Diagnosis and early life risk factors for bronchiectasis in cystic fibrosis: a review

Sly, Peter D.; Wainwright, Claire

doi:10.1080/17476348.2016.1204915

Cited by 14 publications

(8 citation statements)

References 70 publications

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“…116 The expansion of microbes drives robust neutrophil infiltration and activation, which have been shown to be the best markers for disease severity in cystic fibrosis. 117 Neutrophils, in turn, contribute to persistent expansion of lung microbes by suppressing T-cell recruitment and activation 118 and promoting anaerobic respiration in these microbes. 119 Close interactions between neutrophils and the lung microbiome have also been reported for other airway disorders such as chronic obstructive pulmonary disease and asthma.…”

Section: Interplay Between Neutrophils and The Microbiota In Chronic Diseasesmentioning

confidence: 99%

Cross talk between neutrophils and the microbiota

Zhang

Frenette

2019

Blood

102

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The microbiota has emerged as an important regulator of the host immunity by the induction, functional modulation, or suppression of local and systemic immune responses. In return, the host immune system restricts translocation and fine tunes the composition and distribution of the microbiota to maintain a beneficial symbiosis. This paradigm applies to neutrophils, a critical component of the innate immunity, allowing their production and function to be influenced by microbial components and metabolites derived from the microbiota, and engaging them in the process of microbiota containment and regulation. The cross talk between neutrophils and the microbiota adjusts the magnitude of neutrophil-mediated inflammation on challenge while preventing neutrophil responses against commensals under steady state. Here, we review the major molecular and cellular mediators of the interactions between neutrophils and the microbiota and discuss their interplay and contribution in chronic inflammatory diseases and cancer.

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Section: Interplay Between Neutrophils and The Microbiota In Chronic Diseasesmentioning

confidence: 99%

Cross talk between neutrophils and the microbiota

Zhang

Frenette

2019

Blood

102

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“…Nevertheless, positive Aspergillus BAL cultures at age 5 years were associated with an increased probability of air trapping, which in young children with CF correlates with small airways structural lung disease. Moreover, a recent report suggested that air trapping on expiratory chest HRCT scans (also known as mosaic attenuation) may result from both hypoventilation and hypoperfusion, the latter secondary to local low oxygen concentrations and predates irreversible structural lung injury,26 while another found air trapping predicted the development of bronchiectasis 27. It is possible that Aspergillus may either have a direct role in early lung injury, manifested by air trapping, or act as a marker for other pathological events, which ultimately lead to irreversible structural airway wall damage and bronchiectasis.…”

Section: Discussionmentioning

confidence: 99%

Aspergillus and progression of lung disease in children with cystic fibrosis

Harun

Wainwright

Grimwood

et al. 2018

Thorax

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BackgroundThe impact of Aspergillus on lung disease in young children with cystic fibrosis is uncertain.AimsTo determine if positive respiratory cultures of Aspergillus species are associated with: (1) increased structural lung injury at age 5 years; (2) accelerated lung function decline between ages 5 years and 14 years and (3) to identify explanatory variables.MethodsA cross-sectional analysis of association between Aspergillus positive bronchoalveolar lavage (BAL) cultures and chest high-resolution CT (HRCT) scan findings at age 5 years in subjects from the Australasian Cystic Fibrosis Bronchoalveolar Lavage (ACFBAL) study was performed. A non-linear mixed-effects disease progression model was developed using FEV1% predicted measurements at age 5 years from the ACFBAL study and at ages 6–14 years for these subjects from the Australian Cystic Fibrosis Data Registry.ResultsPositive Aspergillus BAL cultures at age 5 years were significantly associated with increased HRCT scores for air trapping (OR 5.53, 95% CI 2.35 to 10.82). However, positive Aspergillus cultures were not associated with either FEV1% predicted at age 5 years or FEV1% predicted by age following adjustment for body mass index z-score and hospitalisation secondary to pulmonary exacerbations. Lung function demonstrated a non-linear decline in this population.ConclusionIn children with cystic fibrosis, positive Aspergillus BAL cultures at age 5 years were associated contemporaneously with air trapping but not bronchiectasis. However, no association was observed between positive Aspergillus BAL cultures on FEV1% predicted at age 5 years or with lung function decline between ages 5 years and 14 years.

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“…In CF, neutrophils are the predominant immune cells infiltrating the airway mucosa and the intralumenal space of bronchioles (accounting for ~70% of the total cell count in BAL fluid) driven by IL-8 and IL-17 secretion; their load as well as the extracellular activity of the protease NE in BAL fluid correlates well with disease progression in CF patients, from infancy to adulthood [ 71 , 72 , 73 ]. In normal homeostatic conditions, neutrophils are short-lived and undergo spontaneous apoptosis to guarantee the termination of the inflammatory insult but, similarly to macrophages, are also characterized by remarkable plasticity.…”

Section: The Role Of Cftr Dysfunction In Phagocyte...mentioning

confidence: 99%

Impact of CFTR Modulators on the Impaired Function of Phagocytes in Cystic Fibrosis Lung Disease

Meoli

Eickmeier

Pisi

et al. 2022

IJMS

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Cystic fibrosis (CF), the most common genetically inherited disease in Caucasian populations, is a multi-systemic life-threatening autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. In 2012, the arrival of CFTR modulators (potentiators, correctors, amplifiers, stabilizers, and read-through agents) revolutionized the therapeutic approach to CF. In this review, we examined the physiopathological mechanism of chronic dysregulated innate immune response in the lungs of CF patients with pulmonary involvement with particular reference to phagocytes, critically analyzing the role of CFTR modulators in influencing and eventually restoring their function. Our literature review highlighted that the role of CFTR in the lungs is crucial not only for the epithelial function but also for host defense, with particular reference to phagocytes. In macrophages and neutrophils, the CFTR dysfunction compromises both the intricate process of phagocytosis and the mechanisms of initiation and control of inflammation which then reverberates on the epithelial environment already burdened by the chronic colonization of pathogens leading to irreversible tissue damage. In this context, investigating the impact of CFTR modulators on phagocytic functions is therefore crucial not only for explaining the underlying mechanisms of pleiotropic effects of these molecules but also to better understand the physiopathological basis of this disease, still partly unexplored, and to develop new complementary or alternative therapeutic approaches.

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Diagnosis and early life risk factors for bronchiectasis in cystic fibrosis: a review

Cited by 14 publications

References 70 publications

Cross talk between neutrophils and the microbiota

Cross talk between neutrophils and the microbiota

Aspergillus and progression of lung disease in children with cystic fibrosis

Impact of CFTR Modulators on the Impaired Function of Phagocytes in Cystic Fibrosis Lung Disease

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