Health-related quality of life of food-allergic children compared with healthy controls and other diseases

Background Food allergy is a potentially life-threatening disease, affecting up to 10% of the pediatric population. Objective The aim of our study was to assess the health-related quality of life (HRQL) of food-allergic patients compared with the general population and patients with other chronic diseases with dietary or allergic burden, in a cross-sectional study. Methods We recruited patients aged 8-17 years diagnosed with food allergy and matched healthy controls recruited in schools. We also included patients with asthma, inflammatory bowel disease, celiac disease, diabetes, obesity, and eating disorders. We used the CHQ-CF87 questionnaire for generic HRQL assessment. Food allergy HRQL was also assessed using specific questionnaires: Food Allergy Quality of Life Questionnaire (FAQLQ) and Food Allergy Independent Measure (FAIM). Results One hundred and thirty-five food-allergic children, 255 children with chronic diseases, and 463 healthy controls were included in the analyses. Food-allergic patients had a better HRQL than healthy controls in the Behavior (BE), Bodily Pain (BP), Family Activities (FA), and Mental Health (MH) domains and a worse HRQL in the General Health Perception (GH) domain (p = .048). Food-allergic patients exhibited a better HRQL than patients affected by other chronic diseases, notably diabetes. Although an epinephrine autoinjector had been prescribed to 87.4% of the food-allergic children, only 54.2% of them carried it at all times. Conclusion Food-allergic patients display overall good HRQL compared with the general population and those with other diseases with daily symptoms and treatments, in line with recent improvements in food allergy management

STAT3 Gain of Function: A New Kid on the Block in Interstitial Lung Diseases

International audienceA 5-year-old girl with failure to thrive and multiorgan disease was referred to our center for chronic hypoxemia. On evaluation, we noted tachypnea (respiratory rate 35/min), supraclavicular retractions, median diurnal oxygen saturation as measured by pulse oximetry (Sp O 2) = 91.7% at rest, percentage of time below Sp O 2 90% at 26% during sleep, and clubbing. A computed tomography scan showed diffuse interstitial lung disease (Figure 1). Spirometry was normal (TLC, 83% of predicted; FEV 1 , 83% of predicted; FEV 1 /FVC, 98%; and forced expiratory flow, midexpiratory phase, 142% of predicted), but it was not possible to measure DL CO

A phase 2 randomized, double-blind, placebo-controlled, proof-of-concept study of oral seletalisib in primary Sjögren's syndrome.

OBJECTIVES This phase 2 proof-of-concept study (NCT02610543) assessed efficacy, safety and effects on salivary gland inflammation of seletalisib, a potent and selective PI3Kδ inhibitor, in patients with moderate-to-severe primary Sjögren's syndrome (PSS). METHODS Adults with PSS were randomized 1:1 to seletalisib 45 mg/day or placebo, in addition to current PSS therapy. Primary end points were safety and tolerability and change from baseline in EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) score at week 12. Secondary end points included change from baseline at week 12 in EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) score and histological features in salivary gland biopsies. RESULTS Twenty-seven patients were randomized (seletalisib n = 13, placebo n = 14); 20 completed the study. Enrolment challenges led to early study termination with loss of statistical power (36% vs 80% planned). Nonetheless, a trend for improvement in ESSDAI and ESSPRI [difference vs placebo: -2.59 (95% CI: -7.30, 2.11; P=0.266) and -1.55 (95% CI: -3.39, 0.28), respectively] was observed at week 12. No significant changes were seen in saliva and tear flow. Serious adverse events (AEs) were reported in 3/13 of patients receiving seletalisib vs 1/14 for placebo and 5/13 vs 1/14 discontinued due to AEs, respectively. Serum IgM and IgG concentrations decreased in the seletalisib group vs placebo. Seletalisib demonstrated efficacy in reducing size and organisation of salivary gland inflammatory foci and in target engagement, thus reducing PI3K-mTOR signalling compared with placebo. CONCLUSION Despite enrolment challenges, seletalisib demonstrated a trend towards clinical improvement in patients with PSS. Histological analyses demonstrated encouraging effects of seletalisib on salivary gland inflammation and organisation. TRIAL REGISTRATION https://clinicaltrials.gov, NCT02610543

