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The fast diffusion of the SARS-CoV-2 pandemic have called for an equally rapid evolution of the therapeutic options.The Human recombinant monoclonal antibodies (mAbs) have recently been approved by the Food and Drug Administration (FDA) and by the Italian Medicines Agency (AIFA) in subjects aged ≥12 with SARS-CoV-2 infection and specific risk factors.Currently the indications are specific for the use of two different mAbs combination: Bamlanivimab+Etesevimab (produced by Eli Lilly) and Casirivimab+Imdevimab (produced by Regeneron).These drugs have shown favorable effects in adult patients in the initial phase of infection, whereas to date few data are available on their use in children.AIFA criteria derived from the existing literature which reports an increased risk of severe COVID-19 in children with comorbidities. However, the studies analyzing the determinants for progression to severe disease are mainly monocentric, with limited numbers and reporting mostly generic risk categories.Thus, the Italian Society of Pediatrics invited its affiliated Scientific Societies to produce a Consensus document based on the revision of the criteria proposed by AIFA in light of the most recent literature and experts’ agreement.This Consensus tries to detail which patients actually have the risk to develop severe disease, analyzing the most common comorbidities in children, in order to detail the indications for mAbs administration and to guide the clinicians in identifying eligible patients.

Composites made of fibers embedded in organic or inorganic matrices are efficient systems for reinforcing historical masonry structures to provide strength and ductility with a negligible mass increment. As it is well known, the structural performance of the composites mainly relies on their adhesion to the substrate. There are different methods to test the adhesion of the composite to the substrate: in laboratory direct shear test is the most commonly employed, while on-site the bond between the reinforcement and the substrate is checked by the pull-off test. In this paper, the adhesion of different composites to the same substrate made of fired-clay bricks is investigated by both the shear test and the pull-off test to qualitatively assess the difference in the two methods. Additionally, to investigate whether the bond is affected by the presence of water in the pores, half of the specimens were tested in water saturated conditions. Three different types of matrix (based on epoxy resin, natural hydraulic lime and Portland cement) were used for the composite matrix, without changing the geometry, the type of masonry substrate and the fibers (galvanized steel cords).

This paper presents the results of single-lap direct shear tests on steel reinforced grout (SRG) strips bonded to fired-clay brick and tuff masonry blocks. For this experimental campaign, fifteen masonry blocks were constructed and reinforced with SRG composite strips. Fired-clay bricks and tuff blocks herein employed were collected from demolished historical structures in Modena and Naples (Italy), respectively. Eight out of fifteen blocks were subjected to an artificial weathering procedure to induce salt crystallization, and therefore reproduce the degradation conditions that the buildings could be subjected to during their life. Bond behavior of unconditioned strengthened specimens (i.e. SRG-masonry joints) was compared with the behavior of conditioned joints. Salt distribution, open porosity, and water absorption of brick and tuff masonry units as well as of the SRG composite matrix were analyzed to understand if and how the salt crystallization influenced the adhesion between the SRG strip and the block.

In this study, a preliminary evaluation of the effectiveness of aqueous solutions of diammonium hydrogen phosphate (DAP) for the consolidation of lime-based mortars is reported. Four types of mortars were considered: 2 lime-based mortars (containing either siliceous or calcareous aggregates), 1 lime-based mortar also containing brick powder as a pozzolanic addition (so-called “cocciopesto” mortar) and 1 mortar based on natural hydraulic lime. Two different formulations of the DAP solution were considered, differing in terms of DAP concentration, CaCl2 addition as a calcium source and ethanol addition to boost formation of calcium phosphate (CaP) phases. First, for the various types of mortar, the nature and the morphology of the new CaP phases were investigated by FT-IR and SEM. Then, the mechanical benefit resulting from the new CaP was evaluated, in terms of increase in dynamic elastic modulus and compressive strength. The microstructural compatibility of the treatments was also evaluated, by determining the alterations in pore size distribution. The results of the study indicate that new CaP phases are formed after 24 hours for both DAP concentrations, the amount of new phases and the mechanical improvement being higher for the more concentrated solution. In no case did dramatic pore occlusion and alteration in the pore size distribution take place, which allows expecting no dramatic alteration in the mortar ability to exchange water and water vapour with the environment. The more concentrated solution caused some visible colour change in the case of mortars not originally white (“cocciopesto” and NHL mortars), which makes the use of less concentrated solutions advisable on those substrates.

