La perception des changements environnementaux : le cas de la collectivité côtière de Shippagan (Nouveau-Brunswick, Canada)

In order to support coastal communities in the process of establishing attenuation and adaptation strategies to environmental changes, it is essential to understand how people perceive these changes. The aim of this study is to verify to what extent people’s perception of environmental changes matches quantitative data computed for the area of interest. Semi-directive interviews were conducted with residents of Shippagan (NB, Canada). A focus group was subsequently organized. Six main topics were discussed : climatic changes, sea level changes, erosion, aquatic pollution, biodiversity and drinking water resources. We also searched databases from government agencies and non government organizations to build time series of environmental variables depicting the aforementioned topics. We were able to gather both local knowledge and quantitative data for eleven variables. The temporal tendencies observed by local people were consistent with quantitative data for six variables. In some of the remaining cases, the apparent discrepancy between the quantitative data and local knowledge might have resulted from the different time frames covered by theses two sources. This correspondence between local knowledge and quantitative environmental data could be translated into incentives for taking concrete actions that would ameliorate the communities’ resilience

La perception des changements environnementaux : le cas de la collectivité côtière de Shippagan (Nouveau-Brunswick, Canada)

Dans un contexte d’accompagnement des communautés côtières visant à mettre en place des stratégies d’atténuation et d’adaptation aux changements environnementaux, il nous paraît capital de comprendre la perception de ces changements par les membres de la collectivité. Le but de notre étude est de vérifier dans quelle mesure une description des changements environnementaux recueillie auprès d’une communauté côtière correspond aux données quantitatives compilées pour la région d’intérêt. Nous avons mené des entrevues semi-dirigées et animé un focus group avec des résidents du territoire de Shippagan (NB, Canada). La discussion ciblait six thèmes généraux (changements climatiques, variation du niveau marin, érosion, pollution du milieu marin, biodiversité, ressources en eau potable). En parallèle, nous avons compilé les données environnementales et climatiques pour la zone de Shippagan, en utilisant les archives de divers ministères et d’organismes non gouvernementaux. Nous avons pu obtenir à la fois des données quantifiées et des informations issues du savoir local pour onze variables environnementales ou climatiques. Les tendances temporelles rapportées par le savoir local concordent avec les données quantitatives pour six variables. Certains cas où les séries chronologiques semblent différer du savoir local pourraient découler d’un décalage entre les échelles temporelles des deux sources de données. En termes de gestion, ces recoupements entre savoir local et séries chronologiques quantifiées pourraient se traduire en motifs d’action et favoriser la mobilisation de la communauté pour la prise en charge de la qualité environnementale et la mise en place de stratégies d’adaptation aux changements climatiques.In order to support coastal communities in the process of establishing attenuation and adaptation strategies to environmental changes, it is essential to understand how people perceive these changes. The aim of this study is to verify to what extent people’s perception of environmental changes matches quantitative data computed for the area of interest. Semi-directive interviews were conducted with residents of Shippagan (NB, Canada). A focus group was subsequently organized. Six main topics were discussed : climatic changes, sea level changes, erosion, aquatic pollution, biodiversity and drinking water resources. We also searched databases from government agencies and non government organizations to build time series of environmental variables depicting the aforementioned topics. We were able to gather both local knowledge and quantitative data for eleven variables. The temporal tendencies observed by local people were consistent with quantitative data for six variables. In some of the remaining cases, the apparent discrepancy between the quantitative data and local knowledge might have resulted from the different time frames covered by theses two sources. This correspondence between local knowledge and quantitative environmental data could be translated into incentives for taking concrete actions that would ameliorate the communities’ resilience

La perception des changements environnementaux : le cas de la collectivité côtière de Shippagan (Nouveau-Brunswick, Canada)

