The pilot dimension method: Reconciling Steering and Conformity in Workshops

International audienceIn machining workshops, workpieces are produced according to dimensions known as manufacturing dimensions. For the same workpiece and the same manufacturing plan, several sets of manufacturing dimensions can be used but none satisfy simultaneously the two main missions workshops need to fulfil: (a) Ensuring conformity of products to their design dimension tolerances (also called blueprint tolerances) and (b) steering machines in order to compensate for tool wear. The set of manufacturing dimensions obtained from the design dimensions using the minimal chain of dimensions method is optimal for a conformity check of workpieces but is practically unusable for steering machines because of the complexity of its relationships toward the tool correctors and tools dimensions. The pilot dimensions method consists in, on the one hand, identifying and representing these tool correctors and these tool/program dimensions on the production drawings (besides the manufacturing dimensions) and, on other the other hand, determining their correction values through a mathematical set of relations after having measured the manufacturing dimensions on a workpiece. Doing so will strongly reduce adjustment time, reduce the number of workpieces used for adjustments and greatly enhance the quality of workpiece batches

PFAPA syndrome is not a sporadic disease

Objectives. To determine whether PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) patients have a positive family history (FH) for recurrent fever syndromes. Method. For all patients with PFAPA seen in two paediatric rheumatology centres (Romandy, Switzerland and Bordeaux, France), parents were interviewed to record the FH for periodic fever. As controls, we interviewed a group of children without history of recurrent fever. Results. We recruited 84 patients with PFAPA and 47 healthy children. The FH for recurrent fever (without an infectious cause and recurring for at least half a year) was positive in 38/84 (45%), and was positive for PFAPA (diagnosis confirmed by a physician) in 10/84 (12%) of the PFAPA patients. For 29 of the 38 patients with positive FH, the affected person was a sibling or a parent. None of the healthy children had a positive FH for recurrent fever or PFAPA. A positive FH for rheumatological diseases was seen in both groups of children. Conclusion. These data show that a significant percentage of PFAPA patients present a positive FH of recurrent fever and PFAPA. This familial susceptibility suggests a potential genetic origin for this syndrom

International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 patients

Objectives. The aims of this study were to describe the clinical features of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) and identify distinct phenotypes in a large cohort of patients from different countries. Methods. We established a web-based multicentre cohort through an international collaboration within the periodic fevers working party of the Pediatric Rheumatology European Society (PReS). The inclusion criterion was a diagnosis of PFAPA given by an experienced paediatric rheumatologist participating in an international working group on periodic fever syndromes. Results. Of the 301 patients included from the 15 centres, 271 had pharyngitis, 236 cervical adenitis, 171 oral aphthosis and 132 with all three clinical features. A total of 228 patients presented with additional symptoms (131 gastrointestinal symptoms, 86 arthralgias and/or myalgias, 36 skin rashes, 8 neurological symptoms). Thirty-one patients had disease onset after 5 years and they reported more additional symptoms. A positive family history for recurrent fever or recurrent tonsillitis was found in 81 patients (26.9%). Genetic testing for monogenic periodic fever syndromes was performed on 111 patients, who reported fewer occurrences of oral aphthosis or additional symptoms. Twenty-four patients reported symptoms (oral aphthosis and malaise) outside the flares. The CRP was >50 mg/l in the majority (131/190) of the patients tested during the fever. Conclusion. We describe the largest cohort of PFAPA patients presented so far. We confirm that PFAPA may present with varied clinical manifestations and we show the limitations of the commonly used diagnostic criteria. Based on detailed analysis of this cohort, a consensus definition of PFAPA with better-defined criteria should be propose

A multicentre, randomised, double-blind, placebo-controlled trial with the interleukin-1 receptor antagonist anakinra in patients with systemic-onset juvenile idiopathic arthritis (ANAJIS trial)

To assess the efficacy of the interleukin 1 receptor antagonist anakinra in systemic-onset juvenile idiopathic arthritis (SJIA).status: publishe

