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Background: The cystic fibrosis (CF) sweat gland is defective in β-adrenergically-stimulated sweat secretion in the coil and chloride reabsorption in the duct. Whereas chloride reabsorption is regularly assessed by quantitative pilocarpine iontophoresis (QPIT), the measurement of β-adrenergic sweat secretion is not yet established in clinical practice.
Methods: A novel sweat bubble imaging protocol was developed that determines sweat secretion rates by automatic recording, processing and quality control of the kinetics of sweat droplet formation.
Results: Treatment of CF patients with the CFTR modulators elexacaftor, tezacaftor and ivacaftor reduced the sweat chloride concentration measured in QPIT in the majority of patients to values in the intermediate or normal range. In contrast, the β-adrenergically-stimulated sweat secretion rate assayed by the automated bubble sweat test was normalized in only 3 patients, slightly increased in 12 patients and remained undetectable in 8 patients.
Conclusions: β-adrenergic sweat stimulation in the coil is apparently rather stringent in its requirements for a wild type CFTR conformation whereas chloride reabsorption in the duct tolerates residual structural and functional deficits of native or pharmacologically rescued mutant CFTR in the apical membrane.
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http://dx.doi.org/10.1016/j.jcf.2021.10.005 | DOI Listing |
Orphanet J Rare Dis
December 2024
Ion Channels and Channelopathies Laboratory, Institute for Biochemistry and Molecular Medicine, University of Bern, Bühlstrasse 28, Bern, CH-3012, Switzerland.
Background: Cystic Fibrosis is caused by recessively inherited variants of the cystic fibrosis transmembrane regulator. It is associated with diverse clinical presentations that can affect the respiratory, digestive, and reproductive systems and inhibit nutrient absorption and growth.
Main Body: The current estimation of people affected by Cystic Fibrosis is likely underestimated as this disease remains undiagnosed in countries with limited diagnostic capacity.
Talanta
December 2024
Key Laboratory for Biorheological Science and Technology of Ministry of Education, Bioengineering College of Chongqing University, Chongqing, 400044, PR China; Liquor Making Biology Technology and Application of Key Laboratory of Sichuan Province, College of Bioengineering, Sichuan University of Science and Engineering, 188 University Town, Yibin, 644000, PR China. Electronic address:
Simultaneous detection of biomarkers in sweat is crucial for comprehensive health assessment and personalized monitoring. However, the low sweat secretion rate and low metabolite concentrations present challenges for developing non-invasive wearable sensors. This study aims to develop a flexible wearable biosensor for simultaneous detection of low-concentration biomarkers in sweat, enabling comprehensive health assessment.
View Article and Find Full Text PDFAnal Chem
December 2024
School of Science, Harbin Institute of Technology, Shenzhen 518055, China.
Oxygen saturation is a crucial indicator in the management of various diseases and in preoperative diagnosis, and the detection of oxygen content is valuable in guiding clinical treatment. However, as the classical and dominant oxygen detection strategies, current photoelectric oximeters and electrochemical-based blood gas analyzers often suffer from significant interindividual variation and poor compliance, respectively. In recent years, wearable microneedles (MNs) for analyzing biomarkers in interstitial fluid (ISF) have received great attention and recognition mainly for the reason that the content of the substances distributed in ISF has a better correlation with that in blood circulation compared with other body fluids such as sweat and saliva.
View Article and Find Full Text PDFClin Cosmet Investig Dermatol
December 2024
Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Eruptive syringoma, a rare subtype of syringoma, is a benign adnexal tumor arising from the eccrine sweat glands. It is characterized by multiple asymptomatic, flat-topped, brown-pigmented papules. The disease typically occurs during adolescence and young adulthood.
View Article and Find Full Text PDFInt J Mol Sci
November 2024
Institute of Experimental Physics, Faculty of Mathematics, Physics and Informatics, University of Gdansk, ul. Wita Stwosza 57, 80-308 Gdańsk, Poland.
The estimation of the time of death represents a highly complex and challenging task within the field of forensic medicine and science. It is essential to approach this matter with the utmost respect for human rights while acknowledging the inherent limitations of the current methods, which require continuous refinement and expansion. Forensic science recognizes the necessity to improve existing techniques and develop new, more accurate, and non-invasive procedures, such as physicochemical approaches, to enhance the precision and reliability of time of death determinations.
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