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QuestionHumanPHOX2Bmutations result in life-threatening sleep-related hypoventilation (congenital central hypoventilation syndrome, CCHS). Most patients retain ventilatory activity when awake through a respiratory-related cortical network. We hypothesised that this need to mobilise cortical resources to breathe would lead to breathing-cognition interferences during cognitive loading.Patients and methodsSeven adult CCHS patients (five women; median age 21) performed standard neuropsychological tests (paced auditory serial addition test – calculation capacity, working memory, sustained and divided attention; trail making test – visuospatial exploration capacity, cognitive processing speed, attentional flexibility; Corsi block-tapping test – visuospatial memory, short-term memory, working memory) during unassisted breathing and under ventilatory support. Ventilatory variables and transcutaneous haemoglobin oxygen saturation were recorded. Cortical connectivity changes between unassisted breathing and ventilatory support were assessed using electroencephalographic recordings (EEG).ResultsBaseline performances were lower than expected in individuals of this age. During unassisted breathing, cognitive loading coincided with increased breathing variability, and decreases in oxygen saturation inversely correlated with an increasing number of apnoeic cycles per minute (rho −0.46, 95% CI −0.76 to −0.06, p=0.01). During ventilatory support, cognitive tasks did not disrupt breathing pattern and were not associated with decreased oxygen saturation. Ventilatory support was associated with changes in EEG cortical connectivity but not with improved test performances.ConclusionsAcute cognitive loads induce oxygen desaturation in adult CCHS patients during unassisted breathing, but not under ventilatory support. This justifies considering the use of ventilatory support during mental tasks in CCHS patients to avoid repeated episodes of hypoxia.

Background: More than a symptom, dyspnoea is an existential experience shaping the lives of those afflicted, particularly when its persistence despite maximal pathophysiological treatments makes it pervasive. It is, however, insufficiently appreciated by concerned people themselves, family members, healthcare professionals and the public (dyspnoea invisibility), limiting access to appropriate care and support. Aim: To provide a better understanding of dyspnoea experiences and its invisibility. Design: Interpretative phenomenological analysis of data collected prospectively through in-depth semi-structured interviews. Setting/Participants: Pulmonary rehabilitation facility of a tertiary care university hospital; 11 people (six men, five women) with severe chronic obstructive pulmonary disease (stages 3 and 4 of the 4-stage international GOLD classification) admitted for immediate post-exacerbation rehabilitation. Results: We identified several types of dyspnoea invisibility depending on temporality and interlocutors: (1) invisibility as a symptom to oneself; (2) invisibility as a symptom to others; (3) invisibility as an experience that cannot be shared; (4) invisibility as an experience detached from objective measurements; (5) invisibility as an experience that does not generate empathic concern. The notion of invisibility was present in all the identified experiential dimensions of dyspnoea. It was seen as worsening the burden of the disease and as self-aggravating through self-isolation and self-censorship. Conclusions: The study confirmed that dyspnoea invisibility is a reality for people with advanced chronic obstructive pulmonary disease. It shows dyspnoea invisibility to be a multifaceted burden. Future research should aim at identifying individual and collective measures to overcome dyspnoea invisibility.

International audience; No abstract available

The association between dyspnea and mortality has not been demonstrated in the ICU setting. We tested the hypothesis that dyspnea (self-reported respiratory discomfort) or its observational correlates (5-item intensive care Respiratory Distress Observation Scale [IC-RDOS]) assessed on ICU admission would be associated with ICU mortality.Ancillary analysis of single-center data prospectively collected from 220 communicative ICU subjects allocated to a derivation cohort of 120 subjects and a separate validation cohort of 100 subjects. Dyspnea was assessed dichotomously (yes/no), with a dyspnea visual analog scale (measured in mm), and IC-RDOS was calculated. Multivariate logistic regression was used to identify factors associated with ICU and hospital mortality.Dyspnea was reported by 69 (58%; median 45 [interquartile range [IQR] 32-60] mm) and 47 (47%; 38 [IQR 26-48] mm) subjects in the derivation and validation cohorts, respectively. IC-RDOS was 2.3 (1.2-3.1) and 2.4 (1.3-2.8), respectively. IC-RDOS values were higher in subjects with dyspnea than in subjects without dyspnea in both the derivation cohort (2.6 [2.2-4.6] vs 1.4 [0.9-2.4],IC-RDOS, an observational correlate of dyspnea, but not dyspnea itself, was associated with higher mortality in ICU subjects.

International audience; Ribeiro Baptista, Bruno, Morgane Faure, Gimbada Benny Mwenge, Capucine Morelot-Panzini, Christian Straus, Thomas Similowski, and Jésus Gonzalez-Bermejo. Feasibility of a hypoxic challenge test under noninvasive ventilation versus oxygen in neuromuscular patients with chronic respiratory insufficiency. High Alt Med Biol. 22:346–350, 2021.Background: The British Thoracic Society recommendations suggest that all patients with an oxygen saturation (SpO2) 90%) by oxygen therapy (+9 [6–12]%, p = 0.0029). Patient's home ventilator also significantly increased the SpO2 by 8 [7–12]% (p = 0.016). Correction of SpO2 during the HCT was not different between oxygen and NIV. NIV was associated with a significant decrease in pressure, end tidal, carbon dioxide (PetCO2) (−10 [−16 to −7.5] mmHg, p = 0.04).Conclusions: The performance of an adapted HCT in home-ventilated patients with a neuromuscular pathology may be useful in a personalized treatment plan for air travel. NIV can be a new alternative to oxygen therapy for neuromuscular patients planning to take a flight.

