Microscopic Description of 2α Decay in ^{212}Po and ^{224}Ra Isotopes.

A microscopic calculation of half-lives for both the α and 2α decays of ^{212}Po and ^{224}Ra is performed, using a self-consistent framework based on energy density functionals. A relativistic density functional and a separable pairing interaction of finite range are used to compute axially symmetric deformation energy surfaces as functions of quadrupole, octupole, and hexadecapole collective coordinates. Dynamical least-action paths are determined, that trace the α and 2α emission from the equilibrium deformation to the point of scission.

Taming Nuclear Complexity with a Committee of Multilayer Neural Networks.

We demonstrate that a committee of deep neural networks is capable of predicting the ground-state and excited energies of more than 1800 atomic nuclei with an accuracy akin to the one achieved by state-of-the-art nuclear energy density functionals (EDFs) and with significantly less computational cost. An active learning strategy is proposed to train this algorithm with a minimal set of 210 nuclei. This approach enables future fast studies of the influence of EDF parametrizations on structure properties over the whole nuclear chart and suggests that for the first time a machine learning framework successfully encoded several correlated aspects of nuclear deformation.

VEGFA variants as prognostic markers for the retinopathy in pseudoxanthoma elasticum.

Pseudoxanthoma elasticum (PXE) is a rare autosomal recessive ectopic mineralization disorder, characterized by skin, eye and cardiovascular symptoms. The most devastating ocular complication is choroidal neovascularization, which is thought to be mediated by vascular endothelial growth factor (VEGF) signaling, a molecule encoded by the VEGFA gene. As early detection and treatment is essential to preserve vision, prioritization of patients at risk is crucial, but impossible because of wide phenotypic variability and a lack of genotype-phenotype correlations for PXE.

A study of optic nerve head drusen in 38 pseudoxanthoma elasticum (PXE) patients (64 eyes). Location of optic nerve head drusen in PXE.

Purpose: To investigate the prevalence and location of optic nerve head drusen and their potential association with other PXE-related ophthalmic abnormalities.

Materials And Methods: Thirty-eight of the 155 patients (57 male and 98 female aged 49±17 years) included in this retrospective study had optic nerve head drusen. All of the patients underwent a comprehensive ophthalmic examination, including color images using red-free, blue and red filters, autofluorescence imaging and late-phase ICG frames.

[Study of fundus examination by age in 158 pseudoxanthoma elasticum patients].

Purpose: To study the prevalence of fundus anomalies among patients with pseudoxanthoma elasticum as a function of their age.

Material And Methods: All patients have had a complete ophthalmologic examination in multidisciplinary consultation for PXE in a national reference center.

Results: Hundred and fifty-eight patients (60 men and 98 women aged from 10 to 90 years old, mean 45±17 years) were included in a cross-sectional retrospective study.

Lorentz-symmetry test at Planck-scale suppression with a spin-polarized 133 Cs cold atom clock.

We present the results of a Local Lorentz Invariance (LLI) test performed with the 133Cs cold atom clock FO2 [1], hosted at SYRTE. Such test, relating the frequency shift between 133Cs hyperfine Zeeman substates to the Lorentz violating coefficients of the Standard Model Extension (SME), has already been realized in [2] and led to state-of-the-art constraints on several SME proton coefficients. In this second analysis we used an improved model, based on a second order Lorentz transformation and a SCRMF nuclear model, which enables us to extend the scope of the analysis from purely proton to both proton and neutron coefficients.

Lorentz-Symmetry Test at Planck-Scale Suppression With a Spin-Polarized Cs Cold Atom Clock.

We present the results of a local Lorentz invariance (LLI) test performed with the Cs cold atom clock FO2, hosted at SYRTE. Such a test, relating the frequency shift between Cs hyperfine Zeeman substates with the Lorentz violating coefficients of the standard model extension (SME), has already been realized by Wolf et al. and led to state-of-the-art constraints on several SME proton coefficients.

Subretinal fibrosis is associated with fundus pulverulentus in pseudoxanthoma elasticum.

Background: Pseudoxanthoma elasticum (PXE) is a rare autosomal recessive disorder caused by mutations in the ABCC6 gene, resulting in various retinal lesions, among other systemic manifestations. Visual loss may occur in PXE, most commonly caused by choroidal neovascularization and macular atrophy, but little is known about the consequences of fundus pulverulentus (FP) in PXE. The aim of this study was to evaluate ophthalmic outcomes in patients with FP associated with PXE in a large series of PXE patients.

Ranibizumab for choroidal neovascularization secondary to pseudoxanthoma elasticum: 4-year results from the PIXEL study in France.

Purpose: To evaluate the long-term effectiveness and safety of ranibizumab 0.5 mg in patients with choroidal neovascularization (CNV) secondary to pseudoxanthoma elasticum (PXE) in a real-world setting.

Methods: A descriptive, observational, multicenter study in a retrospective and prospective cohort was conducted in France that included patients who had received at least one injection of ranibizumab 0.

[Treatment with ranibizumab for choroidal neovascularization secondary to a pseudoxanthoma elasticum: Results of the French observational study PiXEL].

Introduction: Pseudoxanthoma elasticum (PXE), a rare hereditary connective tissue disorder, may be complicated by angioid streaks (AS) and choroidal neovascularization (CNV), which may lead to irreversible loss of visual acuity (VA). Here we describe the safety and efficacy of ranibizumab in patients with CNV secondary to PXE.

Methods: A multicenter (n=23), observational study of a retrospective/prospective cohort, performed under real world conditions in France in all patients with CNV secondary to PXE who received at least one ranibizumab injection as of October 2011.

Access to large cyclic peptides by a one-pot two-peptide segment ligation/cyclization process.

