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In recent years, the growing need for reducing non-structural damage after earthquakes has stimulated a dedicated effort to develop innovative types of fasteners for anchoring non-structural components (NSCs) to reinforced concrete (RC) host-structures. To contribute to such need, and building on previous research, this paper presents the results of a series of uni-directional shake-table tests of simulated NSCs anchored to concrete via: (1) expansion, and (2) chemical anchors; post-installed into: (a) uncracked, and (b) cracked concrete. Considering different construction details, the experimental investigation focused on traditional anchorage systems, alternative solutions comprising mortar filling into the gap clearance, and a low-damage system relying on supplemental damping devices, capable of reducing the acceleration of the NSCs as well as the force of the anchorage during seismic shakings. The experimental tests provided significant evidence on the beneficial effects of a dissipative anchorage protecting both the non-structural component and the anchorage itself, even during strong earthquakes. Moreover, when construction details allow to close the fixture clearance with a mortar filling, this stiffer solution provide an additional reduction of NSCs seismic accelerations and forces. Therefore, suggestions for further improvements of the adopted low-damage solution are also proposed.

In the last decades, recent earthquakes have further highlighted the high vulnerability of non-structural components. Post-earthquake damage due to building envelope, equipment and building contents can lead to substantial economic losses in terms of repair costs and daily activity interruption (downtime). Moreover, non-structural damage can represent a life-safety threat for both occupants and pedestrians. These considerations confirm the crucial need for developing low-damage systems for either structural or non-structural elements. This paper aims to assess the seismic performance of glazed facade systems, widely adopted in modern buildings, focusing on point fixed glass facade systems (PFGFSs), also referred to as “spider glazing”. In this work, a numerical investigation is developed to study the seismic performance of such systems at both local-connection level through a 3D FEM in ABAQUS as well as at global system level through a simplified lumped plasticity model in SAP 2000 to assess the overall in-plane capacity of the facade. Based on the local connection and global facade system behavior, a novel low-damage connection system is herein proposed, and a parametric study is carried out on the key parameters influencing the facade capacity. The benefits of implementing low-damage connection details are highlighted by an increase of the in-plane capacity of the facade system when compared to a traditional solution. To further investigate the potential of the proposed low-damage details in preserving the integrity of the facade system itself, non-linear time history analyses have been carried out on a case-study building equipped with the innovative PFGFSs.

Myeloid sarcoma (MS) is a very rare disease in both adults and children. Prognosis is poor in adults; in the pediatric age, the prognostic impact of extramedullary disease is controversial. Systemic therapy represents the mainstay of treatment even in isolated MS, but a comparison between different induction regimens is very limited in the literature. To date, it is still not clear if induction treatment should differ from that of other acute myeloid leukemias and stem cell transplant is considered for consolidation in both leukemic patients and in those with isolated disease. Our study describes a retrospective series of 13 cases of MS (adults and children), diagnosed and treated at our institute over 18 years. We report the results of immunophenotypic, cytogenetic and molecular studies, therapeutic approaches, and outcome, in order to establish the best strategy for patients’ workup.

Gaucher disease (GD) has been increasingly recognized as a continuum of phenotypes with variable neurological and sensory involvement. No study has yet specifically explored the spectrum of neuropsychiatric and sensory abnormalities in GD patients through a multidisciplinary approach. Abnormalities involving the nervous system, including sensory abnormalities, cognitive disturbances, and psychiatric comorbidities, have been identified in GD1 and GD3 patients. In this prospective study, named SENOPRO, we performed neurological, neuroradiological, neuropsychological, ophthalmological, and hearing assessments in 22 GD patients: 19 GD1 and 3 GD3. First, we highlighted a high rate of parkinsonian motor and non-motor symptoms (including high rates of excessive daytime sleepiness), especially in GD1 patients harboring severe glucocerebrosidase variants. Secondly, neuropsychological evaluations revealed a high prevalence of cognitive impairment and psychiatric disturbances, both in patients initially classified as GD1 and GD3. Thirdly, hippocampal brain volume reduction was associated with impaired short- and long-term performance in an episodic memory test. Fourthly, audiometric assessment showed an impaired speech perception in noise in the majority of patients, indicative of an impaired central processing of hearing, associated with high rates of slight hearing loss both in GD1 and GD3 patients. Finally, relevant structural and functional abnormalities along the visual system were found both in GD1 and GD3 patients by means of visual evoked potentials and optical coherence tomography. Overall, our findings support the concept of GD as a spectrum of disease subtypes, and support the importance of in-depth periodic monitoring of cognitive and motor performances, mood, sleep patterns, and sensory abnormalities in all patients with GD, independently from the patient’s initial classification.

