Territories in the Middle of the Ford. Mapping and Knowledge for Nature-Based Approach in the South Italy

The paper investigates a particular phenomenon of the industrialization of Southern Italy, starting from the second post-war period, and, in particular, the unsolved relationship between some large industrial areas and the territories of the medium and small cities on which such productive “plates” have been installed. The town planning policies and instruments with which the industrialization of Southern Italy was planned, on the contrary, foresaw a strong integration between urban fabric and industrial agglomerations. Through the case study of the province of Caserta, the research illustrated by the paper investigates, first of all, the policies that supported this process of industrialization and what the territorial effects of this phenomenon were. Next, the paper illustrates the method of knowledge and mapping of the territory of the case study of the industrial development area of Caserta. The data will be collected in a GIS platform that will return the following condition that is nowadays not available: analytical maps of the brownfield areas and the identification of the areas still intact, agricultural or green areas, urban areas and infrastructures, which trace a cognitive framework addressed to the project in which it will possible to experiment some possible eco-innovative and nature-based solutions

Morning blood pressure surge: pathophysiology, clinical relevance and therapeutic aspects

Morning hours are the period of the day characterized by the highest incidence of major cardiovascular events including myocardial infarction, sudden death or stroke. They are also characterized by important neurohormonal changes, in particular, the activation of sympathetic nervous system which usually leads to a rapid increase in blood pressure (BP), known as morning blood pressure surge (MBPS). It was hypothesized that excessive MBPS may be causally involved in the pathogenesis of cardiovascular events occurring in the morning by inducing hemodynamic stress. A number of studies support an independent relationship of MBPS with organ damage, cerebrovascular complications and mortality, although some heterogeneity exists in the available evidence. This may be due to ethnic differences, methodological issues and the confounding relationship of MBPS with other features of 24-hour BP profile, such as nocturnal dipping or BP variability. Several studies are also available dealing with treatment effects on MBPS and indicating the importance of long-acting antihypertensive drugs in this regard. This paper provides an overview of pathophysiologic, methodological, prognostic and therapeutic aspects related to MBPS

Eventos agroalimentarios y de productos típicos: instrumentos de promoción para un territorio del sur de Italia

For most of the hilly, mountainous and inland areas, local and typical agri-food products represent an important tool for producing income, guaranteeing food and nutritional security, providing an answer to basic human needs and the desire to improve the quality of life. The survival of these areas is strongly linked to the persistence of these products, which, in addition to having to be profitable for the communities that produce them and who live from them, must also impose themselves within the framework of the competitiveness imposed by the markets. In fact, it happens that, for many of them, although the production conditions guarantee quality and food safety, the same cannot be said about their ability to compete with most of the semi-industrial productions. Thanks to consumers survey the study proposes a reading of the relationships between food and wine events / manifestations of an internal area in Southern Italy and some local food products offered in the stalls and food stands on occasion of such events. From a methodological point of view, it was decided to apply the “Social Network Analysis” (SNA), comparing gastronomic, folkloristic and cultural events that were detected in the area and with some local food products present in the stalls and stands. The area considered is very interesting with reference to the typical and traditional products, among which the less known and widespread truffle has only recently begun to establish itself. The results showed that some of the products taken into consideration are recurrent, that is, always present in the various events in the area, others less so. The data shows the attractiveness that these events and gastronomic events are able to trigger in consumers and the interest and propensity to buy that the food of local tradition. In particular, tourists seek the experience of local gastronomy and the culture and identity of the destination visited. Reading these data provides some information on the competitive strength that food and wine events and local productions are able to arouse. Events and demonstrations such as those highlighted in the work help to preserve the identity and authenticity of local traditions.Para la mayor parte de las zonas serranas, montañosas y del interior, los productos agroalimentarios locales y típicos representan una importante herramienta para generar ingresos, garantizar la seguridad alimentaria y nutricional, dando respuesta a las necesidades humanas básicas y al deseo de mejorar la calidad de vida. La supervivencia de estas áreas está fuertemente ligada a la persistencia de estos productos, que, además de tener que ser rentables para las comunidades que los producen y que viven de ellos, también deben imponerse en el marco de la competitividad que imponen los mercados. De hecho, sucede que, para muchos de ellos, si bien las condiciones de producción garantizan la calidad y la seguridad alimentaria, no se puede decir lo mismo de su capacidad para competir con la mayoría de las producciones semi-industriales. Gracias a una encuesta a los consumidores, este estudio propone una evaluación de las relaciones entre los eventos / manifestaciones gastronómicas y vinícolas de un área de interior del sur de Italia y algunos productos alimenticios locales que se ofrecen en los puestos de comida con motivo de tales eventos. Desde el punto de vista metodológico, se optó por aplicar el “Análisis de Redes Sociales” (ARS), comparando los eventos gastronómicos, folclóricos y culturales que se detectaron en la zona y con algunos productos alimenticios locales presentes en los puestos. El área considerada es muy interesante en cuanto a los productos típicos y tradicionales, entre los que la trufa es la menos conocida y extendida, y sólo ha comenzado a consolidarse recientemente. Los resultados mostraron que algunos de los productos tomados en consideración son recurrentes, es decir, siempre presentes en los diversos eventos de la zona, otros menos. Los datos muestran el atractivo que estos eventos y jornadas gastronómicas son capaces de suscitar en los consumidores y el interés y propensión a comprar los alimentos de tradición local. En particular, los turistas buscan la experiencia de la gastronomía local y la cultura e identidad del destino visitado. El análisis de estos datos proporciona información sobre la fuerza competitiva que pueden suscitar los eventos enogastronómicos y las producciones locales. Eventos y manifestaciones como los destacados en el trabajo ayudan a preservar la identidad y la autenticidad de las tradiciones locales

