In cystic fibrosis the potential difference between interstitial fluid and the surface of respiratory and sweat duct epithelia is abnormally large. This study attempted to confirm this finding and investigate its value for a diagnostic test. With a subcutaneous reference electrode the mean potential difference of the inferior turbinate was -25 mv in 19 patients with cystic fibrosis, which was significantly greater than the difference of -13 mv in 22 normal subjects (p less than 0.02). Of the observations in the patients with cystic fibrosis, 67% were greater than the largest potential difference observed in normal individuals. Potential differences from the lip and forearm were also significantly greater in cystic fibrosis than in normal subjects, but there was considerable overlap. Changes in the electrical properties of nasal epithelium and skin have been confirmed in cystic fibrosis, but these have limited use for diagnosis.
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http://dx.doi.org/10.1136/thx.40.7.493 | 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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