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Interstitial lung disease in anti-U1RNP systemic sclerosis patients: A European Scleroderma Trials and Research analysis
No full textInternational audienceBackground: Interstitial lung disease is the leading cause of morbidity and mortality in systemic sclerosis, but it is characterized by significant heterogeneity in patient outcomes. So far, little is known about the influence of anti-U1RNP antibodies on lung outcomes in systemic sclerosis-associated interstitial lung disease patients.Methods: European Scleroderma Trials and Research group systemic sclerosis patients with radiological-confirmed interstitial lung disease, available %predicted forced vital capacity, and autoantibody status were included. Baseline demographic and disease features were compared between anti-U1RNP positive and anti-U1RNP negative patients. Moreover, longitudinal analyses were done measuring relative change in %predicted forced vital capacity over 12 ± 6, 24 ± 6, and 36 ± 6 months, and changes were classified into stable (⩽ 4%), mild (5%-9%), and major progression (⩾ 10%). Predictors associated with death of any cause or major interstitial lung disease progression were evaluated in systemic sclerosis-associated interstitial lung disease patients with or without anti-U1RNP antibodies. Logistic regression analyses and Cox proportional hazards models adjusted for age and FVC were applied.Results: A total of 6043 systemic sclerosis-associated interstitial lung disease patients were included for the analysis, among which 327 (5.4%) were positive for anti-U1RNP antibodies. Mean age was 56.8 ± 13.2 years and 4971 (82.3%) were women. Anti-U1RNP + systemic sclerosis-associated interstitial lung disease patients had more frequently limited cutaneous systemic sclerosis (63.5.5% vs 53.3%, p < 0.001), higher frequency of joint synovitis (18.1% vs 13.9%, p = 0.039), and myositis (24.0% vs 19.5%, p = 0.048). Anti-U1RNP + patients had a baseline lower mean forced vital capacity (82.0% vs 86.0%, p < 0.001) and lower mean %predicted diffusing capacity for carbon monoxide (57.0% vs 60.5%, p = 0.003). Periods of mild or major FVC decline and mortality rates were not statistically different between the groups.Conclusion: Systemic sclerosis-associated interstitial lung disease patients positive for anti-U1RNP antibodies have more impaired baseline lung function but similar trajectories of forced vital capacity changes and mortality during the first 3 years of follow-up
Tracking ectopic lignification in flax stems following scarification.
No full textInternational audienceWhen flax (Linum usitatissimum L.) stems are scarified, major changes occur in the organization of cell walls within the tissues that border the wound. We sought to characterize the plant's response using a variety of approaches, with a particular focus on lignin deposition within the peripheral fiber cell walls of the stem. Raman spectroscopy and imaging first showed that changes occurred in the polysaccharide matrix of the parenchyma and fiber cell walls. These changes were accompanied by rapid deposition of lignin which initially diffuses centripetally and then, once the vascular cambium was reached, propagates in a periclinal manner until 150 μm from the edges of the wounded zone. Lignin biosynthesis appears to be the result of a de novo activity, as demonstrated by the concomitant accumulation of transcripts corresponding to lignin biosynthesis genes. In addition, using bioorthogonal chemistry approaches, we showed that wounding had enhanced the capacity of fiber cell walls to incorporate modified lignin precursors, in parallel with an increase in transcripts corresponding to peroxidases in the cortical tissues. This incorporation potential was identical for the 3 different types of reporters tested. Our findings demonstrated that mechanical stress can trigger lignification, in a polarized manner within the bast fibers, providing insights into the plasticity of cell wall composition and the potential for modulating fiber properties in flax
Lipodystrophy and severe insulin resistance syndrome: epidemiological data from a French national rare diseases registry
