Severe Achromobacter xylosoxidans infection and loss of sputum bacterial diversity in an adult patient with cystic fibrosis
We report the case of an adult patient with cystic fibrosis and chronic A. xylosoxidans infection who experienced rapid, progressive clinical deterioration. Metagenomic analysis of the sputum revealed that the airway microbiota was almost entirely dominated by A. xylosoxidans. We review the impact of this organism on lung function and the airway microbiome in cystic fibrosis, and discuss the potential for cross-infection between patients. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - June 13, 2016 Category: Respiratory Medicine Authors: Nick P. Talbot, William G. Flight Source Type: research
Pneumothorax in cystic fibrosis: beyond the guidelines
Pneumothorax is a serious but common complication in patients with cystic fibrosis (CF). It has adverse prognostic implications as well as associations with subsequent reduction in lung function and significant risk of recurrence. Management dilemmas frequently occur that are beyond current guidelines. We review the evidence and highlight management difficulties in pneumothoraces in CF. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - June 13, 2016 Category: Respiratory Medicine Authors: Robert W. Lord, Andrew M. Jones, A. Kevin Webb, Peter J. Barry Source Type: research
Treatment of pulmonary exacerbations in cystic fibrosis –could do better?
This article describes the nature and significance of pulmonary exacerbations in cystic fibrosis (CF). The effectiveness and safety of current exacerbation treatment are explored. The article concludes with a summary of clinical trials (completed and ongoing) which aim to improve the efficacy and safety of exacerbation treatment. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - June 13, 2016 Category: Respiratory Medicine Authors: Alan Smyth Source Type: research
Lifestyle treatments in CF –the NHS should not pay
Lifestyle treatments can be defined as those which may have in impact on quality of life but do not affect health outcomes. Particular treatment options may be preferred by patients because they are for example, easier to use, take up less time or taste better. The impact on adherence needs to be considered. Treatment options that promote greater adherence to therapy are likely to be more efficacious and so are not, by definition, lifestyle treatments. The NHS is facing unprecedented financial pressure and resources are limited. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - June 13, 2016 Category: Respiratory Medicine Authors: Jeremy Hull Source Type: research
Treatment of pulmonary exacerbations in cystic fibrosis–could do better?
This article describes the nature and significance of pulmonary exacerbations in cystic fibrosis (CF). The effectiveness and safety of current exacerbation treatment are explored. The article concludes with a summary of clinical trials (completed and ongoing) which aim to improve the efficacy and safety of exacerbation treatment. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - June 13, 2016 Category: Respiratory Medicine Authors: Alan Smyth Source Type: research
Electronic patient records, past, present and future
The health informatics revolution was spear-headed in the 1980s by pioneers in primary care who worked in an opportune environment and were able to successfully implement electronic patient records (EPR) as far back as the 1990s. Although the ambitious and costly National Programme for IT failed to deliver an integrated EPR, the project achieved the creation of the Spine, the N3 Network, choose and book, picture archiving, communication systems and standards which have allowed integration. Real change is taking place within the NHS with the launch of exciting new projects focusing on true integration and secure data flows ...
Source: Paediatric Respiratory Reviews - June 13, 2016 Category: Respiratory Medicine Authors: Daniel Peckham Source Type: research
Lifestyle treatments in cystic fibrosis: The NHS should pay
With the NHS under increasing financial pressure and healthcare costs soaring year on year, it is perhaps not surprising that assessment agencies focus on cost-effectiveness analysis when assessing new therapies. Such an approach does not however, always take sufficient account of treatment burden, lifestyle and patient choice and therefore new equally effective but perhaps “easier to take” formulations and faster delivery systems for current therapies do not always take precedence in current treatment guidelines. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - June 13, 2016 Category: Respiratory Medicine Authors: Robert Ian Ketchell Source Type: research
Lifestyle treatments in CF–the NHS should not pay
Lifestyle treatments can be defined as those which may have in impact on quality of life but do not affect health outcomes. Particular treatment options may be preferred by patients because they are for example, easier to use, take up less time or taste better. The impact on adherence needs to be considered. Treatment options that promote greater adherence to therapy are likely to be more efficacious and so are not, by definition, lifestyle treatments. The NHS is facing unprecedented financial pressure and resources are limited. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - June 13, 2016 Category: Respiratory Medicine Authors: Jeremy Hull Source Type: research
Cystic Fibrosis Papers of the Year 2015
Studies published in the last year have expanded our knowledge of potential disease modifying agents in the treatment of class II, II and IV CFTR mutations, and included the first report of an efficacious gene therapy for CF. There is also an important message on increasing use of conventional chronic therapies even in milder disease, and the pernicious effect of chronic infection on pulmonary function. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - June 13, 2016 Category: Respiratory Medicine Authors: Iolo Doull Source Type: research
Phenotypic variability of R117H-CFTR expression within monozygotic twins
Whilst cystic fibrosis is a monogenic condition, variation in phenotype exists for the same CFTR genotype, which is influenced by multiple genetic and non-genetic (environmental) factors. The R117H-CFTR mutation has variability directly relating to in cis poly-thymidine alleles, producing a differing spectrum of disease. This paper provides evidence of extreme phenotype variability–including fertility status - in the context of male monogenetic twins, discussing mechanisms and highlighting the diagnostic and treatment challenges. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - June 13, 2016 Category: Respiratory Medicine Authors: Michael D. Waller, Nicholas J. Simmonds Source Type: research
Is deafness mutation screening required in cystic fibrosis patients?
