A 27 year-old man initially considered as a schizophrenic patient was later diagnosed as having Kleine-Levin syndrome.

Pulmonary hypertension is a complex and progressive condition that is either idiopathic or heritable, or associated with one or multiple health conditions, with or without congenital or acquired cardiovascular disease. Recent developments have tremendously increased the armamentarium of diagnostic and therapeutic approaches in children and young adults with pulmonary hypertension that is still associated with a high morbidity and mortality. These modalities include non-invasive imaging, pharmacotherapy, interventional and surgical procedures, and supportive measures. The optimal, tailored diagnostic and therapeutic strategies for pulmonary hypertension in the young are rapidly evolving but still face enormous challenges: Healthcare providers need to take the patient’s age, development, disease state, and family concerns into account when initiating advanced diagnostics and treatment. Therefore, there is a need for guidance on core and advanced medical training in paediatric pulmonary hypertension. The Association for European Paediatric and Congenital Cardiology working group “pulmonary hypertension, heart failure and transplantation” has produced this document as an expert consensus statement; however, all recommendations must be considered and applied in the context of the local and national infrastructure and legal regulations.

The influence of a strong external magnetic field on the collimation of a high Mach number plasma flow and its collision with a solid obstacle is investigated experimentally and numerically. The laser irradiation ( $I\sim 2\times 10^{14}~\text{W}\cdot \text{cm}^{-2}$ ) of a multilayer target generates a shock wave that produces a rear side plasma expanding flow. Immersed in a homogeneous 10 T external magnetic field, this plasma flow propagates in vacuum and impacts an obstacle located a few mm from the main target. A reverse shock is then formed with typical velocities of the order of 15–20 $\pm$ 5 km/s. The experimental results are compared with 2D radiative magnetohydrodynamic simulations using the FLASH code. This platform allows investigating the dynamics of reverse shock, mimicking the processes occurring in a cataclysmic variable of polar type.

The Lembo clay deposit occurs on orthogneiss, but it also comprises clays with litho-relicts of volcanic rocks. In this study, ten samples from two sites were investigated. The mineralogical compositions consisted of kaolinite + halloysite + illite + quartz + hematite + anatase ± rutile ± orthoclase ± sanidine ± magnetite ± maghemite ± goethite ± Ba-Al-Fe-phosphates ± carbonates ± sulphates. Kaolinite-halloysite and quartz are the prevailing minerals. Some volcanicderived clays contain Fe-rich kaolinite-halloysite (9.6–14.1 wt.% Fe2O3). The chemical compositions, colours and specific surface area (SSA) measurements reveal two groups of clays: one with a positive whiteness index (IB), small SSA and small Fe content, and the other showing a large SSA, negative IB and relatively large Fe contents. The compositions of the first group are close to those of clays from the Mayouom deposit (Cameroon), and from some European commercial kaolins used in ceramics. Relatively Fe-rich clay materials may conform to most formulations of earthen bricks. As a whole, the Lembo clay deposit comprises various compositions of kaolinitic clays, which may yield the opportunity for extensive application in ceramics.

A new target design is presented to model high-energy radiative accretion shocks in polars. In this paper, we present the experimental results obtained on the GEKKO XII laser facility for the POLAR project. The experimental results are compared with 2D FCI2 simulations to characterize the dynamics and the structure of plasma flow before and after the collision. The good agreement between simulations and experimental data confirms the formation of a reverse shock where cooling losses start modifying the post-shock region. With the multi-material structure of the target, a hydrodynamic collimation is exhibited and a radiative structure coupled with the reverse shock is highlighted in both experimental data and simulations. The flexibility of the laser energy produced on GEKKO XII allowed us to produce high-velocity flows and study new and interesting radiation hydrodynamic regimes between those obtained on the LULI2000 and Orion laser facilities.

