Update on non-cystic fibrosis bronchiectasis. Curr Opin Pulm Med

Royal Brompton Hospital, Sydney Street, London, UK.
Current opinion in pulmonary medicine (Impact Factor: 2.76). 12/2008; 14(6):595-9. DOI: 10.1097/MCP.0b013e328312ed8c
Source: PubMed


There is growing awareness in both adult and paediatric respiratory clinics regarding the importance of non-cystic fibrosis bronchiectasis. There has been debate regarding the requirement for investigations to establish an underlying cause of bronchiectasis. Furthermore, there has been growing interest in establishing the role of bacteria in disease progression.
Post-infection is no longer the most common cause of bronchiectasis in developed countries in children or adults and the importance of identifying immunodeficiency has been underlined. Nontuberculous mycobacteria are recognized as both causing and complicating non-cystic fibrosis bronchiectasis. It has been suggested that infection with Pseudomonas aeruginosa may confer a worse prognosis compared with other pathogens but recent publications produce conflicting views. There is increasing recognition that patients with non-cystic fibrosis bronchiectasis do not necessarily respond to cystic fibrosis treatment regimens in the same way as patients with cystic fibrosis regimens.
Non-cystic fibrosis bronchiectasis can be approached in a systematic fashion to establish the underlying cause and develop treatment strategies.

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    • "Eventually, the inflammation can lead to bronchiectasis characterized by permanent dilation of the airway and thickening of the bronchial wall. Because of these changes, areas like this can function as a seedbed for future infections (Bilton, 2008; Ilowite et al., 2008; Javidan-Nejad & Bhalla, 2009; King, 2009; Goeminne & Dupont, 2010). "
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    ABSTRACT: Mucociliary clearance has long been known to be a significant innate defence mechanism against inhaled microbes and irritants. Important knowledge has been gathered regarding the anatomy and physiology of this system, and in recent years, extensive studies of the pathophysiology related to lung diseases characterized by defective mucus clearance have resulted in a variety of therapies, which might be able to enhance clearance from the lungs. In addition, ways to study in vivo mucociliary clearance in humans have been developed. This can be used as a means to assess the effect of different pharmacological interventions on clearance rate, to study the importance of defective mucus clearance in different lung diseases or as a diagnostic tool in the work-up of patients with recurrent airway diseases. The aim of this review is to provide an overview of the anatomy, physiology, pathophysiology, and clinical aspects of mucociliary clearance and to present a clinically applicable test that can be used for in vivo assessment of mucociliary clearance in patients. In addition, the reader will be presented with a protocol for this test, which has been validated and used as a diagnostic routine tool in the work-up of patients suspected for primary ciliary dyskinesia at Rigshospitalet, Denmark for over a decade.
    Clinical Physiology and Functional Imaging 09/2013; 34(3). DOI:10.1111/cpf.12085 · 1.44 Impact Factor
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    • "P. aeruginosa is the most-frequently isolated Gram-negative bacterium in nosocomial infections and causes acute blood, lung, intra-abdominal space, urogenital tract and wound/burn infections (Gaynes and Edwards, 2005; Jones, 2010; Fujitani et al., 2011). Chronic conditions are also associated with P. aeruginosa infection, including cystic fibrosis (CF), chronic obstructive pulmonary disease, acquired immunodeficiency syndrome , non-CF brochiectasis and situations requiring use of in-dwelling catheters (Murray et al., 2007; Bilton, 2008; Huang et al., 2010). Antimicrobials are the mainstay of current therapy for P. aeruginosa infections; however, new anti-Pseudomonal approaches are needed because: (i) infections are often unresponsive; (ii) pan-drug-resistant P. aeruginosa strains have been isolated; and (iii) biofilms formed by P. aeruginosa are highly drug-resistant (Giamarellou and Kanellakopoulou, 2008; Page and Heim, 2009; Ho et al., 2010). "
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    ABSTRACT: P. aeruginosa infections are commonly associated with cystic fibrosis, pneumonias, neutropenia and burns. The P. aeruginosa quorum sensing molecule N-(3-oxo-dodecanoyl) homoserine lactone (C12) cause multiple deleterious host responses, including repression of NF-κB transcriptional activity and apoptosis. Inhibition of C12-mediated host responses is predicted to reduce P. aeruginosa virulence. We report here a novel, host-targeted approach for potential adjunctive anti-Pseudomonal therapy based on inhibition of C12-mediated host responses. A high-throughput screen was developed to identify C12 inhibitors that restore NF-κB activity in C12-treated, lipopolysaccharide (LPS)-stimulated cells. Triazolo[4,3-a]quinolines with nanomolar potency were identified as C12-inhibitors that restored NF-κB-dependent luciferase expression in LPS- and TNF-stimulated cell lines. In primary macrophages and fibroblasts, triazolo[4,3-a]quinolines inhibited C12 action to restore cytokine secretion in LPS-stimulated cells. Serendipitously, in the absence of an inflammatory stimulus, triazolo[4,3-a]quinolines prevented C12-mediated responses, including cytotoxicity, elevation of cytoplasmic calcium, and p38 MAPK phosphorylation. In vivo efficacy was demonstrated in a murine model of dermal inflammation involving intradermal C12 administration. The discovery of triazolo[4,3-a]quinolines provides a pharmacological tool to investigate C12-mediated host responses, and a potential host-targeted anti-Pseudomonal therapy.
    Cellular Microbiology 08/2013; 48(1). DOI:10.1111/cmi.12176 · 4.92 Impact Factor
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    • "Bronchiectasis is a chronic airway disease characterized by abnormal and irreversible dilation and destruction of bronchial walls and “Mucociliary Clearance System”. As the result, the patient experiences recurrent coughs associate with thick and sometimes bloody sputum, dyspnea, rhinosinusitis and chronic respiratory infections (1–4). Chronic nature of these symptoms directly affects and reduces the quality of life of patients (5). "
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    ABSTRACT: Background Halotherapy is a treatment modality suggested for patients with chronic pulmonary diseases. In this technique, inhalation of crystal salt stones extracted from mines improves patients’ pulmonary function tests and symptoms by facilitating the secretion or expulsion of phlegm and mucus and reducing the risk of bacterial infections. Bronchiectasis is chronic disease of the airways characterized by irreversible dilation of airways. It has a progressive course and despite the available treatments, many of these patients eventually enter the advanced phase of disease. The aim of this study was to evaluate the effect of halotherapy on pulmonary function tests and quality of life of non-CF bronchiectatic patients. Materials and Methods This clinical trial evaluated the results of spirometry and 6-minute walk test as well as the quality of life (according to SF-36 questionnaire) of stable non-CF bronchiectatic patients presenting to the pulmonary clinic before and after the use of salt spray for 2 months. Results Of 40 study patients, 20 were excluded due to various reasons and 20 were evaluated. The mean age of patients was 35±11 years and the underlying cause of disease was chronic pulmonary infection in 65% of cases. Comparison of the results of pulmonary function tests and 6-minute walk test and quality of life indices in SF-36 questionnaire before and after the intervention showed no significant difference (P > 0.05). However, 65% of patients were satisfied with halotherapy and requested to receive the medication again. Conclusion Our study results indicated that 2-month halotherapy with Salitair inhaler containing salt crystals extracted from the Klodawa mine in Poland could not improve the pulmonary function tests or quality of life of non-CF bronchiectatic patients. No significant side effects were noted in understudy patients. Future studies with larger sample size and longer duration of treatment are recommended to better determine the efficacy of this treatment modality.
    Tanaffos 03/2013; 12(2):22-7.
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