Abstract
Background
Cough and sputum are major symptoms in cystic fibrosis (CF) that contribute to the impairment of quality of life.
Methods
This prospective single centre cross-sectional pilot ...study aimed to evaluate the results of a self-administered questionnaire assessing cough and sputum symptoms (2 domains), and their impact (2 domains) on daily activities in the previous week, named the Cough and Sputum Assessment Questionnaire (CASA-Q) in CF adult patients at stable state, and to analyse associations with clinical, functional, microbiological, radiological data, and two quality of life scales: the Cystic Fibrosis Questionnaire Revised (CFQ-R) and the Saint George Respiratory Questionnaire (SGRQ).
Results
Forty-eight patients were included in this analysis (69% men; median age of 27.8
±
8.1 years; median body mass index of 21.8
+
3.3 kg/m²; mean FEV
1
of 64
±
30% of the predicted value). The mean values of the CASA-Q domains were 58
±
23 for cough symptoms, 77
±
24 for cough impact, 62
±
25 for sputum symptoms and 84
±
21 for sputum impact. Impairment in CASA-Q cough and sputum domains was associated with dyspnea mMRC scale (p < 0.005 for all 4 domains of CASA-Q) and exacerbations in the previous year (p < 0.05 for CASA-Q symptoms domains). We also found correlations between all domains of the CASA-Q and quality of life questionnaires including SGRQ (p < 0.001) and to a lesser extend CFQ-R. We identified a clinical phenotype (female gender, ΔF508 heterozygous mutation, dyspnea mMRC scale) associated with an impairment of CASA-Q score and quality of life using a 2-step cluster analysis.
Conclusions
CASA-Q allows the assessment of cough and sputum in CF adult patients and is associated with quality of life impairment. This simple easy-to-use tool could be used in routine clinical practice and in clinical studies to assess cough and sputum in CF patients.
Trial Registration
The study was registered on ClinicalTrials.gov (NCT02924818, first posted on 5th October 2016).
Adult patients with cystic fibrosis (CF) experience daily physical symptoms and disabilities that can be challenging to address for health care teams.
We sought to identify the most frequent topics ...that CF adults need to discuss with health care teams using a custom questionnaire including 62 items.
Fifty patients were included, 70% men, mean age 27.6 years, with a mean body mass index of 21.8 kg/m
. Mean FEV
% was 64% of predicted value. Forty-two percent of patients selected at least one topic. The most frequently selected topics were fatigue (20%), professional or scholar worries (18%), procreation (16%), physical activities (16%) and evolution of CF disease (16%). Women were more frequently concerned about fatigue, procreation and profession/school.
Using a custom questionnaire, we identified that CF adults express various unmet needs that extend beyond usual respiratory and nutritional concerns or treatment adherence. The interest of this questionnaire by health care team for improving therapeutic management of CF patients remains to be validated.
The study was registered on ClinicalTrials.gov (NCT02924818) on 5th October 2016.
Background As urban areas expand, scientists now agree that the city is an important space for biodiversity conservation. Yet, still relatively little is known about how urban forms could have a ...differential impact on terrestrial species and ecosystems. If some reviews have been conducted to examine the link between biodiversity and urban characteristics at an infra-city scale, none have explored the relationship between urban organization and biodiversity and tried to assess the capacity of various urban forms to maintain and possibly favour flora and fauna in the city. The resulting map will present the state of knowledge regarding possible relationships between urban forms and its features on the establishment and settlement of terrestrial and temperate biodiversity at infra-city scale in western cities. Methods The systematic map will follow the Collaboration for Environmental Evidence (CEE) Guidelines. We will collect the relevant peer-reviewed and grey literature in French and English language. The scientific literature will be retrieved with the use of a search string in two publication databases, one environmental and one social science database (Web Of Science Core Collection, and Cairn.info). We will also perform supplementary searches (search engines, call for literature, search for relevant reviews). All references will be screened for relevance using a three-stage process, according to a predefined set of eligibility criteria. Our study will concentrate on urban areas at the infra-city scale in cities of the temperate biogeographical zone. The subject population will include terrestrial species and ecosystems, except for archaea and bacteria. The exposure will consider all types of urban forms described by any urban descriptors or measures including heterogeneity, fragmentation, housing density, organisation of urban matrix, urban fabric) and all types of urban features (e.g. size, age of the buildings, materials, urban artefacts). All relevant outcomes will be considered (e.g. species richness, abundance, behaviour). We will provide an open-access database of the studies included in the map. Our results will also be presented narratively, together with tables and graphs summarising the key information coded from the retained articles (e.g. study characteristics, types and areas of research that has been undertaken, types of exposure, population concerned, etc.). Keywords: Urban biodiversity, Infra-city scale, Ecological connectivity, Density
