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Serious infections in patients with VEXAS syndrome: data from the French VEXAS registry
No full textInternational audienc
Intérêt du test d’activation des basophiles dans le suivi d’une immunothérapie orale à la noix de cajou : à propos d’un cas
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Monocentric prospective study to valid multidisciplinary diagnostic and therapeutic approach for Nutcracker syndrome
No full textInternational audienceBackground: Nutcracker syndrome (NCS) is defined as left renal vein (LRV) compression by the superior mesenteric artery. NCS diagnosis is rendered complex by confounding symptoms. The study objective was to perform a prospective observational analysis of the diagnostic and therapeutic criteria of the patients with suspected NCS. When NCS diagnosis was confirmed, transposition of the LRV was carried out by mini-invasive robotic surgery (MIRS). Method: All patients addressed to the vascular surgery department for suspicion of NCS between January 2022 and June 2023 were included in the study. Patients were subsequently assessed by means of a computed tomography scan, dynamic duplex ultrasound and phlebography associated with an occlusion test of the left gonadic vein (LGV). Diagnostic criteria included aorto-mesenteric angle, LGV diameter and reflux, velocity ratios and diameters and the reno-caval gradient. Result: Thirty two patients aged 37 ± 14 years had suspicion of NCS. Twenty presented an aorto-mesenteric angle below 20°, twenty three had a LGV diameter greater than 5 mm and twenty two of the latter patients also had LGV reflux. A significant reno-caval gradient greater than 5 mmHg was found in ten cases, thereby consolidating NCS diagnosis. Overall, thirteen patients neither presented NCS or pelvic varicosities; eight had pelvic congestion syndrome without NCS and were successfully treated by embolization. Eleven patients with confirmed NCS underwent LRV transposition in the inferior vena cava (IVC). Eight of the latter patients received a complementary pelvic varicosity embolization 2 days later. Two months post-operation 100% of transposed LRV were permeable as assessed by duplex ultrasound controls and all of these patients reported an improvement of symptoms. Conclusion: An innovative multidisciplinary decisional algorithm establishes certitude in NCS diagnosis which can subsequently be treated radically by MIRS
3q29 duplications: A cohort of 46 patients and a literature review
No full textInternational audienceDuplications of the 3q29 cytoband are rare chromosomal copy number variations (CNVs) (overlapping or recurrent ~1.6 Mb 3q29 duplications). They have been associated with highly variable neurodevelopmental disorders (NDDs) with various associated features or reported as a susceptibility factor to the development of learning disabilities and neuropsychiatric disorders. The smallest region of overlap and the phenotype of 3q29 duplications remain uncertain. We here report a French cohort of 31 families with a 3q29 duplication identified by chromosomal microarray analysis (CMA), including 14 recurrent 1.6 Mb duplications, eight overlapping duplications (>1 Mb), and nine small duplications (<1 Mb). Additional genetic findings that may be involved in the phenotype were identified in 11 patients. Focusing on apparently isolated 3q29 duplications, patients present mainly mild NDD as suggested by a high rate of learning disabilities in contrast to a low proportion of patients with intellectual disabilities. Although some are de novo, most of the 3q29 duplications are inherited from a parent with a similar mild phenotype. Besides, the study of small 3q29 duplications does not provide evidence for any critical region. Our data suggest that the overlapping and recurrent 3q29 duplications seem to lead to mild NDD and that a severe or syndromic clinical presentation should warrant further genetic analyses
Analysis of opioid analgesics consumption in Africa: a longitudinal study from a 20-year continental perspective
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Radioprotection dans une cohorte de professionnels de santé : connaissances, attitudes, pratiques, auto-perception et exposition aux ri dans les hôpitaux français -étude experts
