ERS statement on transition of care in childhood interstitial lung diseases.

Interstitial lung diseases (ILD) are a heterogeneous group of rare diffuse diseases affecting the lung parenchyma in children and adults. Childhood interstitial lung diseases (chILD) are often diagnosed at very young age, affect the developing lung, and can have different presentations and prognosis compared to adult forms of these diseases. In addition, chILD in many cases may apparently remit, and have a better response to therapy and better prognosis than adult ILD.

Leriglitazone halts disease progression in adult patients with early cerebral adrenoleukodystrophy.

Cerebral adrenoleukodystrophy (CALD) is an X-linked rapidly progressive demyelinating disease leading to death usually within a few years. The standard of care is haematopoietic stem cell transplantation (HSCT), but many men are not eligible due to age, absence of a matched donor or lesions of the corticospinal tracts (CST). Based on the ADVANCE study showing that leriglitazone decreases the occurrence of CALD, we treated 13 adult CALD patients (19-67 years of age) either not eligible for HSCT (n = 8) or awaiting HSCT (n = 5).

Integrated Stopped-Flow Device for the Study of Porous Materials Using Hyperpolarized Xe NMR.

A simple, low-cost, and efficient device is proposed for the study of porous materials via NMR using small gas probes. Mainly built through additive manufacturing and being equipped with a radiofrequency solenoid microcoil, it only requires tiny quantities of sample and/or gas and is particularly suited for hyperpolarized xenon. The performances of this device have been accessed on a commercial sample of MCM-41 exhibiting multiporosity.

Hubble Tension as a Window on the Gravitation of the Dark Matter Sector.

A simple and minimal extension of the standard cosmological ΛCDM model in which dark matter experiences an additional long-range scalar interaction is demonstrated to alleviate the long-lasting Hubble tension while primordial nucleosynthesis predictions remain unaffected and passing by construction all current local tests of general relativity. The theoretical formulation of this ΛβCDM model and its comparison to astrophysical observations are presented to prove its ability to fit existing data and potentially resolve the tension.

Fertility preservation before cancer treatment: the dilemma of saying 'no' as the price of glory.

A great deal of work has been carried out by professionals in reproductive medicine in order to raise awareness about fertility preservation (FP) techniques, particularly for women, and to ensure that FP is included in the care of young adults treated for cancer or a pathology requiring gonadotoxic treatment. If the importance of the development of our discipline is obvious, our militancy in favour of FP and our emotional projections must not make us forget that medical thinking must be carried out not only on a case-by-case basis, weighing up the benefit-risk balance, but also without losing sight that conceiving a child with one's own gametes is not a vital issue. The cultural importance given to the genetic link with offspring may bias patients' and physicians' decisions, while other ways of achieving parenthood exist, and are often more effective.

Impact of air quality model settings for the evaluation of emission reduction strategies to curb air pollution.

For air quality management, while numerical tools are mainly evaluated to assess their performances on absolute concentrations, this study assesses the impact of their settings on the robustness of model responses to emission reduction strategies for the main criteria pollutants. The effect of the spatial resolution and chemistry schemes is investigated. We show that whereas the spatial resolution is not a crucial setting (except for NO), the chemistry scheme has more impact, particularly when assessing hourly values of the absolute potential of concentrations.

Decomposition of Reinforcement Learning Deficits in Disordered Gambling via Drift Diffusion Modeling and Functional Magnetic Resonance Imaging.

Gambling disorder is associated with deficits in reward-based learning, but the underlying computational mechanisms are still poorly understood. Here, we examined this issue using a stationary reinforcement learning task in combination with computational modeling and functional resonance imaging (fMRI) in individuals that regular participate in gambling (n = 23, seven fulfilled one to three DSM 5 criteria for gambling disorder, sixteen fulfilled four or more) and matched controls (n = 23). As predicted, the gambling group exhibited substantially reduced accuracy, whereas overall response times (RTs) were not reliably different between groups.

The EBI2 receptor is coexpressed with CCR5 in CD4 + T cells and boosts HIV-1 R5 replication.

Objective: CCR5, a G protein-coupled receptor (GPCR), is used by most HIV strains as a coreceptor. In this study, we looked for other GPCR able to modify HIV-1 infection.

Design: We analyzed the effects of one GPCR coexpressed with CCR5, EBI2, on HIV-1 replicative cycle.

Outcome and prognosis of isolated carotid vasculitis.

Objective: To assess the prognosis and outcome of patients with isolated carotid vasculitis.

Methods: We performed a retrospective multicenter study of 36 patients (median age at diagnosis was 37 [IQR 27-45] years and 11 [31 %] patients were men) with initial presentation as isolated carotid vasculitis. Study endpoints included vascular complications, relapses, and progression to large vessel vasculitis (i.

Efficacy and safety of dolutegravir/lamivudine in virologically suppressed female participants: week 48 data from the pooled TANGO and SALSA studies.

Objectives: Women represent >50% of people with HIV globally but have historically been underrepresented in clinical trials. We evaluated the efficacy and safety of switching to dolutegravir/lamivudine (DTG/3TC) vs continuing their current antiretroviral regimen (CAR) by sex assigned at birth (female and male) in virologically suppressed adults with HIV-1 without prior virological failure in a pooled analysis of two randomized controlled trials.

