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Haworth C.S.,Adult Cystic Fibrosis Center
Current Opinion in Pulmonary Medicine | Year: 2010

Purpose of Review: This review summarizes recently published data on the epidemiology, pathophysiology and treatment of cystic fibrosis (CF)-related low bone mineral density (BMD). Recent Findings: A systematic literature review reports that the pooled prevalence of osteoporosis in adults with CF is 23.5% [95% confidence interval (CI) 16.6-31.0] and the pooled prevalences of radiologically confirmed vertebral and nonvertebral fractures are 14% (95% CI 7.8-21.7) and 19.7% (95% CI 6.0-38.8), respectively. Recent data suggest that the cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in bone cells and that CFTR dysfunction affects bone cell activity. The secondary effects of CFTR dysfunction also influence bone metabolism. For example, bone resorption increases during CF pulmonary exacerbations due to the stimulatory effects of proinflammatory cytokines on osteoclast activity. Bisphosphonates inhibit osteoclastic bone resorption and recent data show that both oral and intravenous bisphosphonates improve BMD in patients with CF. Summary: CF-related low BMD is a common but treatable complication of CF. © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins. Source


Lomas P.,Adult Cystic Fibrosis Center
Therapeutic Advances in Respiratory Disease | Year: 2014

Nonadherence to inhaled therapies is a major problem in the treatment of cystic fibrosis that can influence lung function and health outcomes. Potential contributors to nonadherence have been identified, including demographic and psychosocial factors, time and convenience of inhaled therapy, and treatment beliefs. Additional research is clearly needed to clarify the contributors and to determine which interventions and technological advances will enhance adherence to inhaled therapies in patients with cystic fibrosis. Nurses and allied health professionals are ideally positioned to assist patients and families with adherence to inhaled therapies through monitoring, communication, and education about the available therapies and their proper use. This review briefly summarizes the available evidence about contributors to nonadherence, potential interventions, novel delivery devices for inhaled therapies, and opportunities for additional research. © The Author(s), 2014. Source


Kidd T.J.,Queensland Childrens Medical Research Institute | Kidd T.J.,University of Queensland | Grimwood K.,Queensland Childrens Medical Research Institute | Grimwood K.,University of Queensland | And 6 more authors.
Journal of Clinical Microbiology | Year: 2011

Monitoring the emergence and transmission of Pseudomonas aeruginosa strains among cystic fibrosis (CF) patients is important for infection control in CF centers internationally. A recently developed multilocus sequence typing (MLST) scheme is used for epidemiologic analyses of P. aeruginosa outbreaks; however, little is known about its suitability for isolates from CF patients compared with that of pulsed-field gel electrophoresis (PFGE) and enterobacterial repetitive intergenic consensus-PCR (ERIC-PCR). As part of a prevalence study of P. aeruginosa strains in Australian CF clinics, we compared the discriminatory power and concordance of ERIC-PCR, PFGE, and MLST among 93 CF sputum and 11 control P. aeruginosa isolates. PFGE and MLST analyses were also performed on 30 paired isolates collected 85 to 354 days apart from 30 patients attending two CF centers separated by 3,600 kilometers in order to detect within-host evolution. Each of the three methods displayed high levels of concordance and discrimination; however, overall lower discrimination was seen with ERIC-PCR than with MLST and PFGE. Analysis of the 50 ERIC-PCR types yielded 54 PFGE types, which were related by ≤6 band differences, and 59 sequence types, which were classified into 7 BURST groups and 42 singletons. MLST also proved useful for detecting novel and known strains and for inferring relatedness among unique PFGE types. However, 47% of the paired isolates produced PFGE patterns that within 1 year differed by one to five bands, whereas with MLST all paired isolates remained identical. MLST thus represents a categorical analysis tool with resolving power similar to that of PFGE for typing P. aeruginosa. Its focus on highly conserved housekeeping genes is particularly suited for long-term clinical monitoring and detecting novel strains. Copyright © 2011, American Society for Microbiology. All Rights Reserved. Source


Elborn J.S.,Queens University of Belfast | Elborn J.S.,Adult Cystic Fibrosis Center | Henig N.R.,Gilead Sciences Inc.
Expert Opinion on Pharmacotherapy | Year: 2010

Importance of the field: Chronic endobronchial infection in cystic fibrosis (CF) leads to progressive lung function loss and respiratory failure. Most adult CF patients are infected with Pseudomonas aeruginosa, an important predictor of mortality. Suppressing chronic P. aeruginosa infection with inhaled antibiotics is standard of care for CF patients. Areas covered in this review: This review describes the development (2003 2010) of aztreonam lysine 75 mg powder and solvent for nebulizer solution (AZLI; Cayston®), an aerosolized formulation of the monobactam antibiotic aztreonam. What the reader will gain: AZLI was studied in patients with CF and chronic P. aeruginosa airway infection. In placebo-controlled trials, AZLI improved respiratory symptoms, increased forced expiratory volume in 1 sec (FEV1), decreased sputum P. aeruginosa density, and was well tolerated. An open-label follow-on trial of nine 'on/off' courses showed that AZLI was safe and the effect durable with repeated administration. AZLI was recently approved for use in CF patients in Australia and the USA, and conditionally approved in Canada and the European Union. AZLI is given three times daily for 28 days (2 3 min/dose), followed by 28 days off-drug. AZLI is used only with the Altera Nebulizer System™, which provides appropriate particle size and small airway deposition, and has excellent portability. Take home message: AZLI is a new therapy that is safe and effectively improves respiratory symptoms and FEV1 in patients with CF. © 2010 Informa UK Ltd. Source


Salvatore D.,Cystic Fibrosis Center | Buzzetti R.,Italian Cystic Fibrosis Research Foundation | Baldo E.,Cystic Fibrosis Service | Furnari M.L.,Cystic Fibrosis Center | And 7 more authors.
Journal of Cystic Fibrosis | Year: 2012

A total of 53 national cystic fibrosis (CF) patient registry studies published between July 2008 and November 2011 have been reviewed, focusing on the following topics: CF epidemiology, nutrition, microbiology, clinical complications, factors influencing diagnosis and lung disease, effects of socioeconomic status, therapeutic strategy evaluation, clinical trial methodology.The studies describe the clinical characteristics of CF patients, the incidence and prevalence of disease and role of gender gap, as well as the influence of socioeconomic status and environmental factors on clinical outcomes, covering a variety of countries and ethnic groups. Original observations describe patients as they get older, with special reference to the adult presentation of CF and long-term survival. Methodological aspects are discussed, covering the design of clinical trials, survival analysis, auxometry, measures of quality of life, follow up of lung disease, predictability of disease progression and life expectancy. Microbiology studies have investigated the role of selected pathogens, such as Burkholderia species and MRSA. Pulmonary exacerbations are discussed both as a factor influencing morbidity and an endpoint in clinical trials. Finally, some studies give insights on complications, such as CF-related diabetes and hemoptysis, and emerging problems, such as chronic nephropathy. © 2012 European Cystic Fibrosis Society. Source

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