APSIC: Training and fitting amputees during situations of daily living

Today, the prevalence of major amputation in France can be estimated between 90,000 and 100,000 and the incidence is about 8300 new amputations per year (according to French National Authority for Health estimation). This prevalence is expected to increase in the next decade due to the ageing of the population. Even if prosthetic fitting allows amputee people recovering the walking ability, their autonomy remains limited when crossing obstacles such as slopes, stairs or cross-slopes frequently encountered during outdoors displacements. The aim of the project APSIC was to complete scientific knowledge about adaptation strategies to situations of daily living compared to level walking through an extensive motion analysis study of transtibial and transfemoral amputee compared to non-amputee people. APSIC succeeded in identifying physiologic joint functions and current prosthetic joint limitations in the studied situations, which notably resulted in the design of a prototype of ankle-knee prosthesis adapted to multimodal locomotion of transfemoral amputee. Perspectives of the clinical use of motion analysis within the rehabilitation process were explored and proved to be relevant for personalized approach of motor learning

JAK inhibition in Aicardi-Goutières syndrome: a monocentric multidisciplinary real-world approach study

International audienceThe paradigm type I interferonopathy Aicardi-Goutières syndrome (AGS) is most typically characterized by severe neurological involvement. AGS is considered an immune-mediated disease, poorly responsive to conventional immunosuppression. Premised on a chronic enhancement of type I interferon signaling, JAK1/2 inhibition has been trialed in AGS, with clear improvements in cutaneous and systemic disease manifestations. Contrastingly, treatment efficacy at the level of the neurological system has been less conclusive. Here, we report our real-word approach study of JAK1/2 inhibition in 11 patients with AGS, providing extensive assessments of clinical and radiological status; interferon signaling, including in cerebrospinal fluid (CSF); and drug concentrations in blood and CSF. Over a median follow-up of 17 months, we observed a clear benefit of JAK1/2 inhibition on certain systemic features of AGS, and reproduced results reported using the AGS neurologic severity scale. In contrast, there was no change in other scales assessing neurological status; using the caregiver scale, only patient comfort, but no other domain of everyday-life care, was improved. Serious bacterial infections occurred in 4 out of the 11 patients. Overall, our data lead us to conclude that other approaches to treatment are urgently required for the neurologic features of AGS. We suggest that earlier diagnosis and adequate central nervous system penetration likely remain the major factors determining the efficacy of therapy in preventing irreversible brain damage, implying the importance of early and rapid genetic testing and the consideration of intrathecal drug delivery

Évolution de la contribution française à l'upgrade de LHCb

Ce document décrit l'évolution de la contribution française à l'upgrade de LHCb. Il s'inscrit dans le prolongement de la Lettre d'Intention [1], du Framework TDR [2], du document soumis au Conseil scientifique de l'IN2P3 le 21 juin 2012 [3], et des Technical Design Reports soumis au LHCC en novembre 2013 [4, 5]. Ces derniers concernent le détecteur de vertex et les détecteurs utilisés dans l'identification des particules. La contribution française s'est cristallisée autour de quatre grands projets : l'électronique front-end des calorimètres et du trajectographe à fibres scintillantes, le système de déclenchement de premier niveau et la carte de lecture à 40MHz commune à l'ensemble des sous-systèmes. Dans ce document nous décrivons les contributions envisagées et les ressources nécessaires pour mener à bien ces projets

Enrichment of intracellular sulphur cycle –associated bacteria in intertidal benthic foraminifera revealed by 16S and aprA gene analysis

Benthic foraminifera are known to play an important role in marine carbon and nitrogen cycles. Here, we report an enrichment of sulphur cycle -associated bacteria inside intertidal benthic foraminifera (Ammonia sp. (T6), Haynesina sp. (S16) and Elphidium sp. (S5)), using a meta barcoding approach targeting the 16S rRNA and aprA -genes. The most abundant intracellular bacterial groups included the genus Sulfurovum and the order Desulfobacterales. The bacterial 16S OTUs are likely to originate from the sediment bacterial communities, as the taxa found inside the foraminifera were also present in the sediment. The fact that 16S rRNA and aprA -gene derived intracellular bacterial OTUs were species-specific and significantly different from the ambient sediment community implies that bacterivory is an unlikely scenario, as benthic foraminifera are known to digest bacteria only randomly. Furthermore, these foraminiferal species are known to prefer other food sources than bacteria. The detection of sulphur-cycle related bacterial genes in this study suggests a putative role for these bacteria in the metabolism of the foraminiferal host. Future investigation into environmental conditions under which transcription of S-cycle genes are activated would enable assessment of their role and the potential foraminiferal/endobiont contribution to the sulphur-cycle.Peer reviewe

Le devenir à court et moyen terme de 71 enfants victimes de maltraitance admis aux Urgences Pédiatriques en 2001

BORDEAUX2-BU Santé (330632101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF