ArticleRespiratory research2009
Long-term gas exchange characteristics as markers of deterioration in patients with cystic fibrosis.
Article in Respiratory research, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed.
- Arterialized oxygen tension and unfavorable clinical outcomes in pediatric cystic fibrosis.Frontiers in pediatrics · 2025Article
- Assessment of functional diversities in patients with Asthma, COPD, Asthma-COPD overlap, and Cystic Fibrosis (CF).PloS one · 2024Article
- Nocturnal non invasive ventilation in normocapnic cystic fibrosis patients: a pilot study.Acta bio-medica : Atenei Parmensis · 2021Article
- Diagnostic accuracy of methacholine challenge tests assessing airway hyperreactivity in asthmatic patients - a multifunctional approach.Respiratory research · 2016Article
- Naja naja atra venom ameliorates pulmonary fibrosis by inhibiting inflammatory response and oxidative stress.BMC complementary and alternative medicine · 2014Article
- Disease-modifying genes and monogenic disorders: experience in cystic fibrosis.The application of clinical genetics · 2014Review
- Identification of SNPs in the cystic fibrosis interactome influencing pulmonary progression in cystic fibrosis.European journal of human genetics : EJHG · 2013Article
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6 authors.
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Abstract
BACKGROUND AND
aimIn patients with cystic fibrosis (CF) the architecture of the developing lungs and the ventilation of lung units are progressively affected, influencing intrapulmonary gas mixing and gas exchange. We examined the long-term course of blood gas measurements in relation to characteristics of lung function and the influence of different CFTR genotype upon this process.
methodsSerial annual measurements of PaO2 and PaCO2 assessed in relation to lung function, providing functional residual capacity (FRCpleth), lung clearance index (LCI), trapped gas (VTG), airway resistance (sReff), and forced expiratory indices (FEV1, FEF50), were collected in 178 children (88 males; 90 females) with CF, over an age range of 5 to 18 years. Linear mixed model analysis and binary logistic regression analysis were used to define predominant lung function parameters influencing oxygenation and carbon dioxide elimination.
resultsPaO2 decreased linearly from age 5 to 18 years, and was mainly associated with FRCpleth, (p < 0.0001), FEV1 (p < 0.001), FEF50 (p < 0.002), and LCI (p < 0.002), indicating that oxygenation was associated with the degree of pulmonary hyperinflation, ventilation inhomogeneities and impeded airway function. PaCO2 showed a transitory phase of low PaCO2 values, mainly during the age range of 5 to 12 years. Both PaO2 and PaCO2 presented with different progression slopes within specific CFTR genotypes.
conclusionIn the long-term evaluation of gas exchange characteristics, an association with different lung function patterns was found and was closely related to specific genotypes. Early examination of blood gases may reveal hypocarbia, presumably reflecting compensatory mechanisms to improve oxygenation.
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