Rationale: Although peak aerobic capacity (VO(2peak)) has been linked to outcome in patients with cystic fibrosis (CF), measuring is time consuming, and requires expensive equipment and expertise that is not readily available in all centers. Other fitness parameters such as peak anaerobic power, measures of power and strength may be simpler to deliver in the clinic. The relationship between these measures and established outcomes such as forced expiratory volume in one second (FEV(1)) and peak aerobic power (VO(2peak)) in CF remains unclear. Therefore we evaluated (a) aerobic fitness, (b) anaerobic fitness, and (c) upper and lower body muscle strength to determine their relationship to FEV(1) and VO(2peak) in children with CF.
Methods: Eighty-two patients (7-18 years) with CF (40 female) from the CF clinic at The Hospital for Sick Children in Toronto performed a maximal incremental cycling test to exhaustion. Anaerobic power (W) for 10 and 30 sec cycling trials as well as vertical jump (VJ) and hand grip strength (HG) were compared to FEV(1) and VO(2peak).
Results: Absolute VO(2peak) (R(2) = 0.16, P < 0.001), anaerobic power (R(2) = 0.21, P < 0.001), and hand grip strength (R(2) = 0.10, P = 0.003) were significantly correlated to lung function whereas measures of explosive lower body strength (VJ) were not. Anaerobic power (R(2) = 0.16, P = 0.001) and hand grip strength (R(2) = 0.08, P = 0.01) were related to VO(2peak). Vertical jump was correlated with VO(2peak) (R(2) = 0.29, P < 0.001) but not FEV(1).
Conclusions: Simple fitness tests such as hand grip strength and anaerobic cycle tests may be useful indicators of lung health and fitness.
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http://dx.doi.org/10.1002/ppul.22928 | DOI Listing |
J Cyst Fibros
January 2025
Medical University of Innsbruck, Austria, Medical Research Affiliate, Austria.
Pancreatic insufficiency is a major complication of cystic fibrosis (CF), which traditionally has been managed with pancreatic enzyme replacement therapy in the vast majority of CF patients, even in the era of highly effective cystic fibrosis transmembrane conductance regulator modulator (CFTRm) therapy. We report on a 1.7 year old male infant with CF who was exposed to ETI both in utero and postpartum, via breast milk and oral granules.
View Article and Find Full Text PDFJ Cyst Fibros
January 2025
Department of Pediatrics, University of Pittsburgh School Of Medicine, USA.
Background: Males with cystic fibrosis (MwCF) face general and disease-specific sexual and reproductive health (SRH) concerns. Using concept mapping (CM), this study identified the SRH topics valued by members of the CF community.
Methods: MwCF 18 years and older, parents and partners of MwCF, and healthcare providers participated in an online CM study.
Int J Pediatr Otorhinolaryngol
January 2025
Department of Otorhinolaryngology and Head and Neck Surgery, Antwerp University Hospital, Edegem, Belgium; Department of Translational Neuroscience, Faculty of Medicine and Health Science, University of Antwerp, Antwerp, Belgium. Electronic address:
Purpose: Cystic fibrosis (CF) is the most common autosomal recessive disorder in the Caucasian population. Otolaryngological manifestations pose a significant impact on the quality of life of children with CF. The primary aim of this review is to provide a state of the art update of current literature on the otolaryngological manifestations of CF in children.
View Article and Find Full Text PDFRedox Biol
January 2025
Department of Pediatrics, Division of Neonatology, Loma Linda University School of Medicine, Loma Linda, CA, USA; Lawrence D. Longo, MD Center for Perinatal Biology, Loma Linda University School of Medicine, Loma Linda, CA, USA. Electronic address:
Repeated use of nitroglycerin results in a loss of its vasodilatory efficacy which limits its clinical use for the treatment of angina pectoris. This tolerance phenomenon is a defining characteristic of all compounds classified as nitrodilators, which includes NTG as well as S-nitrosothiols and dinitrosyl iron complexes. These compounds vasodilate via activation of soluble guanylate cyclase, although they do not release requisite amounts of free nitric oxide (NO) and some do not even cross the plasma membrane.
View Article and Find Full Text PDFJ Bras Pneumol
January 2025
. Programa de Pós-Graduação em Ciências Pneumológicas, Universidade Federal do Rio Grande do Sul - UFRGS - Porto Alegre (RS) Brasil.
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