Centre Hospitalier Saint-Denis

This national Delphi study provides the first context-adapted POCUS curriculum for pediatric emergency medicine in France. By identifying priority skills and preferred teaching modalities, it offers a structured approach to competency-based POCUS training that may be adapted to other countries facing similar challenges in pediatric emergency education. • Point-of-care ultrasound (POCUS) is a core skill in pediatric emergency medicine. In France, however, training remains absent from the resident's curriculum. International programs cannot be directly implemented due to differences in training structures, scope of practice, and available resources. • This national Delphi study establishes the first context-adapted POCUS curriculum for pediatric emergency medicine in France, identifying 20 core learning objectives and consensus-based teaching modalities to support structured, competency-based training.

<strong class="journal-contentHeaderColor">Abstract.</strong> Winter windstorms cause extensive damage to infrastructure and represent the most significant natural hazard for Generali France in terms of insured losses. This study presents a method to systematically link physical storm events with observed insurance claims, enabling a better understanding of which storms, including weaker depressions, drive losses within Generali's portfolio. The proposed association represents a cornerstone for the calibration of insurance and reinsurance processes such as risk assessment, loss modelling and prevention. Beyond analysing individual events, we assess the impact of storm clusters, defined as multiple storms affecting the same region within a 96-hour window, consistent with reinsurance contract definitions. Our findings reveal that 85 % of windstorm-related losses since 1998 are attributable to clustered events. The most intense storms are frequently preceded or succeeded by smaller, yet damaging, depressions. This is illustrated by the case of Storms Anatol, Lothar and Martin in December 1999 and Storm Klaus in January 2009. Furthermore, we find that storms causing damage are more likely to occur as part of a cluster (50 %) compared to the overall population of depressions affecting France (29 %). These findings highlight the importance of accounting for storm clustering in risk modelling and reinsurance strategies.

In women with multiple sclerosis (MS), disease-modifying therapy (DMT) management during pregnancy might impact relapse risk. To estimate the effect of DMT management during pregnancy on MS relapse rate and compare different therapeutic strategies. This was a multicenter retrospective cohort study using data from January 1990 to December 2023. Data were extracted in December 2023 from the French MS registry. Among 52 955 women in the registry, we included pregnancies identified through childbirths in patients with relapsing-onset MS who were monitored for at least 18 months before delivery and 9 months after. Pregnancies occurring less than 18 months apart or with missing month of birth were excluded. Mediation analysis was used to estimate the total, direct, and indirect (mediated by DMT management) effects of pregnancy. Different therapeutic strategies were compared: DMT interruption, switching to or maintaining interferon β or glatiramer acetate, switching to or maintaining natalizumab until the third trimester, and switching to or maintaining intravenous anti-CD20 and interrupting it 3 months before conception. The primary outcome was the annualized relapse rate (ARR) during the preconception, gestation, and postpartum periods. Within a causal inference framework, counterfactual ARRs were estimated using longitudinal g-computation, combining a random forest algorithm for predicting DMTs, and a mixed-effects Poisson model for relapses. We included 6341 pregnancies occurring in 4998 women (mean [SD] age at conception, 31.5 [4.5] years). DMT management during pregnancy significantly increased ARR during gestation (causal rate ratio [cRR], 1.13; 95% CI, 1.06-1.22) and postpartum (cRR, 1.08; 95% CI, 1.01-1.16) periods. This led to a deleterious total effect of pregnancy on ARR, particularly in women receiving natalizumab before pregnancy with prolonged interruption (ie, interruption before the second trimester or resumption more than 3 months after delivery; cRR, 2.18; 95% CI, 1.76-2.69), and in women receiving fingolimod (cRR, 2.15; 95% CI, 1.60-2.93). Compared to DMT interruption, anti-CD20 strategy was the most effective (cRR, 0.38; 95% CI, 0.25-0.52), followed by the natalizumab strategy with short interruption (cRR, 0.80; 95% CI, 0.71-0.90), whereas interferon β (cRR, 0.93; 95% CI, 0.86-0.99) and glatiramer acetate strategies (cRR, 0.91; 95% CI, 0.84-0.99) were less effective. In this study, DMT management during pregnancy significantly increased relapse risk, particularly in patients receiving natalizumab with prolonged interruption or fingolimod. The strategy based on the use of anti-CD20 before pregnancy was the most effective to mitigate this risk.