Quality Productions, Protection and Recovery of the Internal Areas of Aspromonte: the Role of Truffles

The purpose of this article is to examine the potential of truffle production and  its market in the Aspromonte area. Through interviews with leading operators, collectors, transformers, retailers and restaurateurs, the strengths and weaknesses of the sector, the risks to the external environment and the opportunities offered by the economic system, as well as the needs raised by the operators who in various ways deal with truffles in the Calabria Region was considered. The results show that truffles are natural biological products and play an important role in the management of woodlands: if they are present they are a formidable indicator of environmental health. The results showed that the truffle supply chain can contribute to the promotion of growth strategies for Calabria as indicated among the objectives of the 2030 Strategy Agenda in Goal 11.a Session 1, point 1 which aims at the conservation, redevelopment and promotion of poor or fragile areas with qualitatively significant cultural and environmental heritage. The truffle sector's ability to attract new economic flows generated by production activities, from territorial marketing, from fresh or processed product marketing and from services capable of guaranteeing additional income for farmers and supply chain operators was also highlighted. The results finally showed that the use of SWOT analysis in niche food products provides useful indications for regional development planning actions, for marketing activities, for the promotion of sustainable tourism, for the recovery and enhancement of rural buildings and for the food and wine routes that revolve around the production of truffles and the gastronomy based on fresh and / or processed truffles. Produzioni di pregio, tutela e recupero delle aree interne aspromontane: il ruolo del tartufoScopo di questo articolo è esaminare le potenzialità della produzione e del mercato del tartufo nell’area aspromontana. Attraverso interviste ad opinioni leader, operatori, raccoglitori, trasformatori, rivenditori e ristoratori, sono stati messi in luce i punti di forza e di debolezza del comparto, i rischi dell’ambiente esterno e le opportunità offerte dal sistema economico, nonché le necessità sollevate dagli operatori che a vario titolo si occupano di tartufo nella Regione Calabria.  I risultati mostrano che i tartufi sono prodotti biologici naturali e che svolgono un ruolo importante nella gestione dei terreni boschivi: se sono presenti rappresentano un formidabile indicatore di salubrità ambientale. I risultati hanno evidenziato, altresì che la filiera del tartufo può contribuire alla promozione di strategie di crescita per la Calabria come indicato tra gli obiettivi della Strategia Agenda 2030, Goal 11.a Sessione 1, punto 1, che punta alla conservazione, riqualificazione e promozione di aree disagiate o fragili con patrimoni culturali e ambientali qualitativamente significativi. Si è inoltre evidenziata la capacità del settore tartuficolo di attrarre nuovi flussi economici generati dalle attività di produzione, dal marketing territoriale, dal marketing del prodotto fresco o trasformato e dai servizi idonei a garantire reddito supplementare aggiuntivo per gli agricoltori e per gli operatori della filiera.  