In order to support coastal communities in the process of establishing attenuation and adaptation strategies to environmental changes, it is essential to understand how people perceive these changes. The aim of this study is to verify to what extent people’s perception of environmental changes matches quantitative data computed for the area of interest. Semi-directive interviews were conducted with residents of Shippagan (NB, Canada). A focus group was subsequently organized. Six main topics were discussed : climatic changes, sea level changes, erosion, aquatic pollution, biodiversity and drinking water resources. We also searched databases from government agencies and non government organizations to build time series of environmental variables depicting the aforementioned topics. We were able to gather both local knowledge and quantitative data for eleven variables. The temporal tendencies observed by local people were consistent with quantitative data for six variables. In some of the remaining cases, the apparent discrepancy between the quantitative data and local knowledge might have resulted from the different time frames covered by theses two sources. This correspondence between local knowledge and quantitative environmental data could be translated into incentives for taking concrete actions that would ameliorate the communities’ resilience

Severe hematologic complications after lung transplantation in patients with telomerase complex mutations

International audienceBACKGROUND: Mutations in the telomerase complex (TERT and TR) are associated with pulmonary fibrosis and frequent hematologic manifestations. The aim of this study was to characterize the prognosis of lung transplantation in patients with TERT or TR mutations. METHODS: Patients with documented TERT or TR mutations who received a lung transplant between 2007 and 2013 in France were identified via an exhaustive search of the lung transplantation network, one expert genetic laboratory, and the clinical research network on rare pulmonary diseases. RESULTS: There were 9 patients (7 men) with TERT (n = 6) or TR (n = 3) mutations who received a single (n = 8) or a double (n = 1) lung transplant for pulmonary fibrosis. Median age was 50 years (range, 35-61 years) at diagnosis and 52 years (range, 37-62 years) at the time of lung transplantation. Thrombocytopenia was present in 7 patients before lung transplantation. After lung transplantation, 6 patients developed myelodysplasia and/or bone marrow failure, directly contributing to death in 4 cases. Anemia was observed in 9 patients, and neutropenia was observed in 3 patients. The median survival after lung transplantation was 214 days (range, 59-1,709 days). CONCLUSIONS: Patients with mutations of the telomerase complex are at high risk of severe hematologic complications after lung transplantation, in particular, bone marrow failure. Specific recommendations should be developed for appropriate guidance regarding hematologic risk assessment before transplantation and management of the post-transplantation immunosuppressive regimen

Pilot experience of multidisciplinary team discussion dedicated to inherited pulmonary fibrosis

International audienceBackground Genetic testing is proposed for suspected cases of monogenic pulmonary fibrosis, but clinicians and patients need specific information and recommendation about the related diagnosis and management issues. Because multidisciplinary discussion (MDD) has been shown to improve accuracy of interstitial lung disease (ILD) diagnosis, we evaluated the feasibility of a genetic MDD (geneMDD) dedicated to the indication for and interpretation of genetic testing. The geneMDD group met monthly and included pediatric and adult lung specialists with ILD expertise, molecular and clinical geneticists, and one radiologist. Hematologists, rheumatologists, dermatologists, hepatologists, and pathologists were also invited to attend. Results Since 2016, physicians from 34 different centers in 7 countries have participated in the geneMDD. The medical files of 95 patients (53 males) have been discussed. The median age of patients was 43 years [range 0–77], 10 were ≤ 15 years old, and 6 were deceased at the time of the discussion. Among 85 analyses available, the geneMDD considered the rare gene variants pathogenic for 61: 37 variants in telomere-related genes, 23 variants in surfactant-related genes and 1 variant in MARS . Genetic counseling was offered for relatives of these patients. The geneMDD therapeutic proposals were as follows: antifibrotic drugs ( n = 25), steroids or immunomodulatory therapy ( n = 18), organ transplantation ( n = 21), watch and wait ( n = 21), or best supportive care ( n = 4). Conclusion Our experience shows that a dedicated geneMDD is feasible regardless of a patient’s age and provides a unique opportunity to adapt patient management and therapy in this very rare condition