Genotype/phenotype correlations of childhood-onset congenital sideroblastic anaemia in a European cohort

Congenital sideroblastic anaemia (CSA) is a rare disease caused by germline mutations of genes involved in haem and iron-sulphur cluster formation, and mitochondrial protein biosynthesis. We performed a retrospective multicentre European study of a cohort of childhood-onset CSA patients to explore genotype/phenotype correlations. We studied 23 females and 20 males with symptoms of CSA. Among the patients, the most frequently mutated genes were ALAS2 (n = 10; 23·3%) and SLC25A38 (n = 8; 18·6%), causing isolated forms of microcytic anaemia of varying severity. Five patients with SLC19A2 mutations suffered from thiamine-responsive megaloblastic anaemia and three exhibited the 'anaemia, deafness and diabetes' triad. Three patients with TRNT1 mutations exhibited severe early onset microcytic anaemia associated with thrombocytosis, and two exhibited B-cell immunodeficiency, inflammatory syndrome and psychomotor delay. The prognoses of patients with TRNT1 and SLC2A38 mutations were generally dismal because of comorbidities or severe iron overload. No molecular diagnosis could be established in 14/43 cases. This study emphasizes the frequency of ALAS2 and SLC25A38 mutations and provides the largest comprehensive analysis to date of genotype/phenotype correlations in CSA. Further studies of CSA patients with data recorded in an international registry would be helpful to improve patient management and establish standardized guidelines

Data-driven precision determination of the material budget in ALICE

International audienceThe knowledge of the material budget with a high precision is fundamental for measurements of direct photon production using the photon conversion method due to its direct impact on the total systematic uncertainty. Moreover, it influences many aspects of the charged-particle reconstruction performance. In this article, two procedures to determine data-driven corrections to the material-budget description in ALICE simulation software are developed. One is based on the precise knowledge of the gas composition in the Time Projection Chamber. The other is based on the robustness of the ratio between the produced number of photons and charged particles, to a large extent due to the approximate isospin symmetry in the number of produced neutral and charged pions. Both methods are applied to ALICE data allowing for a reduction of the overall material budget systematic uncertainty from 4.5% down to 2.5%. Using these methods, a locally correct material budget is also achieved. The two proposed methods are generic and can be applied to any experiment in a similar fashion

Study of the p−-p−-K+^+ and p−-p−-K−^- dynamics using the femtoscopy technique

International audienceThe interactions of kaons (K) and antikaons ($\mathrm{\overline{K}}$) with few nucleons (N) were studied so far using kaonic atom data and measurements of kaon production and interaction yields in nuclei. Some details of the three-body KNN and $\mathrm{\overline{K}}$NN dynamics are still not well understood, mainly due to the overlap with multi-nucleon interactions in nuclei. An alternative method to probe the dynamics of three-body systems with kaons is to study the final state interaction within triplet of particles emitted in pp collisions at the Large Hadron Collider, which are free from effects due to the presence of bound nucleons. This Letter reports the first femtoscopic study of p$-$p$-$K$^+$ and p$-$p$-$K$^-$ correlations measured in high-multiplicity pp collisions at $\sqrt{s}$ = 13 TeV by the ALICE Collaboration. The analysis shows that the measured p$-$p$-$K$^+$ and p$-$p$-$K$^-$ correlation functions can be interpreted in terms of pairwise interactions in the triplets, indicating that the dynamics of such systems is dominated by the two-body interactions without significant contributions from three-body effects or bound states

Measurements of jet quenching using semi-inclusive hadron+jet distributions in pp and central Pb−-Pb collisions at sNN=5.02\sqrt{s_{\rm NN}}=5.02 TeV