No full textInternational audience[This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.]Context: Lipodystrophy and extreme insulin resistance syndrome are rare diseases with severe metabolic complications. Reported epidemiological data are conflicting.Objective: We aimed to evaluate nationwide and regional prevalences of lipodystrophy and insulin resistance syndrome in France, and to study diagnostic and care-pathways.Methods: We studied data from the French National Rare Disease Registry (BNDMR), which includes all patients attending rare disease reference centers in France. We analyzed demographic data and age at first signs and at diagnosis in patients with an ORPHA code of lipodystrophy or severe insulin resistance syndrome.Results: The number of patients registered with lipodystrophy/insulin resistance syndrome doubled from 2017 to 2023, with the deployment of nationwide epidemiological tools in specialized rare diseases centers. Currently, 567 of the 652 patients (58% female) had a diagnosis of genetically determined disease with generalized (GL) or partial lipodystrophy (PL), giving an estimated national prevalence of 1.6 and 6.4 per million, respectively. Wide regional differences in estimated prevalence may be partly due to founder pathogen variants, or local spread of clinical skills and knowledge. Median age at first signs and diagnosis were respectively 1 [IQR: 1-3] and 5 years [0-20] for GL, and 22 [14-35] and 40 years [25-52] for PL, with earlier first signs of PL in women than in men.Conclusion: The estimated prevalence of genetic lipodystrophy/extreme insulin resistance syndrome has increases with the use of dedicated epidemiological tools, suggesting persistent underdiagnosis. Knowledge of these diseases needs to be improved to decrease diagnostic delay and reduce regional and gender-associated diagnostic disparities
Periodic membrane fractionation of freshwater organic matter reveals various reactivity patterns during chlorine/chloramine disinfection
No full textInternational audienceAlthough drinking water disinfection has significantly reduced mortality from waterborne diseases, the formation of potentially harmful disinfection by-products (DBPs) remains a concern today. These DBPs emerge from the reaction of dissolved organic matter (DOM) with chlor(am)ine during disinfection. Identifying these DBP precursors from heterogeneous and complex DOM mixtures is challenging and to address this, a novel membrane fractionation protocol was developed to isolate and identify the organic DBP precursors from fresh water sources. A tight ultrafiltration and nanofiltration membrane were carefully selected to partition DOM into three molecular weight (MW) fractions: high (>20 kDa), medium (0.3–20 kDa) and low (<0.3 kDa) as defined by high performance-size exclusion chromatography- total organic carbon analysis. A mathematical tool was developed to optimize the fractionation protocol. Therefore, ultrafiltration was executed before nanofiltration and the concentration factor was maximized without inducing fouling to obtain the purest fractions. The mathematical tool was also set in place to predict the necessary diafiltration factor for each individual fractionation experiment based on the initial organic matter composition of the surface water allowing the fractionation protocol to be effective at all times. The protocol was applied to surface water samples collected six times across three seasons yielding a fraction enriched in high MW compounds (up to 50 %), a fraction having more than 80 % of medium MW compounds and a fraction only containing low MW compounds. Although the medium MW fraction showed the highest reactivity for the majority of the investigated DBPs, the low MW fraction showed high reactivity for iodinated trihalomethanes during chlorination and for haloacetonitriles during chloramination. The high MW fraction had the lowest reactivity towards DBPs, which can have important implications for a drinking water treatment since this fraction is generally the most effectively removed by e.g. coagulation, while the more important fractions for DBP formation such as the medium and low MW compounds remain in the water until the disinfection step
Design of Fast Redox-Controlled Self-Immolative Selenium-Based Cysteine Surrogates
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Sex-dependent effects of maternal high-fat diet during lactation in the offspring of adult THY-Tau22 mice.
