Gene modulation and severity of lung disease in cystic fibrosis

Pillai, A
August 2008
Thorax;Aug2008, Vol. 63 Issue 8, p670
Academic Journal
The article discusses a study which shows that children with cystic fibrosis with mannose binding lectin 2 (MBL2) deficiency acquired Pseudomonas infection earlier and also had a faster rate of decline in lung function than those with intermediate or high levels of MBL2. It emphasizes the need for further studies in evaluating the effects of other pulmonary modifiers and their interactions in what is an increasingly complex genetic disease.


Related Articles

  • Variant Mannose-Binding Lectin Alleles Are Not Associated with Susceptibility to or Outcome of Invasive Pneumococcal Infection in Randomly Included Patients. Kronborg, Gitte; Weis, Nina; Madsen, Hans O.; Pedersen, Svend S.; Wejse, Christian; Nielsen, Henrik; Skinhoj, Peter; Garred, Peter // Journal of Infectious Diseases;5/15/2002, Vol. 185 Issue 10, p1517 

    Examines the relationship between mannose-binding lectin (MBL) gene heterogeneity and susceptibility to infectious diseases. Severity of lung disease in cystic fibrosis; Outcome of invasive pneumococcal disease; Role of MBL in pathophysiology.

  • Altered sialyl- and fucosyl-linkage on mucins in cystic fibrosis patients promotes formation of the sialyl-Lewis X determinant on salivary MUC-5B and MUC-7. Shori, D. K.; Genter, T.; Hansen, J.; Koch, C.; Wyatt, H.; Kariyawasam, H. H.; Knight, R. A.; Hodson, M. E.; Kalogeridis, A.; Tsanakas, I. // Pflugers Archiv European Journal of Physiology;Nov2001 Supplement 1, Vol. 443, pS55 

    Destruction of the lungs as a consequence of recurrent infections with microorganisms such as Pseudomonas aeruginosa remains the underlying cause of most morbidity and mortality in cystic fibrosis (CF). We have hypothesized that changes in the glycosylation of key tracheal mucins such as MUC5B...

  • Use of a modeling framework to evaluate the effect of a modifier gene (MBL2) on variation in cystic fibrosis. McDougal, Kathryn E.; Green, Deanna M.; Vanscoy, Lori L.; Fallin, M. Daniele; Grow, Michael; Cheng, Suzanne; Blackman, Scott M.; Collaco, J. Michael; Henderson, Lindsay B.; Naughton, Kathleen; Cutting, Garry R. // European Journal of Human Genetics;Jun2010, Vol. 18 Issue 6, p680 

    Variants in mannose-binding lectin (MBL2; protein MBL) have shown association with different aspects (eg, lung function, infection, survival) of cystic fibrosis (CF) in some studies but not others. Inconsistent results may be due to confounding among disease variables that were not fully...

  • 'To CT or not to CT? That is the question': outcome surrogates for surveillance in childhood cystic fibrosis. Young, Carolyn; Owens, Catherine // Thorax;Jun2012, Vol. 67 Issue 6, p471 

    The author reflects on the use of computerized tomography (CT) for the monitoring of the patients especially children with cystic fibrosis (CF) who suffer from lung diseases. The use of thin section CT with respect to the imaging modality is highlighted by a study conducted by the Australian...

  • Acute renal failure in people with cystic fibrosis. Southern, Kevin W. // Thorax;Jun2007, Vol. 62 Issue 6, p472 

    The article comments on antibiotic strategies for cystic fibrosis (CF) lung infection, to achieve the goal of increased survival while balancing risks to patients. The cystic fibrosis transmembrane conductance regulator has an important role in transepithelial salt transport. The outlook for...

  • Following Nero: fiddle while Rome burns, or is there a better way? Bush, Andrew; Pavord, Ian // Thorax;May2011, Vol. 66 Issue 5, p367 

    The author comments on the use of the term exacerbation in describing severity of asthma or chronic obstructive pulmonary disease in relation to a proposal to discard the term in favor of the phrase lung attack. The author criticizes the generally accepted view of exacerbation and discusses how...

  • Reasons for heterogeneous change in LCI in children with cystic fibrosis after antibiotic treatment. Yammine, Sophie; Bigler, Anja; Casaulta, Carmen; Singer, Florian; Latzin, Philipp; Horsley, Alex // Thorax;Feb2014, Vol. 69 Issue 2, p183 

    A letter to the editor and a response from the author of the article is presented titled "Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation" in 2013 issue.

  • An unusual cystic lung lesion. Huang, T-W.; Cheng, Y.L.; Lee, S-C. // Thorax;Nov2008, Vol. 63 Issue 11, p980 

    A quiz about an unusual cystic lung lesion is presented.

  • Highlights from this issue. Andrew Bush; Ian Pavord // Thorax;Oct2011, Vol. 66 Issue 10, pi 

    The article discusses various reports published within the issue on topics including lungs attacks in cystic fibrosis patients, lung transplantation and lung cancer screening.


Read the Article


Sign out of this library

Other Topics