Switch from enzyme replacement therapy to oral chaperone migalastat for treating fabry disease: real-life data

The treatment options for Fabry disease (FD) are enzyme replacement therapy (ERT) with agalsidase alfa or beta, and the oral pharmacological chaperone migalastat. Since few data are available on the effects of switching from ERT to migalastat, we performed a single-center observational study on seven male Fabry patients (18-66 years) to assess the effects of the switch on renal, cardiac, and neurologic function, health status, pain, lyso-Gb3, α-Gal A activity and adverse effects. Data were retrospectively collected at time of diagnosis of FD (baseline, T0), and after 12 months of ERT (T1), and prospectively after 1 year of therapy with migalastat (T2). No patient died or reported renal, cardiac, or cerebrovascular events during the study period. The predefined measures for cardiac, renal and neurologic function, and FD-related symptoms and questionnaires were stable between baseline and the switch, and remained unchanged with migalastat. However, a significant improvement was observed in left ventricular mass index from baseline to T2 (p = 0.016), with a significative difference between the treatments (p = 0.028), and in median proteinuria from T2 vs T1 (p = 0.048). Moreover, scores of the BPI improved from baseline to T1, and remained stable with migalastat. Plasma lyso-Gb3 levels significantly decreased from baseline to T1 (P = 0.007) and T2 (P = 0.003), while did not significantly differ between the two treatments. α-Gal A activity increased from T0 to T2 (p < 0.0001). The frequency of adverse effects under migalastat and ERT was comparable (28% for both drugs). In conclusion, switching from ERT to migalastat is valid, safe and well tolerated

Corrigendum: oscillatory activities in neurological disorders of elderly: biomarkers to target for neuromodulation

No abstract availble

SFIDE: challenges towards synchronous interaction in e-learning

History of the development of a synchronous tool for e-learning, with University of Verona in the unusual role of services provider

Collective spontaneous emission of two entangled atoms near an oscillating mirror

We consider the cooperative spontaneous emission of a system of two identical atoms interacting with the electromagnetic field in the vacuum state and in the presence of an oscillating mirror. We assume that the two atoms, one in the ground state and the other in the excited state, are prepared in a correlated (symmetric or antisymmetric) Bell-type state. We also suppose that the perfectly reflecting plate oscillates adiabatically, with the field modes satisfying the boundary conditions at the mirror surface at any given instant, so that the time dependence of the interaction Hamiltonian is entirely enclosed in the instantaneous atom-wall distance. Using time-dependent perturbation theory, we investigate the spectrum of the radiation emitted by the two-atom system, showing how the oscillation of the boundary modifies the features of the emitted spectrum, which exhibits two lateral peaks not present in the case of a static boundary. We also evaluate the transition rate to the collective ground state of the two-atom system in both cases of the superradiant (symmetric) and subradiant (antisymmetric) state. We show that it is modulated in time and that the presence of the oscillating mirror can enhance or inhibit the decay rate compared to the case of atoms in vacuum space or near a static boundary. Our results thus suggest that a dynamical (i.e., time-modulated) environment can offer further possibilities to control and manipulate radiative processes of atoms or molecules nearby, such as the cooperative decay, and strongly indicate a similar possibility for other radiative processes, for example, the resonance interaction and the energy transfer between atoms or molecules

Eradication of HCV in Renal Transplant Recipients and Its Effects on Quality of Life

Background. The use of direct antiviral agents (DAA) has radically modified the course of HCV hepatitis in renal patients. Aim of this study was to assess the effects of HCV eradication on quality of life (QOL) in renal transplant recipients (RTR), measured by CLDQ and SF-36. Methods. Sixteen RTR with well preserved GFR (mean: 60.3±19.3 ml/min) and chronic HCV infection with moderate liver stiffness (9.3±1.7 kPa) were given a sofosbuvir-based regimen for 12 weeks and had a 1 year follow-up. Results. At end of treatment (EOT) a complete viral clearance was observed in all the patients, with normalization of most laboratory data and a consistent reduction in liver stiffness. All these parameters remained stable after 1 year, as well as renal function and proteinuria. Questionnaire data showed consistent amelioration in different “emotional” domains at EOT, which persisted after 1 year and were associated with a globally improved QOL, although there was no change in most of the “physical” domains in both questionnaires. One patient under ribavirin developed an acute anemia and withdrew from the study, but no further adverse episode was observed throughout the study. Conclusions. Our data, while confirming the efficacy of oral DAA, show that HCV infection represents a heavy psychological burden in renal transplant recipients, greatly alleviated by viral eradication, which determines a significant improvement in QOL that represents an important outcome in management of all transplant recipients. This trial is registered with ISRCTN97560076