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#1861 Kidney involvement in MELAS/MIDD syndrome: a multicentric description of 104 patients
No full textMeeting abstractInternational audienceBackground and Aims Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome, along with maternally inherited diabetes and deafness (MIDD) syndrome, are mitochondrial disorders with systemic involvement. These conditions are associated with 46 distinct mutations, most commonly the m.3243A>G mutation. Kidney involvement is a frequent complication, potentially associated with increased mortality risk. However, the precise patterns and progression of kidney disease in these syndromes remain largely unknown. Method We conducted a multicenter retrospective study in 16 French hospitals of patients who with documented renal impairment and carrying a mutation involved in MELAS/MIDD syndrome. Results The study cohort included 104 patients. The median age at onset of the first symptom was 25 years [IQR: 17.5–36.5], with a median delay of 12 years [IQR: 3.0–22.0] between symptom onset and genetic diagnosis. Kidney manifestations first appeared at a median age of 41 years [IQR: 32.0–53.0]. Proteinuria exceeding 1 g/24 h was observed in 41/87 patients (47.1%), while signs of tubulopathy were present in 15/39 patients (38.5%). Kidney biopsies were performed in 41 patients, revealing focal segmental glomerulosclerosis (FSGS) as the predominant lesion in 16 patients (39%), followed by vascular lesions in 11 patients (26.8%) and tubulointerstitial lesions in 9 patients (22%). The median duration of kidney follow-up—defined as the interval between the discovery of kidney manifestations and either kidney failure (KF) or the last clinical visit—was 5 years [IQR: 2–10]. During this period, the estimated glomerular filtration rate (eGFR) declined by 9.17 mL/min/1.73 m² per year (95% CI: 3.31–15.02). KF developed in 47 patients (45.2%) at a median age of 40 years [IQR: 32–49]. Furthermore, 23 patients (22.1%) died during follow-up, with a median age at death of 61.0 years [IQR: 46.0–65.0]. Patients exhibited symptoms in a median of 4 different organs [3–5], with the prevalence of deafness at 88%, diabetes at 76%, cardiac involvement at 66%, neurological involvement at 53%, muscular involvement at 48%, and ophthalmic involvement at 42%. Extrarenal manifestations were significantly different between patients carrying the m.3243A>G mutation and those harboring a different mutation. Poorer kidney outcomes were significantly associated with the onset of the first mitochondrial disease symptom before the age of 20, the presence of a non-m.3243A>G mutation, or proteinuria exceeding 1 g/24 h. Conclusion Kidney involvement in patients with MELAS/MIDD can manifest as glomerular, tubular, or vascular dysfunction and often progresses to kidney failure. Earlier recognition and diagnosis of these rare mitochondrial diseases are critical to improving patient outcomes and optimizing care
Langue ou patois, picard ou ch’ti : comment l’auto-assignation d’une langue met en scène laconscience linguistique des locuteurs
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Résultats des réinterventions pour améliorer la resynchronisation cardiaque chez les patients non-répondeurs
No full textIntroduction: Cardiac resynchronization therapy (CRT) is an effective treatment for heart failure (HF) with reduced LVEF, but 30–50% of patients remain non-responders, with no standardized management strategy. Objective: To evaluate the outcomes of additional interventions aimed at improving resynchronization in CRT non-responders. Methods: This was a retrospective study including 72 CRT non-responders. The interventional group (n = 25) underwent an additional intervention on the pacing system, compared with a control group (n = 47) treated conventionally. The primary endpoints were clinical, electrocardiographic, and echocardiographic efficacy, HF hospitalizations, and mortality. The secondary endpoint was the response rate to resynchronization (defined as an increase in LVEF ≥ 5% and absence of HF hospitalization or cardiovascular death) in the interventional group. Results: Among the 25 patients in the interventional group, 7 received an LV lead in the postero-lateral vein, 10 in the antero-lateral vein, 6 underwent left bundle branch pacing (including 4 LOT-CRT), 1 received a new apical RV lead, and 1 underwent endocardial LV pacing. During 18 months of follow-up, patients in the interventional group showed improvement in NYHA class (2.7 ± 0.6 vs. 2.1 ± 0.5; p = 0.001), QRS width (170 ± 22 vs. 146 ± 22 ms; p = 0.001), LVEF (24.2 ± 6.8% vs. 28.3 ± 9.2%; p = 0.036), and mitral regurgitation grade (1.4 ± 0.8 vs. 0.9 ± 0.7; p = 0.043). Compared with the control group, operated