Long-term gas exchange characteristics as markers of deterioration in patients with cystic fibrosis

Respiratory Research
Richard KraemerUrs Frey

Abstract

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. 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 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. 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 ...Continue Reading

References

Dec 1, 1975·Journal of Chronic Diseases·W J WarwickC J Nesbitt
Jan 1, 1992·Statistical Methods in Medical Research·N M LairdJ H Ware
Jan 1, 1990·Respiration; International Review of Thoracic Diseases·Richard Kraemer, M H Schöni
Jan 1, 1989·Pediatric Pulmonology·J A HirschG J Cropp
Aug 1, 1986·Respiration Physiology·Richard KraemerB Meister
Jun 1, 1985·Chest·D C StokesD J Strieder
Oct 1, 1985·Journal of Applied Physiology·Richard Kraemer, B Meister
Jan 1, 1971·British Journal of Diseases of the Chest·S GodfreyC S Samuels
Sep 14, 1968·British Medical Journal·J MacIntyreG Smith
Nov 1, 1995·American Journal of Respiratory and Critical Care Medicine·K B DominoM P Hlastala
Apr 1, 1994·Thorax·A D PitkinJ A Wedzicha
Dec 1, 1995·Archives of Disease in Childhood·C S Beardsmore
Jan 15, 1998·The Journal of Pediatrics·M CoreyM Knowles
May 15, 1998·The Journal of Pediatrics·B J Rosenstein, G R Cutting
Dec 16, 1998·Pediatric Research·Richard KraemerS Liechti-Gallati
Dec 19, 2001·Lancet·Sarath RanganathanLondon Collaborative Cystic Fibrosis Group
May 10, 2002·Pediatric Pulmonology·C SchaedelL Holmberg
Jul 2, 2002·American Journal of Respiratory and Critical Care Medicine·Nicholas HartBrigitte Fauroux
Feb 3, 2004·American Journal of Respiratory and Critical Care Medicine·Sarath RanganathanColin Wallis
Oct 2, 2004·The European Respiratory Journal·Brigitte FaurouxFrédéric Lofaso
Nov 2, 2004·American Journal of Respiratory and Critical Care Medicine·Paul AuroraLondon Cystic Fibrosis Collaboration
Nov 9, 2004·The American Journal of Clinical Nutrition·Nicholas HartBrigitte Fauroux
Nov 9, 2004·American Journal of Respiratory and Critical Care Medicine·Richard KraemerSabina Gallati
Aug 22, 2006·Respiratory Physiology & Neurobiology·Gerald S ZavorskyJuan M Murias
Sep 9, 2006·American Journal of Respiratory and Critical Care Medicine·Richard KraemerReto Crameri
Nov 24, 2006·Thorax·Sooky LumLondon Cystic Fibrosis Collaboration
Dec 23, 2006·Pediatric Pulmonology·Per M Gustafsson
Sep 12, 2008·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Rajeev SoniPeter P T Bye
Sep 3, 2009·European Journal of Human Genetics : EJHG·Javier SanzSabina Gallati

Citations

Aug 16, 2012·European Journal of Human Genetics : EJHG·Franziska M GislerSabina Gallati
Dec 4, 2014·BMC Complementary and Alternative Medicine·Kui CuiZheng-Hong Qin
Sep 17, 2010·Pediatric Pulmonology·Richard Kraemer, Cindy Thamrin
May 10, 2012·Pediatric Pulmonology·Marie Lémery MagninSylvain Blanchon
Dec 10, 2013·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·L KentEuropean Cystic Fibrosis Society Clinical Trial Network (ECFS-CTN) Standardisation Committee
Sep 6, 2015·The European Respiratory Journal·Lizzie GrilloMichael R Loebinger

Methods Mentioned

BETA
blood draw

Related Concepts

CFTR protein, human
Airway Resistance
Blood Gas Analysis
Carbon Dioxide
Pulmonary Cystic Fibrosis
Forced Expiratory Flow Rates
Forced Expiratory Volume Function
Functional Residual Capacity
Lung
Dioxygen

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