%0 Journal Article %J Ital J Pediatr %D 2017 %T The Italian Society for Pediatric Nephrology (SINePe) consensus document on the management of nephrotic syndrome in children: Part I - Diagnosis and treatment of the first episode and the first relapse. %A Pasini, Andrea %A Benetti, Elisa %A Conti, Giovanni %A Ghio, Luciana %A Lepore, Marta %A Massella, Laura %A Molino, Daniela %A Peruzzi, Licia %A Emma, Francesco %A Fede, Carmelo %A Trivelli, Antonella %A Maringhini, Silvio %A Materassi, Marco %A Messina, Giovanni %A Montini, Giovanni %A Murer, Luisa %A Pecoraro, Carmine %A Pennesi, Marco %K Adrenal Cortex Hormones %K Child %K Child, Preschool %K Consensus %K Dose-Response Relationship, Drug %K Drug Administration Schedule %K Female %K Humans %K Italy %K Male %K Nephrotic Syndrome %K Practice Guidelines as Topic %K Prognosis %K Recurrence %K Retreatment %K Societies, Medical %K Survival Rate %K Treatment Outcome %X

This consensus document is aimed at providing an updated, multidisciplinary overview on the diagnosis and treatment of pediatric nephrotic syndrome (NS) at first presentation. It is the first consensus document of its kind to be produced by all the pediatric nephrology centres in Italy, in line with what is already present in other countries such as France, Germany and the USA. It is based on the current knowledge surrounding the symptomatic and steroid treatment of NS, with a view to providing the basis for a separate consensus document on the treatment of relapses. NS is one of the most common pediatric glomerular diseases, with an incidence of around 2-7 cases per 100000 children per year. Corticosteroids are the mainstay of treatment, but the optimal therapeutic regimen for managing childhood idiopathic NS is still under debate. In Italy, shared treatment guidelines were lacking and, consequently, the choice of steroid regimen was based on the clinical expertise of each individual unit. On the basis of the 2015 Cochrane systematic review, KDIGO Guidelines and more recent data from the literature, this working group, with the contribution of all the pediatric nephrology centres in Italy and on the behalf of the Italian Society of Pediatric Nephrology, has produced a shared steroid protocol that will be useful for National Health System hospitals and pediatricians. Investigations at initial presentation and the principal causes of NS to be screened are suggested. In the early phase of the disease, symptomatic treatment is also important as many severe complications can occur which are either directly related to the pathophysiology of the underlying NS or to the steroid treatment itself. To date, very few studies have been published on the prophylaxis and treatment of these early complications, while recommendations are either lacking or conflicting. This consensus provides indications for the prevention, early recognition and treatment of these complications (management of edema and hypovolemia, therapy and prophylaxis of infections and thromboembolic events). Finally, recommendations about the clinical definition of steroid resistance and its initial diagnostic management, as well as indications for renal biopsy are provided.

%B Ital J Pediatr %V 43 %P 41 %8 2017 Apr 21 %G eng %N 1 %1 http://www.ncbi.nlm.nih.gov/pubmed/28427453?dopt=Abstract %R 10.1186/s13052-017-0356-x %0 Journal Article %J Pharmacogenomics %D 2015 %T Glucocorticoid pharmacogenetics in pediatric idiopathic nephrotic syndrome. %A Cuzzoni, Eva %A De Iudicibus, Sara %A Franca, Raffaella %A Stocco, Gabriele %A Lucafò, Marianna %A Pelin, Marco %A Favretto, Diego %A Pasini, Andrea %A Montini, Giovanni %A Decorti, Giuliana %X

Idiopathic nephrotic syndrome represents the most common type of primary glomerular disease in children: glucocorticoids (GCs) are the first-line therapy, even if considerable interindividual differences in thepir efficacy and side effects have been reported. Immunosuppressive and anti-inflammatory effects of these drugs are mainly due to the GC-mediated transcription regulation of pro- and anti-inflammatory genes. This mechanism of action is the result of a complex multistep pathway that involves the glucocorticoid receptor and several other proteins, encoded by polymorphic genes. Aim of this review is to highlight the current knowledge on genetic variants that could affect GC response, particularly focusing on children with idiopathic nephrotic syndrome.

%B Pharmacogenomics %V 16 %P 1631-48 %8 2015 Sep %G eng %N 14 %1 http://www.ncbi.nlm.nih.gov/pubmed/26419298?dopt=Abstract %R 10.2217/pgs.15.101