BACKGROUND: The assessment before surgical plication for unilateral hemidiaphragm (HD) eventration is not clearly defined and no precise criteria exist to really understand which patient is operated with which results depending on the technique used. The goal of this study was to evaluate the place of dynamic magnetic resonance imaging (dMRI) before and after plication by developing measurement criteria. METHODS: Between 2006 and 2017, 18 patients (group1: Gp1) were operated for eventrations, 15 left-sided (Gp1L) and 3 right-sided (Gp1R). All had preoperative and postoperative evaluations including dMRI and pulmonary function tests. Five healthy volunteer subjects (group2: Gp2) had the same imaging protocol. For each HD, we measured the respiratory excursion at three fixed points (S1, S2, S3) and the height of curvature on sagittal plane. We also searched for upward paradoxical diaphragm movements. RESULTS: Before surgery, no excursion (n=13) or extremely reduced excursion (n=5) was detected on the injured HD (IHD) in Gp1. Upward paradoxical movements were identified only in Gp1L (n=6). Compared with Gp2 subjects, the healthy HD for Gp1L patients had significantly reduced excursion values at three sites S1 (P=0.038), S2 (P=0.006), and S3 (P=0.004). After plication, the decreasing height of curvature confirmed a tightening of the IHD in all patients (median value from 100 to 39.5 mm in Gp1L and 92 to 74 mm in Gp1R, P=0.0001). All upward paradoxical movements disappeared. Healthy HD excursions in Gp1L normalised their values. All those imaging improvements were correlated with postoperative improvements of dyspnoea score (P

Resume La dyspnee resulte d’un desequilibre entre demande (liee a la production metabolique de CO2, au niveau de regulation de PaCO2 et au niveau de ventilation perdue – espace mort physiologique) et capacite (liee au systeme mecanique respiratoire passif – compliance, resistance – et actif – muscles respiratoires) ventilatoires. La spirometrie et la mesure des volumes pulmonaires statiques n’explorent que la capacite ventilatoire. La demande ventilatoire (augmentee notamment lors des atteintes vasculaires pulmonaires) n’est pas evaluee. La mesure du transfert du CO (DLCO), evaluant a la fois demande et capacite, est l’exploration la plus liee au degre de dyspnee, notamment au cours des pathologies obstructives et interstitielles pulmonaires. La dyspnee, composee de plusieurs dimensions, est intrinsequement complexe et mal expliquee par les explorations de repos : la variance expliquee n’atteint pas 50 %. La strategie diagnostique doit distinguer les plaintes en rapport ou non avec l’exercice (il existe des dyspnees sans rapport avec l’effort). L’exploration fonctionnelle a l’exercice avec mesure de V’O2, V’CO2, ventilation (V’E) et volumes pulmonaires dynamiques permet d’apprecier les mecanismes physiopathologiques associes a l’eventuelle limitation de la tolerance a l’effort et peut contribuer a decortiquer les mecanismes physiopathologiques d’une dyspnee mal expliquee meme si sa valeur « diagnostique etiologique » reste mal appreciee.

Purpose Amyotrophic lateral sclerosis (ALS) entails a risk of acute respiratory failure (ARF). The decision to admit such patients to the intensive care unit (ICU) is difficult given the inexorable prognosis of ALS. To fuel this discussion, this study describes the ICU and post-ICU survival of ALS-related ARF. Material and methods Retrospective cohort analysis over 10 years (university hospital setting, ALS reference center). Results Of 90 patients (66 men, median age: 67 [IQR 59–71], median interval since ALS diagnosis: 26.5 months [14–53], ALSFRS-R: 19 [12−30], bulbar signs 73%), 48 were managed by noninvasive ventilation (NIV) only, 7 were already tracheotomized upon admission, 12 were tracheotomized during the ICU stay (advance care planning project), 18 were already intubated before admission, 5 received oxygen and physiotherapy only. Median ICU stay was 4 days [ [2] , [3] , [4] , [5] , [6] , [7] , [8] , [9] ] with 20% mortality. Median hospital stay was 10 days [5–22] with 33% mortality. The 3-month and one year mortality wer 46% and 71%. Hospital mortality was higher in patients with more severe respiratory acidosis and higher simplified acute physiology scores on admission. Conclusions The prognosis of ALS-related ARF requiring ICU admission resembles that of ARF complicating other conditions with high short-term mortality (e.g. lung cancer).

Question addressedIn contrast with pain, dyspnoea is not visible to the general public, who lack the corresponding experiential baggage. We tested the hypothesis that the generalised use of face masks to fight severe acute respiratory syndrome coronavirus 2 dissemination could change this and sensitise people to respiratory health.MethodsGeneral population polling (1012-person panel demographically representative of the adult French population, quota sampling method; 517 (51%) female). 860 (85%) answered “no” to “treated for a chronic respiratory disease” (“respiratory healthy”), and 152 answered “yes” (“respiratory disease”). 14% of respiratory healthy respondents reported having a close family member treated for a chronic respiratory disease (RH-family+). Respondents described mask-related attitudes, beliefs, inconveniencies, dyspnoea and changes in their respiratory health vision.ResultsCompliance with masks was high (94.7%). Dyspnoea ranked first among mask inconveniencies (respiratory disease 79.3%, respiratory healthy 67.3%; p=0.013). “Air hunger” was the main sensory dyspnoea descriptor. Mask-related dyspnoea was independently associated with belonging to RH-family+ (OR 1.85, 95% CI 1.16–2.98) and removing masks to improve breathing (OR 5.21, 95% CI 3.73–7.28). It was negatively associated with considering masks effective to protect others (OR 0.42, 95% CI 0.25–0.75). Half the respondents were more concerned with their respiratory health since wearing masks; 41% reported better understanding patients’ experiences.Answer to the questionWearing protective face masks leads to the mass discovery of breathing discomfort. It raises public awareness of what respiratory diseases involve and sensitivity to the importance of breathing. These data should be used as the fulcrum of respiratory health oriented communication actions.

