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

Étude du fond d’œil en fonction de l’âge dans le pseudoxanthome élastique chez 158 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. All fundus pictures were retrospectively reviewed over 9 years. Peau d\u27orange (55.6 %), was the first fundus feature observed in younger patients and disappeared with age. Angioid streaks (100 %), appeared during the second decade of life: at first hypofluorescent and then hyperfluorescent after 40 years old. Crystalline bodies (54.1 %), optic nerve head drusen (19 %) and peripheral pigmentation (39.9 %) were found in the various age groups. Macular atrophy (33.5 %) could have resulted from subretinal neovascularization (40.8 %), pattern dystrophies (7.9 %) or subretinal fibrosis (5.3 %). The onset of neovascularization appeared during the fourth decade. DISCUSSION: Progressive centrifugal calcification of Bruch\u27s membrane is the cause of the many fundus features observed in PXE patients. CONCLUSION: Knowledge of the natural history of PXE can help the clinician explain and reassure patients about the evolving risks of ophthalmological involvement, particularly the fact that neovascular complications arise from the fourth decade

Administración agrícola integral

Pertenece a la sección: Desarrollo sustentable: naturaleza y economía.Se advierte la necesidad de una práctica administrativa que conciba integralmente a los sectores agrícolas. Se muestra el mundo sin computadoras ni tecnología, una población carente de instrumentos, pero exigida de organización moderna. El deseado desarrollo de las sociedades modernas topa con la carencia educativa en los ámbitos rurales, encuentra su límite en las prácticas agrícolas que se generan para la subsistencia y que se ven condicionadas por políticas que no favorecen ni responden a la satisfacción de necesidades de quienes trabajan en el campo. Arellano pone de relieve la necesidad de pensar en una administración agrícola integral y estratégica que permita la producción de calidad y una explotación sustentable de los recursos

Combining symmetry breaking and restoration with configuration interaction: Extension to z -signature symmetry in the case of the Lipkin model

© 2018 American Physical Society. Background: Ab initio many-body methods whose numerical cost scales polynomially with the number of particles have been developed over the past fifteen years to tackle closed-shell mid-mass nuclei. Open-shell nuclei have been further addressed by implementing variants based on the concept of spontaneous symmetry breaking (and restoration). These methods typically access ground states properties and some restricted aspects of spectroscopy. Purpose: In order to access the spectroscopy of open-shell nuclei more systematically while controlling the numerical cost, we design a novel many-body method that combines the merit of breaking and restoring symmetries with those brought about by low-rank individual excitations. Methods: The recently proposed truncated configuration-interaction method based on optimized symmetry-broken and -restored states is extended to the SU(2) group associated with total angular momentum. Dealing more specifically with the Lipkin Hamiltonian, the present study focuses on the breaking and the restoration of the z-signature symmetry associated with a discrete subgroup of SU(2). The highly-truncated N-body Hilbert subspace within which the Hamiltonian is diagonalized is spanned by a z-signature broken and restored Slater determinant vacuum and associated low-rank, e.g., one-particle/one-hole and two-particle/two-hole, excitations. Furthermore, the extent by which the symmetry-unrestricted vacuum breaks z-signature symmetry is optimized in the presence of projected low-rank particle-hole excitations. The quality of the method is gauged against exact ground- and excited-state eigenenergies for a large range of values of the two-body interaction strength. Furthermore, results are compared to those obtained from the generator coordinate method, the random-phase approximation, and the self-consistent (second) random-phase approximation. Results: The proposed method provides an excellent reproduction of the ground-state energy and of low-lying excitation energies of various z signatures and total angular momenta across the full range of internucleon coupling defining the Lipkin Hamiltonian and driving the normal-to-deformed quantum phase transition. In doing so, the successive benefits of (i) breaking the symmetry, (ii) restoring the symmetry, (iii) including low-rank particle-hole excitations, and (iv) optimizing the amount by which the underlying vacuum breaks the symmetry are illustrated. While the generator coordinate method, built on the same deformed vacua, provides results of similar quality, this is not the case for the symmetry-restricted random-phase and self-consistent (second) random-phase approximations in the strongly interacting regime. Conclusions: The numerical cost of the newly designed variational method is polynomial with respect to the system size. It achieves a good accuracy on the ground-state energy and the low-lying spectroscopy for both weakly and strongly interacting systems. The present study confirms the results obtained previously for the attractive pairing Hamiltonian in connection with the breaking and restoration of U(1) global gauge symmetry. These two studies constitute a strong motivation to apply this method to realistic nuclear Hamiltonians in view of providing a complementary, accurate and versatile ab initio description of mid-mass open-shell nuclei.status: publishe

