TY - JOUR T1 - 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. JF - Ital J Pediatr Y1 - 2017 A1 - Pasini, Andrea A1 - Benetti, Elisa A1 - Conti, Giovanni A1 - Ghio, Luciana A1 - Lepore, Marta A1 - Massella, Laura A1 - Molino, Daniela A1 - Peruzzi, Licia A1 - Emma, Francesco A1 - Fede, Carmelo A1 - Trivelli, Antonella A1 - Maringhini, Silvio A1 - Materassi, Marco A1 - Messina, Giovanni A1 - Montini, Giovanni A1 - Murer, Luisa A1 - Pecoraro, Carmine A1 - Pennesi, Marco KW - Adrenal Cortex Hormones KW - Child KW - Child, Preschool KW - Consensus KW - Dose-Response Relationship, Drug KW - Drug Administration Schedule KW - Female KW - Humans KW - Italy KW - Male KW - Nephrotic Syndrome KW - Practice Guidelines as Topic KW - Prognosis KW - Recurrence KW - Retreatment KW - Societies, Medical KW - Survival Rate KW - Treatment Outcome AB -

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.

VL - 43 IS - 1 U1 - http://www.ncbi.nlm.nih.gov/pubmed/28427453?dopt=Abstract ER -