PURPOSE/OBJECTIVE(S) To evaluate the efficacy and safety of radiotherapy (RT) of the primary salivary glands in a large series of patients with amyotrophic lateral sclerosis (ALS) and Parkinson's disease with sialorrhea. MATERIALS/METHODS Patients treated in our institution between 2010 and 2020 were retrospectively analyzed, including the first 50 patients prospectively analyzed. All patients included were refractory to conventional sialorrhea drug treatments. RT was delivered by a conventional LINAC, with 2 opposite beams including the two submandibular glands and two thirds of the two parotid glands before 2014, and from 2015 2 lateral beams including the submaxillary glands, and 2 opposite oblique beams directed to each parotid gland, with reduced oral cavity irradiation. The total dose delivered was 10 Gy in 2 fractions or 20 Gy in 4 fractions. RT efficacy was assessed using the 9-item Sialorrhea Scoring Scale (SSS), prospectively validated as the most effective and sensitive tool for measuring sialorrhea in ALS patients. RESULTS A total of 235 patients were included, and 281 treatments (45 reirradiations, 1 patient received 3 irradiations). Median age was 69 years (range: 38 - 95), 127 women (54%) and 126 men (46%), 212 had ALS (90%), 17 had Parkinson's disease (7%), and 5 patients (3%) had another neurological disorder. Median interval between the first and second radiotherapy course was 7.5 months (range: 1 - 37). The median SSS score before radiation therapy was 8 (range: 6 - 9), and the median SSS score at 1 month of irradiation was 2 (range: 1 - 6). There was no toxicity of > grade 1. At the end of RT, all but one patient had an improvement in SSS score: 272 had a complete response (CR) (97%, SSS 1-3) and 8 had a partial response (PR) (3%, SSS 4-5); one patient had a stable SSS score (< 1%). A 1-month CR after RT was achieved in 96% of the 235 patients who underwent a primary RT, and 100% of the 45 patients who received re-irradiation. Patients treated with 20Gy versus 10Gy during the first RT treatment were more likely to have a CR at 1 month (99% vs. 91%, P = 0.007), and had a greater decrease in SSS score (mean difference -6 vs. -5 points, P < 0.001). Also, the 45 patients who received reirradiation were more frequently previously treated with the RT 10Gy protocol (25/45 patients, 55%) versus 60/191 (31%) in patients who did not require reirradiation (P < 0.001). The 3-beam irradiation technique, used from 2014 and beyond, did not significantly improve treatment efficacy (reduction of the SSS score by -5.8 points on average versus -5.6 points previously, P = 0.3). Nevertheless, it reduced 1 month toxicity, in particular decreased saliva thickening discomfort (P < 0.001). CONCLUSION A 20 Gy irradiation in 4 fractions is an effective treatment for ALS patients with sialorrhea, with minimal toxicity, particularly by separately targeting parotid and submaxillary glands. A shorter RT (10 Gy in 2 fractions) may be proposed in patients with poor medical condition, subject to its lower efficacy and the greater need for reirradiation.

La Société de Pneumologie de Langue Française (SPLF) propose un guide pour la prise en charge thérapeutique des patients ayant d’éventuelles séquelles respiratoires après avoir présenté une pneumonie à SARS-CoV-2 (COVID-19). Les propositions s’appuient sur les données connues des précédentes épidémies, les données préliminaires publiées sur le suivi après COVID-19 et les avis d’experts. Les propositions ont été élaborées par un groupe d’experts puis soumises selon la méthode Delphi à un panel composé de 22 pneumologues. Dix-sept propositions ont été validées, qui vont des examens complémentaires à réaliser après le bilan minimal proposé dans le guide de suivi de la SPLF à la place de la corticothérapie inhalée ou systémique et des médicaments antifibrosants. Ces propositions pourront évoluer dans le temps au fil des connaissances sur le sujet. Ce guide insiste sur l’importance de la discussion multidisciplinaire.

Key Points Question Can electroencephalography (EEG), combined with clinical, biological, and magnetic resonance imaging (MRI) analyses, help to better characterize patients with neurologic coronavirus disease 2019 (COVID-19) and diagnose specific COVID-19–related encephalopathy? Findings Neurologic manifestations, biological findings, EEG findings, and brain MRI images were analyzed in a cohort study of 78 adult patients with COVID-19. Nine patients had no identified cause of brain injury, as revealed by biological and MRI findings; their injury was defined as COVID-19–related encephalopathy. Meaning This study suggests that, although neurologic manifestations, EEG findings, and MRI findings may appear heterogeneous and nonspecific, multimodal monitoring may better identify patients with COVID-19–related encephalopathy and guide treatment strategy., Importance There is evidence of central nervous system impairments associated with coronavirus disease 2019 (COVID-19) infection, including encephalopathy. Multimodal monitoring of patients with COVID-19 may delineate the specific features of COVID-19–related encephalopathy and guide clinical management. Objectives To investigate clinical, biological, and brain magnetic resonance imaging (MRI) findings in association with electroencephalographic (EEG) features for patients with COVID-19, and to better refine the features of COVID-19–related encephalopathy. Design, Setting, and Participants This retrospective cohort study conducted in Pitié-Salpêtrière Hospital, Paris, France, enrolled 78 hospitalized adults who received a diagnosis of severe acute respiratory syndrome coronavirus 2 (SARS-Cov2) and underwent EEG between March 30 and June 11, 2020. Exposures Detection of SARS-CoV-2 from a nasopharyngeal specimen using a reverse transcription–polymerase chain reaction assay or, in the case of associated pneumonia, on a computed tomography scan of the chest. Main Outcomes and Measures Data on the clinical and paraclinical features of the 78 patients with COVID-19 were retrieved from electronic patient records. Results Of 644 patients who were hospitalized for COVID-19, 78 (57 men [73%]; mean [SD] age, 61 [12] years) underwent EEG. The main indications for EEG were delirium, seizure-like events, and delayed awakening in the intensive care unit after stopping treatment with sedatives. Sixty-nine patients showed pathologic EEG findings, including metabolic-toxic encephalopathy features, frontal abnormalities, periodic discharges, and epileptic activities. Of 57 patients who underwent brain MRI, 41 showed abnormalities, including perfusion abnormalities, acute ischemic lesions, multiple microhemorrhages, and white matter–enhancing lesions. Fifty-five patients showed biological abnormalities, including dysnatremia, kidney failure, and liver dysfunction, the same day as the EEG. The results of cerebrospinal fluid analysis were negative for SARS-Cov-2 for all tested patients. Nine patients who had no identifiable cause of brain injury outside COVID-19 were further isolated; their brain injury was defined as COVID-19–related encephalopathy. They represented 1% (9 of 644) of patients with COVID-19 requiring hospitalization. Six of these 9 patients had movement disorders, 7 had frontal syndrome, 4 had brainstem impairment, 4 had periodic EEG discharges, and 3 had MRI white matter–enhancing lesions. Conclusions and Relevance The results from this cohort of patients hospitalized with COVID-19 suggest there are clinical, EEG, and MRI patterns that could delineate specific COVID-19–related encephalopathy and guide treatment strategy., This cohort study investigates clinical, biological, and brain magnetic resonance imaging (MRI) findings in association with electroencephalographic (EEG) features for patients with coronavirus disease 2019 (COVID-19) and refines the features of COVID-19–related encephalopathy.