The aim of this paper is to examine generic competition in the UK, with a special focus on the role of Health Technology Assessment (HTA) on generic market entry and diffusion. In the UK, where no direct price regulation on pharmaceuticals exists, HTA has a leading role for recommending the use of medicines providing a non-regulatory aspect that may influence the dynamics in the generic market. The paper focuses on the role of Technology Appraisals issued by the National Institute for Health and Care Excellence (NICE). We follow a two-step approach. First, we examine the probability of generic entry. Second, conditional on generic entry, we examine the determinants of generic market share. We use data from IQVIA British Pharmaceutical Index (BPI) for the primary care market for 60 products that lost patent between 2003 and 2012. Our results suggest that market size remains one of the main drivers of generic entry. After controlling for market size, intermolecular substitution and difficulty of manufacturing increase the likelihood of generic entry. After generic entry, our estimates suggest that generic market share is highly state dependent. Our findings also suggest that while NICE recommendations do influence generic uptake, there is only marginal evidence they affect generic entry.

Structural safety and environmental sustainability are major factors in investment decisions for building systems, but are rarely considered simultaneously. Recent research efforts have redressed this by developing assessment methodologies and technical solutions for integrated energy efficiency and seismic performance. These studies are typically limited to existing buildings and retrofit interventions at global building scale, whereas an effective framework could and should be part of the design process of either new or existing buildings at both building and component scales. This paper proposes a probabilistic-based assessment framework to assess the building performance in terms of integrated economic losses and support the selection of resilience-enhanced solutions. The proposed methodology is validated through its application to reinforced concrete case-study buildings consisting of traditional vs low-damage earthquake-resistant technologies coupled with energy efficiency strategies. Seismic and energy risk assessment analyses are performed accounting for both modelling uncertainties and earthquake/weather variability. Probabilistic distributions of the integrated economic losses are finally derived to compare the design solutions in terms of risk and reliability. The research outcomes demonstrate the effectiveness of the probabilistic approach for decision-making in building projects. Specifically, it is found that the economic losses can be highly underestimated (greater than40 %) in the single domains (energy or seismic); greater savings and return on investment can be achieved when the seismic safety is involved in the design process; probabilistic distributions and reliability/risk values can represent an effective tool to assess and compare design solutions.

When dealing with seismic risk assessment at a large scale, the collection of relevant building data is still deemed a challenging task, often leading to limited building knowledge and, consequently, high uncertainties. Therefore, innovative yet standardized frameworks and adaptive tools are needed to support the seismic risk assessment of buildings. Towards this goal, this paper proposes a simplified multi-knowledge seismic assessment methodology involving the analytical-mechanical SLaMA (Simple Lateral Mechanism Analysis) method. An ad-hoc data collection form is first developed to identify the building vulnerabilities by merging and building on existing institutional forms at the international level and integrating new input data. The data are then used to implement the SLaMA-based methodology, at different building knowledge levels, to assess the seismic safety and the economic losses of buildings. The proposed data structure and approach is planned to be included in the “Seismic-Response” module for PELL (Public Energy Living Lab)-School platform, aiming to become a standardized and interoperable database for relevant data of Italian schools and a dashboard for allowing stakeholders to continuously monitor their energetic and static/seismic conditions. The paper discusses the potential and effectiveness of the proposed procedure for large-scale applications and its integration into platforms assessing the energy efficiency of buildings.