No full textInternational audienceObjectifs Le nombre de travailleurs du secteur de la santé exposés professionnellement aux rayonnements ionisants (RI) augmente chaque année. Étant donné que des effets sur la santé dus à l'exposition à de faibles doses de RI ont été observés, la radioprotection (RP) dans le contexte des activités professionnelles est une préoccupation majeure. Sur le plan légal, une surveillance dosimétrique impliquant le port d’un/plusieurs dosimètre(s) doit être mise en place pour tous les travailleurs potentiellement exposés aux RI, lesquels doivent suivre des formations en RP. L’étude EXPERTS (EXposition des Professionnels de santE aux RayonnemenTs ioniSants) a plusieurs objectifs, notamment d’évaluer l’adhésion des travailleurs à ces pratiques de RP et ses associations avec la dose cumulée enregistrée, la profession, ainsi que l’auto-perception de l’exposition aux RI et des risques potentiels.Méthodes Tout salarié d’un service médical (diagnostic ou soins) de l'un des neuf hôpitaux français participants avec au moins un enregistrement dosimétrique en 2009, 2014 et 2019 dans le registre SISERI (Surveillance de l'Exposition aux Rayonnements Ionisants) a été inclus et invité à remplir un questionnaire en ligne comprenant des informations sur son exposition professionnelle, sa perception du risque d'exposition aux RI et ses connaissances générales en matière de RP. Les doses de Hp(10) ont également été fournies par le système SISERI. Des régressions logistiques multivariées ont été utilisées.Résultats428 travailleurs ont répondu au questionnaire, soit un taux de réponse de 29%. Les répondants et les non-répondants étaient similaires en termes de niveau d’exposition aux RI. Parmi les répondants, 80% ont déclaré porter toujours ou souvent leur dosimètre individuel, 82% utilisent des moyens de RP, et 62% participent régulièrement à des sessions de formations en RP. Les principales raisons pour le non-port ou la non-adhésion aux pratiques de RP étaient le manque de temps et le caractère parfois inadapté des outils de RP aux activités des professionnels de santé. Le port du dosimètre et l’adhésion aux pratiques de RP étaient fortement associés au "sentiment d'être exposé aux RI" (OR=3,69, CI95% 2,04-6,66 ; OR=4,60, CI95% 2,28-9,30, respectivement). Toutefois, aucun de ces facteurs n'était associé à la participation aux sessions de formation sur les RI. Enfin, pour les travailleurs qui déclaraient porter toujours ou souvent leur dosimètre, la dose moyenne annuelle Hp(10) sur la période 2009-2019 a diminué progressivement. Cependant, l’analyse par service a montré une augmentation de cette dose moyenne en médecine nucléaire uniquement (β=0.017, IC95% 0.006-0.027 mSv/an).Conclusions Sensibiliser les travailleurs exposés à leur propre exposition aux RI semble être un élément clé à aborder dans les cours de formation en RP. Ce type de questionnaire pourrait être introduit dans des études épidémiologiques de plus grande taille pour mieux évaluer les freins et motivations à l’adhésion aux pratiques de RP
Long-Term Follow-Up of the Prospective Randomized AATT Study (Autologous or Allogeneic Transplantation in Patients With Peripheral T-Cell Lymphoma)
No full textInternational audienceClinical trials frequently include multiple end points that mature at different times. The initial report, typically based on the primary end point, may be published when key planned co-primary or secondary analyses are not yet available. Clinical Trial Updates provide an opportunity to disseminate additional results from studies, published in JCO or elsewhere, for which the primary end point has already been reported.Primary analysis of the phase III randomized AATT study showed that younger patients with peripheral T-cell lymphoma (PTCL) consolidated with autologous or allogeneic transplantation (alloSCT) had similar event-free survival (EFS) and overall survival (OS). Seven-year EFS of patients randomly assigned to alloSCT was 38% (95% CI, 25 to 52) compared with 34% (95% CI, 22 to 47) for patients randomly assigned to autologous transplantation of hematopoietic stem cells (autoSCT); OS was 55% (95% CI, 41 to 69) and 61% (95% CI, 47 to 74). Among patients undergoing alloSCT (n = 26) or autoSCT (n = 41) on study, the cumulative progression/relapse rate was 8% (95% CI, 0 to 19) and 55% (95% CI, 35 to 74). Nonrelapse mortality (NRM) was 31% (95% CI, 13 to 49) and 3% (95% CI, 0 to 8) after alloSCT and autoSCT, respectively. Fifteen of 30 patients with early progression and 11 of 20 patients with progression/relapse after autoSCT received alloSCT. Seven-year OS after salvage alloSCT was 61% (95% CI, 47 to 74); NRM was 23% (95% CI, 6 to 40). Long-term follow-up documents the strong graft versus lymphoma effect of alloSCT independent of the timing of transplantation. Survival of patients unable to undergo transplantation was dismal. AlloSCT is the treatment of choice for younger, transplant-eligible patients with relapsed/refractory PTCL. AlloSCT is currently not recommended as part of first-line consolidation.Trial registration: ClinicalTrials.gov NCT00984412
A pain research strategy for Europe: A European survey and position paper of the European Pain Federation EFIC