Prader-Willi syndrome is a multisystemic genetic disorder that can be associated with epilepsy. There is insufficient information concerning the clinical and electroencephalographic characteristics of epilepsy and the long-term outcome of these patients. The aim of this study is to describe seizure types, electroencephalographic patterns and long-term seizure outcome in Prader-Willi syndrome patients suffering from epilepsy. We retrospectively studied 38 patients with Prader-Willi syndrome and seizures. Results of neuroimaging studies were obtained for 35 individuals. We subdivided these patients into two groups: group A, 24 patients, without brain lesions; and group B, 11 patients, with brain abnormalities. All patients were re-evaluated after a period of at least 10 years. Twenty-one patients (55.2 %) were affected by generalized epilepsy and 17 patients (44.8 %) presented focal epilepsy. The most common seizure type was generalized tonic-clonic seizure. The mean age at seizure onset was 4.5 years (ranged from 1 month to 14 years). In the follow-up period, seizure freedom was achieved in 32 patients (84.2 %). Seizure freedom was associated with electroencephalographic normalization, while the six children presenting drug-resistant epilepsy showed persistence of electroencephalographic abnormalities. Group B patients showed a higher prevalence of drug-resistant epilepsy. Patients with Prader-Willi syndrome were frequently affected by generalized seizures. Most of the patients had a favorable evolution, although, patients with brain abnormalities presented a worse outcome, suggesting that the presence of these lesions can influence the response to antiepileptic therapy. Prader–Willi syndrome is a multisystemic genetic disorder that can be associated with epilepsy. There is insufficient information concerning the clinical and electroencephalographic characteristics of epilepsy and the long-term outcome of these patients. The aim of this study is to describe seizure types, electroencephalographic patterns and long-term seizure outcome in Prader–Willi syndrome patients suffering from epilepsy. We retrospectively studied 38 patients with Prader–Willi syndrome and seizures. Results of neuroimaging studies were obtained for 35 individuals. We subdivided these patients into two groups: group A, 24 patients, without brain lesions; and group B, 11 patients, with brain abnormalities. All patients were re-evaluated after a period of at least 10 years. Twenty-one patients (55.2 %) were affected by generalized epilepsy and 17 patients (44.8 %) presented focal epilepsy. The most common seizure type was generalized tonic– clonic seizure. The mean age at seizure onset was 4.5 years (ranged from 1 month to 14 years). In the follow-up period, seizure freedom was achieved in 32 patients (84.2 %). Seizure freedom was associated with electroencephalographic normalization, while the six children presenting drug-resistant epilepsy showed persistence of electroencephalographic abnormalities. Group B patients showed a higher prevalence of drug-resistant epilepsy. Patients with Prader–Willi syndrome were frequently affected by generalized seizures. Most of the patients had a favorable evolution, although, patients with brain abnormalities presented a worse outcome, suggesting that the presence of these lesions can influence the response to antiepileptic therapy.

Le problematiche relative ai disturbi alimentari psicogeni in età evolutiva costituiscono un fenomeno a sempre maggiore impatto sociale e clinico (Linsheid et. al 2003; Bryant-Waugh et al. 2010). Tra le modificazioni più interessanti del DSM-V riveste un ruolo importante la riorganizzazione della classificazione dei disturbi alimentari dell’età infantile e adolescenziale/adulta che – a partire dalle osservazioni cliniche riportate in svariati lavori (es. Davies et al 2006; Bravender et. al. 2007; Attia et. al. 2013; Bryant-Waugh, 2013; Hartmann et al. 2012) - sono stati raccolti in uno stesso gruppo diagnostico. Da un punto di vista epidemiologico è sempre maggiore il numero di utenti che in età pediatrica afferiscono ai centri specializzati per i disturbi del comportamento alimentare. È questo il caso del Centro a valenza regionale per i Disturbi del Comportamento Alimentare in età evolutiva, dell’Unità Operativa di Neuropsichiatria Infantile del Policlinico Sant’Orsola di Bologna. I due aspetti di maggiore rilevanza per l’attività clinica in questo settore e con questa tipologia di popolazione sono: la valutazione diagnostica e la tipologia di intervento clinico. Verranno presentati i dati epidemiologici e di personalità - misurati con la classificazione 0-3 R (Chatoor, 2000), la classificazione di Lask, Btyan-Waugh (2000), il CBCL (Achenbach, Rescorla, 2001), l’EDI-3 (quando applicabile per la fascia di età) e griglie osservative - relativi alla popolazione di utenti afferenti al centro tra i 4 e i 14 anni di età negli anni dal 2011 al 2014; verranno inoltre illustrate le strategie di intervento articolate per fasce di età (0-3; 4-7; 8-14), alcune valutazioni di efficacia e delineate le criticità più comunemente incontrate nel trattamento degli utenti in età pediatrica e dei loro familiari.