Multi-reference many-body perturbation theory for nuclei: I. Novel PGCM-PT formalism

International audiencePerturbative and non-perturbative expansion methods already constitute a tool of choice to perform ab initio calculations over a significant part of the nuclear chart. In this context, the categories of accessible nuclei directly reflect the class of unperturbed state employed in the formulation of the expansion. The present work generalizes to the nuclear many-body context the versatile method of Burton and Thom (J Chem Theory Comput 16(4):5586, 2020) by formulating a perturbative expansion on top of a multi-reference unperturbed state mixing deformed non-orthogonal Bogoliubov vacua, i.e. a state obtained from the projected generator coordinate method (PGCM). Particular attention is paid to the part of the mixing taking care of the symmetry restoration, showing that it can be exactly contracted throughout the expansion, thus reducing significantly the dimensionality of the linear problem to be solved to extract perturbative corrections. While the novel expansion method, coined as PGCM-PT, reduces to the PGCM at lowest order, it reduces to single-reference perturbation theories in appropriate limits. Based on a PGCM unperturbed state capturing (strong) static correlations in a versatile and efficient fashion, PGCM-PT is indistinctly applicable to doubly closed-shell, singly open-shell and doubly open-shell nuclei. The remaining (weak) dynamical correlations are brought consistently through perturbative corrections. This symmetry-conserving multi-reference perturbation theory is state-specific and applies to both ground and excited PGCM unperturbed states, thus correcting each state belonging to the low-lying spectrum of the system under study. The present paper is the first in a series of three and discusses the PGCM-PT formalism in detail. The second paper displays numerical zeroth-order results, i.e. the outcome of PGCM calculations. Second-order, i.e. PGCM-PT(2), calculations performed in both closed- and open-shell nuclei are the object of the third paper

Recent Results from Lohengrin on Fission Yields and Related Decay Properties

The Lohengrin mass spectrometer is one of the 40 instruments built around the reactor of the Institute Laue-Langevin (France) which delivers a very intense thermal neutron flux. Usually, Lohengrin was combined with a high-resolution ionization chamber in order to obtain good nuclear charge discrimination within a mass line, yielding an accurate isotopic yield determination. Unfortunately, this experimental procedure can only be applied for fission products with a nuclear charge less than about 42, i.e. in the light fission fragment region. Since 2008, a large collaboration has started with the aim of studying various fission aspects, mainly in the heavy fragment region. For that, a new experimental setup which allows isotopic identification by γ-ray spectrometry has been developed and validated. This technique was applied on the 239Pu(nth,f) reaction where about 65 fission product yields were measured with an uncertainty that has been reduced on average by a factor of 2 compared with what was that previously available in nuclear data libraries. The same γ-ray spectrometric technique is currently being applied to the study of the 233U(nth,f) reaction. Our aim is to deduce charge and mass distributions of the fission products and to complete the experimental data that exist mainly for light fission fragments. The measurement of 41 mass yields from the 241Am(2nth,f) reaction has been also performed. In addition to these activities on fission yield measurements, various new nanosecond isomers were discovered. Their presence can be revealed from a strong deformed ionic charge distribution compared to a 'normal' Gaussian shape. Finally, a new neutron long-counter detector designed to have a detection efficiency independent of the detected neutron energy has been built. Combining this neutron device with a Germanium detector and a beta-ray detector array allowed us to measure the beta-delayed neutron emission probability Pn of some important fission products for reactor applications