Cystic fibrosis
Disease description
An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION. Year introduced: 1967
Source: MeSHReferences 20
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Journal | Year | Authors |
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Increased nitrosothiols in exhaled breath condensate in inflammatory airway diseases. | Am J Respir Crit Care Med. | 2001 |
M. Corradi, P. Montuschi, LE. Donnelly, A. Pesci, SA. Kharitonov, PJ. Barnes
Am J Respir Crit Care Med. 2001 Mar;163(4):854-8. PubMed:11282756 |
Increased nitrotyrosine in exhaled breath condensate in cystic fibrosis. | Eur Respir J. | 2001 |
B. Balint, SA. Kharitonov, T. Hanazawa, LE. Donnelly, PL. Shah, ME. Hodson, PJ. Barnes
Eur Respir J. 2001 Jun;17(6):1201-7. PubMed:11491165 |
Airways in cystic fibrosis are acidified: detection by exhaled breath condensate. | Thorax. | 2002 |
S. Tate, G. MacGregor, M. Davis, JA. Innes, AP. Greening
Thorax. 2002 Nov;57(11):926-9. PubMed:12403872 |
Increased leukotriene B4 and interleukin-6 in exhaled breath condensate in cystic fibrosis. | Am. J. Respir. Crit. Care Med. | 2003 |
GE. Carpagnano, PJ. Barnes, DM. Geddes, ME. Hodson, SA. Kharitonov
Am. J. Respir. Crit. Care Med. 2003 Apr;167(8):1109-12. PubMed:12684249 |
Breath condensate pH in children with cystic fibrosis and asthma: a new noninvasive marker of airway inflammation? | Chest | 2004 |
GE. Carpagnano, PJ. Barnes, J. Francis, N. Wilson, A. Bush, SA. Kharitonov
Chest 2004 Jun;125(6):2005-10. PubMed:15189915 |
Elemental and ion composition of exhaled air condensate in cystic fibrosis. | J. Cyst. Fibros. | 2003 |
M. Griese, J. Noss, P. Schramel
J. Cyst. Fibros. 2003 Sep;2(3):136-42. PubMed:15463862 |
Exhaled breath condensate pH and exhaled nitric oxide in allergic asthma and in cystic fibrosis. | Thorax | 2005 |
JC. Ojoo, SA. Mulrennan, JA. Kastelik, AH. Morice, AE. Redington
Thorax 2005 Jan;60(1):22-6. PubMed:15618578 |
Hyperglycemia and cystic fibrosis alter respiratory fluid glucose concentrations estimated by breath condensate analysis. | J Appl Physiol (1985). | 2007 |
EH. Baker, N. Clark, AL. Brennan, DA. Fisher, KM. Gyi, ME. Hodson, BJ. Philips, DL. Baines, DM. Wood
J Appl Physiol (1985). 2007 May;102(5):1969-75. Epub 2007 Feb 15. PubMed:17303703 |
Extracellular purines are biomarkers of neutrophilic airway inflammation. | Eur Respir J. | 2008 |
CR. Esther, NE. Alexis, ML. Clas, ER. Lazarowski, SH. Donaldson, CM. Ribeiro, CG. Moore, SD. Davis, RC. Boucher
Eur Respir J. 2008 May;31(5):949-56. doi: 10.1183/09031936.00089807. Epub 2008 Feb 6. PubMed:18256064 |
Biomarkers in exhaled breath condensate indicate presence and severity of cystic fibrosis in children. | Pediatr Allergy Immunol. | 2008 |
CM. Robroeks, PP. Rosias, D. van Vliet, Q. Jöbsis, JB. Yntema, HJ. Brackel, JG. Damoiseaux, GM. den Hartog, WK. Wodzig, E. Dompeling
Pediatr Allergy Immunol. 2008 Nov;19(7):652-9. doi: 10.1111/j.1399-3038.2007.00693.x. Epub 2008 Feb 27. PubMed:18312532 |
Exhaled 8-isoprostane and prostaglandin E(2) in patients with stable and unstable cystic fibrosis. | Free Radic. Biol. Med. | 2008 |
V. Lucidi, G. Ciabattoni, S. Bella, PJ. Barnes, P. Montuschi
Free Radic. Biol. Med. 2008 Sep;45(6):913-9. PubMed:18634869 |
Oral bacteria--the missing link to ambiguous findings of exhaled nitrogen oxides in cystic fibrosis. | Respir Med. | 2009 |