International audience; Introduction: Lung function in survivors of SARS-Co-V2 pneumonia is poorly known, but concern over the possibility of sequelae exists.Methods: Retrospective study on survivors with confirmed infection and pneumonia on chest-CT. Correlations between PFT and residual radiologic anomalies at three months taking into account initial clinical and radiological severity and steroid use during acute phase.Results: 137 patients (69 men, median age 59 (Q1 50; Q3 68), BMI 27.5 kg/m2 (25.1; 31.7)) were assessed. Only 32.9% had normal PFT, 75 had altered DLCO. Median (Q1; Q3) values were: VC 79 (66; 92) % pred, FEV1 81 (68; 89), TLC 78 (67; 85), DLCO 60 (44; 72), and KCO 89 (77; 105). Ground glass opacities (GGO) were present in 103 patients (75%), reticulations in 42 (30%), and fibrosis in 18 (13%). There were significantly lower FEV1 (p = 0.0089), FVC (p = 0.0010), TLC (p < 0.0001) and DLCO (p < 0.0001) for patients with GGO, lower TLC (p = 0.0913) and DLCO (p = 0.0181) between patients with reticulations and lower FVC (p = 0.0618), TLC (p = 0.0742) DLCO (p = 0.002) and KCO (p = 0.0114) between patients with fibrosis. Patients with initial ≥50% lung involvement had significantly lower FEV1 (p = 0.0019), FVC (p = 0.0033), TLC (p = 0.0028) and DLCO (p = 0.0003) compared to patients with ≤10%. There was no difference in PFT and residual CT lesions between patients who received steroids and those who did not.Conclusion: The majority of patients have altered PFT at three months, even in patients with mild initial disease, with significantly lower function in patients with residual CT lesions. Steroids do not seem to modify functional and radiological recovery. Long-term follow-up is needed.

Background Kennedy’s disease (KD), also known as spinal and bulbar muscular atrophy (SBMA), is a rare, adult-onset, X-linked recessive neuromuscular disease caused by CAG expansions in exon 1 of the androgen receptor gene (AR). The objective of the French national diagnostic and management protocol is to provide evidence-based best practice recommendations and outline an optimised care pathway for patients with KD, based on a systematic literature review and consensus multidisciplinary observations. Results The initial evaluation, confirmation of the diagnosis, and management should ideally take place in a tertiary referral centre for motor neuron diseases, and involve an experienced multidisciplinary team of neurologists, endocrinologists, cardiologists and allied healthcare professionals. The diagnosis should be suspected in an adult male presenting with slowly progressive lower motor neuron symptoms, typically affecting the lower limbs at onset. Bulbar involvement (dysarthria and dysphagia) is often a later manifestation of the disease. Gynecomastia is not a constant feature, but is suggestive of a suspected diagnosis, which is further supported by electromyography showing diffuse motor neuron involvement often with asymptomatic sensory changes. A suspected diagnosis is confirmed by genetic testing. The multidisciplinary assessment should ascertain extra-neurological involvement such as cardiac repolarisation abnormalities (Brugada syndrome), signs of androgen resistance, genitourinary abnormalities, endocrine and metabolic changes (glucose intolerance, hyperlipidemia). In the absence of effective disease modifying therapies, the mainstay of management is symptomatic support using rehabilitation strategies (physiotherapy and speech therapy). Nutritional evaluation by an expert dietician is essential, and enteral nutrition (gastrostomy) may be required. Respiratory management centres on the detection and treatment of bronchial obstructions, as well as screening for aspiration pneumonia (chest physiotherapy, drainage, positioning, breath stacking, mechanical insufflation-exsufflation, cough assist machnie, antibiotics). Non-invasive mechanical ventilation is seldom needed. Symptomatic pharmaceutical therapy includes pain management, endocrine and metabolic interventions. There is no evidence for androgen substitution therapy. Conclusion The French national Kennedy’s disease protocol provides management recommendations for patients with KD. In a low-incidence condition, sharing and integrating regional expertise, multidisciplinary experience and defining consensus best-practice recommendations is particularly important. Well-coordinated collaborative efforts will ultimately pave the way to the development of evidence-based international guidelines.

Introduction La dyspnee semble peu rapportee au cours des pneumopathies causees par le coronavirus 2 du syndrome respiratoire aigu severe (SARS CoV 2), faisant parfois evoquer le terme d’“hypoxemie silencieuse”. La dyspnee, perception d’informations sensorielles liees a la respiration, est associee a un vecu emotionnel negatif. Elle resulterait de l’activation de structures du systeme limbique/paralimbique dont, l’insula. Cette derniere serait le centre d’alarme interoceptif, reliant la perception de sensations viscerales (dont la dyspnee) a l’experience consciente des emotions. Le potentiel neuroinvasif du SARS CoV 2 via les bulbes olfactifs fait suspecter une atteinte limbique/paralimbique mais aussi du tronc cerebral au cours de l’atteinte respiratoire de la Corona VIrus Disease 2019 (COVID19). Nous formulons l’hypothese qu’une attenuation de la composante affective de la dyspnee lors de l’hypoxie, resultant de l’atteinte de l’interoception, est la consequence d’une atteinte de ces structures neurologiques. Methodes Une etude observationnelle, DysCovid, a porte sur l’evaluation de l’interoception et de la dyspnee chez des patients hospitalises en pneumologie et/ou en reanimation. Une etude physiopathologique, CRC COVID, a porte sur l’evaluation de l’interoception et de la dyspnee experimentalement induite chez des patients presentant une forme neurologique pure de la COVID19. Ces etudes comportaient un examen neurologique, une evaluation de l’interoception et de la dyspnee au moyen d’echelles visuelles analogiques (sensorielle et affective) et du questionnaire multidimensional dyspnea profile. Dans CRC-COVID la dyspnee et la reponse ventilatoire ont ete evaluees lors de test en hypoxie et en hypercapnie. Resultats et Conclusion Les inclusions et analyses des donnees, sont en cours.

