Pulmonary function and clinical course in patients with cystic fibrosis after pulmonary colonization with Pseudomonas aeruginosa

Kerem, E; Corey, Mary; Gold, Ronald; Levison, Henry

doi:10.1016/s0022-3476(05)82653-8

Cited by 275 publications

(154 citation statements)

References 18 publications

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“…This population included 107 patients with chronic P. aeruginosa infection and 6 patients with transient infection. Of those included in the cohort, 72 (63%) patients are currently active within the CACFC cohort, 12 (11%) patients were transferred to another CF clinic, and 29 (26%) patients experienced respiratory failure and either received a life-saving lung transplant (24) or succumbed to CF (5). Among the members of the cohort, 101 (89%) patients had received prior care at a pediatric CF care center, and 12 were adults diagnosed with CF late in life, among which 9 received care only at CACFC.…”

Section: Patient Populationmentioning

confidence: 99%

“…Pseudomonas aeruginosa, the archetypical CF pathogen, infects 70% of patients (1)(2)(3). The acquisition of this organism and its conversion to a hyper-alginate-producing mucoid phenotype are associated with deteriorating clinical status, increased treatment requirements, and progression to end-stage disease (4)(5)(6)(7)(8). P. aeruginosa, which is ubiquitous in the environment (9)(10)(11), was initially presumed to be acquired from each patient's local environment and not transmitted among patients (12).…”

mentioning

confidence: 99%

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Twenty-Five-Year Outbreak of Pseudomonas aeruginosa Infecting Individuals with Cystic Fibrosis: Identification of the Prairie Epidemic Strain

et al. 2014

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Transmissible strains of Pseudomonas aeruginosa have been described for cystic fibrosis (CF) and may be associated with a worse prognosis. Using a comprehensive strain biobank spanning 3 decades, we sought to determine the prevalence and stability of chronic P. aeruginosa infection in an adult population. P. aeruginosa isolates from sputum samples collected at initial enrollment in our adult clinic and at the most recent clinic visit were examined by a combination of pulsed-field gel electrophoresis and multilocus sequence typing and compared against a collection of established transmissible and local non-CF bronchiectasis (nCFB) isolates. A total of 372 isolates from 107 patients, spanning 674 patient-years, including 66 patients with matched isolates from initial and final encounters, were screened. A novel clone with increased antibacterial resistance, termed the prairie epidemic strain (PES), was found in 29% (31/107 patients) of chronically infected patients referred from multiple prairie-based CF centers. This isolate was not found in those diagnosed with CF as adults or in a control population with nCFB. While 90% (60/66 patients) of patients had stable infection over a mean of 10.8 years, five patients experienced strain displacement of unique isolates, with PES occurring within 2 years of transitioning to adult care. PES has been present in our cohort since at least 1987, is unique to CF, generally establishes chronic infection during childhood, and has been found in patients at the time of transition of patients from multiple prairie-based CF clinics, suggesting broad endemicity. Studies are under way to evaluate the clinical implications of PES infection.

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Section: Patient Populationmentioning

confidence: 99%

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confidence: 99%

Twenty-Five-Year Outbreak of Pseudomonas aeruginosa Infecting Individuals with Cystic Fibrosis: Identification of the Prairie Epidemic Strain

et al. 2014

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“…Additionally, epidemiological data show that infection with P. aeruginosa is confounded with the degree of pulmonary impairment which already exists [29] and that P. aeruginosa, although associated with some decrease in FEV1, does not cause sudden deterioration in CF patients [30]. Apparently, some patients can tolerate P. aeruginosa colonization for years without decline of lung function.…”

Section: Diagnosis Of Pseudomonas Aeruginosa Lung Infectionmentioning

confidence: 99%

Antibiotic therapy against Pseudomonas aeruginosa in cystic fibrosis: a European consensus