Rare cases of immune thrombocytopenia (ITP) occurring after SARS-CoV-2 mRNA vaccines have recently reached public attention. It has been reported in patients with previous ITP or other autoimmune diseases and in individuals with an apparent negative past medical history. The management and the outcome of these cases are still not well investigated and reported in the medical literature. A 23-year-old female with a past medical history of ITP, in stable complete remission for 3 years and on mycophenolate treatment received SARS-CoV-2 mRNA vaccine. She presented severe ITP recurrence with hemorrhagic symptoms after the second vaccine dose. A combined treatment with high-dose immunoglobulin and prednisone was successfully administered with a full recovery of platelet count. The patient remains in ITP remission and on mycophenolate therapy, five months later. At our Center, none of the other 76 adult “fragile patients” with ITP on immunosuppressive treatment who had received the SARS-CoV-2 mRNA vaccine, developed such a severe thrombocytopenic recurrence. Follow-up of large cohorts of patients receiving mRNA vaccine will answer the question as to whether it increases the risk of autoimmune conditions. So far, the benefits of the vaccination largely outweigh the risk of infection in these patients.

Post-earthquake damage reports have continuously highlighted the significant vulnerability of nonstructural elements to seismic events. Nonstructural damage has severe impact in the building recovery, increasing the socioeconomic losses even for low intensity events. In the last few years, research efforts have focused on the development of innovative nonstructural solutions, to be combined with damage-resistant structural skeletons in order to obtain an overall highperformance building. As part of a European Union (EU)-funded project, the effectiveness of such integrated skeleton&envelope low-damage system in reducing the earthquake related losses was investigated. Tridimensional shake table tests on a 1:2 scaled timber-concrete low-damage structural skeleton, “dressed” by different innovative nonstructural elements (glass/concrete facades, gypsum/ masonry partitions), were performed at the National Laboratory for Civil Engineering (LNEC) in Lisbon, Portugal. The shake table testswere carried out at increasing seismic intensities to investigate the structural and nonstructural performance up to a higher-than Collapse Prevention Limit State according to the Italian Code (975 years return period). This paper focuses on the dynamic behavior of nonstructural elements with detailed discussion on construction detailing, seismic demand and performance of the innovative solutions. The high performance of nonstructural elements proved the potential of the details introduced in the partitions/facades. Minimal or no damage was observed up to the end of the overall testing sequence, which reached moderate-to-high interstory drift ratios (more than 1.00%) - typically expected to cause severe damage to traditional nonstructural partitions/infills/facades.

Introduction: Patients with cancer may be more susceptible to and have higher morbidity and mortality rates from COVID-19 than the general population, while epidemiologic data specifically addressed to hematologic patients are limited. To investigate whether patients with hematologic diseases undergoing therapy are at increased risk for acquiring SARS CoV-2 infection compared to the general population, a retrospective study was carried out at a referral hematologic center in Rome, Italy, during the period of the greatest epidemic spread (March 8 to May 14, 2020). Methods: All adult and pediatric patients with a diagnosis of a neoplastic or a nonneoplastic hematologic disease who underwent treatment (chemotherapy or immunosuppressive or supportive therapy) during the study period or in the previous 6 months were considered. The prevalence of COVID-19 in the overall outpatient and inpatient population undergoing hematologic treatment compared to that of the general population was analyzed. The measures taken to manage patients during the epidemic period are described. Results: Overall, 2,513 patients with hematological diseases were considered. Out of 243 (9.7%) patients who were screened for SARS CoV-2, three of 119 (2.5%) outpatients with fever or respiratory symptoms and none of 124 asymptomatic patients were diagnosed with COVID-19. Three further patients were diagnosed with COVID-19 and managed in other hospitals in Rome. As of May 14, 2020, the prevalence of COVID-19 in our hematologic population accounted for 0.24% (95% CI 0.23–0.25; 6 of 2,513 patients: 1 case in every 419 patients) as compared to 0.12% (7,280 of 5,879,082 residents; 1 case in every 807 residents) in the general population (p = 0.14). Three of 6 patients diagnosed with COVID-19 required critical care and 2 died while still positive for SARS CoV-2. Out of 225 healthcare providers on duty at our Institution during the study period, 2 (0.9%) symptomatic cases were diagnosed with COVID-19. Conclusion: In our experience, the prevalence of COVID-19 in hematologic patients, mainly affected by malignancies, was not significantly higher compared to that of the general population. Definition of adapted strategies for healthcare services, while continuing to administer the standard hematologic treatments, represents the crucial challenge for the management of hematologic diseases in the COVID-19 era.

