Background: The aim of this study was to assess the bone mineral density of cystic fibrosis patients.
Patients And Methods: We have retrospectively studied 45 patients with cystic fibrosis, 22 females and 23 males, aged between six and 27 years (mean 13 +/- 5.5 years). Nutritional status, Shwachman score, pulmonary function, serum calcium and 25-hydroxyvitamin D levels and lumbar bone mineral density by dual energy X-ray absorptiometry were assessed.
Results: The average height of the patients was 97 +/- 4% (range: 86-106) and body mass index 93 +/- 10% (range 77-114) of the normal values of age. The average Shwachman score was 80 +/- 11/100 (range: 47-96). The forced expiratory volume in 1s was 80 +/- 29% (21-128) and the vital capacity 85 +/- 21% (range: 40-122) of the predicted values. The serum levels of calcium and 25-hydroxyvitamin D were lower than normal values in six cases and one case respectively. Thirty-eight patients had a mean lumbar spine bone mineral Z-score which was -1.35 +/- 1.2 DS and 84.7 +/- 13.5% of the normal values. It was correlated with the height, the body mass index, the Shwachman score, the FEV 1 and the vital capacity.
Conclusion: Decreased bone density is frequent among cystic fibrosis subjects. It shows a lack of control of the illness. It remains unknown with usual investigation; the dual X-ray absorptiometry should, each time it is possible, take part of investigations in those patients.
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http://dx.doi.org/10.1016/s0929-693x(99)80353-4 | DOI Listing |
ACS Nano
January 2025
Centre de recherche du Centre hospitalier de l'Université de Montréal (CRCHUM), Montréal, Québec H2X 0A9, Canada.
The abnormally viscous and thick mucus is a hallmark of cystic fibrosis (CF). How the mutated CF gene causes abnormal mucus remains an unanswered question of paramount interest. Mucus is produced by the hydration of gel-forming mucin macromolecules that are stored in intracellular granules prior to release.
View Article and Find Full Text PDFHum Mol Genet
January 2025
Laboratory Medicine and Pathology, University of Minnesota, 420 Delaware Street SE, Minneapolis, MN, 55455, USA.
Background: Individuals with cystic fibrosis (CF; a recessive disorder) have an increased risk of colorectal cancer (CRC). Evidence suggests individuals with a single CFTR variant may also have increased CRC risk.
Methods: Using population-based studies (GECCO, CORECT, CCFR, and ARIC; 53 785 CRC cases and 58 010 controls), we tested for an association between the most common CFTR variant (Phe508del) and CRC risk.
Adv Exp Med Biol
January 2025
Department of Clinical Microbiology, Rigshospitalet, Copenhagen, Denmark.
Despite advances in healthcare, bacterial pathogens remain a severe global health threat, exacerbated by rising antibiotic resistance. Lower respiratory tract infections, with their high death toll, are of particular concern. Accurately replicating host-pathogen interactions in laboratory models is crucial for understanding these diseases and evaluating new therapies.
View Article and Find Full Text PDFClinical trials demonstrate the short-term efficacy of dual CFTR modulators, but long-term real-world data is limited. We aimed to investigate the effects of 24-month lumacaftor/ivacaftor (LUM/IVA) therapy in pediatric CF patients (pwCF). This observational study included pwCF homozygous for F508del mutation treated between 2021 and 2023.
View Article and Find Full Text PDFSci Rep
January 2025
Hubrecht Institute-KNAW and University Medical Center Utrecht, Utrecht, The Netherlands.
Pseudomonas aeruginosa is a Gram-negative bacterium that is notorious for airway infections in cystic fibrosis (CF) subjects. Bacterial quorum sensing (QS) coordinates virulence factor expression and biofilm formation at population level. Better understanding of QS in the bacterium-host interaction is required.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!