patients showed greater QRS narrowing (−23 ± 17 vs. −3 ± 17 ms, p < 0.001) and a trend toward increased LVEF (3.7 ± 8.7 vs. −0.1 ± 5.0, p = 0.058), but no benefit regarding HF hospitalization (p = 0.285) or survival (p = 0.245). Ten patients (40%) in the interventional group were identified as responders. Placement of a lead in the postero-lateral vein, alone or in combination with LOT-CRT, was the only independent predictor of response (p = 0.011). Conclusion: Additional interventions aiming to optimize CRT in non-responders improve electrical and echocardiographic parameters in 40% of cases, achieving a positive response without significant impact on HF hospitalizations or mortality. These procedures should be considered in non-responders and further evaluated in prospective trials.Introduction: La resynchronisation cardiaque (CRT) est un traitement efficace de l’IC avec FEVG altérée, mais 30 à 50 % des patients restent non-répondeurs, sans stratégie standardisée de prise en charge. Objectif: Évaluer les résultats des réinterventions visant à améliorer la resynchronisation chez les patients non répondeurs à la CRT. Méthodes: Étude rétrospective, incluant 72 patients non-répondeurs a la CRT. Le groupe interventionnel (n = 25) a bénéficié d’une réintervention sur le système de stimulation, comparé à un groupe contrôle (n = 47) traité de façon conventionnelle. Les critères de jugement principal étaient l’efficacité clinique, électrocardiographique et échographique, les hospitalisations pour IC et la mortalité. Le critère de jugement secondaire était : le taux de réponse à la resynchronisation (définie comme augmentation de la FEVG ≥ 5 %, et absence d’hospitalisation pour IC ou de décès cardiovasculaire) dans le groupe interventionnel. Résultats: Parmi les 25 patients du groupe interventionnel, 7 ont eu une sonde VG ajoutée dans la veine postéro-latérale, 10 dans la veine antéro-latérale, 6 une stimulation de la branche gauche (dont 4 LOT-CRT), 1 une nouvelle sonde VD apicale et 1 une stimulation intracavitaire du VG. Dans le suivi de 18 mois les patients du groupe interventionnel améliorent leur classe NYHA (2,7 ± 0,6 vs 2,1 ± 0,5 ; p = 0,001), largeur des QRS (170 ± 22 vs 146 ± 22 ms ; p = 0,001), FEVG (24,2 ± 6,8 % vs 28,3 ± 9,2 % ; p = 0,036) et grade d’insuffisance mitrale (1,4 ± 0,8 vs 0,9 ± 0,7 ; p = 0,043). En comparaison au groupe contrôle les patients réopérés présentent l’affinement des QRS (-23 ± 17 vs -3 ± 17, p < 0.001 ) et l’augmentation de la FEVG (3.7 ± 8.7 vs -0.1 ± 5.0, p = 0.058) , mais pas de bénefice en terme d’hospitalisation pour IC (p = 0.285) ou survie (p = 0.245). Dix patients (40 %) du groupe interventionnel ont été identifiés comme répondeurs. L’ajout d’une sonde dans la veine postéro-latérale seul ou associé à une LOT-CRT était le seul facteur prédictif indépendant de réponse (p = 0,011). Conclusion: Les réinterventions visant à optimiser la CRT chez les patients non répondeurs améliorent les paramètres électriques et échographiques dans 40% des cas, permettant d’obtenir la réponse positive sans impact significative sur les hospitalisations pour IC et la mortalité. Ces procédures devraient être discutées chez les non-répondeurs et évaluées dans des essais prospectifs
« Le quotidien au masculin : l’impensé du genre dans les théories du quotidien et les études littéraires »
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Valorisation de culture végétales locales par le développement d'un support cosmétique sec plus respectueux de l'environnement
No full textInternational audienceAu cours de ces dernières années, un intérêt croissant s'est porté au niveau mondial pour les soins cosmétiques. On retrouve parmi ces produits des crèmes hydratantes, des crèmes solaires, des sérums, ou encore des masques cosmétiques. Parmi les masques cosmétiques, la catégorie de type « masque-tissus » applicable directement sur le visage est une gamme qui se développe de plus en plus. Ce type de produit est principalement composée d'un film biodégradable ou non, inerte, faisant office de support pour un principe actif amené sous forme liquide, la plupart du temps présent en excès. Ce type de produits, dont les consommateurs sont friands, posent des problèmes écologiques majeurs : (1) l'excédent de principes actifs et de produits chimiques à traiter dans les eaux usées ; (2) le traitement du support inerte après utilisation (mauvais tri sélectif, recyclage, destruction) ; (3) une pollution probable aux microplastiques engendrant un risque pour l'Homme et l'Environnement.Dans une optique d'économie circulaire et de réduction de l'empreinte environnementale, notre étude propose une alternative innovante aux masques traditionnels. Basée sur l'utilisation de biomasses végétales comme matière