Cittadini senza diritti: abitare e lavorare a Milano da clandestini

Lo scopo di questo rapporto è di presentare le principali caratteristiche socio-demografiche dell’utenza Naga (Associazione Volontaria di Assistenza SocioSanitaria e per i Diritti di Stranieri e Nomadi Onlus), che costituisce una delle più grandi banche dati sull’immigrazione irregolare. A tal fine analizzeremo i dati contenuti nelle cartelle mediche del Naga per i pazienti che sono stati registrati tra il gennaio 2000 e il dicembre 2006, soffermandoci sulla loro nazionalità, genere, situazione familiare, anzianità migratoria, livello di istruzione, condizione lavorativa e situazione abitativa. Dall’analisi emerge un quadro delle caratteristiche e delle condizioni dei migranti privi di permesso di soggiorno del tutto diverso da quello spesso proposto nel dibattito politico e mediatico italiano. Gli immigrati irregolari sono giovani giunti di recente in Italia, ma hanno livelli di istruzione e tassi di occupazione che eguagliano – se non addirittura superano – quelli della popolazione residente in Italia. Le loro condizioni socioabitative, invece, sono assolutamente critiche e lontanissime dagli standard italiani

Fibre-reinforced geopolymer concretes for sensible heat thermal energy storage: Simulations and environmental impact

Power plants based on solar energy are spreading to accomplish the incoming green energy transition. Besides, affordable high-temperature sensible heat thermal energy storage (SHTES) is required. In this work, the temperature distribution and thermal performance of novel solid media for SHTES are investigated by finite element method (FEM) modelling. A geopolymer, with/without fibre reinforcement, is simulated during a transient charging/discharging cycle. A life cycle assessment (LCA) analysis is also carried out to investigate the environmental impact and sustainability of the proposed materials, analysing the embodied energy, the transport, and the production process. A Multi-Criteria Decision Making (MCDM) with the Analytical Hierarchy Process (AHP) approach, taking into account thermal/environmental performance, is used to select the most suitable material. The results show that the localized reinforcement with fibres increases thermal storage performance, depending on the type of fibre, creating curvatures in the temperature profile and accelerating the charge/discharge. High-strength, high-conductivity carbon fibres performed well, and the simulation approach can be applied to any fibre arrangement/material. On the con-trary, the benefit of the fibres is not straightforward according to the three different scenarios developed for the LCA and MCDM analyses, due to the high impact of the fibre production processes. More investigations are needed to balance and optimize the coupling of the fibre material and the solid medium to obtain high thermal performance and low impacts

Migration and public finances in the EU

We provide novel and comprehensive evidence on the net fiscal contributions of natives and migrants to the governmental budgets of EU countries. We account for income taxes and cash benefits, along with indirect taxes and in-kind benefits, which are often missing in standard datasets. We find that on average, migrants were net contributors to public finances over the period of 2014–2018 in the EU and, moreover, that they contribute approximately €1.5 thousand more per capita each year than natives. We also show that this difference is partly due to the selection on characteristics that make migrants net fiscal contributors, such as demographic factors and employment probability

Big Data in Critical Infrastructures Security Monitoring: Challenges and Opportunities

Critical Infrastructures (CIs), such as smart power grids, transport systems, and financial infrastructures, are more and more vulnerable to cyber threats, due to the adoption of commodity computing facilities. Despite the use of several monitoring tools, recent attacks have proven that current defensive mechanisms for CIs are not effective enough against most advanced threats. In this paper we explore the idea of a framework leveraging multiple data sources to improve protection capabilities of CIs. Challenges and opportunities are discussed along three main research directions: i) use of distinct and heterogeneous data sources, ii) monitoring with adaptive granularity, and iii) attack modeling and runtime combination of multiple data analysis techniques.Comment: EDCC-2014, BIG4CIP-201

Hereditary neuropathy with liability to pressure palsy (HNPP): Report of a family with a new point mutation in PMP22 gene

Background: Hereditary neuropathy with liability to pressure palsy (HNPP) is an autosomal dominant disorder most commonly presenting with acute-onset, non-painful focal sensory and motor mononeuropathy. Approximately 80% of patients carry a 1.5 Mb deletion of chromosome 17p11.2 involving the peripheral myelin protein 22 gene (PMP22), the same duplicated in Charcot-Marie-Tooth 1A patients. In a small proportion of patients the disease is caused by PMP22 point mutations. Case presentation: We report on a familial case harbouring a new point mutation in the PMP22 gene. The proband is a 4-years-old girl with acute onset of focal numbness and weakness in her right hand. Electroneurography demonstrated transient sensory and motor radial nerves involvement. In her father, reporting chronic symptoms (cramps and exercise-induced myalgia), we uncovered mild atrophy and areflexia on clinical examination and a mixed (predominantly demyelinating) polyneuropathy with sensory-motor involvement on electrophysiological study. Both carried a nucleotidic substitution c.178 + 2 T > C on intron 3 of the PMP22 gene, involving the splicing donor site, not reported on databases but predicted to be likely pathogenic. Conclusions: We described a previously unreported point mutation in PMP22 gene, which led to the development of a HNPP phenotype in a child and her father. In children evaluated for a sensory and motor transient episode, HNPP disorder due to PMP22 mutations should be suspected. Clinical and electrophysiological studies should be extended to all family members even in the absence of previous episodes suggestive for HNPP

An exo-cell assay for examining real-time γ-secretase activity and inhibition

γ-Secretase is an aspartyl protease that cleaves multiple substrates that are involved in broad biological processes ranging from stem cell development to neurodegeneration. The investigation of γ-secretase has been limited by currently available assays that require genetic or biochemical manipulation in the form of substrate transfection or membrane preparation. Here we report an exo-cell assay that is capable of characterizing γ-secretase activity in any cellular system without limitation. Using a highly active, recombinant substrate this assay can quickly and easily ascertain the status of γ-secretase activity in cell systems and patient samples. We have applied this method to determine the activity of γ-secretase in primary cell samples where transfection and/or membrane isolation are not viable options. Importantly, it allows for the detection of real time γ-secretase activity after inhibitor or drug treatment. The application of this assay to determine the role of γ-secretase in physiological and pathological conditions will greatly facilitate our characterization of this complex protease and help in the development and evaluation of γ-secretase-targeted therapies in Alzheimer's disease or a variety of neoplasms

Neuropsychological and behavioral disorders as presentation symptoms in two brothers with early-infantile niemann-pick type C

Background: Niemann-Pick disease type C (NPC) is a lysosomal storage disease caused by mutations in NPC1 or NPC2 genes. Case presentation: We present two brothers with the same compound heterozygous variants in exon 13 of the NPC1 gene (18q11.2), the first one (c.1955C> G, p. Ser652Trp), inherited from the mother, the second (c.2107T>A p.Phe703Ile) inherited from the father, associated to the classical biochemical phenotype of NPC. The two brothers presented unspecific neurologic symptoms with difference in age of onset: one presented and previously described dyspraxia and motor clumsiness at age 7 years, the other showed a systemic presentation with hepatosplenomegaly noted at the age of two months and neurological symptoms onset at age 4 with speech disturbance. Clinical evolution and neuroimaging data led to the final diagnosis. Systemic signs did not correlate with the onset of neurological symptoms. Miglustat therapy was started in both patients. Conclusions: We highlight the extreme phenotypic heterogeneity of NP-C in the presence of the same genetic variant and the unspecificity of neurologic signs at onset as previously reported. We report some positive effects of miglustat on disease progression assessed also with neuropsychological follow-up, with an age-dependent response