Background - A new modified-release (MR) granule formulation of valproate (VPA) has been recently developed for the treatment of children with epilepsy. It consists of tasteless microspheres that can be sprinkled on soft foods and easily swallowed. There are no data on the effectiveness of this formulation in pediatric age. Aim of the study -To evaluate the effects of the abrupt switch from solution to VPA MR granules in children undergoing chronic treatment. Methods -We enrolled children receiving VPA solution as sole or adjunctive therapy and switched them to MR granules at identical dosages. VPA blood level, treatment efficacy (clinical and EEG data), tolerability (adverse reactions), palatability, ease of administration, and compliance were evaluated before switching (T0) and after 4 weeks (T1). Results -Out of 112 enrolled children, 108 (96.4%) completed the evaluation. We observed no significant differences between the patients at T0 and T1 in VPA blood levels, treatment efficacy, tolerability, and compliance. MR granules were judged more palatable (P < 0.05) and easier to administer (P < 0.05) than solution by children and parents. At 6-month follow-up, all patients continued to use MR granules. Conclusion -Modified-release granule formulation of VPA may be a reliable alternative to solution for its convenience of use

Information on the effects of early treatment of seizures on mortality is scarce. The authors assessed the survival of patients with a first generalised tonic-clonic seizure, randomised to immediate treatment (treated) versus treatment only in the event of seizure recurrence (untreated), over a 20-year period. METHODS: The authors followed 419 patients. The median follow-up was 19.7 years (range 0.2-21.5) for a total of 7867 person-years. RESULTS: 40 persons (9.6%) died during follow-up, 19 (8.9%) treated and 21 (10.3.%) untreated. The probability of surviving was 100% at 1 year, 97% (95% CI 95% to 99%) at 5 years, 94% (91-97) at 10 years and 91% (87-95) at 20 years in treated patients and 100%, 98% (95-100), 97% (94-99) and 89% (85-94), respectively, in untreated patients (p=0.7). After adjustment for treatment of first seizure and putative risk factors (gender, age, seizure type, previous uncertain seizures, family history of seizures, pre-, peri- and postnatal risk factors, remote aetiological factors for epilepsy, abnormal neurological examination, CT or MRI abnormalities, EEG abnormalities and acute treatment), only the presence of aetiological factors for epilepsy predicted a higher mortality (HR 3.4, 95% CI 2.5 to 4.3%; p

AIM: To describe a spectrum of intracerebral large artery disease in Aicardi-Goutières syndrome (AGS) associated with mutations in the AGS5 gene SAMHD1. METHOD: We used clinical and radiological description and molecular analysis. RESULTS: Five individuals (three males, two females) were identified as having biallelic mutations in SAMHD1 and a cerebral arteriopathy in association with peripheral vessel involvement resulting in chilblains and ischaemic ulceration. The cerebral vasculopathy was primarily occlusive in three patients (with terminal carotid occlusion and basal collaterals reminiscent of moyamoya syndrome) and aneurysmal in two. Three of the five patients experienced intracerebral haemorrhage, which was fatal in two individuals. Post-mortem examination of one patient suggested that the arteriopathy was inflammatory in origin. INTERPRETATION: Mutations in SAMHD1 are associated with a cerebral vasculopathy which is likely to have an inflammatory aetiology. A similar disease has not been observed in patients with mutations in AGS1 to AGS4, suggesting a particular role for SAMHD1 in vascular homeostasis. Our report raises important questions about the management of patients with mutations in SAMHD1