The objective was to evaluate the distribution of coagulase-negative staphylococci (CNS) involved in periprosthetic-joint infections (PJIs) and to describe their susceptibility profile to antibiotics. We conducted a multicentre retrospective study in France, including 215 CNS PJIs between 2011 and 2015. CNS PJIs involved knees in 54% of the cases, hips in 39%, other sites in 7%. The distribution of the 215 strains was: Staphylococcus epidermidis 129 (60%), Staphylococcus capitis 24 (11%), Staphylococcus lugdunensis 21 (10%), Staphylococcus warneri 8 (4%), Staphylococcus hominis 7 (3%), Staphylococcus haemolyticus 7 (3%). More than half of the strains (52.1%) were resistant to methicillin, 40.9% to ofloxacin, 20% to rifampicin. The species most resistant to antibiotics were S. hominis, S. haemolyticus, S. epidermidis, with 69.7% of the strains resistant to methicillin and 30% simultaneously resistant to clindamycin, cotrimoxazole, ofloxacin and rifampicin. No strain was resistant to linezolid or daptomycin. In this study on CNS involved in PJIs, resistance to methicillin is greater than 50%. S. epidermidis is the most frequent and resistant species to antibiotics. Emerging species such S. lugdunensis, S. capitis and Staphylococcus caprae exhibit profiles more sensitive to antibiotics. The antibiotics most often active in vitro are linezolid and daptomycin.

Animal health planning activities are not always providing a satisfactory positive impact on herd health and welfare. Moreover, evaluating the impact of advisory programmes is complex due to multiple interacting elements that influence its outcome. Therefore, measuring solely health outcomes is not sufficient: the whole process of the implementation and use of such programmes should be evaluated. In order to evaluate the impact of an intervention with a Herd Health and Production Management (HHPM) programme a process evaluation framework was designed and used. The intervention involved 20 organic dairy cattle farmers and their advisors, in both France and Sweden. In both countries 20 organic dairy farms were selected as control herds. The evaluation of the HHPM programme was based on: (a) the compliance to the programme; (b) the programme’s functions influencing herd health management practices and stimulating dialogue between farmers and advisors; (c) its effectiveness in terms of improving herd health compared with control farms. Complete compliance to the programme was fulfilled by 21 out of 40 farmers–advisors. Results from a questionnaire showed that the programme functioned as intended (e.g. by allowing early identification of herd health problems), stimulated change in farmers’ herd health management practices and farmer–advisor dialogue. Even though the majority of the users perceived that the programme contributed to herd health improvements, no significant differences in health outcomes were found when compared with control farms 12 months after the start of the intervention. The programme allowed creating an environment promoting the exchange of information between farmers and advisors, necessary to define pertinent advice in a farm-specific situation. Future research should aim at improving methods for the evaluation of the effect of advisory programmes, by identifying early indicators for effective advice and developing methods to evaluate the quality of advisory situations without interfering with them.

Different treatment options for pulmonary hypertension have emerged in recent years, and evidence-based management strategies have improved quality of life and survival in adults. In children with pulmonary vascular disease, therapeutic algorithms are not so clearly defined; this study determined current treatment initiation in children with pulmonary hypertension in participating centres of a registry.

Through the multinational Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension registry, patient demographics, diagnosis, and treatment as judged and executed by the local physician were collected. Inclusion criteria were >3 months and <18 years of age and diagnostic cardiac catheterisation consistent with pulmonary hypertension (mean pulmonary arterial pressure ⩾25 mmHg, pulmonary vascular resistance index ⩾3 Wood units×m2, and mean pulmonary capillary wedge pressure ⩽12 mmHg).

At diagnostic catheterisation, 217/244 patients (88.9%) were treatment naïve for pulmonary hypertension-targeted therapy. Targeted therapy was initiated after catheterisation in 170 (78.3%) treatment-naïve patients. A total of 19 patients received supportive therapy, 28 patients were not started on therapy, and 26 patients (10.7%) were on targeted treatment before catheterisation. Among treatment-naïve subjects, treatment was initiated with one targeted drug (n=112, 51.6%), dual therapy (n=39, 18%) or triple-therapy (n=5, 2.3%), and calcium channel blockers with one targeted medication in one patient (0.5%). Phosphodiesterase inhibitors type 5 were used frequently; some patients with pulmonary hypertension related to lung disease received targeted therapy.

There is a diverse therapeutic approach for children with pulmonary hypertension with a need of better-defined treatment algorithms based on paediatric consensus for different aetiologies including the best possible diagnostic workup.