Background: Cystic Fibrosis (CF) adult patients experience daily physical symptoms and disabilities that may impact their quality of life and mental health. Methods: This prospective study aimed to ...evaluate the relative contribution of the familial, occupational, and social environment, besides that of the main physical and mental health factors, to the quality of life of CF adult patients using the Cystic Fibrosis Questionnaire-Revised (CFQ-R) in a multivariate model. Results: Fifty patients were analyzed (70% of men; median age of 25 years; median body mass index of 21 kg/m²; median FEV1 of 57%). Anxiety and depression scores were negatively associated with 9 of the 12 CFQ-R domains. When controlling for anxiety and depression, FEV1% and BMI were significant positive predictors of several domains of the CFQ-R. All the familial, occupational, and social components analyzed but one (professional training) were predictors of at least one domain of the CFQ-R. Conclusion: Anxiety and depression explained a greater proportion of the variance than physical variables (age, sex, BMI, FEV1%, and exacerbation in the last year) in CF HRQoL. Many familial, occupational, and social components were also specifically and independently predictors of some HRQoL domains. Their screening might help identifying CF patients eligible for specific interventions, focusing on the impaired QoL dimensions.
With the improvement of cystic fibrosis (CF) patient survival, the prevalence of long-term complications increased, among them rheumatologic disorders.
The aim of this prospective study was to ...evaluate the prevalence of spinal and joint pain, and their impact on disability, anxiety, depression, and quality of life in CF adult patients.
Forty-seven patients were analyzed, 72% of men, mean aged 28 years, with a mean body mass index of 22 kg/m
and a mean FEV
% of 63%. Twenty-two patients (47%) described rheumatologic pain either spinal (
= 15, 32%) and/or joint pain (
= 14, 30%). Patients with spinal and/or joint pain were shorter (
= 0.023), more frequently colonized with
(
< 0.008), had more frequent ΔF508 homozygous mutations (
= 0.014), and a trend for more impairment of the 6-min walking distance (
= 0.050). The presence of rheumatologic pain tended to be associated with disability according to the Health Assessment Questionnaire (HAQ) and anxiety. Compared with patients with no pain patients with both spinal and joint pain exhibited a more pronounced impact on the St George's Respiratory Questionnaire (SGRQ).
Rheumatologic pain is frequent in CF adult patients, and may affect daily living, anxiety and quality of life. Systematic assessment of rheumatologic pain should be included in the management of CF patients.
Construit sur la base de conférences présentées à l’Ifla dans le cadre du groupe d’intérêt spécial « Publics LGBT », cet ouvrage a été conçu par la commission Légothèque qui a traduit les productions ...de nos collègues étrangers, convaincus que la bibliothèque peut être cet « endroit sûr au sein de la culture dominante ». Ces conférences sont présentées sous une forme éditorialisée, soulignant les problématiques professionnelles, présentant des outils pratiques et contextualisant, selon la zone géographique, le métier du bibliothécaire au service de toutes les communautés. Un éclairage inédit en France sur les pratiques des bibliothèques à destination et en faveur des minorités sexuelles et de genre.
Abstract Mutations in dynamin 2 (DNM2), an ubiquitously-expressed large GTPase, cause autosomal dominant centronuclear myopathy (DNM2-CNM) and AD Charcot-Marie-Tooth disease type 2B (DNM2-CMT2B). We ...report a series of 5 patients from the same family who all presented with dominant centronuclear myopathy, mild cognitive impairment, mild axonal peripheral nerve involvement, and the novel E368Q mutation in the DNM2 gene. This study suggests that the phenotypes of dynamin 2 related centronuclear myopathy and Charcot-Marie-Tooth disease overlap and that DNM2 mutations may alter cerebral function. This report extends the clinical knowledge of DNM2-centronuclear myopathy and shows that the role of DNM2 mutations in the central nervous system should be further studied.
To describe neuroimaging findings and to report the epidemiologic and clinical characteristics of patients with coronavirus disease 2019 (COVID-19) with neurologic manifestations.
In this ...retrospective multicenter study (11 hospitals), we included 64 patients with confirmed COVID-19 with neurologic manifestations who underwent a brain MRI.