No full textInternational audienceAbstract Background Pain is the leading cause of disability and reduced quality of life worldwide. Despite the increasing burden for patients and healthcare systems, pain research remains underfunded and under focused. Having stakeholders identify and prioritize areas that need urgent attention in the field will help focus funding topics, reduce ‘research waste’, improve the effectiveness of pain research and therapy and promote the uptake of research evidence. In this study, the European Pain Federation (EFIC) developed a Pain Research Strategy for Europe. Methods The study used multiple methods, including literature searches, multidisciplinary expert debate, a survey and a final consensus meeting. The cross‐sectional survey was conducted among 628 European pain researchers, clinicians, educators and industry professionals to obtain the rating and hierarchy of pain research priorities. The final consensus meeting involved a multidisciplinary expert panel including people with lived experience from 23 countries. The survey results guided discussions where top priorities were agreed. Results Content analysis identified nine survey themes, of which five emerged as top priorities: (i) understand the pathophysiology of pain; (ii) understand and address comorbidities; (iii) critically assess current therapies; (iv) develop new treatments; and (v) explore the biopsychosocial impacts of pain. Physical, psychological and social approaches were prioritized at the same level as pharmacological treatments. The top priorities were endorsed by a multidisciplinary expert panel. The panel emphasized the importance of also clearly communicating the concepts of prediction, prevention self‐management and personalized pain management in the final strategy. Conclusions The content of the final top research priorities' list reflects a holistic approach to pain management. The equal importance given to physical, psychological and social aspects alongside pharmacological treatments highlights the importance of a comprehensive biopsychosocial‐orientated research strategy. The expert panel's endorsement of five top priorities, coupled with an emphasis on communicating the concepts of prediction, prevention, self‐management and personalized pain management, provides a clear direction for future basic, translational and clinical research. Significance EFIC has developed a Pain Research Strategy for Europe that identifies pain research areas deserving the most focus and financial support. Implementation and wide dissemination of this Strategy is vital to increase the conduct of urgent pain projects, pain research funding and the implementation of research findings into practice, to ultimately decrease the personal, societal and financial burden of pain
Dietary Protein in a Challenge Meal Does Not Alleviate Postprandial Impairments in Vascular Endothelial Function in Healthy Older Adults with Cardiometabolic Risk: A Randomized Crossover-Controlled Trial
No full textInternational audienceBackground: Postprandial vascular endothelial dysfunction is an early marker of atherosclerosis. Meal protein has been reported to reduce endothelial dysfunction in adults and the effect could be mediated by the amino acid content. Objective: This trial aims to assess the effect of a specifically designed plant protein blend that contains high leucine, arginine and cysteine on postprandial endothelial function in the elderly.Methods: In a randomized, double-blind, 3-period crossover (2-wk washout), controlled trial, we compared the vascular effects of 3 high-saturated-fat high-sucrose (HFHS) meals based on either our specific plant-protein blend, milk protein, or without added protein. The trial was conducted on 29 healthy adults >65y presenting at least 2 cardiometabolic risk factors. Postprandial vascular function was evaluated at fasting, 3 hours, and 5 hours postprandially, using brachial flow-mediated dilation (FMD), hand microvascular reactivity (by Flowmetry Laser Doppler, FLD), and finger reactive hyperemia index (using Peripheral Arterial Tonometry, RHI). Immune cells count and gene expression in peripheral blood mononuclear cells (PBMC) was also assessed postprandially. Data were analyzed using mixed linear models with repeated measurements on participants for meal composition and time of sampling. This trial was registered at clinicaltrials.gov as NCT04923555.Results: FMD iAUC decreased after meals (time effect P<0.01), with no significant differences between meals. RHI also decreased with time (P<0.01). PBMC count and MCP1, IL-1b, IL-6 expression increased after meals showing postprandial endothelial activation (P<0.05). Overall, meal composition had no effect on any of the postprandial changes (Ps>0.10).Conclusions: In healthy adults over 65 years presenting cardiometabolic risk, adding protein to an HFHS challenge meal does not mitigate postprandial impairments in vascular endothelial function and inflammatory activation. Further studies are needed to explore the potential differences with younger adults