Infections in Patients with Cystic Fibrosis

Gilligan, Peter H.

doi:10.1016/j.cll.2014.02.001

Cited by 76 publications

(78 citation statements)

References 108 publications

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“…Staphylococcus aureus , Pseudomonas aeruginosa , Burkholderia cepacia and Haemophilus influenzae ), filamentous fungi and/or yeasts. NTM infections are increasingly being reported in adults with CF , but children are also vulnerable . The frequency of NTM infections in CF children from the UK has been estimated at 3.3% .…”

Section: Inherited Conditions Affecting the Lungs And Conferring A Prmentioning

confidence: 99%

Inherited and acquired immunodeficiencies underlying tuberculosis in childhood

Boisson–Dupuis

Bustamante

El-Baghdadi

et al. 2015

Immunological Reviews

191

165

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Summary Tuberculosis (TB), caused by Mycobacterium tuberculosis (M.tb) and a few related mycobacteria, is a devastating disease, killing more than a million individuals per year worldwide. However, its pathogenesis remains largely elusive, as only a small proportion of infected individuals develop clinical disease either during primary infection or during reactivation from latency or secondary infection. Subacute, hematogenous, and extrapulmonary disease tends to be more frequent in infants, children, and teenagers than in adults. Life-threatening primary TB of childhood can result from known acquired or inherited immunodeficiencies, although the vast majority of cases remain unexplained. We review here the conditions conferring a predisposition to childhood clinical diseases caused by mycobacteria, including not only M.tb but also weakly virulent mycobacteria, such as BCG vaccines and environmental mycobacteria. Infections with weakly virulent mycobacteria are much rarer than TB, but the inherited and acquired immunodeficiencies underlying these infections are much better known. Their study has also provided genetic and immunological insights into childhood TB, as illustrated by the discovery of single-gene inborn errors of IFN-γ immunity underlying severe cases of TB. Novel findings are expected from ongoing and future human genetic studies of childhood TB in countries that combine a high proportion of consanguineous marriages, a high incidence of TB, and an excellent clinical care, such as Iran, Morocco, and Turkey.

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Section: Inherited Conditions Affecting the Lungs And Conferring A Prmentioning

confidence: 99%

Inherited and acquired immunodeficiencies underlying tuberculosis in childhood

Boisson–Dupuis

Bustamante

El-Baghdadi

et al. 2015

Immunological Reviews

191

165

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“…Cystic fibrosis (CF) is characterized by chronic microbial infections within the lower respiratory tract (Gilligan, 2014;LiPuma, 2010). It is now well established that the CF airways harbour a complex microbiota in addition to classical pathogens, including Pseudomonas aeruginosa, Staphylococcus aureus, Haemophilus influenzae and the Burkholderia cepacia complex (Dickson et al, 2013;Lynch & Bruce, 2013;Rabin & Surette, 2012;Rogers et al, 2015;Surette, 2014), and diverse commensal streptococci (LiPuma, 2012;Lynch & Bruce, 2013;Rogers et al, 2010;Sibley et al, 2006), some of which may become pathogenic (Parkins et al, 2008;Sibley et al, 2008aSibley et al, , 2009Sibley et al, , 2010.…”

Section: Introductionmentioning

confidence: 99%

Antibiotic susceptibility and molecular mechanisms of macrolide resistance in streptococci isolated from adult cystic fibrosis patients

Thornton

Grinwis

Sibley

et al. 2015

Journal of Medical Microbiology

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The cystic fibrosis (CF) airways are colonized by polymicrobial communities with high bacterial load and are influenced by frequent antibiotic exposures. This community includes diverse streptococci, some of which have been directly or indirectly associated with pulmonary exacerbations. As many streptococci are naturally competent, horizontal transfer of antibioticresistant determinants coupled with frequent and/or chronic antibiotic exposure may contribute to high resistance rates. In this study, we assessed antibiotic resistance in 413 streptococcal isolates from adult CF patients against nine antibiotics relevant in CF treatment. We observed very low rates of cephalosporin resistance [cefepime and ceftriaxone (,2 %)], and higher rates of resistance to tetracycline (,34 %) and sulfamethoxazole/trimethoprim (,45 %). The highest rate of antibiotic resistance was to the macrolides [azithromycin (56.4 %) and erythromycin (51.6 %)]. We also investigated the molecular mechanisms of macrolide resistance and found that only half of our macrolide-resistant streptococci isolates contained the mef (efflux pump) or erm (methylation of 23S ribosomal target site) genes. The majority of isolates were, however, found to have point mutations at position 2058 or 2059 of the 23S ribosomal subunit -a molecular mechanism of resistance not commonly reported in the non-pyogenic and non-pneumococcal streptococci, and unique in comparison with previous studies. The high rates of resistance observed here may result in poor outcomes where specific streptococci are contributing to CF airway disease and serve as a reservoir of resistance genes within the CF airway microbiome.

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“…P. aeruginosa is considered the main bacterium that affects people with CF, causing chronic lung infections, which leads to high rates of morbidity and mortality, and once the bacteria is established, it is difficult to eradicate it (Winstanley et al, 2016). On the other hand, patients colonized in a chronic manner with B. cepacia present a worse prognosis of the disease, as the use of antibiotics becomes more frequent they also have a greater deterioration of lung function and the rate of mortality is higher compared to that patients colonized by only P. aeruginosa (Gilligan, 2014; Folescu et al, 2015). It has been reported that B. cepacia and P. aeruginosa can form mixed biofilms in the lungs of people with CF, since, P. aeruginosa through its extracellular products can increase the attachment of B. cepacia by modifying the lung epithelial cells on its surface; however, the same does not occur in patients previously colonized with B. cepacia (Saiman et al, 1990).…”

Section: Nets Formation By Microorganisms Of Cystic Fibrosismentioning

confidence: 99%

Understanding the Entanglement: Neutrophil Extracellular Traps (NETs) in Cystic Fibrosis

Martínez-Alemán

Campos-García

Palma-Nicolás

et al. 2017

Front. Cell. Infect. Microbiol.

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Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the gene that codes for the CF trans-membrane conductance regulator. These mutations result in abnormal secretions viscous airways of the lungs, favoring pulmonary infection and inflammation in the middle of neutrophil recruitment. Recently it was described that neutrophils can contribute with disease pathology by extruding large amounts of nuclear material through a mechanism of cell death known as Neutrophil Extracellular Traps (NETs) into the airways of patients with CF. Additionally, NETs production can contribute to airway colonization with bacteria, since they are the microorganisms most frequently found in these patients. In this review, we will discuss the implication of individual or mixed bacterial infections that most often colonize the lung of patients with CF, and the NETs role on the disease.

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Infections in Patients with Cystic Fibrosis

Cited by 76 publications

References 108 publications

Inherited and acquired immunodeficiencies underlying tuberculosis in childhood

Inherited and acquired immunodeficiencies underlying tuberculosis in childhood

Antibiotic susceptibility and molecular mechanisms of macrolide resistance in streptococci isolated from adult cystic fibrosis patients

Understanding the Entanglement: Neutrophil Extracellular Traps (NETs) in Cystic Fibrosis

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