The cohort included 43 men (67%) and 21 women (33%); their median age was 66 (range 20-92) years. Thirty-six (56%) brain MRIs were considered abnormal, possibly related to severe acute respiratory syndrome coronavirus. Ischemic strokes (27%), leptomeningeal enhancement (17%), and encephalitis (13%) were the most frequent neuroimaging findings. Confusion (53%) was the most common neurologic manifestation, followed by impaired consciousness (39%), presence of clinical signs of corticospinal tract involvement (31%), agitation (31%), and headache (16%). The profile of patients experiencing ischemic stroke was different from that of other patients with abnormal brain imaging: the former less frequently had acute respiratory distress syndrome (
= 0.006) and more frequently had corticospinal tract signs (
= 0.02). Patients with encephalitis were younger (
= 0.007), whereas agitation was more frequent for patients with leptomeningeal enhancement (
= 0.009).
Patients with COVID-19 may develop a wide range of neurologic symptoms, which can be associated with severe and fatal complications such as ischemic stroke or encephalitis. In terms of meningoencephalitis involvement, even if a direct effect of the virus cannot be excluded, the pathophysiology seems to involve an immune or inflammatory process given the presence of signs of inflammation in both CSF and neuroimaging but the lack of virus in CSF.
NCT04368390.
L’assistanat spécialisé à temps partagé (ASTP) est un projet régional de santé piloté par l’Agence régionale de la santé (ARS). Il s’agit d’un partenariat entre un médecin en post-internat, thésé, et ...au moins deux établissements de santé (CH, CHRU ou structure ambulatoire en zone sous-dense). Ses objectifs sont de consolider les effectifs médicaux, soutenir les projets professionnels des jeunes médecins et parfaire leur formation post-internat. Ce dispositif est en expansion ces dernières années dans la Région Grand-Est. La neurologie y figure en 4e place des spécialités les plus représentées, avec 5 postes pourvus sur la campagne 2021/2023 et 12 postes en cours au 1er janvier 2022.
La densité médicale est en flux tendu depuis plusieurs années et la pandémie COVID-19 a porté un coup supplémentaire au sein d’équipes déjà fragilisées. Ce fut notamment le cas du service de neurologie du CH de Mulhouse, suite à l’hospitalisation en réanimation de deux neurologues souffrant du SARS-CoV-2. En juin 2020, on ne comptait plus que 4 neurologues permanents pour assurer la continuité des soins et la gestion de 22 lits d’hospitalisation dont 4 de soins intensifs neurovasculaires, 8 lits d’hospitalisation de semaine et 6 d’hôpital de jour. Le dispositif d’ASTP a enrichi l’effectif d’1 praticien sur plusieurs semestres consécutifs.
Les avantages pour le praticien sont multiples :
– la poursuite de la formation en post-internat, le partage d’expérience d’équipes variées et surspécialisées et la possibilité d’encadrer des internes et des externes ;
– un avantage financier avec la prime d’exercice multi-sites ;
– l’alternance hebdomadaire permet un suivi plus prolongé des patients, ne se limitant plus à une seule période de 6 mois.
Le principal inconvénient est la nécessité de déplacements inter-sites, impliquant un allongement du temps de trajet domicile–travail voire un changement de domicile. De plus, l’activité multi-site complexifie la gestion et le suivi des patients qui se fait simultanément sur plusieurs sites et donc parfois à distance. Enfin, l’ASTP est un contrat limité à 2 ans et ne solutionne pas les tensions d’effectifs sur le long cours.
Pour conclure, l’ASTP est un dispositif bénéfique pour le praticien et les établissements partenaires, n’offrant néanmoins qu’un soutien transitoire des effectifs médicaux.
Severe asthma is characterized by frequent exacerbations, altered lung function, and impaired quality of life. Tailored patient education allows for the improvement of both asthma management and ...quality of life. Our study aimed to assess the needs of severe asthma patient in therapeutic education, according to previous therapeutic patient education background and asthma phenotype.
Consecutive patients monitored for severe asthma in a tertiary referral center were considered for inclusion and answered a questionnaire detailing their patient education needs and the topics they would like to discuss. Asthma history, clinical and biological data, and lung function results were recorded.
Fifty-three patients were included and 47 (88.7%) expressed at least one need. The most frequently selected topics were "life with asthma" (83%), "treatment use" (68%), and "exacerbation management" (60%), independent of previous participation in a patient education program dedicated to asthma. Patients of older age at inclusion, uncontrolled asthma, and T2-high phenotypes were associated with different profiles of patient education needs.
Our study identified frequent and various patient educational needs among severe asthmatics, highlighting the importance of an in-depth assessment of severe asthmatics expectations and the crucial need for the development of dedicated educational tools.
PDF
