Sant'Ambrogio di Torino, Italy
Sant'Ambrogio di Torino, Italy

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Deschenes G.,Paris-Sorbonne University | Vivarelli M.,Bambino Gesu Childrens Hospital And Research Institute | Peruzzi L.,Regina Margherita Childrens Hospital
European Journal of Pediatrics | Year: 2017

The aim of the surveys conducted by the Idiopathic Nephrotic Syndrome Working Group of the ESPN was to study the possible variability of treatment in Europe at different stages of the disease by means of questionnaires sent to members of the Working Group. Four surveys have been completed: treatment of the first flare, treatment of the first relapse and the issue of steroid dependency, use of rituximab, and the management of steroid-resistant patients. A uniform treatment of the first flare was applied in only three countries, and ten additional centers have adopted one of the three main protocols. Reported treatment of the first relapse was relatively uniform, whereas the use of additional immunosuppressants in steroid dependency was widely variable. Rituximab had already been used in hundreds of patients, although the formal evidence of efficiency in steroid dependency was relatively recent at the time of the survey. The definition of steroid resistance was variable in the European centers, but strikingly, the first-line treatment was uniform throughout the centers and included the combination of prednisone plus calcineurin antagonists. Conclusion: The variability in the approach of idiopathic nephrotic syndrome is unexpectedly large and affects treatment of the first flare, strategies in the case of steroid dependency, as well as the definitions of steroid resistance.(Table presented.) © 2017 Springer-Verlag Berlin Heidelberg


Baldovino S.,University of Turin | Montin D.,University of Turin | Montin D.,Regina Margherita Childrens Hospital | Martino S.,University of Turin | And 5 more authors.
Autoimmunity Reviews | Year: 2013

Common variable immunodeficiency is a collection of diseases characterized by primary hypogammaglobulinemia. The causes of CVID are extremely heterogeneous and may affect virtually every pathway linked to B cell development and function. Clinical manifestations of CVID mainly include recurrent bacterial infections, but autoimmune, gastrointestinal, lymphoproliferative, granulomatous, and malignant disorders have also been frequently reported as associated conditions. We aimed to focus on the state of the art of the relationship between infections, inflammation and autoimmunity in CVID. © 2012 Elsevier B.V.


Coppo R.,Regina Margherita Childrens Hospital | Feehally J.,University of Leicester | Glassock R.J.,University of California at Los Angeles
Kidney International | Year: 2010

On May 26-28, 2009, an international symposium on IgA nephropathy was convened in Stresa, Italy, as a Satellite Symposium of the World Congress of Nephrology held in Milan. This meeting was attended by a large number of scientists and clinicians working in the field of IgA nephropathy. The oral and poster presentations (over 70) ranged from very fundamental structural biology to clinical management. This article attempts to summarize the main findings of the meeting and to put forth some new perspectives and hypotheses regarding human IgA nephropathy on the 41st anniversary of its original description by Berger and Hinglais in 1968. © 2010 International Society of Nephrology.


Garazzino S.,University of Turin | Scolfaro C.,University of Turin | Raffaldi I.,University of Turin | Barbui A.M.,University of Turin | And 2 more authors.
Pediatric Pulmonology | Year: 2014

Objective To report our experience on the safety and tolerability of moxifloxacin for treating children affected by pulmonary TB. Study Design Children receiving a moxifloxacin-containing anti-TB regimen were included in the study. Their medical records were revised at the end of follow-up. Methods We describe nine children treated with moxifloxacin for pulmonary TB at Regina Margherita Children's Hospital (Turin, Italy) between 2007 and 2012. Moxifloxacin was administered orally at 10 mg/kg/day once daily (maximum dose = 400 mg/day) following World Health Organization indications. During treatment, patients were systematically assessed for the development of side effects. Results Eight children were considered cured at the end of treatment; one child was lost to follow-up after 3 months of treatment. Two children had side effects during treatment: one developed arthritis of the ankle; the other had liver toxicity, whose relationship with moxifloxacin could not be ruled out. We did not observe any case of QT prolongation, central nervous system disorders, growth defects or gastrointestinal disturbances. Conclusions A moxifloxacin-containing regimen might be considered for the treatment of TB in children, especially for drug-resistant and extensive forms. However, vigilance for possible side effects is recommended, especially if other drugs are concomitantly used. Studies on wider populations are needed to better define the impact of long-term treatments with quinolones on children's growth and psychomotor development and to outline regulatory indications on moxifloxacin use in the pediatric setting. Pediatr Pulmonol. 2014; 49:372-376. © 2013 Wiley Periodicals, Inc. © 2013 Wiley Periodicals, Inc.