Introduction Au cours du mois de mars 2020, de nombreux cas d’hyposmie et hypogueusie soudaines associees au SARS-CoV-2 ont ete signales partout dans le monde. Notre objectif etait de decrire la prevalence de l’hyposmie et de l’hypogueusie et de la comparer chez les patients COVID-19 hospitalises et non hospitalises pour evaluer l’association de ces symptomes avec la gravite de la maladie. Materiels et methodes Nous avons realise une etude transversale pendant 5 jours consecutifs en mars 2020, au sein d’un hopital centre de reference, du secteur de consultation ambulatoire de ce meme hopital et de deux centres de sante rattaches dans la meme ville. Tous les patients testes positifs pour le SARS-CoV-2 hospitalises pendant la periode d’etude et pouvant etre interroges (n = 198), les patients vus en consultation externe testes positifs pour le SARS-CoV-2 au cours du mois precedent (n = 129), et tous les patients hautement suspects de COVID-19 vus dans les deux centres de sante (n = 63) ont ete inclus. Resultats Les patients hospitalises etaient significativement plus souvent de sexe masculin (64 % vs 40 %) et plus âges (66 vs 43 ans en mediane) et avaient significativement plus de comorbidites que les patients ambulatoires. Au total, une hyposmie et une hypogueusie etaient rapportees par 33 % des patients, significativement moins frequemment par les patients hospitalises (12 % et 13 %, respectivement) que par les patients vus dans les centres de sante (33 % et 43 % respectivement) et en consultation a l’hopital (65 % et 60 %, respectivement). L’hyposmie et l’hypogueusie apparaissaient plus frequemment apres les autres symptomes de COVID-19. Les patients atteints d’hyposmie et/ou d’hypogueusie etaient significativement plus jeunes et avaient significativement moins de criteres de gravite respiratoire que les patients sans ces symptomes. Conclusion Un dysfonctionnement olfactif et gustatif survient frequemment au COVID-19, en particulier chez les patients jeunes et non severes. Ces symptomes sont un outil utile dans la demarche diagnostique chez les patients suspects de COVID-19.

BackgroundIdentification of prognostic factors in COVID-19 remains a global challenge. The role of smoking is still controversial.ObjectiveTo evaluate the rate of daily smokers in patients with COVID-19.MethodsCOVID-19 in-and outpatients from a large French university hospital were systematically interviewed for their smoking status, use of e-cigarette and nicotinic substitutes. The rates of daily smokers in in-and outpatients were compared to those in the 2019 French general population, after standardization for sex and age.ResultsThe inpatient group was composed of 340 patients, median age 66 years: 203 men (59.7%) and 137 women (40.3%), median age for both 66 years, with a daily smokers rate of 4.1 % CI95% [2.3–6.9] (5.4% of men, 2.2% of women). The outpatient group was composed of 139 patients, median age 44 years: 62 men (44.6%, median age 43 years), and 77 women (55.4%, median age 44 years). The daily smoker rate was 6.1 % CI 95% [2.7 - 11.6] (5.1% of men, 6.8% of women). In the 2019 French population, the daily smoker rate was 24.0% (27.5% of men, 20.7% of women). Among inpatients, daily smokers represented 2.2% and 3.4% of the 45 dead patients and of the 29 patients transferred to ICU, respectively.The rate of daily smokers was significantly lower in COVID-19 patients, as compared to that in the French general population after standardization by age and sex, with Standardized Incidence Ratios of 0.24 [0.12-0.48] for outpatients and 0.24 [0.14-0.40] for inpatients.ConclusionDaily smokers rate in patients with symptomatic COVID-19 is lower as compared to the general population

Since December 2019, SARS‐CoV‐2 has rapidly spread worldwide, challenging the clinician and focusing the entire globe on critical illness high mortality.1 Apart from common respiratory symptoms, patients often present with symptoms suggestive of SARS‐CoV‐2 neuroinvasiveness.2 Some are very general (like headache, nausea, or vomiting), while others are more specific, such as hypoacousis, agueusia, or anosmia. SARS‐CoV‐2 is suspected to enter the central nervous system through the olfactive bulb and to progress transsynaptically to the brain—a mechanism demonstrated in animal models of SARS‐CoV‐1 infection.

Importance: As the pandemic of COVID-19 is still under progression, identification of prognostic factors remains a global challenge. The role of smoking has been suggested among the disease risk factors, although it is highly controversial. Objective: To evaluate whether the rate of daily smokers in patients with COVID-19 was different to that in the French population. Participants: COVID-19-infected in- and outpatients in a large French university hospital between February 28, 2020 and March 30, 2020 for outpatients and from March 23, till April 9, 2020 for inpatients. Design: We systematically interviewed the patients on their smoking status, use of e-cigarette and nicotinic substitutes. The rate of daily smokers in inpatients and outpatients were compared to those in the 2018 French general population, after standardization for sex and age. Results: The inpatient group was composed of 340 patients, median age 66 years: 203 men (59.7%, median age 66 years) and 137 women (40.3%, median age 66 years),with a rate of daily smokers of 4.1% CI95% [2.3 – 6.9] (5.4% of men and 2.2% of women). The outpatient group was composed of 139 patients, median age 44 years: 62 men (44.6%, median age 43 years, and 77 women (55.4 %, median age 44 years). The daily smokers’ rate was 6.1 % CI95% [2.7 - 11.6] (5.1% of men and 6.8 % of women). In the French population, the daily smokers’ rate was 25.4% (28.2% of men and 22.9% of women). The rate of daily smokers was significantly lower in COVID-19 patients, as compared to that in the French general population after standardization by age and sex, with Standardized Incidence Ratios of 0.23 [0.11 - 0.45] for outpatients and 0.23 [0.14 - 0.39] for inpatients. These ratios did not significantly differ between the two groups (P=0.94). Conclusions and relevance: This cross sectional study in both COVID-19 out- and inpatients shows that daily smokers rate in patients with symptomatic COVID-19 is lower as compared to the general population.

