The natural history of hypertrophic cardiomyopathy

In the early years of the disease recognition, hypertrophic cardiomyopathy (HCM) was viewed as an ominous disease with unfavourable prognosis and with an annual mortality between 4% and 6%. At that time, 73% of the patients reported in the literature came from only two referral centres. With the introduction of echocardiography, our understanding of HCM has improved and non-selected patient populations were assembled in several centres. A more benign prognostic profile was documented with an annual mortality rate of 1.5% or less. In the 2000s, important therapeutic interventions further improved the prognosis of patients with HCM: implantable-cardioverter defibrillator for prevention of sudden death, heart transplantation for treatment of severe refractory heart failure, and an extensive treatment with myectomy for relief of left ventricular outflow tract gradient. The natural history of HCM has changed substantially with contemporary treatment achieving an annual mortality rate less than 1% with extended longevity and a greatly improved quality of life

Power consumption evaluation of circuit-switched versus packet-switched optical backbone networks

While telecommunication networks have historically been dominated by a circuit-switched paradigm, the last decades have seen a clear trend towards packet-switched networks. In this paper we evaluate how both paradigms perform in optical backbone networks from a power consumption point of view, and whether the general agreement of circuit switching being more power-efficient holds. We consider artificially generated topologies of various sizes, mesh degrees and not yet previously explored in this context transport linerates. We cross-validate our findings with a number of realistic topologies. Our results show that, as a generalization, packet switching can become preferable when the traffic demands are lower than half the transport linerate. We find that an increase in the network node count does not consistently increase the energy savings of circuit switching over packet switching, but is heavily influenced by the mesh degree and (to a minor extent) by the average link length

Clinical translation of genetic testing in TTR Amyloidosis. genotype-phenotype correlations, management of asymptomatic carriers and familial screening

Transthyretin (TTR)-related amyloidosis (ATTR) is a heterogeneous disease with different organ involvement depending on the type of TTR infiltration [mutated (vTTR) or wild-type (wtTTR)]. Genetic testing in ATTR is required to define diagnosis and identify asymptomatic at-risk family members. Since new therapies are maximally effective in the early stages of the disease, there is a growing agreement about the need for close monitoring of genotype-positive, phenotype-negative individuals to assure a prompt treatment when minor disease signs are detected. This review summarizes the complexity of genotype-phenotype correlation and revises the current indications with respect to familiar screening and management of asymptomatic carriers

One year of COVID-19 pandemic in Italy: Effect of sedentary behavior on physical activity levels and musculoskeletal pain among university students

The COVID-19 outbreak forced Italian students to reduce their daily activities, inducing a seden-tary attitude that was worsened by distanced learning. This study aimed to survey the physical activity levels that were maintained before and during the social restrictions following the pan-demic, their correlation to musculoskeletal pain, as well as analyzing the impact of these COVID-19 restrictions on pain and fatigue that affects daily life activities. A total of 2044 students completed the online questionnaire, of which the results of 1654 participants were eligible. Before the pandemic, the levels of physical activity were distributed as: 19.9% no activity, 30.1% light ac-tivity, 21.5% moderate activity, and 28.5% high activity. After one year of the pandemic, 30.6% of the participants were inactive, 48.1%, 10.9%, and 10.5% stated as maintaining, respectively, light, moderate and high levels of physical activity. Furthermore, 43.5% reported neck pain and 33.5% stated to experience low back pain. Physical activity levels lower than 150 min/week may have predisposed students to suffer from neck pain (1.95 OR at 95% CI, 1.44–2.64) and low back pain (1.79 OR at 95% CI, 1.29–2.49). A positive correlation between physical activity levels, Verbal Descriptive Scale (VDS), and pain frequency have been observed for neck and low back pain (p-value < 0.05). Finally, low physical activity levels were associated with musculoskeletal pain onset and pain worsening

Neuroprotective Effects of Physical Activity via the Adaptation of Astrocytes

The multifold benefits of regular physical exercise have been largely demonstrated in human and animal models. Several studies have reported the beneficial effects of physical activity, both in peripheral tissues and in the central nervous system (CNS). Regular exercise improves cognition, brain plasticity, neurogenesis and reduces the symptoms of neurodegenerative diseases, making timeless the principle of "mens sana in corpore sano" (i.e., a healthy mind in a healthy body). Physical exercise promotes morphological and functional changes in the brain, acting not only in neurons but also in astrocytes, which represent the most numerous glial cells in the brain. The multiple effects of exercise on astrocytes comprise the increased number of new astrocytes, the maintenance of basal levels of catecholamine, the increase in glutamate uptake, the major release of trophic factors and better astrocytic coverage of cerebral blood vessels. The purpose of this review is to highlight the effects of exercise on brain function, emphasize the role of astrocytes in the healthy CNS, and provide an update for a better understanding of the effects of physical exercise in the modulation of astrocyte function

[Assessment of pulmonary function in a follow-up of premature infants: our experience].