Rituximab for auto-immune alveolar proteinosis, a real life cohort study

International audienceBackground: Whole lung lavage is the current standard therapy for pulmonary alveolar proteinosis (PAP) that is characterized by the alveolar accumulation of surfactant. Rituximab showed promising results in auto-immune PAP (aPAP) related to anti-GM-CSF antibody.Methods: We aimed to assess efficacy of rituximab in aPAP in real life and all patients with aPAP in France that received rituximab were retrospectively analyzed.Results: Thirteen patients were included. No patients showed improvement 6 months after treatment, but, 4 patients (30%) presented a significant decrease of alveolar-arterial difference in oxygen after 1 year. One patient received lung transplantation and one patient was lost of follow-up within one year. Although a spontaneous improvement cannot be excluded in these 4 patients, improvement was more frequent in patients naive to prior specific therapy and with higher level of anti-GM-CSF antibodies evaluated by ELISA. No serious adverse event was evidenced. Conclusions: These data do not support rituximab as a second line therapy for patients with refractory aPAP

Relevance of partitioning DLCO to detect pulmonary hypertension in systemic sclerosis.

We investigated whether partitioning DLCO into membrane conductance for CO (DmCO) and pulmonary capillary blood volume (Vcap) was helpful in suspecting precapillary pulmonary (arterial) hypertension (P(A)H) in systemic sclerosis (SSc) patients with or without interstitial lung disease (ILD). We included 63 SSc patients with isolated PAH (n=6), isolated ILD (n=19), association of both (n=12) or without PAH and ILD (n=26). Partitioning of DLCO was performed by the combined DLNO/DLCO method. DLCO, DmCO and Vcap were equally reduced in patients with isolated PAH and patients with isolated ILD but Vcap/alveolar volume (VA) ratio was significantly lower in the isolated PAH group. In patients without ILD, DLCO, DmCO, Vcap and Vcap/VA ratio were reduced in patients with isolated PAH when compared to patients without PAH and both Vcap/VA and DLCO had the highest AUC to detect PAH. In patients with ILD, Vcap had the highest AUC and performed better than DLCO to detect PH in this subgroup. In conclusion, Vcap/VA was lower in PAH than in ILD in SSC whereas DLCO was not different. Vcap/VA ratio and DLCO had similar high performance to detect PAH in patients without ILD. Vcap had better AUC than DLCO, DmCO and FVC/DLCO ratio to detect PH in SSC patients with ILD. These results suggest that partitioning of DLCO might be of interest to detect P(A)H in SSC patients with or without ILD

Impact of Gender on the Characteristics of Patients with Idiopathic Pulmonary Fibrosis Included in the RaDiCo-ILD Cohort

International audienceBackground: There is growing evidence of gender-specific phenotypic differences among patients with idiopathic pulmonary fibrosis (IPF), which may affect patient outcomes. Objectives: We present the characteristics of patients with IPF at inclusion in the French Rare Disease Cohort - Interstitial Lung Disease (RaDiCo-ILD) with the aim of characterizing gender-specific phenotypic differences. Methods: Patients with IPF who were enrolled in the national, multicentre RaDiCo-ILD cohort were included. Demographic characteristics, comorbidities, health-related quality of life (HRQoL) scores, pulmonary function, chest imaging, and IPF treatment were collected at inclusion and described by gender. Results: The cohort included 724 patients with IPF (54% of RaDiCo-ILD cohort), of whom 82.9% were male. The proportion of male and female patients with a prior history of smoking was 75.0% and 26.8%, respectively. Emphysema was present in 17.0% (95% confidence interval [CI]: 10.0, 24.0) of men and 5.4% (95% CI: 1.2, 9.6) of women. At inclusion, females had poorer HRQoL than males based on St. George's Respiratory Questionnaire scores (48.5 [95% CI: 43.9, 53.0] and 41.5 [39.4, 43.6], respectively). The mean forced vital capacity per cent predicted was 77.7% (95% CI: 76.2, 79.3) and 87.4% (83.4, 91.4) for males and females, respectively. Honeycombing on high-resolution computed tomography (HRCT) was present in 70.8% (95% CI: 61.0, 80.6) of males and 45.8% (95% CI: 35.1, 56.5) of females. Conclusions: This analysis of patients with IPF at inclusion in the RaDiCo-ILD cohort provides evidence that comorbid emphysema, lung volume reduction, and honeycombing on HRCT are more common characteristics of males than females