Cystic fibrosis mortality trends in France

Bellis, Gil; Cazes, Marie-Hélène; Parant, Alain; Gaimard, Maryse; Travers, Cécile; Roux, E; Ravilly, S.; Rault, G.

doi:10.1016/j.jcf.2006.07.001

Cited by 56 publications

(36 citation statements)

References 10 publications

(9 reference statements)

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“…Although AET has considerably decreased the prevalence of P. aeruginosa in younger CF patients, the pathogen is still present in the majority of older CF patients [3,5,57,58]. Chronic suppressive antibiotics have proven successful as a treatment of chronic airways infection in the maintenance of lung health [59].…”

Section: Optimizing Antibiotic Therapy For Treatment Of Chronic P Aementioning

confidence: 99%

Treatment of lung infection in patients with cystic fibrosis: Current and future strategies

Döring

Flume

Heijerman³

et al. 2012

Journal of Cystic Fibrosis

426

446

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In patients with cystic fibrosis (CF) lung damage secondary to chronic infection is the main cause of death. Treatment of lung disease to reduce the impact of infection, inflammation and subsequent lung injury is therefore of major importance. Here we discuss the present status of antibiotic therapy for the major pathogens in CF airways, including prophylaxis against infection, eradication of early infection, suppression of chronic infection, and the treatment of infective exacerbations. We outline measures to optimize maintenance treatment for infection in the light of novel antibiotic drug formulations. We discuss new developments in culture-independent microbiological diagnostic techniques and the use of tools for monitoring the success of antibiotic treatment courses. Finally, cost-effectiveness analyses for antibiotic treatment in CF patients are discussed.

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Section: Optimizing Antibiotic Therapy For Treatment Of Chronic P Aementioning

confidence: 99%

Treatment of lung infection in patients with cystic fibrosis: Current and future strategies

Döring

Flume

Heijerman³

et al. 2012

Journal of Cystic Fibrosis

426

446

View full text Add to dashboard Cite

show abstract

“…In the USA, 40% of patients reach adult age and almost a third live beyond the age of 30 years [9]. The median survival age has been put at about 30 years in the United Kingdom [10] and Canada [11], and at 35 years in the USA in 2004 [12], and at 22 years in France [13]. Incidence of CF cases in Spain varies from 1 in 2,810 [14] to 1 in 5,532 newborn infants [15].…”

Section: Introductionmentioning

confidence: 99%

Cystic fibrosis mortality trends in Spain among infants and young children: 1981–2004

et al. 2008

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Data on cystic fibrosis deaths were drawn from the National Statistics Institute (Instituto Nacional de Estadística), which collects data from all death certificates in Spain. During the period 1981-2004, overall CF mortality in Spain decreased by an annual average of 4% in both sexes. A breakdown by age showed that patients under 15 years registered a declining and those over 15 years a rising mortality rate over the study period. Mean and median age at death from CF increased with time, from a median of 4.4 years (males) and 3.8 years (females) in 1981 to 20.1 years (males) and 17.7 years (females) in 2004. The results of this study show that, as in other Western countries, CF is no longer a major cause of death in childhood, and that the challenge now lies in caring for adults who suffer from this disease. The fact that our study was descriptive meant that the reasons for the decrease in CF mortality in Spain could not be identified. Other authors have shown that this decrease is associated with improved treatment for pulmonary complications, better nutritional control and lung transplants.

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“…With a frequency of about 1/2,500 births in France, cystic fibrosis (CF) is the most common genetic inherited disease in the European Caucasian population (3). The disease is caused by mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gene, which encodes a chloride channel in the plasma membranes of various epithelial cell types.…”

mentioning

confidence: 99%

Geosmithia argillacea : an Emerging Pathogen in Patients with Cystic Fibrosis

et al. 2010

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We report eight cases of airway colonization by Geosmithia argillacea in patients with cystic fibrosis. This filamentous fungus, resembling members of the genera Penicillium and Paecilomyces, was identified by molecular analysis. All patients carried a mutation on each CFTR (cystic fibrosis transmembrane conductance regulator) allele, with at least one copy of the F508del mutation. The first isolation of this fungus occurred from F508del-homozygous patients at a younger age than in F508del-heterozygous patients. Before recovery of G. argillacea, all patients were treated with itraconazole; two of them had also received voriconazole for an Aspergillus fumigatus infection. However, antifungal susceptibility patterns showed high MICs of voriconazole for all isolates, and high MICs of amphotericin B and itraconazole for the majority of them, but mostly low minimum effective concentrations (MECs) of caspofungin. The appearance and persistence of G. argillacea in the airways were not associated with exacerbation of the disease. However, the clinical implications of G. argillacea, particularly in immunocompromised patients, remain a concern, particularly given recent observations suggesting that this fungus may also cause disseminated infections.

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Cystic fibrosis mortality trends in France

Abstract: These provisional results demonstrate the need to convert the ONM observatory into a registry providing exhaustive coverage of all patients.

Cited by 56 publications

References 10 publications

Treatment of lung infection in patients with cystic fibrosis: Current and future strategies

Treatment of lung infection in patients with cystic fibrosis: Current and future strategies

Cystic fibrosis mortality trends in Spain among infants and young children: 1981–2004

Geosmithia argillacea : an Emerging Pathogen in Patients with Cystic Fibrosis

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