W. Zetterquist, H. Marteus, P. Kalm-Stephens, E. Näs, L. Nordvall, M. Johannesson, K. Alving
Respir Med. 2009 Feb;103(2):187-93. doi: 10.1016/j.rmed.2008.09.009. Epub 2008 Nov 9. PubMed:19006660 |
Mass spectrometric analysis of biomarkers and dilution markers in exhaled breath condensate reveals elevated purines in asthma and cystic fibrosis. | Am J Physiol Lung Cell Mol Physiol. | 2009 |
CR. Esther, G. Boysen, BM. Olsen, LB. Collins, AJ. Ghio, JW. Swenberg, RC. Boucher
Am J Physiol Lung Cell Mol Physiol. 2009 Jun;296(6):L987-93. doi: 10.1152/ajplung.90512.2008. Epub 2009 Mar 20. PubMed:19304910 |
Exhaled breath condensate pH and ammonia in cystic fibrosis and response to treatment of acute pulmonary exacerbations. | Pediatr Pulmonol. | 2009 |
S. Newport, N. Amin, AJ. Dozor
Pediatr Pulmonol. 2009 Sep;44(9):866-72. doi: 10.1002/ppul.21078. PubMed:19670404 |
NMR spectroscopy metabolomic profiling of exhaled breath condensate in patients with stable and unstable cystic fibrosis. | Thorax. | 2012 |
P. Montuschi, D. Paris, D. Melck, V. Lucidi, G. Ciabattoni, V. Raia, C. Calabrese, A. Bush, PJ. Barnes, A. Motta
Thorax. 2012 Mar;67(3):222-8. doi: 10.1136/thoraxjnl-2011-200072. Epub 2011 Nov 21. PubMed:22106016 |
Exhaled breath condensate purines correlate with lung function in infants and preschoolers. | Pediatr Pulmonol. | 2013 |
K. Patel, SD. Davis, R. Johnson, CR. Esther
Pediatr Pulmonol. 2013 Feb;48(2):182-7. doi: 10.1002/ppul.22573. Epub 2012 May 21. PubMed:22615171 |
Exhaled breath condensate pH in patients with cystic fibrosis. | Inflamm. Res. | 2012 |
B. Antus, I. Barta, E. Csiszer, K. Kelemen
Inflamm. Res. 2012 Oct;61(10):1141-7. PubMed:22706320 |
Nitrites and nitrates in exhaled breath condensate in cystic fibrosis: relation to clinical parameters. | Bratisl Lek Listy. | 2013 |
L. Fila, J. Chladek, M. Maly, J. Musil
Bratisl Lek Listy. 2013;114(9):503-7. PubMed:24020705 |
Exhaled breath condensate detects baseline reductions in chloride and increases in response to albuterol in cystic fibrosis patients. | Clin Med Insights Circ Respir Pulm Med | 2013 |
CM. Wheatley, WJ. Morgan, NA. Cassuto, WT. Foxx-Lupo, CL. Daines, MA. Morgan, H. Phan, EM. Snyder
Clin Med Insights Circ Respir Pulm Med 2013 ;7:79-90. PubMed:24367235 |
Comparison of Airway and Systemic Malondialdehyde Levels for Assessment of Oxidative Stress in Cystic Fibrosis. | Lung. | 2015 |
B. Antus, O. Drozdovszky, I. Barta, K. Kelemen
Lung. 2015 Aug;193(4):597-604. doi: 10.1007/s00408-015-9739-1. Epub 2015 May 8. PubMed:25951912 |
Compounds 22
Download compoundsAcetate | PubChem CID: 175 |
Acetone | PubChem CID: 180 |
Chloride | PubChem CID: 312 |
Ethanol | PubChem CID: 702 |
Nitrate | PubChem CID: 943 |
Nitrite | PubChem CID: 946 |
Urea | PubChem CID: 1176 |
Isopropanol | PubChem CID: 3776 |
D-Glucose | PubChem CID: 5793 |
Adenosine triphosphate | PubChem CID: 5957 |
Malondialdehyde | PubChem CID: 10964 |
Zinc | PubChem CID: 23994 |
Nitric oxide | PubChem CID: 145068 |
3-Nitrotyrosine | PubChem CID: 235719 |
Prostaglandin E2 | PubChem CID: 5280360 |
Leukotriene B4 | PubChem CID: 5280492 |
8-epi-PGF2alpha | PubChem CID: 5282263 |
Sodium | PubChem CID: 5360545 |
Potassium | PubChem CID: 5462222 |
Thionitrous acid | PubChem CID: 11389353 |
Methanol | PubChem CID: 101954077 |
Adenosine | PubChem CID: 60961 |
MeSH information
MeSH ID | D003550 |
MeSH name | Cystic Fibrosis |