SARS-CoV-2 can cause severe pneumonia requiring invasive mechanical ventilation [2], in the context of atypical acute respiratory distress syndrome (ARDS) [3]. The magnitude of the epidemic places an unprecedented pressure on intensive care units (ICU), making avoidance of intubation a critical issue., COVID-19 pneumonia can be life-threatening. Given the unprecedented burden place on ICU resources by the epidemic, avoiding intubation is a major issue. This study suggests that CPAP can achieve this objective.

IntroductionPreventing in-flight hypoxia in pilots is typically achieved by wearing oxygen masks. These masks must be as comfortable as possible to allow prolonged and repeated use. The consequences of mask-induced facial contact pressure have been extensively studied, but little is known about mask-induced breathing discomfort. Because breathlessness is a strong distractor and engages cerebral resources, it could negatively impact flying performances.MethodsSeventeen volunteers (age 20–32) rated respiratory discomfort while breathing with no mask and with two models of quick-donning full-face crew oxygen masks with regulators (mask A, mask B). Electroencephalographic recordings were performed to detect a putative respiratory-related cortical activation in response to inspiratory constraint (experiment 1, n=10). Oxygen consumption was measured using indirect calorimetry (experiment 2, n=10).ResultsWith mask B, mild respiratory discomfort was reported significantly more frequently than with no mask or mask A (experiment 1: median respiratory discomfort on visual analogue scale 0.9 cm (0.5–1.4), experiment 1; experiment 2: 2 cm (1.7–2.9)). Respiratory-related cortical activation was present in 1/10 subjects with no mask, 1/10 with mask A and 6/10 with mask B (significantly more frequently with mask B). Breathing pattern, sigh frequency and oxygen consumption were not different.ConclusionsIn a laboratory setting, breathing through high-end aeronautical full-face crew oxygen masks can induce mild breathing discomfort and activate respiratory-related cortical networks. Whether or not this can occur in real-life conditions and have operational consequences remains to be investigated. Meanwhile, respiratory psychometric and neuroergonomic approaches could be worth integrating to masks development and evaluation processes.

Introduction Les consequences a long terme des formes severes de pneumonie a SARS-CoV-2 (COVID-19) restent meconnues mais le risque de sequelles pulmonaires, de handicap fonctionnel, notamment respiratoire, d’impact psychologique et d’alteration de la qualite de vie des patients semble indeniable. Methodes RE 2 COVERI est une etude de cohorte observationnelle multicentrique (14 centres) francaise visant a inclure 625 participants. Les criteres d’inclusion sont: âge ≥ 18 ans; PCR SARS-CoV-2 positive ou TDM typique de COVID-19 au diagnostic; infection COVID-19 severe (hospitalisation ≥ 7 jours et oxygenotherapie ≥ 3L/min) ou tres severe (SDRA necessitant le recours a la ventilation mecanique pendant au moins 48 h). L’objectif principal de l’etude est de determiner la trajectoire de recuperation de la fonction respiratoire (spirometrie, plethysmographie, diffusion du monoxyde de carbone) des pneumonies COVID-19 severes ou tres severes (evaluation a M3, M6 et M12). Les objectifs secondaires sont de: determiner la trajectoire de recuperation et la proportion de patients presentant des anomalies en termes de capacite d’exercice (test de marche, test du lever de chaise), de lesions pulmonaires (scanner thoracique) et de force musculaire (hand grip et pinch test; mesure de la force des muscles respiratoires); determiner les relations entre les alterations fonctionnelles, la capacite d’exercice et l’etendue initiale des lesions; evaluer la dyspnee et les autres symptomes residuels, la fatigue, l’anxiete et la depression, le stress post-traumatique et l’impact sur la qualite de vie; comparer les alterations fonctionnelles et de la capacite d’exercice selon la severite clinique initiale, en tenant compte de l’impact des comorbidites et des therapeutiques et interventions proposees, tant initialement que secondairement; evaluer la consommation de soins de sante chez les patients severes ou tres severes, en mettant l’accent sur l’utilisation des soins de rehabilitation. Cette etude a recu un soutien financier de la Fondation du Souffle dans le cadre de l’appel a projet “SARSCohorte-Covid2020”. Resultats Les resultats preliminaires de cette etude (evaluation a M3 et M6) seront presentes lors du congres. Conclusion Nos resultats devraient permettre de preciser les facteurs influencant la recuperation a distance d’une forme severe a tres severe de pneumonie a SARS-CoV-2 et de preciser quels patients justifient d’une attention et d’un suivi particuliers.

International audience; Exercise induces release of cytokines and increase of circulating natural killers (NK) lymphocyte during strong activation of respiratory muscles. We hypothesised that non-fatiguing respiratory muscle loading during exercise causes an increase in NK cells and in metabolic stress indices. Heart rate (HR), ventilation (VE), oesophageal pressure (Pes), oxygen consumption (VO2), dyspnoea and leg effort were measured in eight healthy humans (five men and three women, average age of 31 ± 4 years and body weight of 68 ± 10 kg), performing an incremental exercise testing on a cycle ergometer under control condition and expiratory flow limitation (FL) achieved by putting a Starling resistor. Blood samples were obtained at baseline, at peak of exercise and at iso-workload corresponding to that reached at the peak of FL exercise during control exercise. Diaphragmatic fatigue was evaluated by measuring the tension time index of the diaphragm. Respiratory muscle overloading caused an earlier interruption of exercise. Diaphragmatic fatigue did not occur in the two conditions. At peak of flow-limited exercise compared to iso-workload, HR, peak inspiratory and expiratory Pes, NK cells and norepinephrine were significantly higher. The number of NK cells was significantly related to ΔPes (i.e. difference between the most and the less negative Pes) and plasmatic catecholamines. Loading of respiratory muscles is able to cause an increase of NK cells provided that activation of respiratory muscles is intense enough to induce a significant metabolic stress.

