanding, and renal prognosis is poor. Numerous causative gene mutations have been identified in SRNS that affect the renal podocyte. In the era of high-throughput sequencing techniques, @entity1 with nongenetic SRNS frequently escape the scientific interest. We here present the long-term data of the German @entity3441 /SRNS Follow-Up Study, focusing on the response to @entity205 ( @entity9472 ) in @entity1 with nongenetic @entity74 . DESIGN, SETTING, @entity1 , _ MEASUREMENTS: Cross-sectional and longitudinal clinical data were collected from 231 @entity1 with @entity3441 /SRNS treated at eight university pediatric nephrology units with a median observation time of 113 months (interquartile range, 50-178). Genotyping was performed systematically in all @entity1 . RESULTS: The overall mutation detection rate was high at 57% (97% in @entity3441 and 41% in SRNS); 85% of all mutations were identified by the analysis of three single genes only ( @entity6333 , @entity21188 , and @entity8587 ), accounting for 92% of all mutations in @entity1 with @entity3441 and 79% of all mutations in @entity1 with SRNS. Remission of the disease in nongenetic SRNS was observed in 78% of @entity1 after a median treatment period of 2.5 months; 82% of nongenetic @entity1 responded within 6 months of therapy, and 98% of @entity1 with nongenetic SRNS and @entity9472 -induced complete remission (normalbuminemia and no @entity644 ) maintained a normal renal function. Genetic SRNS, on the contrary, is associated with a high rate of @entity454 in 66% of @entity1 . Only 3% of @entity1 with genetic SRNS experienced a complete remission and 16% of @entity1 with genetic SRNS experienced a partial remission after @entity9472 therapy. CONCLUSIONS: The efficacy of @entity9472 is high in nonhereditary SRNS, with an excellent prognosis of renal function in the large majority of @entity1 . @entity9472 should be given for a minimum period of 6 months in these @entity1 with nongenetic SRNS. In genetic SRNS, response to @entity9472 was low and restricted to exceptional @entity1 .
