@article {10483, title = {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.}, journal = {Ital J Pediatr}, volume = {43}, year = {2017}, month = {2017 Apr 21}, pages = {41}, abstract = {

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.

}, keywords = {Adrenal Cortex Hormones, Child, Child, Preschool, Consensus, Dose-Response Relationship, Drug, Drug Administration Schedule, Female, Humans, Italy, Male, Nephrotic Syndrome, Practice Guidelines as Topic, Prognosis, Recurrence, Retreatment, Societies, Medical, Survival Rate, Treatment Outcome}, issn = {1824-7288}, doi = {10.1186/s13052-017-0356-x}, author = {Pasini, Andrea and Benetti, Elisa and Conti, Giovanni and Ghio, Luciana and Lepore, Marta and Massella, Laura and Molino, Daniela and Peruzzi, Licia and Emma, Francesco and Fede, Carmelo and Trivelli, Antonella and Maringhini, Silvio and Materassi, Marco and Messina, Giovanni and Montini, Giovanni and Murer, Luisa and Pecoraro, Carmine and Pennesi, Marco} } @article {10563, title = {Outcome of childhood-onset full-house nephropathy.}, journal = {Nephrol Dial Transplant}, volume = {32}, year = {2017}, month = {2017 Jul 01}, pages = {1194-1204}, abstract = {

Background: Patients with full-house nephropathy (FHN) present renal lesions that are indistinguishable from those of lupus nephritis (LN) but lack the systemic features necessary to meet diagnostic criteria for systemic lupus erithematosus (SLE). Some have been reported to develop a delayed SLE with time. The clinical outcome of children having FHN without SLE has never been reported.

Methods: Children with biopsy-proven FHN were selected after excluding SLE cases by the absence of America College of Rheumatology criteria. The proportion of patients with complete (proteinuria <0.5 g/day) or partial remission (proteinuria <=50\% from baseline), relapse (estimated glomerular filtration rate <25\% and/or proteinuria >=50\% from baseline) and progression to Stage III chronic kidney disease (CKD) was described according to age and gender groups with the Kaplan-Meier curve and compared with the Log-rank test. Entity of treatment was summarized by a score at induction (0-6 months) and maintenance (6-18 months). Cox-regression model was performed to test predictors of remission, relapse and progression to CKD.

Results: Among 42 patients (28 pre-pubertal) who met the inclusion criteria, 39 (92.9\%) achieved partial and 32 (76.2\%) complete remission of nephropathy over 2.78 and 7.51 months of follow-up. At 10 years, the probability of progressing to CKD was 4.8\%. Of those achieving remission, 18\% had a renal flare mainly within 4 years after remission. Pre-pubertal males achieved complete remission more frequently than other patients but often relapsed; pre-pubertal females were treated more aggressively. Cox-regression analysis did not find independent predictors of remission or relapse.

Conclusions: The outcome of the patients with FHN we investigated was encouraging. Recurrences are limited to the first 4 years following diagnosis, allowing progressive withdrawal of immunosuppression in patients achieving remission. Evaluation of risk factors for adverse outcome is necessary especially in pre-pubertal children.

}, keywords = {Adolescent, Age of Onset, Child, Disease Progression, Female, Glomerular Filtration Rate, Glomerulonephritis, Humans, Kidney Diseases, Lupus Erythematosus, Systemic, Lupus Nephritis, Male, Proteinuria, Recurrence, Remission Induction, Retrospective Studies, Risk Factors, Survival Rate}, issn = {1460-2385}, doi = {10.1093/ndt/gfw230}, author = {Ruggiero, Barbara and Vivarelli, Marina and Gianviti, Alessandra and Pecoraro, Carmine and Peruzzi, Licia and Benetti, Elisa and Ventura, Giovanna and Pennesi, Marco and Murer, Luisa and Coppo, Rosanna and Emma, Francesco} } @article {3497, title = {Rituximab in steroid-dependent or frequently relapsing idiopathic nephrotic syndrome.}, journal = {J Am Soc Nephrol}, volume = {25}, year = {2014}, month = {2014 Apr}, pages = {850-63}, abstract = {

The outcome of steroid-dependent or frequently relapsing nephrotic syndrome of minimal change disease (MCD), mesangial proliferative GN (MesGN), or FSGS may be poor and with major treatment toxicity. This academic, multicenter, off-on trial (ClinicalTrials.gov $\#$NCT00981838) primarily evaluated the effects of rituximab therapy followed by immunosuppression withdrawal on disease recurrence in 10 children and 20 adults with MCD/MesGN (n=22) or FSGS who had suffered >=2 recurrences over the previous year and were in steroid-induced remission for >=1 month. Participants received one dose (n=28) or two doses of rituximab (375 mg/m(2) intravenously). At 1 year, all patients were in remission: 18 were treatment-free and 15 never relapsed. Compared with the year before rituximab treatment, total relapses decreased from 88 to 22 and the per-patient median number of relapses decreased from 2.5 (interquartile range [IQR], 2-4) to 0.5 (IQR, 0-1; P<0.001) during 1 year of follow-up. Reduction was significant across subgroups (children, adults, MCD/MesGN, and FSGS; P<0.01). After rituximab, the per-patient steroid maintenance median dose decreased from 0.27 mg/kg (IQR, 0.19-0.60) to 0 mg/kg (IQR, 0-0.23) (P<0.001), and the median cumulative dose to achieve relapse remission decreased from 19.5 mg/kg (IQR, 13.0-29.2) to 0.5 mg/kg (IQR, 0-9.4) (P<0.001). Furthermore, the mean estimated GFR increased from 111.3{\textpm}25.7 to 121.8{\textpm}29.2 ml/min per 1.73 m(2) (P=0.01), with the largest increases in children and in FSGS subgroups. The mean height z score slope stabilized in children (P<0.01). Treatment was well tolerated. Rituximab effectively and safely prevented recurrences and reduced the need for immunosuppression in steroid-dependent or frequently relapsing nephrotic syndrome, and halted disease-associated growth deficit in children.

}, keywords = {Adolescent, Adrenal Cortex Hormones, Adult, Antibodies, Monoclonal, Murine-Derived, Child, Female, Glomerulonephritis, Membranoproliferative, Glomerulosclerosis, Focal Segmental, Humans, Male, Middle Aged, Nephrosis, Lipoid, Nephrotic Syndrome, Recurrence, Rituximab}, issn = {1533-3450}, doi = {10.1681/ASN.2013030251}, author = {Ruggenenti, Piero and Ruggiero, Barbara and Cravedi, Paolo and Vivarelli, Marina and Massella, Laura and Maras{\`a}, Maddalena and Chianca, Antonietta and Rubis, Nadia and Ene-Iordache, Bogdan and Rudnicki, Michael and Pollastro, Rosa Maria and Capasso, Giovambattista and Pisani, Antonio and Pennesi, Marco and Emma, Francesco and Remuzzi, Giuseppe} }