The ALICE Collaboration reports measurements of the semi-inclusive distribution of charged-particle jets recoiling from a high transverse momentum (high $p_{\rm T}$) charged hadron, in pp and central Pb$-$Pb collisions at center of mass energy per nucleon$-$nucleon collision $\sqrt{s_{\rm NN}}=5.02$ TeV. The large uncorrelated background in central Pb$-$Pb collisions is corrected using a data-driven statistical approach, which enables precise measurement of recoil jet distributions over a broad range in $p_{\rm T,ch\,jet}$ and jet resolution parameter $R$. Recoil jet yields are reported for $R=0.2$, 0.4, and 0.5 in the range $7 < p_{\rm T,ch\, jet} < 140$ GeV$/c$ and $\pi/2<\Delta\varphi<\pi$, where $\Delta\varphi$ is the azimuthal angular separation between hadron trigger and recoil jet. The low $p_{\rm T,ch\,jet}$ reach of the measurement explores unique phase space for studying jet quenching, the interaction of jets with the quark-gluonnplasma generated in high-energy nuclear collisions. Comparison of $p_{\rm T,ch\,jet}$ distributions from pp and central Pb$-$Pb collisions probes medium-induced jet energy loss and intra-jet broadening, while comparison of their acoplanarity distributions explores in-medium jet scattering and medium response. The measurements are compared to theoretical calculations incorporating jet quenching.The ALICE Collaboration reports measurements of the semi-inclusive distribution of charged-particle jets recoiling from a high transverse momentum (high $p_{\rm T}$) charged hadron, in pp and central Pb$-$Pb collisions at center of mass energy per nucleon$-$nucleon collision $\sqrt{s_{\rm NN}}=5.02$ TeV. The large uncorrelated background in central Pb$-$Pb collisions is corrected using a data-driven statistical approach, which enables precise measurement of recoil jet distributions over a broad range in $p_{\rm T,ch\,jet}$ and jet resolution parameter $R$. Recoil jet yields are reported for $R=0.2$, 0.4, and 0.5 in the range $7 < p_{\rm T,ch\, jet} < 140$ GeV$/c$ and $\pi/2<\Delta\varphi<\pi$, where $\Delta\varphi$ is the azimuthal angular separation between hadron trigger and recoil jet. The low $p_{\rm T,ch\,jet}$ reach of the measurement explores unique phase space for studying jet quenching, the interaction of jets with the quark-gluonnplasma generated in high-energy nuclear collisions. Comparison of $p_{\rm T,ch\,jet}$ distributions from pp and central Pb$-$Pb collisions probes medium-induced jet energy loss and intra-jet broadening, while comparison of their acoplanarity distributions explores in-medium jet scattering and medium response. The measurements are compared to theoretical calculations incorporating jet quenching

Dielectron production in central Pb−-Pb collisions at sNN\sqrt{s_\mathrm{NN}} = 5.02 TeV

International audienceThe first measurement of the e$^+$e$^-$ pair production at midrapidity and low invariant mass in central Pb$-$Pb collisions at $\sqrt{s_{\mathrm{NN}}}=5.02$ TeV at the LHC is presented. The yield of e$^+$e$^-$ pairs is compared with a cocktail of expected hadronic decay contributions in the invariant mass ($m_{\rm ee}$) and pair transverse momentum ($p_{\rm T,ee}$) ranges $m_{\rm ee} < 3.5$ GeV$/c^2$ and $p_{\rm T,ee} < 8$ GeV$/c$. For $0.18 < m_{\rm ee} < 0.5$ GeV$/c^2$ the ratio of data to the cocktail of hadronic contributions without $\rho$ mesons amounts to $1.42 \pm 0.12 \ ({\rm stat.}) \pm 0.17 \ ({\rm syst.}) \pm 0.12 \ ({\rm cocktail})$ and $1.44 \pm 0.12 \ ({\rm stat.}) \pm 0.17 \ ({\rm syst.}) ^{+0.17}_{-0.21} \ ({\rm cocktail})$, including or not including medium effects in the estimation of the heavy-flavor background, respectively. It is consistent with predictions from two different models for an additional contribution of thermal e$^+$e$^-$ pairs from the hadronic and partonic phases. In the intermediate-mass range ($1.2 < m_{\rm ee} < 2.6$ GeV$/c^2$), the pair transverse impact parameter of the e$^+$e$^-$ pairs (DCA$_{\rm ee}$) is used for the first time in Pb$-$Pb collisions to separate displaced dielectrons from heavy-flavor hadron decays from a possible (thermal) contribution produced at the interaction point. The data are consistent with a suppression of e$^+$e$^-$ pairs from ${\rm c\overline{c}}$ and an additional prompt component. Finally, the first direct-photon measurement in the 10% most central Pb$-$Pb collisions at $\sqrt{s_{\mathrm{NN}}}=5.02$ TeV is reported via the study of virtual direct photons in the transverse momentum range $1 < p_{\rm T} < 5$ GeV$/c$. A model including prompt photons, as well as photons from the pre-equilibrium and fluid-dynamic phases, can reproduce the result, while being at the upper edge of the data uncertainties