Both white and brown adipose tissues are recognized to be differently involved in energy metabolism and are also able to secrete a variety of factors called adipokines that are involved in a wide range of physiological and metabolic functions. Brown adipose tissue is predominant around birth, except in pigs. Irrespective of species, white adipose tissue has a large capacity to expand postnatally and is able to adapt to a variety of factors. The aim of this review is to update the cellular and molecular mechanisms associated with pre- and postnatal adipose tissue development with a special focus on pigs and ruminants. In contrast to other tissues, the embryonic origin of adipose cells remains the subject of debate. Adipose cells arise from the recruitment of specific multipotent stem cells/progenitors named adipose tissue-derived stromal cells. Recent studies have highlighted the existence of a variety of those cells being able to differentiate into white, brown or brown-like/beige adipocytes. After commitment to the adipocyte lineage, progenitors undergo large changes in the expression of many genes involved in cell cycle arrest, lipid accumulation and secretory functions. Early nutrition can affect these processes during fetal and perinatal periods and can also influence or pre-determinate later growth of adipose tissue. How these changes may be related to adipose tissue functional maturity around birth and can influence newborn survival is discussed. Altogether, a better knowledge of fetal and postnatal adipose tissue development is important for various aspects of animal production, including neonatal survival, postnatal growth efficiency and health.

Vigabatrin (ɣ-vinyl GABA; GVG), an irreversible inhibitor of GABA-transaminase, at a daily dose of 2-4 g, and a placebo were each administered orally for 4 months to 14 patients with cerebellar ataxia (9 with Friedreich's ataxia, 5 with olivopontocerebellar atrophy), in a double-blind, placebo-controlled crossover study. For the group as a whole, there was no significant difference between the GVG and placebo periods in any of the parameters of cerebellar symptomatology measured. Individually, one patient showed some improvement after 3 months of treatment with 2 g/day GVG. Tolerance to 4 g/day GVG was poor, whereas 2 g/day was well tolerated. The results suggest that agents which increase central GABA concentrations are not likely to be of benefit to patients with Friedreich's ataxia or olivopontocerebellar atrophy.

The paramyxean parasite Marteilia refringens infects several bivalve species including European flat oysters Ostrea edulis and Mediterranean mussels Mytilus galloprovincialis. Sequence polymorphism allowed definition of three parasite types ‘M’, ‘O’ and ‘C’ preferably detected in oysters, mussels and cockles respectively. Transmission of the infection from infected bivalves to copepods Paracartia grani could be experimentally achieved but assays from copepods to bivalves failed. In order to contribute to the elucidation of the M. refringens life cycle, the dynamics of the infection was investigated in O. edulis, M. galloprovincialis and zooplankton over one year in Diana lagoon, Corsica (France). Flat oysters appeared non-infected while mussels were infected part of the year, showing highest prevalence in summertime. The parasite was detected by PCR in zooplankton particularly after the peak of prevalence in mussels. Several zooplanktonic groups including copepods, Cladocera, Appendicularia, Chaetognatha and Polychaeta appeared PCR positive. However, only the copepod species Paracartia latisetosa showed positive signal by in situ hybridization. Small parasite cells were observed in gonadal tissues of female copepods demonstrating for the first time that a copepod species other than P. grani can be infected with M. refringens. Molecular characterization of the parasite infecting mussels and zooplankton allowed the distinguishing of three Marteilia types in the lagoon.

N-3 long-chain polyunsaturated fatty acids (n-3 LCPUFA) are subject of growing interest as they are of particular relevance for meat quality and human health. However, their content in the muscles of cattle is generally low probably as the complex result of their biosynthesis from dietary n-3 PUFA in the muscle and/or in other tissues/organs and of their subsequent uptake by the muscle. In view of this, this study aimed at understanding whether the changes in the muscle n-3 LCPUFA content, depending on the diet (maize silage v. grass) or the muscle type (Rectus abdominis, RA v.Semitendinosus, ST) in 12 Charolais steers, were related to variations in the gene expression of proteins involved in n-3 LCPUFA biosynthesis or cellular uptake. Tissue fatty acid composition was analysed by gas-liquid chromatography and mRNA abundance of proteins by quantitative real-time PCR. The grass-based diet resulted in a 2.3-fold (P < 0.0002) increase in both RA and ST n-3 LCPUFA content compared with the maize silage-based diet, whereas no difference in the expression of genes involved in n-3 LCPUFA biosynthesis and uptake was observed between diets. ST exhibited a 1.5-fold higher n-3 LCPUFA content than RA (P < 0.003), whereas the gene expression of proteins involved in n-3 LCPUFA biosynthesis and uptake was 1.3- to 18-fold higher in RA than in ST (P < 0.05). In conclusion, diet- or muscle type-dependent changes in the muscle n-3 LCPUFA content of Charolais steers did not seem to be mediated by the gene expression regulation of proteins involved in the biosynthesis or uptake of these fatty acids.

