Xeroderma Pigmentosum

Xeroderma pigmentosum (XP) is defined by extreme sensitivity to sunlight, resulting in sunburn, pigment changes in the skin and a greatly elevated incidence of skin cancers. It is a rare autosomal recessive disorder and has been found in all continents and racial groups. Estimated incidences vary from 1 in 20, 000 in Japan to 1 in 250, 000 in the USA, and approximately 2.3 per million live births in Western Europe. The first features are either extreme sensitivity to sunlight, triggering severe sunburn, or, in patients who do not show this sun-sensitivity, abnormal lentiginosis (freckle-like pigmentation due to increased numbers of melanocytes) on sun-exposed areas. This is followed by areas of increased or decreased pigmentation, skin aging and multiple skin cancers, if the individuals are not protected from sunlight. A minority of patients show progressive neurological abnormalities. There are eight XP complementation groups, corresponding to eight genes, which, if defective, can result in XP. The products of these genes are involved in the repair of ultraviolet (UV)- induced damage in DNA. Seven of the gene products (XPA through G) are required to remove UV damage from the DNA. The eighth (XPV or DNA polymerase h) is required to replicate DNA containing unrepaired damage. There is wide variability in clinical features both between and within XP groups. Diagnosis is made clinically by the presence, from birth, of an acute and prolonged sunburn response at all exposed sites, unusually early lentiginosis in sun-exposed areas or onset of skin cancers at a young age. The clinical diagnosis is confirmed by cellular tests for defective DNA repair. These features distinguish XP from other photodermatoses such as solar urticaria and polymorphic light eruption, Cockayne Syndrome (no pigmentation changes, different repair defect) and other lentiginoses such as Peutz-Jeghers syndrome, Leopard syndrome and Carney complex (pigmentation not sunassociated), which are inherited in an autosomal dominant fashion. Antenatal diagnosis can be performed by measuring DNA repair or by mutation analysis in CVS cells or in amniocytes. Although there is no cure for XP, the skin effects can be minimised by rigorous protection from sunlight and early removal of pre-cancerous lesions. In the absence of neurological problems and with lifetime protection against sunlight, the prognosis is good. In patients with neurological problems, these are progressive, leading to disabilities and a shortened lifespan

The Environmental, Economic and Social Impact of Industry 4.0 in the Food Sector: a Descriptive Literature Review

Industries today are facing new technological changes. Thanks to the introduction of new tools and technologies of the so-called Industry 4.0, industrial processes are becoming more automated and interconnected. In the meantime, laws and international meetings, such as the 2030 Agenda, ask companies to be more sustainable. Considering these premises, this study aims to analyse the available studies that focus on a possible link between these two topics, investigating the sustainability of 4.0 innovations in the food sector. The work carried out a literature analysis on the Scopus scientific database, looking for papers published in English all over the world. Results highlight that among the three pillars of sustainability, the environmental is the most studied one. More efforts should be done, instead, from an economic and social point of view. However, even if the available studies are still few, they are increasing over the last few years, also spreading geographically, underling the importance of these topics in the near future

Time stability of asymmetric Fabry-Perot modulator based analog lightwave links

Lightwave links for analog signal transfer are being developed and evaluated for application in high-density interconnects. The reflective links are based on compact electro-optic intensity modulators connected by ribbons of single-mode fibres to remotely located transceivers (lasers and photoreceivers) and read-out electronics. For long-term characterization, four Asymmetric Fabry-Perot Modulator (AFPM) prototypes were continuously operated and monitored over a period of eight monthes. The collected data allow evaluation of the system time stability and simulation of the possible recalibration procedures. The recalibration requirements to achieve the desirable accuracy and reliability are inferred statistically

Driving the transition to a circular economic model: A systematic review on drivers and critical success factors in circular economy

The circular economy (CE) is arising as a novel economic system that is restorative by design. In light of its capability to boost sustainable economic development and to cope with environmental challenges, it has recently attracted increasing attention from academics, practitioners, policymakers, and intergovernmental organizations. Despite the wide speculation on this issue, the scientific literature lacks a wide-ranging, systematic, and updated identification and classification of the main drivers and Critical Success Factors of CE initiatives, which appears increasingly necessary to facilitate future scientific work, practical implementations, and policy guidelines. With this aim, this paper develops a systematic literature review by starting with over 400 manuscripts. A final set of 55 selected papers was selected for singling out and classifying drivers and Critical Success Factors in the CE context. The results may provide clear indications for further research, may help business organizations in evaluating CE initiatives, and may guide policy makers in developing and refining CE normative frameworks

Space and Time pattern of mid-velocity IMF emission in peripheral heavy-ion collisions at Fermi energies

The emission pattern in the V_perp - V_par plane of Intermediate Mass Fragments with Z=3-7 (IMF) has been studied in the collision 116Sn + 93Nb at 29.5 AMeV as a function of the Total Kinetic Energy Loss of the reaction. This pattern shows that for peripheral reactions most of IMF's are emitted at mid-velocity. Coulomb trajectory calculations demonstrate that these IMF's are produced in the early stages of the reaction and shed light on geometrical details of these emissions, suggesting that the IMF's originate both from the neck and the surface of the interacting nuclei.Comment: 4 pages, 3 figures, RevTex 3.1, submitted to Phys. Rev. Letter

Energy and angular momentum sharing in dissipative collisions

Primary and secondary masses of heavy reaction products have been deduced from kinematics and E-ToF measurements, respectively, for the direct and reverse collisions of 93Nb and 116Sn at 25 AMeV. Light charged particles have also been measured in coincidence with the heavy fragments. Direct experimental evidence of the correlation of energy-sharing with net mass transfer has been found using the information from both the heavy fragments and the light charged particles. The ratio of Hydrogen and Helium multiplicities points to a further correlation of angular momentum sharing with net mass transfer.Comment: 21 pages, 20 figures. Submitted to European Physics Journal