AIMS: To estimate the incidence and describe the clinical presentation and outcome ( @entity134 responsiveness, clinical course, complications) of @entity3441 in @entity1 in a population-based retrospective study. METHODS: Using local registries and the hospital discharge diagnosis system from two centers, all new cases of @entity3441 were identified in Gironde (France) between January  1992  and May  2008. To estimate incidence, population-based denominators were obtained from the National Institute for Statistics and Economic Studies (INSEE). Clinical data were collected from medical charts. RESULTS: Ninety-nine cases of @entity3441 were reported (66  @entity1 , 18  non-Caucasians) with an incidence of 2.3/100,000 (CI, 1.8-3.0) @entity1 less than 15years. Ninety @entity1 (91%) had @entity134 -sensitive @entity1985 (SSNS) and nine (9%) were @entity134 -resistant (SRNS). The median time to remission in SSNS was 11days. Relapses occurred in 75 (83%) @entity1 with SSNS with a median of four relapses (range, 1-32). The cumulative relapse-free incidence was 60% at 10years after diagnosis in SSNS and 13% of @entity1 aged 18years old or over still had active disease. In SSNS, the only significant factor associated with @entity134 dependency or use of non @entity134 drugs was the time to initial response to steroids greater than 14days. Nineteen @entity1 (19%) experienced severe complications of @entity1985 including 11  @entity1031 . Two @entity1 with SRNS, of whom one was initially @entity134 -responsive, developed @entity454 . CONCLUSION: The incidence and outcome of @entity3441 in Gironde are comparable to the rates found in other studies. The disease may have a long course and the time for response to @entity230 at disease onset is the main predictor of @entity134 dependency and of use of non @entity134 agents.

[Idiopathic @entity1985 in XXXX : Incidence, clinical presentation, and outcome in the county of Gironde, France].

@entity3441 :: ('MESH:C535761', 'Disease') :: ['idiopathic nephrotic syndrome'] @entity1 :: ('9606', 'Species') :: ['children', 'patients', 'boys'] @entity230 :: ('MESH:D013256', 'Chemical') :: ['steroids'] @entity134 :: ('CHEBI:35341', 'Chemical') :: ['steroid'] @entity1031 :: ('MESH:D013923', 'Disease') :: ['bacterial infections and two thromboembolic complications'] @entity1985 :: ('MESH:D009404', 'Disease') :: ['nephrotic syndrome'] @entity454 :: ('MESH:D007676', 'Disease') :: ['end-stage renal failure']
@entity1 :: (9606,Species) :: ['children']