Abstract. Winter windstorms cause extensive damage to infrastructure and represent the most significant natural hazard for Generali France in terms of insured losses. This study presents a method to systematically link physical storm events with observed insurance claims, enabling a better understanding of which storms, including weaker depressions, drive losses within Generali's portfolio. The proposed association represents a cornerstone for the calibration of insurance and reinsurance processes such as risk assessment, loss modelling and prevention. Beyond analysing individual events, we assess the impact of storm clusters, defined as multiple storms affecting the same region within a 96-hour window, consistent with reinsurance contract definitions. Our findings reveal that 85 % of windstorm-related losses since 1998 are attributable to clustered events. The most intense storms are frequently preceded or succeeded by smaller, yet damaging, depressions. This is illustrated by the case of Storms Anatol, Lothar and Martin in December 1999 and Storm Klaus in January 2009. Furthermore, we find that storms causing damage are more likely to occur as part of a cluster (50 %) compared to the overall population of depressions affecting France (29 %). These findings highlight the importance of accounting for storm clustering in risk modelling and reinsurance strategies.

Congenital neutropenia (CN) comprises a heterogeneous group of rare genetic disorders. While some CN cases present only with neutropenia, others present with additional extra-hematological manifestations. The most common cause of CN is variants in ELANE; however, approximately 30 other genes have been implicated. Despite this, the genetic basis remains unknown in roughly 30% of cases. The clinical and genetic heterogeneity of CN makes diagnosis particularly challenging. To address this, we conducted exome or genome sequencing of 60 patients with a suspected diagnosis of CN that remained unresolved following targeted sequencing. A genetic diagnosis was established in 25 patients (42%). Variants were identified in 15 different genes. Half of these cases involved genes traditionally associated with hereditary immunodeficiencies (GINS4, CARD11, ADA2, GINS1, LCP1, SASH3, and WAS). One-third of the cases carried variants in genes linked to syndromic disorders (VPS13B, TAFAZZIN, CLPB, and TONSL), demonstrating variable penetrance of extra-hematological phenotypes. A smaller subset (15%) harbored variants in genes associated with inherited bone marrow failure syndromes (BLM, RPL18, SAMD9, and SRP72), identified incidentally due to atypical presentations. Compared to patients with ELANE-CN, these individuals were diagnosed later, had fewer severe bacterial infections and gingivitis, exhibited less profound neutropenia, lacked monocytosis, and had a granulocytic maturation arrest, often beyond the promyelocytic stage. A shared feature among these cases was a tendency toward reduced lymphocyte subsets, particularly NK cells. This study highlights the significant contribution of exome and genome sequencing in diagnosing CN, given the phenotypic overlap, genetic heterogeneity, and variable penetrance of immunological and extra-hematological features.

These past few years have marked a growing interest in multimodality, interaction and eye-gaze in the interpretation and understanding of discourse. Eye-gaze, for example, plays a central role in face-to-face interaction and stance taking because it helps discourse participants coordinate with each other. Such visual markers help the interlocutors/audience to intersubjectively connect to the same common ground on which they construe their meanings. The case of humor has also received more attention from a multimodal perspective since it follows the same patterns of meaning construction and coordination. Elements that are salient to the humorous interpretation will be emphasized using either prosodic cues or visual markers, such as facial expressions and head movements. In this paper, we explore the use of such nonverbal discourse markers with the use of humor in the American presidential debates of 2016 and 2020, analyzing their role on the humorous stance.