Effets extra-ventilatoires de la ventilation non-invasive au cours de la sclérose latérale amyotrophique

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease. Respiratory failure (RF) develops when phrenic neurons are involved. Noninvasive ventilation (NIV) is the only treatment for diaphragm weakness. Patients with ALS-related RF often exhibit strong activity of inspiratory neck muscles (INM) as a compensatory mechanism to maintain adequate ventilation.In chapter 1 of thesis, resting energy expenditure (REE) is lower under NIV (median decrease of 7%). Chapter 2 confirms that standard tests to measure respiratory muscle endurance (maximal voluntary ventilation and repeated maximal inspiratory pressures) are not adapted to ALS patients with diaphragm involvement while 90 % of them complete a series of 10 maximal sniff pressures (SNIP). This test is sufficient to initiate fatigue in ALS patients with a progressive decrease in sniff amplitude and preserved maximal relaxation rate. These results suggest that central fatigue contribute to RF in ALS. Different tests of respiratory muscle endurance were not linked to ALS severity or NIV use. Chapter 3 shows that 57 % of ALS patients with RF exhibit respiratory-related cortical activity during spontaneous breathing. Pre-inspiratory potentials (PIP) almost disappear on NIV. Presence of PIP interferes with dyspnea and INM activity.NIV can reduce REE probably by alleviating the ventilator burden imposed on INM to compensate ALS-related RF. This positive contribution to a better nutritional equilibrium supports the hypothesis that starting NIV early in the course of ALS could be beneficial. To determine the good timing to initiate NIV stay difficult. Recording PIP could provide a useful tool.La sclérose latérale amyotrophique (SLA) est une maladie neurodégénérative. Une insuffisance diaphragmatique apparaît quand les neurones phréniques sont atteints. La ventilation non-invasive (VNI) est un traitement efficace. Une hyperactivité des muscles inspiratoires extradiaphragmatiques (MIED) est fréquente. C'est un phénomène compensateur vital pour assurer une ventilation correcte mais ses conséquences extraventilatoires restent inconnues. La VNI, mettant au repos les MIED, pourrait avoir d'autres effets bénéfiques que la correction des échanges gazeux. Dans la partie 1, la dépense énergétique (DE) diminue de 7% sous VNI. Dans la partie 2, les tests d'endurance (VMM et PiMAX répétés) ne sont pas adaptés aux patients SLA avec faiblesse diaphragmatique. Une série de 10 SNIP est réussie par 90% d'eux. La réalisation de SNIP répétés suffit à induire une fatigue aux caractéristiques évocatrices de fatigue centrale : la pression chute alors que la vitesse de relaxation normalisée est conservée. La fatigue n'est pas corrélée à la sévérité de la SLA ou à l'utilisation de la VNI. Dans la partie 3, 57% des patients SLA ont un potentiel préinspiratoire (PPI) en ventilation spontanée (VS), réponse corticale au déséquilibre charge-capacité. L'activité corticale préinspiratoire influence l'activité des MIED et la perception de la dyspnée. La VNI inhibe quasi-complètement le PPI. La VNI réduit la DE en soulageant le travail respiratoire imposé aux MIED pour compenser la faiblesse diaphragmatique. Cette contribution métabolique plaide en faveur d'une initiation précoce de la VNI. Juger du moment pour initier la VNI est délicat. La présence d’un PPI en VS peut guider la décision

Diaphragm pacing failure secondary to deteriorated chest wall mechanics: When a good diaphragm does not suffice to take a good breath in

AbstractDiaphragm pacing allows certain quadriplegic patients to be weaned from mechanical ventilation. Pacing failure can result from device dysfunction, neurotransmission failure, or degraded lung mechanics (such as atelectasis). We report two cases where progressive pacing failure was attributed to deteriorated chest wall mechanics. The first patient suffered from cervical spinal cord injury at age 45, was implanted with a phrenic stimulator (intrathoracic), successfully weaned from ventilation, and permanently paced for 7 years. Pacing effectiveness then slowly declined, finally attributed to rib cage stiffening due to ankylosing spondylitis. The second patient became quadriplegic after meningitis at age 15, was implanted with a phrenic stimulator (intradiaphragmatic) and weaned. After a year hypoventilation developed without obvious cause. In relationship with complex endocrine disorders, the patient had gained 31 kg. Pacing failure was attributed to excessive mechanical inspiratory load. Rib cage mechanics abnormalities should be listed among causes of diaphragm pacing failure and it should be kept in mind that a “good diaphragm” is not sufficient to produce a “good inspiration”

AI-based diagnosis in mandibulofacial dysostosis with microcephaly using external ear shapes