Döring¹,

Conway²,

Heijerman³

et al. 2000

Eur Respir J

557

478

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Cystic fibrosis (CF) is the most common lethal hereditary disorder with autosomal recessive heredity in caucasians. The majority of CF patients suffer from chronic respiratory infection with the opportunistic bacterial pathogen Pseudomonas aeruginosa. No consensus among clinicians has been reached so far concerning antibiotic treatment against P. aeruginosa in CF patients.Consensus answers to 24 important questions in this context, based on current evidence, are presented, given by a panel of 34 European experts. Questions addressed and answered are: The diagnosis of P. aeruginosa lung colonization in CF; The impact of P. aeruginosa on the clinical state of CF patients; The assessment of P. aeruginosa susceptibility against antibiotics and the importance of these results for the clinician; The use of monotherapy versus combination therapy; The development of microbial resistance; The achievement of optimal airway concentrations; The effects of subinhibitory concentrations of antibiotics on P. aeruginosa; Statements on the pharmacokinetics of antibiotics in CF patients; Recommendations for doses and dosing intervals and length of treatment regimens; and Toxic side effects due to repeated antibiotic therapy was addressed.The expert panel answered further questions on the use of fluoroquinolones in children with CF, on the administration of nebulized antibiotics and whether prevention of P. aeruginosa lung colonization is possible in CF using antibiotic therapy.Problems of antibiotic therapy at home and in the hospital were addressed, a consensus statement on regular maintenance treatment, or treatment on demand, was given and different routes of administration of antibiotics were recommended for different clinical situations.Finally, the factors which determine the choice of the antibiotic, the dosage, and the duration of the treatment in cystic fibrosis patients were addressed and the design of future antibiotic studies in the context of Pseudomonas aeruginosa lung infection in cystic fibrosis patients were recommended. Eur Respir J 2000; 16: 749±767.

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“…More specifically, survival to 16 years of age was 53% in the P aeruginosa-positive patients compared with 84% in patients without this organism. Subsequently, Kerem et al 18 found that at 7 years of age, CF patients colonized with P aeruginosa had a significantly lower forced expiratory volume in 1 second compared with uninfected patients. Other data 19,20 also indicate a relationship between pulmonary dysfunction and pseudomonas-positive cultures.…”

Section: Lished Wilmott Et Almentioning

confidence: 99%

Acquisition ofPseudomonas aeruginosain Children With Cystic Fibrosis

et al. 1997

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ABSTRACT. Objective. This study was pursued as an extension of a randomized clinical investigation of neonatal screening for cystic fibrosis (CF). The project included assessment of respiratory secretion cultures for pathogens associated with CF. The objective was to determine whether patients diagnosed through neonatal screening and treated in early infancy were more likely to become colonized with Pseudomonas aeruginosa compared with those identified by standard diagnostic methods.Methodology. The design involved prospective cultures of respiratory secretions obtained generally by oropharyngeal swabs at least every 6 months and more often if clinically indicated. Patients were managed with a standardized evaluation and treatment protocol at the two Wisconsin certified CF centers; however, there were community and environmental variations associated with the follow-up period as described below.Results. Overall, there were no differences in acquisition of respiratory pathogens between the screened and the control (standard diagnosis) groups. Evaluation of the data between and within the two centers, however, revealed significant differences with earlier acquisition of P aeruginosa in the center with the following distinguishing characteristics: urban location; following patients with the standard US approach in which newly diagnosed, young children were interspersed with older CF patients; and where there were more opportunities for social interactions with other CF patients. The differences were confined to the screened group followed in the urban center in which the median pseudomonas-free survival period was 52 weeks contrasted with 289 weeks in the other center. In addition, assessment of data for the entire CF populations followed at the two centers revealed that the urban center showed a significantly higher prevalence of P aeruginosa colonization in patients between the ages of 3 and 9 years.Conclusions. These results present questions and generate hypotheses on risk factors for acquisition of P aeruginosa in CF and suggest that clinic exposures and/or social interactions may predispose such patients to pseudomonas infections. Pediatrics 1997;100(5). URL: http://www.pediatrics.org/cgi/content/full/100/5/e2; cystic fibrosis, Pseudomonas aeruoginosa, transmission, epidemiology, pulmonary disease.

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Pulmonary function and clinical course in patients with cystic fibrosis after pulmonary colonization with Pseudomonas aeruginosa

Cited by 275 publications

References 18 publications

Twenty-Five-Year Outbreak of Pseudomonas aeruginosa Infecting Individuals with Cystic Fibrosis: Identification of the Prairie Epidemic Strain

Twenty-Five-Year Outbreak of Pseudomonas aeruginosa Infecting Individuals with Cystic Fibrosis: Identification of the Prairie Epidemic Strain

Antibiotic therapy against Pseudomonas aeruginosa in cystic fibrosis: a European consensus

Acquisition ofPseudomonas aeruginosain Children With Cystic Fibrosis

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