The Société de Pneumologie de Langue Française proposes a decision algorithm on long-term pharmacological COPD treatment. A working group reviewed the literature published between January 2009 and May 2016. This document lays out proposals and not guidelines. It only focuses on pharmacological treatments except vaccinations, smoking cessation treatments and oxygen therapy. Any COPD diagnosis, based on pulmonary function tests, should lead to recommend smoking cessation, vaccinations, physical activity, pulmonary rehabilitation in case of activity limitation, and short-acting bronchodilators. Symptoms like dyspnea and exacerbations determine the therapeutic choices. In case of daily dyspnea and/or exacerbations, a long-acting bronchodilator should be suggested (beta-2 agonist, LABA or anticholinergics, LAMA). A clinical and lung function reevaluation is suggested 1 to 3 months after any treatment modification and every 3-12 months according to the severity of the disease. In case of persisting dyspnea, a fixed dose LABA+LAMA combination improves pulmonary function (FEV1), quality of life, dyspnea and decreases exacerbations without increasing side effects. In case of frequent exacerbations and a FEV1≤70%, a fixed dose long-acting bronchodilator combination or a LABA+ inhaled corticosteroids (ICS) combination can be proposed. A triple combination (LABA+LAMA+ICS) is indicated when exacerbations persist despite one of these combinations. Dyspnea in spite of a bronchodilator combination or exacerbations in spite of a triple combination should lead to consider other pharmacological treatments (theophylline if dyspnea, macrolides if exacerbations, low-dose opioids if refractory dyspnea).

Background Breathlessness is the prominent symptom of chronic obstructive pulmonary disease (COPD). Despite optimal therapeutic management including pharmacological and non-pharmacological interventions, many COPD patients exhibit significant breathlessness. Chronic breathlessness is defined as breathlessness that persists despite optimal treatment of the underlying disease. Because of the major disability related to chronic breathlessness, symptomatic treatments including opioids have been recommended by several authors. The prevalence of chronic breathlessness in COPD and its management in routine clinical practice have been poorly investigated. Our aim was to examine prevalence, associated characteristics and management of chronic breathlessness in patients with COPD recruited in a real-life tertiary hospital-based cohort. Methods A prospective study was conducted among 120 consecutive COPD patients recruited, in stable condition, at Nancy University Hospital, France. In parallel, 88 pulmonologists of the same geographical region were asked to respond to an on-line questionnaire on breathlessness management. Results Sixty four (53%) patients had severe breathlessness (modified Medical Research Council scale≥3), despite optimal inhaled medications for 94% of them; 40% had undergone pulmonary rehabilitation within the past 2 years. The severity of breathlessness increased with increasing airflow limitation. Breathlessness was associated with increased symptoms of anxiety, depression and with osteoporosis. No relation was found with other symptoms, exacerbation rate, or cardiovascular comorbidities. Among the patients with chronic breathlessness and Hospitalized Anxiety and/or Depression score > 10, only 25% were treated with antidepressant or anxiolytic. Among the pulmonologists 46 (52%) answered to the questionnaire and expressed a high willingness to prescribe opioids forchronic breathlessness, which contrasted with the finding that none of these patients received such treatments against breathlessness. Conclusion Treatment approaches to breathlessness and associated psychological distress are insufficient in COPD. This study highlights underuse of pulmonary rehabilitation and symptomatic treatment for breathlessness. Electronic supplementary material The online version of this article (10.1186/s12890-019-0851-5) contains supplementary material, which is available to authorized users.

International audience; Non-invasive ventilation (NIV) has become an essential part of the treatment of amyotrophic lateral sclerosis (ALS) since 2006. NIV very significantly improves survival, quality of life and cognitive performances. The initial NIV settings are simple, but progression of the disease, ventilator dependence and upper airway involvement sometimes make long-term adjustment of NIV more difficult, with a major impact on survival. Unique data concerning the long-term adjustment of NIV in ALS show that correction of leaks, management of obstructive apnoea and adaptation to the patient's degree of ventilator dependence improve the prognosis. Non-ventilatory factors also impact the efficacy of NIV and various solutions have been described and must be applied, including cough assist techniques, control of excess salivation and renutrition. NIV in ALS has been considerably improved as a result of application of all of these measures, avoiding the need for tracheostomy in the very great majority of cases. More advanced use of NIV also requires pulmonologists to master the associated end-of-life palliative care, as well as the modalities of discontinuing ventilation when it becomes unreasonable.

Amyotrophic lateral sclerosis (ALS) patients show progressive respiratory muscle weakness leading to death from respiratory failure. However, there are no data on diaphragm histological changes in ALS patients and how they correlate with routine respiratory measurements.We collected 39 diaphragm biopsies concomitantly with laparoscopic insertion of intradiaphragmatic electrodes during a randomised controlled trial evaluating early diaphragm pacing in ALS (https://clinicaltrials.gov; NCT01583088). Myofibre type, size and distribution were evaluated by immunofluorescence microscopy and correlated with spirometry, respiratory muscle strength and phrenic nerve conduction parameters. The relationship between these variables and diaphragm atrophy was assessed using multivariate regression models.All patients exhibited significant slow- and fast-twitch diaphragmatic atrophy. Vital capacity (VC), maximal inspiratory pressure, sniff nasal inspiratory pressure (SNIP) and twitch transdiaphragmatic pressure did not correlate with the severity of diaphragm atrophy. Inspiratory capacity (IC) correlated modestly with slow-twitch myofibre atrophy. Supine fall in VC correlated weakly with fast-twitch myofibre atrophy. Multivariate analysis showed that IC, SNIP and functional residual capacity were independent predictors of slow-twitch diaphragmatic atrophy, but not fast-twitch atrophy.Routine respiratory tests are poor predictors of diaphragm structural changes. Improved detection of diaphragm atrophy is essential for clinical practice and for management of trials specifically targeting diaphragm muscle function.

International audience; Obstructive sleep apnoea syndrome (OSAS) patients do not report breathing discomfort in spite of abnormal upper airway mechanics. We studied respiratory sensations in OSAS patients without and with mandibular advancement device (MAD).Fifty-seven moderate to severe non obese OSAS patients were asked about breathing discomfort using visual analogue scales (VAS) in the sitting position (VAS-1), after lying down (VAS-2), then with MAD (VAS-3). Awake critical closing pressure (awake Pcrit) was measured in 15 patients without then with MAD.None of the patients reported breathing discomfort when sitting but 19 patients (33%) did when lying (VAS-2: -20% or less). A feeling of "easier breathing" with MAD was observed and was more marked in patients reporting breathing discomfort when supine (VAS-3: +66.0% [49.0; 89.0]) than in those not doing so (VAS-3: +28.5% [1.0; 56.5], p = 0.007). MAD-induced change in awake Pcrit was correlated to VAS-3.In conclusion, MAD revealed “latent dyspnea” related to the severity of upper airways mechanics abnormalities in OSAS patients.

