1. Drug induced complications; can we do more?
Journal of Cystic Fibrosis 12 (2013) 547-558 

Daniel Peckham, Paul Whitaker

Abstract: Drug induced complication are becoming increasingly common in CF. In this review we discuss some of the key issues relating to drug side in an ageing population of patients with CF. Strategies to reduce drug complications are also discussed.

2. Pain in CF: Review of the literature
Journal of Cystic Fibrosis 12 (2013) 423-430

Trudy Havermans, Kristine Colpaert, Kris De Boeck, Lieven Dupont, Janice Abbott


Background: This review evaluated pain research in cystic fibrosis (CF).
Methods: OVID MEDLINE, CINAHL, AMED, Web of Science, Pubmed, PsychINFO and PsychARTICLES were searched from January 1995–December 2012 to locate papers assessing pain in CF. A proforma was used to record the rationale for the study, characteristics of the sample, pain assessment tools, pain location, frequency and severity, treatment/self-management, coping and the impact on daily activities and quality of life.
Results: All studies (n = 13) were retrospective. Chest and abdominal pains were most commonly reported. Pain was negatively associated with pulmonary exacerbations, quality of life and treatment adherence. Approximately 50% of patients do not consult their GP or CF team about pain, with many patients reporting self-management.
Conclusion: A high incidence of pain is reported in CF although there is little standardization of CF pain measurement. The way forward is to develop guidelines on how to assess pain and provide adequate treatment for pain in CF.

3. A review of renal disease in cystic fibrosis
Journal of Cystic Fibrosis 12 (2013) 309-317

Dilip Nazareth, Martin Walshaw

Abstract: Kidney disease is becoming increasingly common in CF. This review looks at the effect of CFTR on the kidney, the problems with measuring renal function effectivel.

4. Update on cystic fibrosis-related diabetes
Journal of Cystic Fibrosis 12 (2013) 318-331

Andrea Kelly, Antoinette Moran

Abstract: Diabetes mellitus has emerged as a common comorbidity in cystic fibrosis and is considered a clinical entity (cystic fibrosis-related diabetes, CFRD) distinct from that of type 1 diabetes (T1DM) and type 2 diabetes (T2DM). The relevance of this diagnosis extends not only from its imposition of additional medical burden but its association with worse health outcomes in individuals with CF. This paper will review the 2010 U.S. and other international guidelines for screening and treating CFRD. It will highlight newer data regarding early glucose and insulin secretion defects, mechanisms linking CFRD to worse outcomes, and recent advances in T2DM that may provide insights for CFRD; insulin secretion will be reviewed as background for these recent developments.

5. Measures of body habitus are associated with lung function in adults with cystic fibrosis: A population-based study
Journal of Cystic Fibrosis 12 (2013) 284-289

Doug L. Forrester, Alan J. Knox, Alan R. Smyth, Andrew W. Fogarty


Background: Body habitus differences may explain some of the variation in lung function between individuals with cystic fibrosis (CF). We tested the hypothesis that measures of lean muscle mass and obesity are independently associated with lung function in CF.
Methods: Cross-sectional study design using UK CF registry data from 2096 clinically stable adults.
Results: Serum creatinine and BMI were positively and independently associated with FEV1 and FVC. One standard deviation increment in serum creatinine was associated with an FEV1 increase of 171 ml (95% confidence intervals CI: +116 to +227 ml) in males and 90 ml (95% CI: +46 to +133 ml) in females. Compared to the reference group of 20–24.9 kg/m2, those with a BMIb20 kg/m2 had lower FEV1 with values of −642 ml (95%CI: −784 to −500 ml) for males and −468 ml (95%CI: −564 to −372 ml) for females.
Conclusions: Prospective studies and controlled trials are required to ascertain if these associations have therapeutic potential in modifying disease progression.

6. Molecular detection of CF lung pathogens: Current status and future potential
Journal of Cystic Fibrosis 12 (2013) 194-205

Sally H. Pattison, Geraint B. Rogers, Martin Crockard, J. Stuart Elborn, Michael M. Tunney

Abstract: Molecular diagnostic tests, based on the detection and identification of nucleic acids in human biological samples, are increasingly employed in the diagnosis of infectious diseases and may be of future benefit to CF microbiology services. Our growing understanding of the complex polymicrobial nature of CF airway infection has highlighted current and likely future shortcomings in standard diagnostic practices. Failure to detect fastidiousorslow growing microbes and misidentification of newly emerging pathogens could potentially be addressed using culture-independent molecular technologies with high target specificity. This review considers existing molecular diagnostic tests in the context of the key requirements for an envisaged CF microbiology focussed assay. The issues of assay speed, throughput, detection of multiple pathogens, data interpretation and antimicrobial susceptibility testing are discussed.

7. Nutritional intervention in patients with Cystic Fibrosis: A systematic review
Journal of Cystic Fibrosis 12 (2013) 102-115  

J.W. Woestenenk, S.J.A.M. Castelijns, C.K. van der Ent, R.H.J. Houwen


Background: To systematically assess the literature published after 1997 describing the effectiveness of nutritional interventions in Cystic Fibrosis patients.
Methods: An online search in PUBMED, EMBASE and COCHRANE databases was conducted. Original studies with 4 patients or more, describing a nutritional intervention and giving at least weight as an outcome parameter were included.
Results: The inclusion criteria were met by 17 articles, focusing on respectively behavioural interventions (n=6), oral supplementation (n=4) or enteral tube feeding (n=7). This latter intervention was universally successful to induce weight gain. One behavioural study and 2 oral supplementation studies also reported significant weight gain.
Conclusion: Enteral tube feeding is effective to improve nutritional status, while the described effects of behavioural intervention and oral supplementation are not consistent at present.

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