The reliability of reverse transcription quantitative real-time PCR (RT-qPCR) depends on normalising the mRNA abundance using carefully selected, stable reference genes. Our aim was to propose sets of reference genes for normalisation in bovine or caprine adipose tissue (AT), mammary gland, liver and muscle. All of these tissues contribute to nutrient partitioning and metabolism and, thus, to the profitability of ruminant productions (i.e. carcasses, meat and milk). In this study, eight commonly used reference genes that belong to different functional classes (CLN3, EIF3K, MRPL39, PPIA, RPLP0, TBP, TOP2B and UXT) were analysed using the geNorm procedure to determine the most stable reference genes in bovine and/or caprine tissues. Abundances and rankings of reference genes varied between tissues, species and the combination of tissues and/or species. Therefore, we proposed 29 sets of reference genes that differed depending on the tissue and/or species. As examples of the 29 sets, EIF3K, TOP2B and UXT were proposed as the most stable reference genes in bovine AT; UXT, EIF3K and RPLP0 were the most stable reference genes in bovine and caprine AT. The optimal number of reference genes for data normalisation was 3 for 27 of the proposed 29 sets. In two of the 29 sets, four to five reference genes were necessary for data normalisation when the number of studied tissues was increased. For example, UXT, EIF3K, TBP, TOP2B and CLN3 were required for data normalisation in bovine mammary gland, AT, muscle and liver. We have evaluated some of our proposed sets of reference genes for the normalisation of CD36 gene expression. Normalisation using the three most stable reference genes has revealed downregulation of CD36 gene expression in bovine mammary gland by a concentrate-based diet that is supplemented with sunflower oil and upregulation of CD36 gene expression in caprine liver by including a rapidly degradable starch in the diet. The dietary regulation of the gene expression of CD36 has been erased by normalisation with the least stable reference genes, which may result in misinterpretation of CD36 gene regulation. To conclude, our results provide valuable reference gene sets for other studies that aim to measure tissue and/or species-specific mRNA abundance in ruminants.

Compte tenu de la radiosensibilité accrue des enfantsaux rayonnements ionisants, notre objectif est d’évaluer les risquesde cancers solides et de leucémies à long terme associés aux expositionsmédicales reçues pendant l’enfance. Deux études de cohorte sontréalisées par le Laboratoire d’épidémiologie de l’Institut de radioprotectionet de sûreté nucléaire (IRSN). L’une, la « Cohorte Enfant Scanner »porte sur les enfants nés après le 01/01/1995 et exposés aux rayonnementsionisants lors de scanners réalisés entre 2000 et 2013. L’autreporte sur les enfants exposés lors de procédures de cardiologie interventionnelleavant l’âge de 10 ans entre 2000 et 2013. Pour chacune des cohortes,une évaluation rétrospective individuelle de l’exposition sera réaliséepar l’Unité d’expertise en radioprotection médicale de l’IRSN. Unsuivi passif en termes de cancers solides et de leucémies sera réaliségrâce aux données des registres de cancers pédiatriques et aux donnéesde mortalité. La « Cohorte Enfant Scanner » est constituée de plus80 000 enfants et s’inscrit dans le projet européen EPI-CT, quiregroupe 9 cohortes nationales. À terme, la cohorte des enfantsen cardiologie interventionnelle regroupera plus de 8 000 enfants.Ces études de cohortes, avec un suivi prolongé, permettront d’étudierl’impact sur la santé des expositions radiologiques reçues pendantl’enfance.