I risultati hanno infine mostrato che l'uso dell'analisi SWOT in prodotti alimentari di nicchia fornisce indicazioni utili per le azioni di pianificazione dello sviluppo regionale, per le attività di marketing, per la promozione del turismo sostenibile, per il recupero e la valorizzazione dei fabbricati rurali e per i percorsi enogastronomici che ruotano intorno alla produzione di tartufi e alla gastronomia a base di tartufi freschi e/o trasformati.The purpose of this article is to examine the potential of truffle production and  its market in the Aspromonte area. Through interviews with leading operators, collectors, transformers, retailers and restaurateurs, the strengths and weaknesses of the sector, the risks to the external environment and the opportunities offered by the economic system, as well as the needs raised by the operators who in various ways deal with truffles in the Calabria Region was considered. The results show that truffles are natural biological products and play an important role in the management of woodlands: if they are present they are a formidable indicator of environmental health. The results showed that the truffle supply chain can contribute to the promotion of growth strategies for Calabria as indicated among the objectives of the 2030 Strategy Agenda in Goal 11.a Session 1, point 1 which aims at the conservation, redevelopment and promotion of poor or fragile areas with qualitatively significant cultural and environmental heritage. The truffle sector's ability to attract new economic flows generated by production activities, from territorial marketing, from fresh or processed product marketing and from services capable of guaranteeing additional income for farmers and supply chain operators was also highlighted. The results finally showed that the use of SWOT analysis in niche food products provides useful indications for regional development planning actions, for marketing activities, for the promotion of sustainable tourism, for the recovery and enhancement of rural buildings and for the food and wine routes that revolve around the production of truffles and the gastronomy based on fresh and / or processed truffles. Produzioni di pregio, tutela e recupero delle aree interne aspromontane: il ruolo del tartufoScopo di questo articolo è esaminare le potenzialità della produzione e del mercato del tartufo nell’area aspromontana. Attraverso interviste ad opinioni leader, operatori, raccoglitori, trasformatori, rivenditori e ristoratori, sono stati messi in luce i punti di forza e di debolezza del comparto, i rischi dell’ambiente esterno e le opportunità offerte dal sistema economico, nonché le necessità sollevate dagli operatori che a vario titolo si occupano di tartufo nella Regione Calabria.  I risultati mostrano che i tartufi sono prodotti biologici naturali e che svolgono un ruolo importante nella gestione dei terreni boschivi: se sono presenti rappresentano un formidabile indicatore di salubrità ambientale. I risultati hanno evidenziato, altresì che la filiera del tartufo può contribuire alla promozione di strategie di crescita per la Calabria come indicato tra gli obiettivi della Strategia Agenda 2030, Goal 11.a Sessione 1, punto 1, che punta alla conservazione, riqualificazione e promozione di aree disagiate o fragili con patrimoni culturali e ambientali qualitativamente significativi. Si è inoltre evidenziata la capacità del settore tartuficolo di attrarre nuovi flussi economici generati dalle attività di produzione, dal marketing territoriale, dal marketing del prodotto fresco o trasformato e dai servizi idonei a garantire reddito supplementare aggiuntivo per gli agricoltori e per gli operatori della filiera.  I risultati hanno infine mostrato che l'uso dell'analisi SWOT in prodotti alimentari di nicchia fornisce indicazioni utili per le azioni di pianificazione dello sviluppo regionale, per le attività di marketing, per la promozione del turismo sostenibile, per il recupero e la valorizzazione dei fabbricati rurali e per i percorsi enogastronomici che ruotano intorno alla produzione di tartufi e alla gastronomia a base di tartufi freschi e/o trasformati