Lessons from recent earthquakes have provided a tough reality check of the current seismic design approach, highlighting a critical mismatch between societal expectations over the reality of seismic performance of modern buildings. The performance-based design criteria and objectives need a paradigm shift towards low-damage design philosophy and technologies that should The project provided the opportunity to coordinate a unique research effort for the development and possible future implementation in the European Environment of an integrated low-damage building systems, including structural skeleton and non-structural components for the next generation of buildings.embrace the entire building system. The final goal is the development of a cost-affordable high-performance building system, including structural skeleton, non-structural elements, services and soil foundation systems, capable of sustaining a design level earthquake with limited damage, controllable socio-economic losses and minimum disruption of business. The modern society is waiting for an “earthquake proof” and eco-friendly building at affordable costs. This paper provides an overview and the preliminary results of the EU-funded SERA project titled “(Towards the) Ultimate Earthquake proof Building System: development and testing of integrated low-damage technologies for structural and non-structural elements”. A damage-control building system based on post-tensioned rocking and dissipative mechanisms that combines self-centering and dissipating capabilities was “dressed” with traditional or lowdamage enclosures and fit-outs. A two-storey 1:2 scaled fully prefabricated and dry-assembled building with two bays timber-concrete low-damage seismic frames in one direction and post-tensioned rocking dissipative timber low-damage seismic walls in the other direction was designed and pre-assembled in Italy, then transported and tested on the 3D shaking table of the National Laboratory of Civil Engineering (LNEC) in Lisbon. The building also comprised two different flooring systems, namely a timber-concrete composite floor (TCC) at the first level and a prestressed timberconcrete floor (3PT) at the second level. Three test stages were performed: 1) the bare structural skeleton solution; 2) the structural skeleton including internal gypsum partitions and 3) the integrated skeleton + envelope system. Exterior GFRC panels in the frame direction and spider glazing systems in the wall direction with an internal low-damage clay brick infill wall were investigated during the last stage. Preliminary test results will be herein discussed After many cycles of input motions at different seismic intensities, the integrated low-damage system proved a very high seismic performance, confirming the unique potential of low-damage building system and the opportunity for their widespread implementation in the design and construction practice.

Recent lessons from past earthquakes repeatedly showed the vulnerability of existing buildings and of non-structural elements. In the past decades, efforts have mainly focused on improving the seismic performance of the structural skeleton. Although buildings designed according to modern and recent seismic codes generally performed as expected by securing the human life, the observed damage was often deemed too expensive to be repaired and stakeholders often preferred to demolish and rebuild the buildings. For this reason, within a performance-based seismic design approach, the attention is nowadays focusing on two main objectives: 1) the harmonization of performance objectives between structural and non-structural elements; 2) the development of damage-control or low-damage structural and nonstructural systems. Within the framework of an EU-funded SERA project, titled “(Towards the) Ultimate Earthquake proof Building System: development and testing of integrated low-damage technologies for structural and non-structural elements”, a two-storey 1:2 scale fully prefabricated and dry-assembled timber-concrete low-damage integrated building system, comprising different high performance or damage-resistant non-structural elements (facades, light and heavy partitions), has been tested on a 3D shake-table at the Laboratório Nacional de Engenharia Civil (LNEC) in Lisbon. The experimental program consisted in one - two - and three - dimensional seismic tests performed at increasing intensities. The selected input ground motions were representative of spectral-compatible earthquakes at various level of code-based limit states up to Collapse Prevention. This paper provides the preliminary results of the experimental campaign focusing on the seismic performance of the two typologies of partition wall tested, specifically 1) the fiber-reinforced ceramic gypsum partitions and 2) the lowdamage masonry infill wall. After an initial description of the detailing, the assembly/construction phases and the monitoring system of these elements, the test results are presented in terms of peak floor accelerations and maximum inter-storey drifts in order to investigate the seismic demand at which the non-structural elements were subjected. The seismic performance of the partition walls is then described, focusing on both their global behaviour and the observed damage. Finally, dynamic identification by impact hammer is also provided for the two partition walls.

