Sotto il Monte Giovanni XXIII, Italy
Sotto il Monte Giovanni XXIII, Italy

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Pasini A.,Nephrology and Dialysis Unit | Aceto G.,Giovanni Xxiii Childrens Hospital | Ammenti A.,University of Parma | Ardissino G.,Pediatric Nephrology and Dialysis Unit | And 24 more authors.
Pediatric Nephrology | Year: 2015

Background: The optimal therapeutic regimen for managing childhood idiopathic nephrotic syndrome (INS) is still under debate. We have evaluated the choice of steroid regimen and of symptomatic treatment adopted by pediatricians and pediatric nephrologists in a large number of centers as the first step towards establishing a shared protocol.Methods: This was a multicenter, retrospective study. A total of 231 children (132 admitted to pediatric units) aged 6 months to <15 years who presented with onset of nephrotic syndrome to 54 pediatric units and six pediatric nephrology units in Italy between 2007 and 2009 were eligible for entry into the study.Results: Median steroid dosing was 55 (range 27–75) mg/m2/day. The overall median cumulative dose regimen for the first episode was 3,440 (1,904–6,035) mg/m2, and the median duration of the therapeutic regimen was 21 (9–48) weeks. The total duration and cumulative steroid dose were significantly higher in patients treated by pediatricians than in those treated by pediatric nephrologists (p = 0.001 and p = 0.008). Among the patient cohort, 55, 64 and 22 % received albumin infusions, diuretics and acetyl salicylic acid treatment, respectively, but the laboratory and clinical data did not differ between children treated or not treated with symptomatic drugs. Albumin and diuretic use did not vary between patients in pediatric units and those in pediatric nephrology units.Conclusions: This study shows major differences in steroid and symptomatic treatment of nephrotic syndrome by pediatricians and pediatric nephrologists. As these differences can influence the efficacy of the treatments and the appearance of side-effects, shared guidelines and their implementation through widespread educational activities are necessary. © 2014, The Author(s).

Magnasco A.,IRCCS Giannina Gaslini Children Hospital | Ravani P.,University of Calgary | Edefonti A.,Nephrology | Murer L.,University of Padua | And 11 more authors.
Journal of the American Society of Nephrology | Year: 2012

Idiopathic nephrotic syndrome resistant to standard treatments remains a therapeutic dilemma in pediatric nephrology. To test whether the anti-CD20 monoclonal antibody rituximab may benefit these patients, we conducted an open-label, randomized, controlled trial in 31 children with idiopathic nephrotic syndrome unresponsive to the combination of calcineurin inhibitors and prednisone. All children continued prednisone and calcineurin inhibitors at the doses prescribed before enrollment, and one treatment group received two doses of rituximab (375 mg/m 2 intravenously) as add-on therapy. Themean age was 8 years (range, 2-16 years). Rituximab did not reduce proteinuria at 3 months (change, 212% [95% confidence interval, 273% to 110%]; P=0.77 in analysis of covariance model adjusted for baseline proteinuria). Additional adjustment for previous remission and interaction terms (treatment by baseline proteinuria and treatment by previous remission) did not change the results. In conclusion, these data do not support the addition of rituximab to prednisone and calcineurin inhibitors in children with resistant idiopathic nephrotic syndrome. Copyright © 2012 by the American Society of Nephrology.

Turolo S.,Pediatric Nephrology and Dialysis | Edefonti A.,Pediatric Nephrology and Dialysis | Lepore M.,Pediatric Nephrology and Dialysis | Ghio L.,Pediatric Nephrology and Dialysis | And 8 more authors.
Pharmacogenomics | Year: 2016

Aim: of the study was to analyse the impact of SXR rs3842689 polymorphism on the response to corticosteroids in pediatric idiopathic nephrotic syndrome. Patients & methods: 66 children (56 steroid-sensitive, ten steroid-resistant) were studied for SXR gene polymorphism distribution. Results: Steroid sensitive patients accounted for 96% of cases with In/In polymorphism, but only for 53% of cases with Del/Del polymorphism At odds ratio analysis, Del/Del represented a clear risk factor of steroid resistance (OR: 20.57; p = 0.009), while In/In was a favourable prognostic factor of steroid sensitivity. Conclusion: The analysis of SXR polymorphism is a promising tool to predict both the favourable response to corticosteroids and the risk of developing steroid resistance. © 2016 Future Medicine Ltd.

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