With the increasing use of highly effective modulator therapy (HEMT) in adults with cystic fibrosis (awCF), it is necessary to determine the evolution of the most dynamic physiological markers of this disease, such as the 6-minute walk test (6MWT) and the Glittre-activities of daily living test (TGlittre). The present study aimed to evaluate the 1-year changes in the 6- minute walking distance (6MWD), TGlittre time, and quality of life (QoL) in awCF before the initiation of HEMT and to determine the impact of habitual physical activity (HPA) and chest physiotherapy (CP). This longitudinal study enrolled 24 awCF who completed the 6MWT and TGlittre. Pulmonary function tests, handgrip strength (HGS), and the Cystic Fibrosis Questionnaire-Revised (CFQ-R) were conducted. Measurements were collected at baseline (T1) and 1 year later (T2). The median body mass index increased between T1 and T2 [19.8 (18-24) vs. 21.4 (19-24) kg/m2, p=0.038]. TGlittre time decreased both in relation to the absolute values [3.10 (2.52-3.39) vs. 2.40 (2.00-3.00) minutes, p=0.001] and in relation to the predicted values [127 (116-150) vs. 108 (102-140) % predicted, p=0.001]. Although there was no increase in 6MWD relative to the predicted values, it increased relative to the absolute values [545 (463-654) vs. 617 (540-658) meters, p=0.041]. In relation to the group that did not engage in HPA, individuals who had HPA showed an increase in HGS between T1 and T2 [7.1 (0-20) vs. 0 (-12-3) kgf, p=0.031]. In relation to the group that did not undergo CP, individuals undergoing CP showed an increase in the 'treatment burden'-CFQ-R between T1 and T2 [16.1 (-3-18) vs. -11.2 (-28-1) points, p=0.049]. In conclusion, awCF performed better on TGlittre than on 6MWT. They experienced an improvement in body composition. HPA was correlated with peripheral muscle strength, as were CP and QoL.
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http://dx.doi.org/10.4081/monaldi.2025.3068 | DOI Listing |
PLoS One
January 2025
INSERM U1151, Université Paris Cité, Centre de Références Maladies Rares Mucoviscidose et Maladies Apparentées, Hôpital Necker Enfants Malades, Paris, France.
The Cystic Fibrosis (CF) Impact Questionnaire (CF-IQ) was qualitatively developed to assess the impact of CF in the context of treatment advancements and increased longevity. This study reports the CF-IQ validation. In this noninterventional validation study, people with CF completed the 40-item CF-IQ and validating patient-reported outcome measures (PROMs) via electronic diaries at enrollment (baseline) and at the 4-week follow-up.
View Article and Find Full Text PDFIndian J Pediatr
January 2025
Pediatric Respiratory Unit, Department of Pediatrics, Christian Medical College, Vellore, Tamilnadu, 632004, India.
Mol Biol Rep
January 2025
Centre for Applied Molecular Biology, University of the Punjab, Lahore, Pakistan.
Background: Pathogenic mutations in the CFTR gene disrupt the normal function of the chloride ion channel CFTR protein, resulting in Cystic Fibrosis (C.F.).
View Article and Find Full Text PDFMicrob Genom
January 2025
Department of Pediatrics, Division of Pulmonary, Asthma, Cystic Fibrosis, and Sleep, Emory University School of Medicine, Atlanta, Georgia, USA.
Bacteria from the complex (Smc) are important multidrug-resistant pathogens that cause a broad range of infections. Smc is genomically diverse and has been classified into 23 lineages. Lineage Sm6 is the most common among sequenced strains, but it is unclear why this lineage has evolved to be dominant.
View Article and Find Full Text PDFBMC Health Serv Res
January 2025
Department of Planning and Development, Region Västra Götaland, NU Hospital Group, Trollhättan, Sweden.
Background: Remote patient monitoring implies continuous follow-up of health-related parameters of patients outside healthcare facilities. Patients share health-related data with their healthcare unit and obtain feedback (which may be automatically generated if data are within a predefined range). The goals of remote patient monitoring are improvements for patients and reduced healthcare costs.
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