Physiological Responses of the Modified Shuttle Test in Adults With Cystic Fibrosis

International audienc

Use of the Peak Heart Rate Reached During Six-Minute Walk Test to Predict Individualized Training Intensity in Patients With Cystic Fibrosis: Validity and Reliability

International audienceUse of the peak heart rate reached during six-minute walk test to predict individualized training intensity in patients with cystic fibrosis: validity and reliability

L’endurance des muscles respiratoires dans la mucoviscidose : évaluation et impact clinique

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Deprescribing in older adults in a French community: a questionnaire study on patients’ beliefs and attitudes

International audienceBackground; General practitioners (GPs) have a central role to play on reduction of polypharmacy and deprescribing. This study aimed to assess beliefs and attitudes towards deprescribing in patients, aged 65 years or older in primary care, and to identify factors associated with deprescribing and their willingness to stop medication.Methods: A questionnaire study was performed between 23 May and 29 July 2022 on patients aged 65 years or older attending a GP’s surgery in a French area. We used the French version of the revised Patients’ Attitudes Towards Deprescribing self-report questionnaire (rPATD), which measures four subscales (“Burden”, “Appropriateness”, “Concerns about stopping” and, “Involvement”), patients’ willingness to stop one of their regular medicines, and patients’ satisfaction with their current medicines. Results The study enrolled 200 patients. Median age was 76 years old (IQR 71–81), 55% were women, and 42.5% took 5 or more medications per day. Although most patients (92.5%) were satisfied with their current medicines, 35% were reluctant to stop medications they had been taking for a long time, and 89.5% were willing to stop medication if asked to by their GP. Patients aged less than 75 years old reported more concerns about stopping. Women and patients with higher educational attainment showed significantly higher involvement in medication management. Conclusions The majority of older adults were willing to stop one or more of their regular medicines if asked to do so by their GP. GPs should address deprescribing into their current practice

Clinical practice versus guidelines for the screening of cystic fibrosis-related diabetes: A French survey from the 47 centers

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Unusual Aspergillus species in patients with cystic fibrosis

Poorly sporulating Aspergillus isolates from patients with cystic fibrosis (CF) are generally identified in routine procedures as Aspergillus spp. In this study, we identified and characterized 11 isolates belonging to two unusual Aspergillus species of the section Fumigati (A. lentulus and Neosartorya pseudofischeri) recovered from four different patients. Aspergillus lentulus was found occasionally during a 10-year follow-up study of one CF patient colonized by A. fumigatus. Neosartorya pseudofischeri was isolated from three patients followed in different European hospitals. This species was recovered from two sputum samples of one patient, and from four successive samples of the two other patients, suggesting that it may be responsible for chronic colonization. Both species were isolated together with A. fumigatus. Isolates from both species did not grow at 50°C, and DNA sequence analysis, together with further morphological observations permitted identification at the species level. Growth at different temperatures and antifungal susceptibility were also investigated. All the isolates of N. pseudofischeri exhibited a very low susceptibility to voriconazole (VRZ) whereas a very low susceptibility to VRZ and amphotericin B was seen with the A. lentulus isolates

L’activité physique adaptée chez les patients atteints de mucoviscidose : étude qualitative des barrières et facilitateurs

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Identification of a novel duplication CFTRdup2 and functional impact of large rearrangements identified in the CFTR gene

International audienceIn European populations, large rearrangements contribute to approximately 2% of CF mutations. Here, we reported a novel duplication, the CFTRdup2, identified in a patient heterozygous for Phe508del and suffering from a mild CF. Using a combination of functional tests, we studied the impact of duplication/deletion on CFTR expression. We showed that the copy number variations of exon 2, in addition to abolishing the rate of the mature CFTR protein, affect the CFTR mRNA levels. These data illustrate the importance to perform functional analysis to better understand the molecular basis responsible for cystic fibrosis. Determining the impact of deletions or duplications is relevant for a more comprehensive diagnosis and prognosis of patients

Geosmithia argillacea: an Emerging Pathogen in Patients with Cystic Fibrosis▿

We report eight cases of airway colonization by Geosmithia argillacea in patients with cystic fibrosis. This filamentous fungus, resembling members of the genera Penicillium and Paecilomyces, was identified by molecular analysis. All patients carried a mutation on each CFTR (cystic fibrosis transmembrane conductance regulator) allele, with at least one copy of the F508del mutation. The first isolation of this fungus occurred from F508del-homozygous patients at a younger age than in F508del-heterozygous patients. Before recovery of G. argillacea, all patients were treated with itraconazole; two of them had also received voriconazole for an Aspergillus fumigatus infection. However, antifungal susceptibility patterns showed high MICs of voriconazole for all isolates, and high MICs of amphotericin B and itraconazole for the majority of them, but mostly low minimum effective concentrations (MECs) of caspofungin. The appearance and persistence of G. argillacea in the airways were not associated with exacerbation of the disease. However, the clinical implications of G. argillacea, particularly in immunocompromised patients, remain a concern, particularly given recent observations suggesting that this fungus may also cause disseminated infections