Acute lymphoblastic leukemia is the most frequent hematologic malignancy in children. Almost 95% of children potentially achieve a complete remission after the induction treatment, but over the last years, new insights in the genomic disease profile and in minimal residual disease detection techniques have led to an improvement in the prognostic stratification, identifying selected patients' subgroups with peculiar therapeutic needs.According to a comprehensive search of peer-review literature performed in Pubmed, in this review we summarize the recent evidences on the induction treatment strategies comprised in the children acute lymphoblastic leukemia scenario, focusing on the role of key drugs such as corticosteroids and asparaginase and discussing the crucial significance of the genomic characterization at baseline which may drive the proper induction treatment choice.Current induction strategies already produce durable remissions in a significant proportion of standard-risk children with acute lymphoblastic leukemia. A broader knowledge of the biologic features related to acute lymphoblastic leukemia subtypes with worse prognosis, and an optimization of targeted drugs now available, might lead to the achievement of long-term molecular remissions in this setting.

Autonomic signs and symptoms could be of epileptic or nonepileptic origin, and the differential diagnosis depends on a number of factors which include the nature of the autonomic manifestations themselves, the occurrence of other nonictal autonomic signs/symptoms, and the age of the patient. Here, we describe twelve children (aged from ten months to six years at the onset of the symptoms) with Panayiotopoulos syndrome misdiagnosed as gastroesophageal reflux disease. Gastroesophageal reflux disease and Panayiotopoulos syndrome may represent an underestimated diagnostic challenge. When the signs/symptoms occur mainly during sleep, a sleep EEG or, if available, a polysomnographic evaluation may be the most useful investigation to make a differential diagnosis between autonomic epileptic and nonepileptic disorders. An early detection can reduce both the high morbidity related to mismanagement and the high costs to the national health service related to the incorrect diagnostic and therapeutic approaches. To decide if antiseizure therapy is required, one should take into account both the frequency and severity of epileptic seizures and the tendency to have potentially lethal autonomic cardiorespiratory involvement. In conclusion, we would emphasize the need to make a differential diagnosis between gastroesophageal reflux disease and Panayiotopoulos syndrome in patients with "an unusual" late-onset picture of GERD and acid therapy-resistant gastroesophageal reflux, especially if associated with other autonomic symptoms and signs.

Background Varicella is an acute infectious disease common during childhood. It has mostly an uncomplicated course in early childhood. Neverthless, it may result in severe complications, especially in particular age groups and clinical conditions. Down Syndrome represents a risk factor for developing complications, because of the frequent comorbidities and their immunodeficiency. Case presentation A 2-year-old white Caucasian female affected by Down Syndrome was referred to our hospital for cardiac arrest in course of varicella disease. After cardiopulmonary resuscitation and stabilization, her clinical conditions didn’t improve and she developed a massive pulmonary hemorrage, which led her to exitus. Conclusions Mortality due to varicella infection is rare, but it is more common in subjects with immune deficit or chronic pathologies, and in particular age-groups. The importance of the vaccine for preventable infectious diseases is stressed in this paper, in which we present a case of death in an unvaccinated cardiopathic child with Down Syndrome affected by varicella.

Objectives Results of epidemiological studies of lipid profiles in individuals with Down Syndrome (DS) in different settings showed discordant results but laboratory norms for this population has been lacking. The aim of our study is to evaluate lipid profiles in a large population of Italian children with DS. Methods Lipid profiles of 357 patients with diagnosis of DS were recorded. RESULTS: Multiple linear regression was employed to estimate models for each lipid fraction as a function of sex and age in patients with DS. Conclusions The main contribution of this paper is to provide data about lipid profile on a large cohort of people with Down syndrome. Long-term surveillance will be crucial to establish if this specific lipid profile may translate into increased morbidity and mortality from cardiovascular diseases (CVD)