IntroductionMandibulo-Facial Dysostosis with Microcephaly (MFDM) is a rare disease with a broad spectrum of symptoms, characterized by zygomatic and mandibular hypoplasia, microcephaly, and ear abnormalities. Here, we aimed at describing the external ear phenotype of MFDM patients, and train an Artificial Intelligence (AI)-based model to differentiate MFDM ears from non-syndromic control ears (binary classification), and from ears of the main differential diagnoses of this condition (multi-class classification): Treacher Collins (TC), Nager (NAFD) and CHARGE syndromes.MethodsThe training set contained 1,592 ear photographs, corresponding to 550 patients. We extracted 48 patients completely independent of the training set, with only one photograph per ear per patient. After a CNN-(Convolutional Neural Network) based ear detection, the images were automatically landmarked. Generalized Procrustes Analysis was then performed, along with a dimension reduction using PCA (Principal Component Analysis). The principal components were used as inputs in an eXtreme Gradient Boosting (XGBoost) model, optimized using a 5-fold cross-validation. Finally, the model was tested on an independent validation set.ResultsWe trained the model on 1,592 ear photographs, corresponding to 1,296 control ears, 105 MFDM, 33 NAFD, 70 TC and 88 CHARGE syndrome ears. The model detected MFDM with an accuracy of 0.969 [0.838–0.999] (p < 0.001) and an AUC (Area Under the Curve) of 0.975 within controls (binary classification). Balanced accuracies were 0.811 [0.648–0.920] (p = 0.002) in a first multiclass design (MFDM vs. controls and differential diagnoses) and 0.813 [0.544–0.960] (p = 0.003) in a second multiclass design (MFDM vs. differential diagnoses).ConclusionThis is the first AI-based syndrome detection model in dysmorphology based on the external ear, opening promising clinical applications both for local care and referral, and for expert centers

Extracorporeal Membrane Oxygenation for Severe Acute Respiratory Distress Syndrome associated with COVID-19: An Emulated Target Trial Analysis.

RATIONALE: Whether COVID patients may benefit from extracorporeal membrane oxygenation (ECMO) compared with conventional invasive mechanical ventilation (IMV) remains unknown. OBJECTIVES: To estimate the effect of ECMO on 90-Day mortality vs IMV only Methods: Among 4,244 critically ill adult patients with COVID-19 included in a multicenter cohort study, we emulated a target trial comparing the treatment strategies of initiating ECMO vs. no ECMO within 7 days of IMV in patients with severe acute respiratory distress syndrome (PaO2/FiO2 <80 or PaCO2 ≥60 mmHg). We controlled for confounding using a multivariable Cox model based on predefined variables. MAIN RESULTS: 1,235 patients met the full eligibility criteria for the emulated trial, among whom 164 patients initiated ECMO. The ECMO strategy had a higher survival probability at Day-7 from the onset of eligibility criteria (87% vs 83%, risk difference: 4%, 95% CI 0;9%) which decreased during follow-up (survival at Day-90: 63% vs 65%, risk difference: -2%, 95% CI -10;5%). However, ECMO was associated with higher survival when performed in high-volume ECMO centers or in regions where a specific ECMO network organization was set up to handle high demand, and when initiated within the first 4 days of MV and in profoundly hypoxemic patients. CONCLUSIONS: In an emulated trial based on a nationwide COVID-19 cohort, we found differential survival over time of an ECMO compared with a no-ECMO strategy. However, ECMO was consistently associated with better outcomes when performed in high-volume centers and in regions with ECMO capacities specifically organized to handle high demand. This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivatives License 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/)

Noninvasive ventilation has extraventilatory effects in lateral amyotrophic sclerosis

La sclérose latérale amyotrophique (SLA) est une maladie neurodégénérative. Une insuffisance diaphragmatique apparaît quand les neurones phréniques sont atteints. La ventilation non-invasive (VNI) est un traitement efficace. Une hyperactivité des muscles inspiratoires extradiaphragmatiques (MIED) est fréquente. C'est un phénomène compensateur vital pour assurer une ventilation correcte mais ses conséquences extraventilatoires restent inconnues. La VNI, mettant au repos les MIED, pourrait avoir d'autres effets bénéfiques que la correction des échanges gazeux. Dans la partie 1, la dépense énergétique (DE) diminue de 7% sous VNI. Dans la partie 2, les tests d'endurance (VMM et PiMAX répétés) ne sont pas adaptés aux patients SLA avec faiblesse diaphragmatique. Une série de 10 SNIP est réussie par 90% d'eux. La réalisation de SNIP répétés suffit à induire une fatigue aux caractéristiques évocatrices de fatigue centrale : la pression chute alors que la vitesse de relaxation normalisée est conservée. La fatigue n'est pas corrélée à la sévérité de la SLA ou à l'utilisation de la VNI. Dans la partie 3, 57% des patients SLA ont un potentiel préinspiratoire (PPI) en ventilation spontanée (VS), réponse corticale au déséquilibre charge-capacité. L'activité corticale préinspiratoire influence l'activité des MIED et la perception de la dyspnée. La VNI inhibe quasi-complètement le PPI. La VNI réduit la DE en soulageant le travail respiratoire imposé aux MIED pour compenser la faiblesse diaphragmatique. Cette contribution métabolique plaide en faveur d'une initiation précoce de la VNI. Juger du moment pour initier la VNI est délicat. La présence d’un PPI en VS peut guider la décision.Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease. Respiratory failure (RF) develops when phrenic neurons are involved. Noninvasive ventilation (NIV) is the only treatment for diaphragm weakness. Patients with ALS-related RF often exhibit strong activity of inspiratory neck muscles (INM) as a compensatory mechanism to maintain adequate ventilation.In chapter 1 of thesis, resting energy expenditure (REE) is lower under NIV (median decrease of 7%). Chapter 2 confirms that standard tests to measure respiratory muscle endurance (maximal voluntary ventilation and repeated maximal inspiratory pressures) are not adapted to ALS patients with diaphragm involvement while 90 % of them complete a series of 10 maximal sniff pressures (SNIP). This test is sufficient to initiate fatigue in ALS patients with a progressive decrease in sniff amplitude and preserved maximal relaxation rate. These results suggest that central fatigue contribute to RF in ALS. Different tests of respiratory muscle endurance were not linked to ALS severity or NIV use. Chapter 3 shows that 57 % of ALS patients with RF exhibit respiratory-related cortical activity during spontaneous breathing. Pre-inspiratory potentials (PIP) almost disappear on NIV. Presence of PIP interferes with dyspnea and INM activity.NIV can reduce REE probably by alleviating the ventilator burden imposed on INM to compensate ALS-related RF. This positive contribution to a better nutritional equilibrium supports the hypothesis that starting NIV early in the course of ALS could be beneficial. To determine the good timing to initiate NIV stay difficult. Recording PIP could provide a useful tool

