Early intervention and prevention of lung disease in cystic fibrosis: a European consensus

Döring, Gerd; Høiby, Niels

doi:10.1016/j.jcf.2004.03.008

Cited by 282 publications

(272 citation statements)

References 285 publications

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“…Thus, routine intravenous treatment courses may be of particular benefit in treating this disease. Whether the reduction in cyanide levels following in-patient antibiotics was due to alterations in bacterial numbers or to other nonbactericidal effects was not assessed in this study, but it is worth speculating that the excellent outcomes reported in some countries may be due in part to suppression of cyanide production through regular admissions, irrespective of clinical status, for intravenous antibiotic courses [23,24].…”

Section: Discussionmentioning

confidence: 99%

Bacterial cyanogenesis occurs in the cystic fibrosis lung

Sanderson¹,

Wescombe²,

Kirov³

et al. 2008

European Respiratory Journal

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The cystic fibrosis (CF) lung environment is poorly defined, but data suggest that bacteria may encounter reduced oxygen tensions and possibly an anaerobic environment. Pseudomonas aeruginosa produces the potent toxin cyanide under strictly microaerobic conditions. Evidence of bacterial cyanogenesis in the CF lung was investigated in the present study by measuring sputum cyanide concentrations.Sputum cyanide was measured in seven stable CF patients, as well as before and after intravenous antibiotic therapy during a hospital admission in a further eight patients experiencing acute exacerbations. All patients were chronically infected with P. aeruginosa. Comparative sputum data were obtained from nine CF patients with no documented P. aeruginosa infection and 10 healthy, nonsmoking normal controls.High levels of cyanide were detected in all the P. aeruginosa-infected stable CF patients (median (range) 0.56 (0.37-2.81) mg?mL -1 ), and in seven out of eight acute sputum samples (0.73 (0-1.43) mg?mL -1 ). In contrast, cyanide was not detectable in sputum from eight out of nine CF patients without P. aeruginosa infection or in any of the normal controls. Intravenous antibiotic treatment significantly reduced sputum cyanide levels (median 0.73 to median 0.0 mg?mL -1 ).The cyanide detected indicates that the cystic fibrosis lung provides a predominantly microaerobic environment for Pseudomonas aeruginosa. Cyanide is likely to be a potentially important virulence factor in Pseudomonas aeruginosa-infected cystic fibrosis patients.

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Section: Discussionmentioning

confidence: 99%

Bacterial cyanogenesis occurs in the cystic fibrosis lung

Sanderson¹,

Wescombe²,

Kirov³

et al. 2008

European Respiratory Journal

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“…It is also possible that these early changes are reversible and, therefore, should be treated before structural damage causes irreversible functional deficits. There is increasing evidence that early and aggressive therapy is improving quality of life and longevity in CF [102,[156][157][158][159][160], and, thus, a reasonable case can be made for regular routine CT scans to detect the earliest indication of airway disease. Ultimately, only a randomised clinical trial will answer the question of whether routine CT scanning is warranted in children with CF.…”

Section: Future Directionsmentioning

confidence: 99%

Computed tomographic imaging of the airways: relationship to structure and function

Jong

Müller²,

Paré³

et al. 2005

European Respiratory Journal

156

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Alterations in the structure of the airways, collectively termed airway remodelling, contribute to airflow obstruction in a variety of chronic lung diseases. While histology has provided valuable insights into the structure of airway wall remodelling, this technique is invasive and does not allow the longitudinal analysis of airway wall dimensions. Technical advances in computed tomography allow the assessment of airway wall dimensions, and are ideally suited for the noninvasive investigation of the pathogenesis of airway wall remodelling and the evaluation of new therapeutic interventions. The aim of this article is to review the use of computed tomography in the investigation of airway structure and function in health and disease.

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“…From our study and in agreement with other studies mentioned, we can conclude that such goals might also be achieved for patients with cystic fibrosis. If the rehabilitation programs are run for patients with cystic fibrosis at a maximum level of hygienic precautions to prevent cross-infections [22], together with individual improvements of health, the overall health care costs might be reduced. However, it is necessary in the short term, to improve the quality of the reports received from the rehabilitation sites and to receive non-biased and standardized assessments of acknowledged outcomes from all subjects.…”

Section: Resultsmentioning

confidence: 99%

Rehabilitation Programs for Cystic Fibrosis - View from a CF Center

Griese¹,

Busch²,

Caroli³

et al. 2010

TORMJ

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Abstract:Background: Rehabilitation programs are comprehensive interventions which effectively improve the health status and reduce costs in chronic respiratory illnesses. Because patients with cystic fibrosis have been discouraged to participate for concerns of microbial cross infection, the efficacy of systematic rehabilitation is unknown for this group. Methods:We retrospectively studied 142 cystic fibrosis patients aged 2-46 years who participated in rehabilitation programs taking place in Germany/Switzerland and in Israel, focusing on changes in lung function and weight.Results: During 172 stays in 97 patients in Israel and 68 stays in 45 patients rehabilitating in Germany/Switzerland, overall lung function and weight improved. Outcome did not differ between Israel and German/Swiss sites. Interestingly, lung function improved during the initial phase of the stay, whereas weight gain was sustained throughout. The study uncovered gaps in reporting sufficient individual outcome information back to the admitting centre.Conclusions: Rehabilitation programs specified for cystic fibrosis patients need to be assessed prospectively to optimize treatment of this life limiting condition.

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Early intervention and prevention of lung disease in cystic fibrosis: a European consensus

Cited by 282 publications

References 285 publications

Bacterial cyanogenesis occurs in the cystic fibrosis lung

Bacterial cyanogenesis occurs in the cystic fibrosis lung

Computed tomographic imaging of the airways: relationship to structure and function

Rehabilitation Programs for Cystic Fibrosis - View from a CF Center

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