The use of the N-acetoacetyl protecting group for N-terminal cysteine residue enabled creation of an efficient and mild one-pot native chemical ligation/SEA ligation sequence giving access to large cyclic peptides.

Progressive outer retinal necrosis and immunosuppressive therapy in myasthenia gravis.

Introduction: Progressive outer retinal necrosis (PORN) is a rare but devastating infectious retinitis associated with varicella zoster virus (VZV) and responsible for severe visual loss.

Case Report: A 59-year-old man treated for generalized myasthenia with oral azathioprine and prednisone presented with severe unilateral necrotizing retinitis. Polymerase chain reaction of the aqueous and vitreous humors was diagnostic for VZV PORN.

Blue light-filtering intraocular lenses and post-operative mood: a pilot clinical study.

The purpose of the study was to determine if implantation of blue-filtering intraocular lenses (IOLs) affects post-operative mood, inducing more depression, compared to patients undergoing implantation with conventional IOLs. The study was conducted at the Angers University Hospital, France. This was a prospective with a lowercase pilot study, including consecutive patients planned to undergo cataract surgery in both eyes within 1 week.

Experimental study of the two-body spin-orbit force in nuclei.

Energies and spectroscopic factors of the first 7/2-, 3/2-, 1/2-, and 5/2- states in the (35)Si21 nucleus were determined by means of the (d, p) transfer reaction in inverse kinematics at GANIL using the MUST2 and EXOGAM detectors. By comparing the spectroscopic information on the Si35 and S37 isotones, a reduction of the p3/2-p1/2 spin-orbit splitting by about 25% is proposed, while the f7/2-f5/2 spin-orbit splitting seems to remain constant. These features, derived after having unfolded nuclear correlations using shell model calculations, have been attributed to the properties of the two-body spin-orbit interaction, the amplitude of which is derived for the first time in an atomic nucleus.

[Systemic conditions associated with central and branch retinal artery occlusions].

Introduction: Retinal artery occlusions (RAO) are severe conditions threatening vision, affecting the subsequent mortality of these patients.

Patients And Methods: We retrospectively reviewed the work-up performed in all patients diagnosed with retinal artery occlusions evaluated in two university hospitals in France (Tours and Angers).

Results: A total of 131 patients (131 eyes) with RAO were included, with a mean age of 69.

[Myopia in pseudoxanthoma elasticum].

Introduction: Refraction in patients with pseudoxanthoma elasticum (PXE) has not yet been studied in the literature. The purpose of our study was to determine if PXE may be associated with myopia, similarly to Marfan syndrome, another collagen-vascular disease.

Patients And Methods: This cross-sectional study evaluated 53 consecutive patients (32 women and 21 men, mean age 50 ± 16) from September 2009 to June 2011, with 53 age- and sex-matched controls.

Direct mass measurements of 19B, 22C, 29F, 31Ne, 34Na and other light exotic nuclei.

We report on direct time-of-flight based mass measurements of 16 light neutron-rich nuclei. These include the first determination of the masses of the Borromean drip-line nuclei (19)B, (22)C, and (29)F as well as that of (34)Na. In addition, the most precise determinations to date for (23)N and (31)Ne are reported.

How atomic nuclei cluster.

Nucleonic matter displays a quantum-liquid structure, but in some cases finite nuclei behave like molecules composed of clusters of protons and neutrons. Clustering is a recurrent feature in light nuclei, from beryllium to nickel. Cluster structures are typically observed as excited states close to the corresponding decay threshold; the origin of this phenomenon lies in the effective nuclear interaction, but the detailed mechanism of clustering in nuclei has not yet been fully understood.

Carbohydrate microarrays in 96-well polystyrene microtiter plates.

A method for the preparation of carbohydrate microarrays inside 96-well polystyrene microtiter plates is described. The key step in this strategy represents the synthesis of carbohydrate-dextran conjugates by copper (I)-catalyzed [3 + 2] cycloaddition between alkyne carbohydrate derivative and a specially designed azido dextran polymer. The conjugates adsorb efficiently on polystyrene surface and can be printed inside 96-well plates using a non-contact piezoelectric microarrayer.

Three-component synthesis of neoglycopeptides using a Cu(II)-triggered aminolysis of peptide hydrazide resin and an azide-alkyne cycloaddition sequence.

Copper(II)-induced oxidative aminolysis of hydrazides generates Cu(I), the catalyst of the azide-alkyne cycloaddition. This feature was exploited to design a novel solid phase detaching three-component reaction permitting the conversion of supported peptide hydrazides into 1,2,3-triazole linked C-terminal neoglycopeptides.

Carotid rete mirabile and pseudoxanthoma elasticum: an accidental association?

We report the case of a young female patient with a transient amaurosis due to a carotid rete mirabile (CRM), a rare congenital carotid malformation, and pseudoxanthoma elasticum (PXE), an inherited autosomal recessive systemic metabolic disorder characterised by fragmentation and mineralisation of elastic fibres in connective tissues (skin, eyes) and the vascular system. CRM is a rare form of intracranial carotid malformation whose association with PXE (6 cases at present) would appear not to be accidental. This observation suggests a new link between congenital arterial remodelling and the PXE.

[Retinal lesions in pseudoxanthoma elasticum: 51 patients].

Objective: Description of retinal lesions in patients with pseudoxanthoma elasticum (PXE).

Patients And Methods: This cross sectional study evaluated 51 consecutive patients (31 women and 20 men, mean age, 47 years±16) from June 2008 to September 2010. Clinical and fundoscopic evaluations were performed for each patient with fundus images: colour, red-free, autofluorescence of the central retina, and red-free of the retinal periphery.