International audienceThe Cap de Creus canyon, northwestern Mediterranean Sea, belongs to a complex network of submarine canyons cutting the western Gulf of Lion continental shelf and opening into the larger Sète canyon. Swath bathymetry data, MAK-1M deep-towed side-scan sonar imagery and 5 kHz high resolution seismic reflection profiles show striking morphologies in the Cap de Creus canyon floor and walls. As a consequence of the canyon head and the upper reach severe incision, the continental shelf dramatically narrows in front of the Creus Cape promontory. The upper canyon has a flat-bottomed thalweg incised in a mega-scale sediment furrow field displaying hyperbolic seismic facies. The tens of kilometres long linear furrows extend also over the middle canyon down to 1400 m of water depth. The furrows on either side of the canyon are not parallel but oblique and display varying degrees of excavation. Mid-channel sediment bars are locally present in the thalweg, which is made of sandy lag deposits, as revealed by its acoustic response and verified by sediment samples. The middle canyon is linear and steep, with an up to 700 m high southern wall, contrasting with the sinuous, smooth lower canyon, which is controlled by flowage of the underlying Messinian evaporites. Large sections of the canyon are affected by sediment instability processes. The lower Cap de Creus canyon hangs up to 260 m over its distalmost reach and the Sète canyon through a narrow, less than 1 km wide, gorge. Numerous scours up to 10 m deep suggesting bed load transport occupy the lower Sète canyon immediately downstream of the Cap de Creus canyon mouth. The data set provides the first complete very-high resolution imaging of a submarine canyon from its upper part down to its distalmost reach. The observations evidence a wide set of erosion, transport and deposition processes along the Cap de Creus canyon, including sediment entrapment at the canyon head, furrow-generating dense water cascading through the southern wall, along-channel currents strong enough to excavate specific sections of the channel floor and bed load sediment transport as demonstrated by the presence of mega-ripples, crescent scours and grooves in the lower canyon

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Background Information regarding inborn error of immunity (IEI) as a risk factor for severe COVID-19 is scarce. We aimed to determine if paediatric patients with moderate/severe IEI got COVID-19 at the same level as the general population, and to describe COVID-19 expression. Material and methods We included patients with moderate/severe IEI aged 0–21 years old: cross-sectional study (June2020) to determine the prevalence of COVID-19; prospective study (January2020-January2021) including IEI patients with COVID-19. Assays used: nasopharyngeal swab SARS-CoV-2 PCR and SARS-CoV-2-specific immunoglobulins. Results Seven from sixty-five patients tested positive (prevalence: 10.7% (7%–13%)) after the first SARS-COV-2 wave and 13/15 patients diagnosed with COVID-19 had an asymptomatic/mild course. Conclusions In our area, prevalence of COVID-19 in moderate/severe IEI paediatric patients after the first wave was slightly higher than in the general population. The majority of patients presented a benign course, suggesting a possible protective factor related with age despite IEI.

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Introduction Since the first description of gain of function (GOF) mutations in signal transducer and activator of transcription (STAT) 1, more than 300 patients have been described with a broad clinical phenotype including infections and severe immune dysregulation. Whilst Jak inhibitors (JAKinibs) have demonstrated benefits in several reported cases, their indications, dosing, and monitoring remain to be established. Methods A retrospective, multicenter study recruiting pediatric patients with STAT1 GOF under JAKinib treatment was performed and, when applicable, compared with the available reports from the literature. Results Ten children (median age 8.5 years (3–18), receiving JAKinibs (ruxolitinib (n = 9) and baricitinib (n = 1)) with a median follow-up of 18 months (2–42) from 6 inborn errors of immunity (IEI) reference centers were included. Clinical profile and JAKinib indications in our series were similar to the previously published 14 pediatric patients. 9/10 (our cohort) and 14/14 patients (previous reports) showed partial or complete responses. The median immune deficiency and dysregulation activity scores were 15.99 (5.2–40) pre and 7.55 (3–14.1) under therapy (p = 0.0078). Infection, considered a likely adverse event of JAKinib therapy, was observed in 1/10 patients; JAKinibs were stopped in 3/10 children, due to hepatotoxicity, pre-HSCT, and absence of response. Conclusions Our study supports the potentially beneficial use of JAKinibs in patients with STAT1 GOF, in line with previously published data. However, consensus regarding their indications and timing, dosing, treatment duration, and monitoring, as well as defining biomarkers to monitor clinical and immunological responses, remains to be determined, in form of international prospective multicenter studies using established IEI registries.