Man with cystic fibrosis becomes champion BODYBUILDER

James Boudreau, 29, from Michigan, U.S, has a lung capacity of just 48 percent and sleeps with an oxygen mask at night to prevent potentially fatal infections.
Source: the Mail online | Health - Category: Consumer Health News Source Type: news

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Conclusions: The pattern of airway colonisation in the Indian setting is different from the Caucasian population, and P. aeruginosa and Burkholderia cepacia complex appear early. Colonisation with P. aeruginosa benefits from therapy. In case of infection, care must be taken while initiating empiric therapy. It should be based on local antibiograms to prevent the emergence of resistant microbes.
Source: Indian Journal of Medical Microbiology - Category: Microbiology Authors: Source Type: research
Condition:   Cystic Fibrosis Interventions:   Dietary Supplement: Resveratrol;   Dietary Supplement: NR;   Dietary Supplement: Placebo Sponsor:   Virginia Commonwealth University Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
AbstractPurposeCystic fibrosis (CF) is a multisystem genetic disease caused by dysfunction of the epithelial anionic channel Cystic Fibrosis Transmembrane conductance Regulator (CFTR). Decreased mucociliary clearance because of thickened mucus is part of the pulmonary disease pathophysiology. It is controversial if the thickened  airway surface liquid (ASL) is caused by the deficient chloride secretion and excessive sodium (through ENaC) and water hyperabsorption from the periciliar fluid or by the lack of bicarbonate secretion with relative acidification of the ASL. Correlations between the magnitude of in vivo chlor...
Source: Lung - Category: Respiratory Medicine Source Type: research
Publication date: Available online 15 November 2019Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Claudio D'Amore, Christian Borgo, Valentina Bosello-Travain, Jordi Vilardell, Valentina Salizzato, Lorenzo A. Pinna, Andrea Venerando, Mauro SalviAbstractF508del-CFTR, the most common mutation in cystic fibrosis (CF) patients, impairs CFTR trafficking to plasma membrane leading to its premature proteasomal degradation. Several post-translational modifications have been identified on CFTR with multiple roles in stability, localization and channel function, and the possibility to control the e...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - Category: Molecular Biology Source Type: research
Cystic fibrosis (CF) results from deficient CF transmembrane conductance regulator (CFTR) protein activity leading to defective epithelial ion transport. Pulmonary degradation due to excessive inflammation is the main cause of morbidity and mortality in CF patients. By analysing miRNAs (small RNAseq) in human primary air-liquid interface cell cultures, we measured the overexpression of miR-636 in CF patients compared to non-CF controls. We validated these results in explant biopsies and determined that the mechanism underlying miR-636 overexpression is linked to inflammation. To identify specific targets, we used bioinform...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Cystic fibrosis (CF) is characterised by a failure of mucociliary clearance and accumulation of viscid secretions in the airway, leading to recurrent infection. To mitigate this process, CF treatment guidelines recommend the regular use of an airway clearance technique (ACT), with exercise as an adjunct [1]. In people with CF (pwCF), exercise such as running or cycling, increases ease of sputum expectoration, minute ventilation and respiratory flow [2]. A comparison of one ACT with treadmill exercise showed that these approaches performed similarly over a single session [3].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Short Communication Source Type: research
Abstract Pathogenic variants in the Cystic Fibrosis Transmembrane Conductance Regulator gene (CFTR) are responsible for cystic fibrosis (CF), the commonest monogenic autosomal recessive disease, and CFTR-related disorders in infants and youth. Diagnosis of such diseases relies on clinical, functional, and molecular studies. To date, over 2,000 variants have been described on CFTR (~40% missense). Since few of them have confirmed pathogenicity, in silico analysis could help molecular diagnosis and genetic counseling. Here, the pathogenicity of 779 CFTR missense variants was predicted by consensus predictor PredictSNP and co...
Source: Genetics and Molecular Biology - Category: Genetics & Stem Cells Source Type: research
A new treatment for lung disease in cystic fibrosis (CF) for which there remains no cure could potentially benefit all patients, according to a University of Bristol study published in Chemical Science. The findings are an important step towards a new therapy addressing the fundamental cause of cystic fibrosis.
Source: University of Bristol news - Category: Universities & Medical Training Tags: Health, Research; Faculty of Life Sciences, Faculty of Life Sciences, School of Physiology, Pharmacology and Neuroscience, Faculty of Science, Faculty of Science, School of Chemistry; Press Release Source Type: news
The James Lind Alliance Priority Setting Partnership (JLA PSP) in cystic fibrosis (CF) used a robust methodology to develop the top 10 clinical research questions, through discussions with the clinical and patient community [1]. A number of the top 10 questions are complex and need to be explored further before they can be transformed into testable hypotheses for clinical study. An example is the first question: “What are effective ways of simplifying the treatment burden of people with CF”. The recent US Cystic Fibrosis Foundation Insight CF survey ranked ‘Making it easier to do daily treatments’ i...
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Short Communication Source Type: research
FIS of intestinal organoids varied between subjects with homozygous F508del and correlated with pulmonary and nutritional parameters. These findings suggest that differences at low CFTR residual function may contribute to clinical heterogeneity in F508del homozygous patients and small changes in CFTR residual function might impact long-term disease expression.
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Original Article Source Type: research
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