Pregnancy and delivery are challenging in women affected by Pompe disease with respiratory involvement. We describe a 28-year-old woman, who continued to receive enzyme replacement therapy during pregnancy and had an uneventful vaginal birth. Before pregnancy the patient's vital capacity was 52% in sitting position and 51% in supine position. At 32 weeks gestation her vital capacity in sitting position was 46% and 35% in supine position. Nocturnal non-invasive mechanical ventilation was introduced at this time. Labor was induced at 34 weeks following premature rupture of membranes, under epidural anesthesia. A 2590 g healthy baby was delivered by vacuum extraction. Assisted ventilation was continued throughout labor and post-partum. This observation suggests a successful pregnancy and a normal vaginal delivery can be achieved in patients with symptomatic Pompe Disease, provided multidisciplinary care is offered.

Introduction Alors que la pandemie de COVID-19 reste encore active, l’identification de facteurs pronostiques de la maladie reste un enjeu important. Parmi ceux-ci, le tabagisme a un role qui reste controverse. Nous avons donc voulu verifier si le taux de fumeur chez les patients COVID-19 etait different de celui de la population francaise. Materiels et methodes Nous avons recueilli par entretien de facon systematique chez les patients COVID-19 ambulatoires (du 28/02 au 30/03/2020) et hospitalises (du 23/03 au 09/04/2020) d’un unique centre hospitalo-universitaire, leur consommation actuelle de tabac, de e-cigarette et de substituts nicotiniques. Les taux de fumeurs actifs quotidiens parmi les patients ambulatoires et hospitalises ont ete compares a ceux de la population generale francaise de 2019 apres standardisation pour l’âge et le sexe. Resultats Le groupe des patients hospitalises etait compose de 340 patients, âge median 66 ans : 203 hommes (59,7 %) et 137 femmes (40,3 %), âge median pour les deux sexes 66 ans, avec un taux de fumeurs actifs quotidiens de 4,1 %, IC95 % [2,3–6,9] (5,4 % des hommes, 2,2 % des femmes). Le groupe des patients ambulatoires etait compose de 139 patients, âge median 44 ans : 62 hommes (44,6 %, âge median 43 ans), et 77 femmes (55,4 %, âge median 44 ans). Le taux de fumeurs actifs quotidiens etait de 6,1 % ; IC95 % [2,7–11,6] (5,1 % d’hommes, et 6,8 % de femmes). Dans la population generale francaise de 2019, le taux de fumeurs actifs quotidiens etait de 24,0 % (27,5 % chez les hommes, 20,7 % chez les femmes). Chez les patients hospitalises, les fumeurs actifs quotidiens representaient 2,2 % et 3,4 % des 45 patients decedes et des 29 patients transferes en soins intensifs respectivement. Le taux de fumeurs actifs quotidiens etait significativement plus faible chez les patients COVID-19 que celui de la population generale francaise de 2019 apres standardisation par âge et par sexe, avec un ratio d’incidence standardise calcule a 0,24 [0,12–0,48] pour les patients ambulatoires et de 0,24 [0,14–0,40] pour les patients hospitalises. Conclusion Le taux de fumeurs actifs quotidiens est faible parmi les malades atteints de COVID-19 symptomatique.

International audience; No abstract available

International audience; Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder leading to chronic respiratory failure. Few studies have investigated ALS-related dyspnoea, and none have characterised the emotional distress it inflicts. We hypothesised that ALS-related dyspnoea has a strong affective component that relates to quality of life.Methods: This prospective, observational study was conducted in 41 ALS patients >18 with chronic respiratory failure and an indication for noninvasive ventilation (NIV). Dyspnoea was assessed using the Multidimensional Dyspnea Profile (MDP) at baseline and 1 month after NIV initiation. Correlations between scores evaluating the sensory and affective dimensions of dyspnoea and other patient-reported outcomes and pulmonary function tests were analysed.Results: Dyspnoea was described as intense (median [IQR] score on a 0-10 scale: 6.5 [4.0-7.5]). The sensory dimension of dyspnoea was polymorphic, but «air hunger» was the most common (48.8%) and the most intense (6 [4-8]) sensory descriptor. In the affective domain, most patients rated «anxious» (85.4%) and «afraid» (60.9%) above 0. The MDP affective dimension correlated significantly with other patient-reported outcomes, with the strongest correlation being between MDP «anxious» and the anxiety component of the Hospital Anxiety Depression Scale (Pearson's R = 0.70). One month after initiation of NIV, dyspnoea during unassisted breathing was described in virtually the same terms, particularly the affective dimension.Discussion: ALS-related dyspnoea is intense and fear-provoking, persists during unassisted breathing between NIV sessions, and significantly impacts health-related quality of life. This study highlights the need for increased awareness of and research into ALS-related dyspnoea.

The benefit of inspiratory muscle training (IMT) combined with a pulmonary rehabilitation programme (PRP) is uncertain. We aimed to demonstrate that, in severe and very severe chronic obstructive pulmonary disease (COPD) patients, IMT performed during a PRP is associated with an improvement of dyspnoea.In a single-blind randomised controlled trial, 150 severe or very severe COPD patients were allocated to follow PRP+IMTversusPRP alone. The evaluations were performed at inclusion and after 4 weeks. The primary outcome was the change in dyspnoea using the Multidimensional Dyspnoea Profile questionnaire at the end of a 6-min walk test (6MWT) at 4 weeks. Secondary outcomes were changes in dyspnoea using the Borg (end of the 6MWT) and modified Medical Research Council scales and in functional parameters (maximal inspiratory pressure (PImax), inspiratory capacity, 6MWT and quality of life). All analyses were performed on an intention-to-treat basis.Dyspnoea decreased significantly in both groups; however, the improvement of dyspnoea was not statistically different between the two groups. We only found a statistically significant greater increase ofPImaxafter IMT+PRP than after PRP alone.In this trial including severe or very severe COPD patients, we did not find a significant benefit of IMT during PRP+IMT as compared to PRP alone on dyspnoea, despite a significantly higher improvement ofPImaxin the IMT group.