No full textInternational audienceThe perinatal environment has been suggested to participate in the development of tauopathies and Alzheimer’s disease but the molecular and cellular mechanisms involved remain contradictory and under-investigated. Here, we evaluated the effects of a maternal high-fat diet (HFD) during lactation on the development of tauopathy in the THY-Tau22 mouse strain, a model of progressive tau pathology associated with cognitive decline.During lactation, dams were fed either a chow diet (13.6% of fat) or a HFD (58% of fat). At weaning, offspring were fed a chow diet until sacrifice at 4 months of age (the onset of tau pathology) or 7 months of age (the onset of cognitive impairment).During lactation, maternal HFD increased body weight gain in offspring. At 3 months of age, maternal HFD led to a mild glucose intolerance only in male offspring. Moreover, it impaired spatial memory in both male and female 6-month-old offspring, with males being more impacted. These cognitive deficits were associated with increased phosphorylation of hippocampal tau protein-observed at 4 months in males and at 7 months in females, highlighting a sex-specific temporal shift. Additionally, maternal HFD modified adult hippocampal neurogenesis (AHN), leading to an increase of mature neuronal cells number in females and of dendritic arborization length in males. Synaptic analysis further revealed that maternal HFD led to synaptic loss only in males. Finally, multi-omics approaches showed that maternal HFD has long-term consequences on both transcriptome, proteome and regulome, this effect being also sex-dependent with mitochondrial pathways, ribosomal activity, cilium and the extracellular matrix predominantly impacted in males, while gliogenesis, myelination and synaptic plasticity were primarily affected in females. Regulome analysis suggested that this sex-dependent phenotype was more related to a temporal shift rather than distinct sex-specific alterations. Collectively, our data suggest that maternal HFD accelerates the development of tauopathy in THY-Tau22 offspring, with sex-dependent effects, males being impacted earlier than females. These findings highlight that exposure to maternal HFD represents a critical window of vulnerability, and potentially of opportunity, for interventions aimed at preventing the development of neurodegenerative diseases
Épisode spécial : Interview de Maïlie Saint-Hilaire par Amélie Elouin : chlordécone, science & santé caribéenne: Voyage au centre de la thèse fondé par Amélie Elouin (Laboratoire d'Optique et Biosciences)
No full textÀ l’occasion du mois des femmes, Amélie Elouin interviewe Maïlie Saint-Hilaire, une scientifique inspirante originaire de la Martinique. Maïlie a soutenu sa thèse en France métropolitaine et dirige aujourd’hui un laboratoire de chimie à l’Institut Pasteur de Guadeloupe. Dans cette interview, elle nous partage son parcours, ses recherches sur la chlordécone, ainsi que son rôle de responsable de laboratoire. Passionnée par la valorisation des talents scientifiques caribéens, elle a créé le podcast Caribéenne & Scientifique. Elle accompagne également les doctorants grâce à un programme en ligne, PhD BOOST, qui les aide à aborder leur thèse avec confiance et sérénité.- enregistrement et montage des interviews par les doctorants du Laboratoire d'Optique et Biosciences. - dépôt sur MediHal par Elsa BALDUZZ
Fieldwork on animals living in extreme conditions as a source of biomedical innovation
No full textInternational audienceMost biomedical research on animals is based on the handful of the so-called standard model organisms, i.e. laboratory mice, rats or Drosophila, but the keys to some important biomedical questions may simply not be found in these. However, compared with the high number of molecules originating from plants in clinical use, and with the countless unique adaption mechanisms that animals have developed over the course of evolution to cope with environmental constrains, there is still few investigations on wild animals with biomedical objectives, and field studies are far fewer. A major limitation is insufficient funding, the main causes of which we analyze. We argue, however, that fieldwork is a key driver in generating new scientific knowledge as part of a One Health approach, by observing/documenting and understanding the diverse and largely unexplored biological processes evolved by animals adapted to unusual environmental conditions, which would be extreme conditions for humans. These conditions do not only refer to extreme temperatures, since lack of food or water, high pressures or lack of oxygen, are clearly extreme constraints. To conduct this research, there are serious limitations we propose to address. Specific techniques and methods are requested, not only to work in extreme environments, but also to minimize the ecological footprint of field work. The erosion of biodiversity is a major threat. The reduction of animal disturbance, a key issue, requires specific technologies and expertise. An ethical approach is requested, for the sake of transparency and to comply with the Nagoya Protocol on genetic resources. An interdisciplinary expertise and a meticulous planning are requested to overcome the field constraints and interface the associated laboratory work. We recommend focusing on the major threats to global human health today, which wild animals appear to resist particularly well, such as antibioresistance and diseases associated with lifestyle and senescence