Ivani G.,Regina Margherita Childrens Hospital | Walker I.,Great Ormond Street Hospital NHS Foundation Trust | Enright A.,Royal Jubilee Hospital
Paediatric Anaesthesia | Year: 2012

Summary Pediatric anesthesia is no longer a small subspecialty, but an important sector where developments in the new century have brought effective and safe management to children in the perioperative period. Unfortunately, what is common daily practice in the high-income countries with all the guidelines, checklists, instruments, and dedicated pediatric anesthesiologists is often only a dream in the low- and middle-income countries where the basic anesthesia services for improving the high rate of morbidity and mortality still are lacking. Anesthesia given by nonphysicians, with no monitoring, lack of elementary supplies, poor control of infections and hemorrhage, and no water or electricity are very often the 'usual' conditions. The World Health Organization is working hard to offer teams, basic equipments, and teaching and what is needed to offer children of these countries the same opportunities given in the industrialized countries. Other projects such as the Lifebox Project have a similar aim. This paper outlines some of what organizations are doing around the world, with different strategies all having the same target: safe pediatric anesthesia. © 2012 Blackwell Publishing Ltd.


Coppo R.,Regina Margherita Childrens Hospital
Contributions to Nephrology | Year: 2013

Various drugs have been used for the treatment of focal segmental glomerular sclerosis (FSGS) or minimal change disease (idiopathic nephrotic syndrome, INS) including methylprednisolone pulses, alkylating agents and calcineurin inhibitors, often without a strong rationale. For some drugs the rationale has been recently provided by the identification of mechanisms regulating proteinuria. The characterization of molecules acting as permeability factors, including hemopexin, soluble urokinase receptor and cardiotrophin-like cytokine-1, supports plasma exchange in severe cases of INS, particularly in patients at high risk of recurrence of FSGS after transplantation. The collaboration of B and T cells for the production of permeability factors has provided a rationale for targeting B cells using rituximab. Several studies in children and adults, mostly in steroid-dependent cases, or after recurrence in grafted kidneys, have reported improved outcomes after rituximab. Podocyte actin cytoskeleton is a new target for therapy. Calcineurin inhibitors block the dephosphorylation of the cytoskeleton component synaptopodin, and steroids increase actin polymerization and stability. High doses of methylprednisolone and cyclosporine proved to be beneficial in patients with a high risk of FSGS recurrence. An interesting new target for blunting the INS pathogenetical mechanisms is the transcription factor nuclear factor-κB (NF-κB) which was reported to be activated in circulating mononuclear cells of these patients. NF-κB is regulated by the proteasome and saquinavir, a HIV protease inhibitor, is provided with antiproteasome activity. Using saquinavir associated with small doses of calcineurin inhibitors, we treated a small series of very difficult cases of INS with insufficient response to steroid therapy and multiple immunosuppressive drugs. Saquinavir allowed a significant reduction of steroid cumulative doses and disappearance of features of steroid toxicity. In conclusion, recent reports have allowed a new insight into the pathogenetical mechanism regulating proteinuria in INS, offering new targets for treating severe cases. Copyright © 2013 S. Karger AG, Basel.


Vigo A.,Regina Margherita Childrens Hospital
Pediatric Emergency Care | Year: 2016