There is increasing evidence to indicate that nutrition is an important factor involved in the onset and development of several chronic human diseases including cancer, cardiovascular disease (CVD), type II diabetes and obesity. Clinical studies implicate excessive consumption of medium-chain saturated fatty acids (SFA) and trans-fatty acids (TFA) as risk factors for CVD, and in the aetiology of other chronic conditions. Ruminant-derived foods are significant sources of medium-chain SFA and TFA in the human diet, but also provide high-quality protein, essential micronutrients and several bioactive lipids. Altering the fatty acid composition of ruminant-derived foods offers the opportunity to align the consumption of fatty acids in human populations with public health policies without the need for substantial changes in eating habits. Replacing conserved forages with fresh grass or dietary plant oil and oilseed supplements can be used to lower medium-chain and total SFA content and increase cis-9 18:1, total conjugated linoleic acid (CLA), n-3 and n-6 polyunsaturated fatty acids (PUFA) to a variable extent in ruminant milk. However, inclusion of fish oil or marine algae in the ruminant diet results in marginal enrichment of 20- or 22-carbon PUFA in milk. Studies in growing ruminants have confirmed that the same nutritional strategies improve the balance of n-6/n-3 PUFA, and increase CLA and long-chain n-3 PUFA in ruminant meat, but the potential to lower medium-chain and total SFA is limited. Attempts to alter meat and milk fatty acid composition through changes in the diet fed to ruminants are often accompanied by several-fold increases in TFA concentrations. In extreme cases, the distribution of trans 18:1 and 18:2 isomers in ruminant foods may resemble that of partially hydrogenated plant oils. Changes in milk fat or muscle lipid composition in response to diet are now known to be accompanied by tissue-specific alterations in the expression of one or more lipogenic genes. Breed influences both milk and muscle fat content, although recent studies have confirmed the occurrence of genetic variability in transcript abundance and activity of enzymes involved in lipid synthesis and identified polymorphisms for several key lipogenic genes in lactating and growing cattle. Although nutrition is the major factor influencing the fatty acid composition of ruminant-derived foods, further progress can be expected through the use of genomic or marker-assisted selection to increase the frequency of favourable genotypes and the formulation of diets to exploit this genetic potential.

The analysis of seismic wave propagation and amplification in complex geological structures requires efficient numerical methods. In this article, following up on recent studies devoted to the formulation, implementation and evaluation of 3-D single- and multi-region elastodynamic fast multipole boundary element methods (FM-BEMs), a simple preconditioning strategy is proposed. Its efficiency is demonstrated on both the single- and multi-region versions using benchmark examples (scattering of plane waves by canyons and basins). Finally, the preconditioned FM-BEM is applied to the scattering of plane seismic waves in an actual configuration (alpine basin of Grenoble, France), for which the high velocity contrast is seen to significantly affect the overall efficiency of the multi-region FM-BEM.

This study reports the metabolic and morphological characteristics of bovine intermuscular adipose tissue (AT) throughout foetal growth. Our hypothesis was that the histological and molecular features of intermuscular AT would be different from those previously reported for foetal perirenal AT, based on its anatomical location near the muscle and the recent identification of two distinct adipocyte precursors in mouse AT depending on their locations. To address this question, intermuscular AT was sampled from Charolais and Blond d'Aquitaine foetuses at 180, 210 and 260 days post conception (dpc). The two bovine breeds were chosen because of the higher adiposity of Charolais than Blond d'Aquitaine cattle during the postnatal life. Regardless of the breed, adipocyte volume increased slightly (+38%, P < 0.01) with increasing foetal age. This was concomitant with a decrease (P < 0.05) in the activity of enzymes involved in de novo fatty acid (FA) synthesis (FA synthase and glucose-6-phosphate dehydrogenase) and FA esterification (glycerol-3-phosphate dehydrogenase) when expressed per million adipocytes, and with an increase (P ⩽ 0.01) in mRNA abundances for uncoupling protein 1, adiponectin and leptin (LEP) between 180 and 260 dpc. No difference was observed in the adipocyte volume between breeds, which was consistent with the lack of major between-breed differences in mRNA abundances or activities of enzymes involved in lipid metabolism. The mRNA abundance of lipoprotein lipase was maintained across ages, suggesting a storage of circulating FA rather than of FA synthesized de novo. Plasma LEP increased with foetal age, but only in the Charolais breed (+71%, P ⩽ 0.01), and was two- to threefold higher in Charolais than Blond d'Aquitaine foetuses. Regardless of the breed, bovine intermuscular AT contained predominantly unilocular adipocytes believed to be white adipocytes that were larger at 260 dpc than at 180 dpc. These data thus challenge current concepts of the largely brown nature of bovine foetal AT (based on histological and metabolic features of perirenal AT as previously reported a few days before or after birth).