première pour leur fabrication, ces supports prennent la forme de patch secs. Ces derniers visent à valoriser des ressources renouvelables, locales et peu transformées, en minimisant les traitements mécaniques et chimiques, et en réduisant les paramètres énergivores (comme la température), afin de rendre le procédé de fabrication plus durable. Par ailleurs, dans une logique de rationalisation de l'usage des ressources et notamment en eau, nous cherchons à éliminer complètement la phase liquide des masques traditionnels. Les supports développés sont ainsi conçus pour incorporer, conserver et libérer efficacement les principes actifs au contact de la peau, tout en étant résistants à l'usage et compostables après utilisation. Ce dispositif permettrait d'optimiser la dose de produit appliquée, réduisant ainsi le gaspillage en molécules actives
To a Familiar New World : Narrating Post-Colonial Jewish Migration from Tunisia and Morocco to France
No full textInternational audienceThis article draws on original interviews with Jews who attended the schools of the Alliance Israélite Universelle in Morocco and Tunisia and subsequently immigrated to France. Building on recent approaches to Jewish migration history that move beyond persecution narratives as the primary motive for emigration, incorporate network analysis, and recognize agency in how and why people decide to emigrate to one place rather than another, it enriches our understanding of the how and why of the post-1948 Jewish departure from the Muslim world. As the interviewees’ departure narratives well demonstrate, political pressure points and personal circumstances combined with long-range economic, cultural, and educational forces to draw them from the Maghreb to France over the course of the 1950s, 1960s, and 1970s
S-adic characterization of minimal dendric shifts
No full textInternational audienceDendric shifts are defined by combinatorial restrictions of the extensions of the words in their languages. This family generalizes well-known families of shifts such as Sturmian shifts, Arnoux-Rauzy shifts and codings of interval exchange transformations. It is known that any minimal dendric shift has a primitive -adic representation where the morphisms in are positive tame automorphisms of the free group generated by the alphabet. In this paper we give an -adic characterization of this family by means of two finite graphs. As an application, we are able to decide whether a shift space generated by a uniformly recurrent morphic word is (eventually) dendric
Real-world comparison of effectiveness between ustekinumab and a second-line anti-TNF agent in patients with symptomatic stricturing Crohn’s disease failing to respond to a first-line anti-TNF agent: the USTEKNOSIS study
No full textInternational audienceBackground: Anti-tumor necrosis factor (TNF) agents are now accepted as the first-line medical treatment for stricturing Crohn’s disease (CD). However, data are lacking about the effectiveness of advanced therapies after anti-TNF failure. Objectives: To compare the effectiveness of ustekinumab and a second-line anti-TNF agent after failing to respond to a first-line anti-TNF for a symptomatic stricturing CD. Design: Multicenter retrospective study. Methods: We included consecutive adult patients with CD treated with ustekinumab or anti-TNF for symptomatic stricture (confirmed on imaging or endoscopy) after prior failure of one anti-TNF for the current stricture. Short-term endpoints were symptomatic remission (composite endpoint) at 6 months, defined as no abdominal pain, no vomiting, no food restriction, no sub-occlusive episode, no steroid, no surgery, and no drug discontinuation or symptomatic response (same definition except for tolerating mild abdominal pain). Long-term endpoints were time to drug discontinuation for treatment failure and to bowel damage progression. The comparisons were performed after using propensity score analysis. Results: Seventy patients were included (34 on ustekinumab, 36 on anti-TNF). After propensity score adjustment, symptomatic remission at 6 months was achieved in 73.9% of patients receiving ustekinumab compared to 42.7% in the anti-TNF group ( p = 0.24), while symptomatic response was observed in 84.0% and 49.5%, respectively ( p = 0.13). Predictors of remission in the ustekinumab group were prior bowel resection ( p = 0.001) and stricture length <12 cm ( p = 0.042). The risk of treatment discontinuation (hazard ratio (HR) = 2.86 (1.33–6.15); p = 0.008) and bowel damage progression (HR = 3.90 (1.64–9.24); p = 0.003) were higher in the anti-TNF group. Conclusion: Ustekinumab appears more effective than a second-line anti-TNF in patients with symptomatic stricturing CD after failing to respond to a first-line anti-TNF