Sequence-specific modification of a β-thalassemia locus by small DNA fragments in human erythroid progenitor cells

Gene therapy has been proposed as a definitive cure of beta-thalassemia. We applied a gene targeting approach, based on the introduction of small DNA fragments (SDF) into erythroid progenitor cells, to specifically modify the beta-globin gene sequence at codon 39. The strategy was first tested in normal individuals by delivering mutant SDF that were able to produce the beta-39 (C-T) mutation. Secondly, wild-type SDF were electroporated into target cells of beta-3i9/beta-39 b-thalassemic patients to correct the endogenous mutation. In both cases, gene modification was assayed by allele-specific polymerase chain reaction of DNA and mRNA, by restriction fragment length polymorphism analysis and by direct sequencing

In vitro and in vivo evaluation of BMAP-derived peptides for the treatment of cystic fibrosis-related pulmonary infections

Patients with cystic fibrosis require pharmacological treatment against chronic lung infections. The alpha-helical antimicrobial peptides BMAP-27 and BMAP-28 have shown to be highly active in vitro against planktonic and sessile forms of multidrug-resistant Pseudomonas aeruginosa, Staphylococcus aureus, and Stenotrophomonas maltophilia cystic fibrosis strains. To develop small antibacterial peptides for therapeutic use, we tested shortened/modified BMAP fragments, and selected the one with the highest in vitro antibacterial activity and lowest in vivo acute pulmonary toxicity. All the new peptides have shown to roughly maintain their antibacterial activity in vitro. The 1-18 N-terminal fragment of BMAP-27, showing MIC90 of 16 A mu g/ml against P. aeruginosa isolates and strain-dependent anti-biofilm effects, showed the lowest pulmonary toxicity in mice. However, when tested in a murine model of acute lung infection by P. aeruginosa, BMAP-27(1-18) did not show any curative effect. If exposed to murine broncho-alveolar lavage fluid BMAP-27(1-18) was degraded within 10 min, suggesting it is not stable in pulmonary environment, probably due to murine proteases. Our results indicate that shortened BMAP peptides could represent a starting point for antibacterial drugs, but they also indicate that they need a further optimization for effective in vivo use

Functional analysis of splicing mutations in exon 7 of NF1 gene

BACKGROUND: Neurofibromatosis type 1 is one of the most common autosomal dominant disorders, affecting about 1:3,500 individuals. NF1 exon 7 displays weakly defined exon-intron boundaries, and is particularly prone to missplicing. METHODS: In this study we investigated the expression of exon 7 transcripts using bioinformatic identification of splicing regulatory sequences, and functional minigene analysis of four sequence changes [c.910C>T (R304X), c.945G>A/c.946C>A (Q315Q/L316M), c.1005T>C (N335N)] identified in exon 7 of three different NF1 patients. RESULTS: Our results detected the presence of three exonic splicing enhancers (ESEs) and one putative exonic splicing silencer (ESS) element. The wild type minigene assay resulted in three alternative isoforms, including a transcript lacking NF1 exon 7 (NF1ΔE7). Both the wild type and the mutated constructs shared NF1ΔE7 in addition to the complete messenger, but displayed a different ratio between the two transcripts. In the presence of R304X and Q315Q/L316M mutations, the relative proportion between the different isoforms is shifted toward the expression of NF1ΔE7, while in the presence of N335N variant, the NF1ΔE7 expression is abolished. CONCLUSION: In conclusion, it appears mandatory to investigate the role of each nucleotide change within the NF1 coding sequence, since a significant proportion of NF1 exon 7 mutations affects pre-mRNA splicing, by disrupting exonic splicing motifs and modifying the delicate balance between aberrantly and correctly spliced transcripts

Case Report: Lymphocytosis Associated With Fatal Hepatitis in a Thymoma Patient Treated With Anti-PD1: New Insight Into the Immune-Related Storm

