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: MeSH

References 20

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Journal Year Authors
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

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Acetate 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