Primary intestinal lymphangiectasia: French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins)
No full textInternational audienceBackground Primary intestinal lymphangiectasia or Waldmann’s disease (ORPHA code: 90362) is a very rare disorder of unknown etiology, characterized by digestive lymphatic vessel dilations.Main body of the abstract The objective of the French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins) is to provide health professionals with information about the optimal management and care for patients, based on a critical literature review and multidisciplinary expert consensus. The PNDS, written by members of the French National Reference Centers for Rare Vascular diseases and Rare Digestive diseases,is available from the French Health Authority website. The latter allow lymph leakage (chyle) into the intestinal lumen that is responsible for protein‑losing gastroenteropathy, combining hypoalbuminemia, lymphopenia and hypogam‑ maglobulinemia. Diagnosis is usually made before the age of 3, but primary intestinal lymphangiectasia may be discovered in adults. Edema of the lower limbs is the main clinical sign and serous effusions (pleura, peritoneum, peri‑cardium) are sometimes abundant. Exudative gastroenteropathy is confirmed by increased α1‑antitrypsin clearance.Esophagogastroduodenoscopy finds milky lesions corresponding to lymphangiectasias; duodenal biopsies confirmthe diagnosis. Endoscopic videocapsule may be useful to evaluate the extent of the disease and/or if esophagogas‑troduodenoscopy is not contributory. In rare cases, the disease may be complicated by digestive or extra‑digestiveB‑cell lymphoma in adults. Management is mainly based on a strict fat‑free diet, combined with supplementationwith medium‑chain triglycerides, essential fatty acids and fat‑soluble vitamins. Octreotide, a somatostatin analogue,has inconsistent efficacy, in combination with the fat‑free diet and the sometimes‑prescribed mammalian targetof rapamycin‑receptor inhibitor sirolimus, occasionally achieving positive effects. Diuretics and albumin infusionsmay be useful in addition to the fat‑free diet. Intestinal resections are proposed for rare, localized, segmental formsof the disease.Short conclusion Primary intestinal lymphangiectasia is a chronic disease requiring a prolonged restrictive and con‑straining strict low‑fat diet supplemented with medium‑chain triglycerides and fat‑soluble vitamins. Its evolution canbe complicated by more‑or‑less severe serous effusions and rare lymphoma. Life‑long clinical and biological monitor‑ing is required
Clé d'identification des genres de Moustiques (Diptera : Culicidae), mâles et femelles, Région Afrotropicale
No full textClé illustrée (photographies macroscopiques) et commentée, en français, pour l'identification des genres de moustiques (DIPTERA : Culicidae) de la région afrotropicale. Elle reprend et adapte des travaux antérieurs, notamment: Service MW. Handbook to the Afrotropical Toxorhynchitine and Culicine Mosquitoes, Excepting Aedes and Culex. British Museum (Natural History); 1990. Robert V, Ndiaye EH, Rahola N, Le Goff G, Boussès P, Diallo D, et al. Clés dichotomiques illustrées d’identification des femelles et des larves de moustiques (Diptera : Culicidae) du Burkina Faso, Cap-Vert, Gambie, Mali, Mauritanie, Niger, Sénégal et Tchad. Montpellier: IRD; 2022. Available: https://doi.org/10.23708/fdi:010084866 [accessed 30 Sep 2025] Baldacchino F, Paupy C, Rahola N. Clé de détermination des Culicidae présents en Afrique Centrale et au GABON (document de travail). 2014. Les photographies ont majoritairement été réalisées sur des individus de la Collection ARIM (https://arim.ird.fr/)Clé illustrée (photographies macroscopiques) et commentée, en français, pour l'identification des genres de moustiques (DIPTERA : Culicidae) de la région afrotropicale. La clé est valide pour l'identification des mâles et femelles. Elle reprend et adapte des travaux antérieurs, notamment: Service MW. Handbook to the Afrotropical Toxorhynchitine and Culicine Mosquitoes, Excepting Aedes and Culex. British Museum (Natural History); 1990. Robert V, Ndiaye EH, Rahola N, Le Goff G, Boussès P, Diallo D, et al. Clés dichotomiques illustrées d’identification des femelles et des larves de moustiques (Diptera : Culicidae) du Burkina Faso, Cap-Vert, Gambie, Mali, Mauritanie, Niger, Sénégal et Tchad. Montpellier: IRD; 2022. Available: https://doi.org/10.23708/fdi:010084866 [accessed 30 Sep 2025] Baldacchino F, Paupy C, Rahola N. Clé de détermination des Culicidae présents en Afrique Centrale et au GABON (document de travail). 2014. Les photographies ont majoritairement été réalisées sur des individus de la Collection ARIM (https://arim.ird.fr/