Recent advances in tumor immunotherapy have made it possible to efficiently unleash immune effectors, reacting against neoplastic cells. Although these approaches primarily aim to eradicate malignancy, immune-related adverse events (irAEs) often influence patients’ prognosis, constituting a new spectrum of side effects. Taking into account the typical microenvironment and the intricate equilibrium between the anti-tumor response and the immune cells, the thymoma constitutes a unicum in the immune-oncology field. We report a fatal immune-mediated adverse events’ storm in a thymoma patient treated with Pembrolizumab, leading to hepatotoxicity accompanied by lymphocytosis, thrombocytopenia, and thyroid dysfunction, unveiling a novel potential pathophysiological effect of immunotherapy. The clinical proficiency of the immune checkpoint inhibitors in thymoma patients warrants timely prevention and management of off-target consequences in order to optimize this promising therapeutic option. This case report describes a unique consequence of irAEs, emerging as a red flag warranting a multidisciplinary approach

Novel α-actin gene mutation p.(ala21val) causing familial hypertrophic cardiomyopathy, myocardial noncompaction, and transmural crypts. clinical-pathologic correlation

.Background: Mutations of α-actin gene (ACTC1) have been phenotypically related to various cardiac anomalies, including hypertrophic cardiomyopathy and dilated cardiomyopathy and left ventricular (LV) myocardial noncompaction. A novel ACTC mutation is reported as cosegregating for familial hypertrophic cardiomyopathy and LV myocardial noncompaction with transmural crypts. Methods and results: In an Italian family of 7 subjects, 4 aged 10 (II-1), 14 (II-2), 43 (I-4) and 46 years (I-5), presenting abnormal ECG changes, dyspnea and palpitation (II-2, I-4, and I-5), and recurrent cerebral ischemic attack (I-5), underwent 2-dimensional echo, cardiac magnetic resonance, Holter monitoring, and next-generation sequencing gene analysis. Patients II-2 and I-5 with ventricular tachycardia underwent a cardiac invasive study, including coronary with LV angiography and endomyocardial biopsy. In all the affected members, ECG showed right bundle branch block and left anterior hemiblock with age-related prolongation of QRS duration. Two-dimensional echo and cardiac magnetic resonance documented LV myocardial noncompaction in all and in I-4, I-5, and II-2 a progressive LV hypertrophy up to 22-mm maximal wall thickness. Coronary arteries were normal. LV angiography showed transmural crypts progressing to spongeous myocardial transformation with LV dilatation and dysfunction in the oldest subject. At histology and electron microscopy detachment of myocardiocytes were associated with cell and myofibrillar disarray and degradation of intercalated discs causing disanchorage of myofilaments to cell membrane. Next-generation sequencing showed in affected members an unreported p.(Ala21Val) mutation of ACTC. Conclusions: Novel p.(Ala21Val) mutation of ACTC1 causes myofibrillar and intercalated disc alteration leading to familial hypertrophic cardiomyopathy and LV myocardial noncompaction with transmural crypt

Myocardial fibrosis and diastolic dysfunction in patients on chronic haemodialysis

BACKGROUND: Left ventricular (LV) diastolic dysfunction is linked to myocardial collagen content in many cardiac diseases. There are no data regarding such relationship in patients with end-stage renal disease (ESRD) undergoing haemodialysis. METHODS: Twenty-five patients with ESRD undergoing haemodialysis were studied by echocardiography. LV diastolic function was investigated by Doppler echocardiography, by analysing LV filling velocities at rest and during loading manoeuvres, which represent an estimate of LV filling pressure. According to the Doppler pattern, LV filling pressure in a given patient was judged to be normal or slightly increased or to be moderately or severely increased. The presence of myocardial fibrosis was estimated by ultrasound tissue characterization with integrated backscatter, which in diastole correlates with the collagen content of the myocardium. RESULTS: Integrated backscatter was higher in patients with moderate or severely increased than in patients with normal or slightly increased LV filling pressure (integrated backscatter: 51.0 +/- 9.8 vs 41.6 +/- 5.6%; P = 0.008). Integrated backscatter was a strong and independent determinant of diastolic dysfunction (odds ratio = 1.212; P = 0.040). CONCLUSION: Our data support the hypothesis that, in a selected population of patients with ESRD undergoing haemodialysis, myocardial fibrosis is associated with LV diastolic myocardial propertie