Respiratory diseases are a major cause of morbidity in neonates, especially preterm infants; a long term complication of prematurity such as bronchopulmonary dysplasia (BPD) is particularly relevant today. The exact role of the Pulmonary Function Test (PFT) in this area is not yet well defined; the PFT in newborns and infants - in contrast to what happens in uncooperative children and adults - are routinely used only in a few centers. The assessment of pulmonary function in newborns and infants, however, is nowadays possible with the same reliability that in cooperative patients with the possibility to extend the assessment of polmonary function from bench to bed. The assessment of pulmonary function must be carried out with non invasive and safe methods, at the bedside, with the possibility of continuous monitoring and providing adequate calculation and management of data. The ability to assess lung function helps to define the mechanisms of respiratory failure, improving the treatment and its effects and is therefore a useful tool in the follow-up of newborn and infant with pulmonary disease

Safety of aortic aneurysm repair 8 weeks after percutaneous coronary intervention for coronary artery disease: a cohort study

Guidelines advice against dual antiplatelet therapy (DAPT) discontinuation less than 12 months after percutaneous coronary intervention with drug-eluting stents (DES-PCI). However, any delay of necessary surgery in patients with descending thoracic (DTA) or abdominal aortic aneurysm (AAA), treated by DES-PCI, increases the risk of aneurysm rupture/dissection. We evaluated the safety of 8-week waiting time between DES-PCI and endovascular aortic repair (EVAR). 1152 consecutive patients with coronary artery disease (CAD) needing elective DTA or AAA repair were enrolled and divided into two groups. Group A included 830 patients treated by DES-PCI for significant CAD who underwent surgery 8 weeks after implantation. Group B included 322 patients treated by DES-PCI at least 6 months before with no residual significant CAD and treated by elective EVAR. Groups were compared according to a composite of death, myocardial infarction, stent thrombosis, cerebrovascular events and bleeding. No aneurysm rupture/dissection occurred while waiting for surgery. Hospital averse events occurred in 6.2% (52/830) group A patients versus 6.5% (21/322) group B patients (p = 0.8). Mortality was 0.7% (6/830) in group A and 0.9% (3/322) in group B (p = 0.7). Multivariate predictors of events were triple vessel DES-PCI (p 3 stents implanted (p 30 mm (p = 0.02). Eight weeks of waiting after DES-PCI in addition to an adequate management of DAPT were safe in terms of cardiac morbidity and bleeding complications. No aneurysm rupture occurred in the interval before surgery

The deep-sea hub of the ANTARES neutrino telescope

The ANTARES neutrino telescope, currently under construction at 2500 m depth off the French Mediterranean coast, will contain 12 detection lines, powered and read out through a deep-sea junction box (JB) hub. Electrical energy from the shore station is distributed through a transformer with multiple secondary windings and a plugboard with 16 deep sea-mateable electro-optic connectors. Connections are made to the JB outputs using manned or remotely operated submersible vehicles. The triply redundant power management and slow control system is based on two identical AC-powered systems, communicating with the shore through 160 Mb/s fibre G-links and a third battery-powered system using a slower link. We describe the power and slow control systems of the underwater hub

Machine learning approach for computing optical properties of a photonic crystal fiber

Photonic crystal fibers (PCFs) are the specialized optical waveguides that led to many interesting applications ranging from nonlinear optical signal processing to high-power fiber amplifiers. In this paper, machine learning techniques are used to compute various optical properties including effective index, effective mode area, dispersion and confinement loss for a solid-core PCF. These machine learning algorithms based on artificial neural networks are able to make accurate predictions of above-mentioned optical properties for usual parameter space of wavelength ranging from 0.5-1.8 µm, pitch from 0.8-2.0 µm, diameter by pitch from 0.6-0.9 and number of rings as 4 or 5 in a silica solid-core PCF. We demonstrate the use of simple and fast-training feed-forward artificial neural networks that predicts the output for unknown device parameters faster than conventional numerical simulation techniques. Computation runtimes required with neural networks (for training and testing) and Lumerical MODE solutions are also compared

Real-world versus trial patients with transthyretin amyloid cardiomyopathy

Transthyretin (TTR) amyloid cardiomyopathy (ATTR‐AC) is caused either by single‐point mutations in the TTR gene (ATTRv‐AC) or by deposition of the wild‐type protein (ATTRwt‐AC).1 Long been considered a rare disease, ATTR‐AC has been increasingly recognized in recent years, particularly among the elderly,1 mostly due to the possibility of a non‐invasive diagnosis through bone scintigraph