Long-term gas exchange characteristics as marker of deterioration in patients with cystic fibrosis
Background and Aim. In 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.
Methods: Serial 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 164 children (83 males; 81 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.
Results: PaO2 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.
Conclusions: In the long-term evaluation of gas exchange characteristics, associations to different lung function patterns are found, closely related to specific genotypes.
Early exploration of blood gases may disclose a certain hypocarbia, presumably serving as compensatory mechanisms for better oxygenation.
Author: Richard KraemerPhilipp LatzinIsabelle PramanaPietro BallinariSabina GallatiUrs Frey
Credits/Source: Respiratory Research 2009, 10:106
Published on: 2009-11-12
Copyright by the authors listed above - made available via BioMedCentral (Open Access). Please
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