OBJECTIVES: Apparent life-threatening event (ALTE) refers to a constellation of unexpected events suddenly occurring in infants that extremely alarm the observers. The objectives of this study were to evaluate 1) intervention of Emergency Service (ES) at home, 2) parental behavior before ES intervention, 3) patientsʼ outcome at follow-up of a minimum of 6 months. METHODS: Retrospective study of infants younger than 12 months whose parents called ES and were evaluated for ALTE from 2005 to 2014. Tactile stimulation (TS) was defined as any maneuver performed by parents or ES staff aimed at rescuing patients without cardiopulmonary resuscitation attempts. Cardiopulmonary resuscitation was defined according to American Heart Association Guidelines 2010. RESULTS: One hundred eighty-eight patients were eligible. Emergency Service provided intervention for 178 infants (10 were assisted only by phone). All patients received TS by parents before ES arrival. Mean time for ES to reach patient location was 15 ± 10 minutes. On examination, 136 patients (76.5%) seemed normal and 42 symptomatic. One hundred sixty-three patients were brought to the emergency department where 23 patients were found symptomatic. One hundred six of 163 patients underwent capillary blood gas determination and, in 28 (26%) of 106, alterations were found. No infant had subsequent cardiopulmonary arrest or clinically evident adverse neurological outcome. Six were found to be epileptic. No infant died during the episode or during follow-up. CONCLUSIONS: Our findings suggest that ALTE is an alarming but self-limiting phenomenon that can be resolved either spontaneously or by simple TS in most cases. Emergency Service should solicit patientsʼ physiological responses through TS first while considering a cardiopulmonary resuscitation maneuver. Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved.


Saglio F.,Regina Margherita Childrens Hospital | Hanley P.J.,Sheikh Zayed Institute for Pediatric Surgical Innovation | Bollard C.M.,Sheikh Zayed Institute for Pediatric Surgical Innovation
Cytotherapy | Year: 2014

Adoptive immunotherapy-in particular, T-cell therapy-has recently emerged as a useful strategy with the potential to overcome many of the limitations of antiviral drugs for the treatment of viral complications after hematopietic stem cell transplantation. In this review, we briefly summarize the current methods for virus-specific T-cell isolation or selection and we report results from clinical trials that have used these techniques, focusing specifically on the strategies aimed to broaden the application of this technology. © 2014 International Society for Cellular Therapy.


Monti G.,Regina Margherita Childrens Hospital
Journal of biological regulators and homeostatic agents | Year: 2012

Not exclusively breastfed children with cow's milk allergy (CMA) require a formula or other alternative food, but past and present guidelines differ concerning the best choice. Our aim was to investigate the clinical tolerability, palatability and nutritional adequacy of donkey's milk (DM) in children with proven CMA. It was important to identify a CM replacement for these children, highly problematic from the feeding standpoint, in spite of their age. A prospective study was conducted on 92 children with CMA, diagnosed through a CM elimination diet, followed by double-blind, placebo-controlled food challenge (DBPCFC) unless contraindicated. Maternal milk was unavailable and current CM substitutes could not be used. Moreover, 89 percent were affected by multiple FA, and subjected to very restricted diets. Within 3 months after the last CM challenge, DBPCFC for DM was performed. CM or DM skin prick test and sIgE determination preceded the CM or DM challenge, respectively. Native electrophoresis and immunoblotting were used to identify CM and DM cross-reactive proteins. Z-scores of weight and length/stature for age were calculated at DM food challenge (T0) and during DM assumption. 83 children (90.2 percent) liked and tolerated DM, at challenge and during follow-up, with increased Z-score for weight and length/stature and improved nutritional parameters. Bovine beta-lactoglobulin was identified as the cross-reacting protein among the DM allergic patients. DM was found to be a valid alternative foodstuff, in terms of clinical tolerability, palatability and nutritional adequacy, in subjects with CMA who were highly problematic from the feeding standpoint.


Mossetti V.,Regina Margherita Childrens Hospital | Ivani G.,Regina Margherita Childrens Hospital
Paediatric Anaesthesia | Year: 2012

Summary Regional anesthesia in children has reached in the last decades a widespread approval and today is a valid and effective technique used in the daily activity in many pediatric centres. Data from surveys and studies show the very low rate of complications and suggest that the use of regional techniques in children is at least as safe as general anesthesia alone. Despite these evidences there are aspects still creating discussions on the best approach to avoid or prevent risks and on the way to make this techniques more and more reliable and safe. Compartment syndrome, test dose, loss-of-resistance technique and blocks performed under general anesthesia are examples of, sometimes, never-ending stories; this review without the presumption to solve every problem shows the more recent data to offer, if possible, the best updated-answer on these topics. © 2011 Blackwell Publishing Ltd.

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