System size dependence of hadronic rescattering effect at LHC energies

International audienceThe first measurements of $\mathrm{K^{*}(892)^{0}}$ resonance production as a function of charged-particle multiplicity in Xe$-$Xe collisions at $\sqrt{s_{\mathrm{NN}}}=$ 5.44 TeV and pp collisions at $\sqrt{s}=$ 5.02 TeV using the ALICE detector are presented. The resonance is reconstructed at midrapidity ($|y|< 0.5$) using the hadronic decay channel $\mathrm{K^{*0}} \rightarrow \mathrm{K^{\pm} \pi^{\mp}}$. Measurements of transverse-momentum integrated yield, mean transverse-momentum, nuclear modification factor of $\mathrm{K^{*0}}$, and yield ratios of resonance to stable hadron ($\mathrm{K^{*0}}$/K) are compared across different collision systems (pp, p$-$Pb, Xe$-$Xe, and Pb$-$Pb) at similar collision energies to investigate how the production of $\mathrm{K^{*0}}$ resonances depends on the size of the system formed in these collisions. The hadronic rescattering effect is found to be independent of the size of colliding systems and mainly driven by the produced charged-particle multiplicity, which is a proxy of the volume of produced matter at the chemical freeze-out. In addition, the production yields of $\mathrm{K^{*0}}$ in Xe$-$Xe collisions are utilized to constrain the dependence of the kinetic freeze-out temperature on the system size using HRG-PCE model

Measurement of inclusive charged-particle jet production in pp and p-Pb collisions at sNN=5.02\sqrt{s_{\rm NN}}=5.02 TeV

International audienceMeasurements of inclusive charged-particle jet production in pp and p-Pb collisions at center-of-mass energy per nucleon-nucleon collision $\sqrt{s_{\rm NN}} = 5.02$ TeV and the corresponding nuclear modification factor $R_{\rm pPb}^{\rm ch\,jet}$ are presented, using data collected with the ALICE detector at the LHC. Jets are reconstructed in the central rapidity region $|\eta_{\rm jet}| < 0.5$ from charged particles using the anti-$k_{\rm T}$ algorithm with resolution parameters $R = 0.2$, 0.3, and 0.4. The $p_{\rm T}$-differential inclusive production cross section of charged-particle jets, as well as the corresponding cross-section ratios, are reported for pp and p-Pb collisions in the transverse momentum range $10 < p^{\rm ch}_{\rm T,jet} < 140$ GeV/$c$ and $10 < p^{\rm ch}_{\rm T,jet} < 160$ GeV/$c$, respectively, together with the nuclear modification factor $R_{\rm pPb}^{\rm ch\,jet}$ in the range $10 < p^{\rm ch}_{\rm T,jet} < 140$ GeV/$c$. The analysis extends the $p_{\rm T}$ range of the previously-reported charged-particle jet measurements by the ALICE Collaboration. The nuclear modification factor is found to be consistent with one and independent of the jet resolution parameter with the improved precision of this study, indicating that the possible influence of cold nuclear matter effects on the production cross section of charged-particle jets in p-Pb collisions at $\sqrt{s_{\rm NN}} = 5.02$ TeV is smaller than the current precision. The obtained results are in agreement with other minimum bias jet measurements available for RHIC and LHC energies, and are well reproduced by the NLO perturbative QCD POWHEG calculations with parton shower provided by PYTHIA8 as well as by JETSCAPE simulations