Aminoglycosides are widely used in cystic fibrosis management. The m.1555A>G mutation predisposes to aminoglycoside ototoxicity. It may cause later onset hearing loss in the absence of aminoglycosides use and gradual hearing loss may be an inevitable consequence of the mutation. Given that aminoglycoside therapy forms the backbone of IV protocols in CF, this article recommends screening for this mutation to allow informed decision-making prior to aminoglycoside administration, to avoid preventable deafness. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - June 13, 2016 Category: Respiratory Medicine Authors: Rania Abusamra, Donna McShane Source Type: research
Severe Achromobacter xylosoxidans infection and loss of sputum bacterial diversity in an adult patient with cystic fibrosis
We report the case of an adult patient with cystic fibrosis and chronic A. xylosoxidans infection who experienced rapid, progressive clinical deterioration. Metagenomic analysis of the sputum revealed that the airway microbiota was almost entirely dominated by A. xylosoxidans. We review the impact of this organism on lung function and the airway microbiome in cystic fibrosis, and discuss the potential for cross-infection between patients. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - June 13, 2016 Category: Respiratory Medicine Authors: Nick P. Talbot, William G Flight Source Type: research
Pneumothorax in cystic fibrosis: beyond the guidelines
Pneumothorax is a serious but common complication in patients with cystic fibrosis (CF). It has adverse prognostic implications as well as associations with subsequent reduction in lung function and significant risk of recurrence. Management dilemmas frequently occur that are beyond current guidelines. We review the evidence and highlight management difficulties in pneumothoraces in CF. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - June 13, 2016 Category: Respiratory Medicine Authors: Robert W. Lord, Andrew M. Jones, A. Kevin Webb, Peter J. Barry Source Type: research
Slipping Rib Syndrome: An elusive diagnosis
Slipping rib syndrome remains rarely recognized and frequently undiagnosed or misdiagnosed. Awareness of this condition may prevent extensive and unnecessary diagnostic evaluation and avoid chronic debilitating pain. Persistent lower chest and/or upper abdomen pain after analgesic treatment may suggest the possibility of this disorder. The diagnosis of this syndrome, is a clinical one, based on history and the hooking maneuver. A few cases have been published in the literature, giving no clear consensus about the treatment of this condition. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 11, 2016 Category: Respiratory Medicine Authors: Nelson L Turcios Tags: Clinical Usefulness Source Type: research
Neurocognitive and Behavioural Outcomes Following Intervention for Obstructive Sleep Apnoea Syndrome in Children
The obstructive sleep apnoea syndrome (OSAS) is common in children and results in several complications when untreated. Considering that children are undergoing important brain development, recent research has focused on neurocognitive and behavioural (NCB) complications of OSAS. Studies have shown that treatment of paediatric OSAS may improve a child's long-term cognitive and social potential and school performance. There is demonstrated benefit in terms of behaviour, attention, and social interactions, as well as likely improvement in cognitive abilities with academic and social achievements. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 11, 2016 Category: Respiratory Medicine Authors: Sofia Konstantinopoulou, Ignacio E. Tapia Tags: Review Source Type: research