Methods: This analysis included 48-week data from the phase 3 TANGO and SALSA studies.

On-Chip Magnetic Extraction of Circulating Cell-Free DNA from Biological Samples.

In recent years, the analysis of circulating cell-free DNA (cfDNA) containing tumor-derived DNA has emerged as a noninvasive means for cancer monitoring and personalized medicine. However, the isolation of cfDNA from peripheral blood has remained a challenge due to the low abundance and high fragmentation of these molecules. Here, we present a dynamic Magnetic ExTRactiOn (METRO) protocol using microfluidic fluidized bed technology to isolate circulating cfDNA from raw biological materials such as undiluted serum.

Micrometeorite collections: a review and their current status.

Micrometeorites are estimated to represent the main part of the present flux of extraterrestrial matter found on the Earth's surface and provide valuable samples to probe the interplanetary medium. Here, we describe large and representative collections of micrometeorites currently available to the scientific community. These include Antarctic collections from surface ice and snow, as well as glacial sediments from the eroded top of nunataks-summits outcropping from the icesheet-and moraines.

Critical care beyond organ support: the importance of geriatric rehabilitation.

Very old critically ill patients pose a growing challenge for intensive care. Critical illness and the burden of treatment in the intensive care unit (ICU) can lead to a long-lasting decline of functional and cognitive abilities, especially in very old patients. Multi-complexity and increased vulnerability to stress in these patients may lead to new and worsening disabilities, requiring careful assessment, prevention and rehabilitation.

Pulmonary arteries in coelacanths shed light on the vasculature evolution of air-breathing organs in vertebrates.

To date, the presence of pulmonary organs in the fossil record is extremely rare. Among extant vertebrates, lungs are described in actinopterygian polypterids and in all sarcopterygians, including coelacanths and lungfish. However, vasculature of pulmonary arteries has never been accurately identified neither in fossil nor extant coelacanths due to the paucity of fossil preservation of pulmonary organs and limitations of invasive studies in extant specimens.

A roadmap to precision treatments for familial pulmonary fibrosis.

Interstitial lung diseases (ILDs) in adults and children (chILD) are a heterogeneous group of lung disorders leading to inflammation, abnormal tissue repair and scarring of the lung parenchyma often resulting in respiratory failure and death. Inherited factors directly cause, or contribute significantly to the risk of developing ILD, so called familial pulmonary fibrosis (FPF), and monogenic forms may have a poor prognosis and respond poorly to current treatments. Specific, variant-targeted or precision treatments are lacking.

Locally adaptive inversions in structured populations.

Inversions have been proposed to facilitate local adaptation, by linking together locally coadapted alleles at different loci. Prior work addressing this question theoretically has considered the spread of inversions in "continent-island" scenarios in which there is a unidirectional flow of maladapted migrants into the island population. In this setting, inversions capturing locally adaptive haplotypes are most likely to invade when selection is weak, because stronger local selection (i) more effectively purges maladaptive alleles and (ii) generates linkage disequilibrium between adaptive alleles, thus lessening the advantage of inversions.

Microglial TNFα controls daily changes in synaptic GABAARs and sleep slow waves.

Microglia sense the changes in their environment. How microglia actively translate these changes into suitable cues to adapt brain physiology is unknown. We reveal an activity-dependent regulation of cortical inhibitory synapses by microglia, driven by purinergic signaling acting on P2RX7 and mediated by microglia-derived TNFα.

Comprehensive Genetic Profiling Reveals Frequent Alterations of Driver Genes on the X Chromosome in Extranodal NK/T-cell Lymphoma.

Extranodal NK/T-cell lymphoma (ENKTCL) is an Epstein-Barr virus (EBV)-related neoplasm with male dominance and a poor prognosis. A better understanding of the genetic alterations and their functional roles in ENKTCL could help improve patient stratification and treatments. In this study, we performed a comprehensive genetic analysis of 178 ENKTCL cases to delineate the landscape of mutations, copy number alterations (CNA), and structural variations, identifying 34 driver genes including six previously unappreciated ones, namely, HLA-B, HLA-C, ROBO1, CD58, POT1, and MAP2K1.

Large-N Principal Chiral Model in Arbitrary External Fields.

We report the explicit solution for the vacuum state of the two-dimensional SU(N) principal chiral model at large N for an arbitrary set of chemical potentials and any interaction strength, a unique result of such kind for an asymptotically free quantum field theory. The solution matches one-loop perturbative calculation at weak coupling, and in the opposite strong-coupling regime exhibits an emergent spacial dimension from the continuum limit of the SU(N) Dynkin diagram.

Rocking- and diffusion-based culture of tumor spheroids-on-a-chip.

Tumor spheroids are now intensively investigated toward preclinical and clinical applications, necessitating the establishment of accessible and cost-effective methods for routine operations. Without losing the advantage of organ-chip technologies, we developed a rocking system for facile formation and culture of tumor spheroids in hydrogel microwells of a suspended membrane under microfluidic conditions. While the rocking is controlled with a step motor, the microfluidic device is made of two plastic plates, allowing plugging directly syringe tubes with Luer connectors.