Background Immunodeficiency is an integral aspect of Down syndrome, as demonstrated by the increased susceptibility to infection of affected. Mortality is still higher than in general population, with respiratory infections among the major causes of death. As more people with Down syndrome are living today than ever before, it is indispensable to develop strategies to prevent and cure the associated disorders. Vaccination is the most successful instrument of preventive medicine. Special seasonal influenza and pneumococcal vaccination strategies have been designed for individuals with risk conditions of all ages. Down syndrome individuals are not included in the high-risk categories. Methods We enrolled in our study 15 children with Down syndrome and their siblings, vaccinated for the first time with seasonal influenza vaccine and receiving a booster dose of a glyco-conjugated pneumococcal vaccine. We compared the immunological features and response to vaccination measuring serum antibody titers and frequency of specific memory B cells. Results We confirm that a severe reduction of switched memory B cells is always associated to Down syndrome. After primary vaccination Down syndrome children generate significantly less specific switched memory B cells than their siblings. The response to a booster dose of vaccine is instead comparable in both groups. The production of specific antibodies was equally effective in Down syndrome and controls both after primary and secondary immunization. Conclusions Down syndrome individuals should be considered a high risk group, because of their increased susceptibility to infection and reduced number of switched memory B cells. Tailored vaccination protocols are needed in order to reduce their burden of infections throughout life.

Introduction Discoid lupus erythematosus may lead to significant orbital inflammation syndrome. Ocular manifestations related to discoid lupus erythematosus are uncommon and few cases of eye inflammation are reported. Case presentation A 37-year-old Caucasian woman with 5-year history of discoid lupus erythematosus presented with exophthalmos, periorbital pain and blurred vision in her right eye. Orbital computed tomography and laboratory tests were performed. Computed tomography imaging revealed an enlargement of the right medial rectus muscle. Thyroid eye disease and orbital cellulites were excluded. Corticosteroid treatment completely resolved the symptoms. Conclusion This is the first case of orbital myositis in a patient with discoid lupus erythematosus presenting with acute proptosis, diplopia and single extraocular muscle involvement.

Panayiotopoulos syndrome is “a benign age related focal seizure disorder occurring in early and mid-childhood. It is characterized by seizures, often prolonged, with predominantly autonomic symptoms, and by an EEG that shows shifting and/or multiple foci, often with occipital predominance” (definition of International League Against Epilepsy). In literature the clinical features of PS was frequently mistaken as non-epileptic conditions such as acute encephalitis, syncope, migraine, cyclic vomiting syndrome, motion sickness, sleep disorder, or gastroenteritis. In our experience during the last 2 years we have observed 4 children misdiagnosed by clinical history for GERD. Until now PS is underestimated with the consequences of high morbidity and costly mismanagement. These 4 children were referred to general pediatrician at the age of 2–3 years because they started suffering from gastrointestinal emetic symptoms: nausea, retching, and vomiting. The attacks occurred mainly during sleep without sensory-motor clinical signs of seizure. One child also had monthly episodes of severe headache with clinical characteristics of migraine without aura. After a treatment with proton pump inhibitors (PPI) and anti-acid without benefits, according to the literature, they were sent to neurologist for suspection of PS which was confirmed by EEGs occipital features. Because of the importance of the symptomatology, the children have been treated with Lamotrigine with benefit. Conclusions When a child presents symptoms suggestive of GERD but non responding to specific GERD therapy, there is indication to effect an EEG to investigate a PS.

PELL, Public Energy Living Lab, is a platform conceived to support the Public Administration in Italy towards the definition and implementation of interventions on public buildings, aiming to jointly pursue their seismic safety, energy efficiency and environmental certification. This paper presents the on-going activities for the development of the platform PELL-Schools, focusing, in particular, on the “Seismic-Response” module that aims to become a standardized and interoperable database for the collection and collation of relevant data for the seismic vulnerability assessment and for the seismic monitoring of Italian schools. In order to test, tune and modify, if necessary, the proposed PELL-School Seismic-Response module and to contribute to its implementation on Italian school buildings a Working Group has been set up involving several Italian municipalities and stakeholders. The paper concludes with a call to action in viting researchers working on the seismic vulnerability assessment and monitoring of strategic buildings to join the Working Group to contribute their knowledge towards the commo n goal of guarantying the seismic safety of schools and other strategic buildings in Italy