Le sevrage tabagique : un pilier du traitement du cancer broncho-pulmonaire

Cet article fait partie du numéro supplément Congrès annuel de l’American Thoracic Society (ATS) : "Respiratory Innovation Summit 2022", 13-18 mai 2022

Cigarettes électroniques : actualités en 2022

Cet article fait partie du numéro supplément Congrès annuel de l’American Thoracic Society (ATS) : "Respiratory Innovation Summit 2022", 13-18 mai 2022

Réadaptation respiratoire : actualités et futures recommandations

Correspondance : [email protected] audienc

Evaluation comparative de différentes stratégies simplifiées de surveillance de la ventilation non invasive

DIJON-BU Médecine Pharmacie (212312103) / SudocSudocFranceF

Spatial planning in Kinshasa (DRC) - The residential function - The case of “Limited Access Residential Areas” and the informal housing areas / Teaching unit

editorial reviewedComment aménager et gérer de manière durable les territoires ? Comment faciliter à tous les citoyens l’accès aux fonctions dont ils ont besoin ? Ces questions importantes pour les responsables publics ainsi que pour les citoyens sont également au cœur des nouveaux programmes scolaires de géographie (en Belgique francophone). Et, dans le cadre des apprentissages, comment intégrer et exploiter les représentations numériques de l’espace – mentionnées comme ressources incontournables – afin que chacun soit capable de mobiliser et de manipuler ces supports numériques à différentes échelles ? Cet article présente un dispositif pédagogique complet adapté à l’enseignement en dernière année de l’enseignement secondaire (terminale). Ce travail est issu d’un projet collaboratif initié par le Laboratoire de Méthodologie de la Géographie (LMG – ULiège) dans le cadre des cours et des stages de didactique disciplinaire en sciences géographiques (Master ULiège) en 2021. Concernant l’étude de l’aménagement du territoire à Kinshasa (RDC), la réalisation d’un diagnostic territorial est privilégiée par l’analyse de deux types d’habitats caractéristiques (les Cités Résidentielles à Accès Limité et les zones d’habitat informel), la mise en évidence des potentialités et vulnérabilités de ces zones d’habitat, la lecture et l’analyse de vues aériennes spécifiques ainsi que la manipulation d’un globe virtuel. La séquence comporte le dispositif général, une synthèse théorique spécifique à l’attention de l’enseignant, les liens vers les outils numériques à exploiter ainsi qu’une proposition de structure de cours avec des aides à la correction.How can territories be developed and managed in a sustainable way? How can we facilitate access for all citizens to the functions they need? These important questions for public officials and citizens are also at the core of the new geography school programmes (in French-speaking Belgium). And, within the framework of learning, how can digital representations of space – mentioned as essential resources – be integrated and exploited so that everyone is able to mobilise and manipulate these digital supports at different scales? This paper provides a complete teaching unit adapted to teaching in the final year of secondary education (high school). This is the result of a collaborative project initiated by the Laboratoire de Méthodologie de la Géographie (LMG - ULiège) as part of the training courses and internships in didactics of geographical sciences (Master ULiège) in 2021. Concerning the study of spatial planning in Kinshasa (DRC), the realisation of a territorial diagnosis is favoured by the analysis of two types of characteristic habitat areas (the “Limited Access Residential Areas” and the informal housing areas), the highlighting of the potentialities and vulnerabilities of these habitat areas, the reading and the analysis of specific aerial views as well as the manipulation of a virtual globe. The unit includes the general structure, a specific theoretical summary for the teacher, links for the digital tools to be used as well as a proposed course design with correction aids