A paradigm shift in cystic fibrosis nutritional care: Clinicians' views on the management of patients with overweight and obesity.

Background: Overweight and obesity among people with cystic fibrosis (pwCF) has become more prevalent since the widespread adoption of CF transmembrane conductance regulator (CFTR) modulator therapies and presents a new challenge for nutritional care. We aimed to explore how clinicians working in CF care approach the management of adults with overweight and obesity.

Methods: We conducted semi-structured interviews with n = 20 clinicians (n = 6 physiotherapists, n = 6 doctors and n = 8 dietitians) working in 15 adult CF centres in the United Kingdom.

Respiratory Viruses and Cystic Fibrosis.

The threat of respiratory virus infection to human health and well-being has been clearly highlighted by the coronavirus disease 2019 (COVID-19) pandemic. For people with cystic fibrosis (CF), the clinical significance of viral infections long predated the emergence of severe acute respiratory syndrome coronavirus 2. This article reviews the epidemiology, diagnosis, and treatment of respiratory virus infection in the context of CF as well as the current understanding of interactions between viruses and other microorganisms in the CF lung.

Clinical associations with carriage of pulmonary .

Background: causes opportunistic respiratory infections and is associated with declining lung function in patients with cystic fibrosis (CF). Risk factors for carrying remain unclear.

Methods: We conducted a retrospective study of patients yielding ⩾1 respiratory isolate at the Oxford University Hospitals Trust between 2014 and 2019 and a cohort study of carriage in CF patients attending annual review in 2018.

Successful awake proning is associated with improved clinical outcomes in patients with COVID-19: single-centre high-dependency unit experience.

The SARS-CoV-2 can lead to severe illness with COVID-19. Outcomes of patients requiring mechanical ventilation are poor. Awake proning in COVID-19 improves oxygenation, but on data clinical outcomes is limited.

Gastrointestinal malignancy in cystic fibrosis.

Cystic fibrosis (CF) is a multisystem disease affecting the gastrointestinal (GI) tract as well as the lungs. As survival has increased significantly over the past few decades, complications not seen previously have become apparent. There is an overall increased rate of malignancy in CF, particularly from the GI tract and in the post-transplant population.

Outcomes of pulmonary infection.

Background: Treatment of Mycobacterium abscessus pulmonary disease (PD) is challenging with frequent side effects and uncertain rates of success.

Methods: We performed a retrospective review of all patients at our center with at least one respiratory sample positive for M. abscessus between 2014 and 2019.

Severe Achromobacter xylosoxidans infection and loss of sputum bacterial diversity in an adult patient with cystic fibrosis.

Achromobacter spp. are emerging pathogens in the lungs of patients with cystic fibrosis. We report the case of an adult patient with cystic fibrosis and chronic A.

Rapid Detection of Emerging Pathogens and Loss of Microbial Diversity Associated with Severe Lung Disease in Cystic Fibrosis.

Respiratory infection in cystic fibrosis (CF) is polymicrobial, but standard sputum microbiology does not account for the lung microbiome or detect changes in microbial diversity associated with disease. As a clinically applicable CF microbiome surveillance scheme, total sputum nucleic acids isolated by a standard high-throughput robotic method for accredited viral diagnosis were profiled for bacterial diversity using ribosomal intergenic spacer analysis (RISA) PCR. Conventional culture and RISA were performed on 200 paired sputum samples from 93 CF adults; pyrosequencing of the 16S rRNA gene was applied to 59 patients to systematically determine bacterial diversity.

Incidence and clinical impact of respiratory viruses in adults with cystic fibrosis.

Background: Viral respiratory infection (VRI) is a common cause of pulmonary exacerbations in children with cystic fibrosis (CF). The importance of VRI in adult CF populations is unclear.

Objective: To determine the incidence and clinical impact of VRI among adults with CF.

Chronic rhinovirus infection in an adult with cystic fibrosis.

Rhinovirus is a common cause of exacerbations of cystic fibrosis (CF) and is usually considered a self-limiting infection. We report a case of chronic infection with rhinovirus A type 33 in a 43-year-old male with CF which has persisted for over 2 years.

A therapeutic conundrum: recurrent cystic-fibrosis-related haemoptysis complicated by acute pulmonary embolism.

The authors present the case of an older patient with cystic fibrosis (CF) and recurrent haemoptysis complicated by acute pulmonary embolism. The patient was treated successfully with a combination of anticoagulation and bronchial artery embolisation. The management of CF-related haemoptysis, the impact of an ageing CF population and the incidence of thromboembolic disease in CF are discussed.

Long-term non-invasive ventilation in cystic fibrosis -- experience over two decades.

Background: Non-invasive ventilation (NIV) is accepted as a bridge to lung transplantation in cystic fibrosis (CF) but there is little evidence to support its use outside this setting.

Methods: We reviewed the records of all patients with CF who received domiciliary NIV at our centre between 1991 and 2010.

Results: Of 47 patients studied, 36% underwent lung transplantation, 28% died without transplantation and 30% remain alive on NIV.

Persistent oseltamivir-resistant pandemic influenza A/H1N1 infection in an adult with cystic fibrosis.

The authors report the case of a 25-year-old patient with cystic fibrosis (CF) who developed pandemic influenza A/H1N1 during a visit to the USA in August 2010. The patient has severe CF lung disease and takes maintenance oral corticosteroids. The influenza virus was positive for the H275Y oseltamivir-resistance mutation despite the patient never having received oseltamivir.