Anti-inflammatory effects of elexacaftor/tezacaftor/ivacaftor in adults with cystic fibrosis heterozygous for F508del.

Inflammation is a key driver in the pathogenesis of cystic fibrosis (CF). We assessed the effectiveness of elexacaftor/tezacaftor/ivacaftor (ETI) therapy on downregulating systemic and immune cell-derived inflammatory cytokines. We also monitored the impact of ETI therapy on clinical outcome.

Urinary bicarbonate and metabolic alkalosis during exacerbations in cystic fibrosis.

https://bit.ly/3NFPkUu.

Nasal high-flow therapy as an adjunct to exercise in patients with cystic fibrosis: A pilot feasibility trial.

Background: Exercise tolerance in people with CF and advanced lung disease is often reduced. While supplemental oxygen can improve oxygenation, it does not affect dyspnoea, fatigue or comfort. Nasal high-flow therapy (NHFT), thanks to its pathophysiological mechanisms, could improve exercise tolerance, saturation and dyspnoea.

Noninvasive Ventilation in Cystic Fibrosis: Clinical Indications and Outcomes in a Large UK Adult Cystic Fibrosis Center.

Background: Noninvasive ventilation (NIV) is routinely used to treat patients with cystic fibrosis and respiratory failure. However, evidence on its use is limited, with no data on its role in disease progression and outcomes. The aim of this study was to assess the indications of NIV use and to describe the outcomes associated with NIV in adults with cystic fibrosis in a large adult tertiary center.

Different CFTR modulator combinations downregulate inflammation differently in cystic fibrosis.

Previously, we showed that serum and monocytes from patients with CF exhibit an enhanced NLRP3-inflammasome signature with increased IL-18, IL-1β, caspase-1 activity and ASC speck release (Scambler et al. eLife 2019). Here we show that CFTR modulators down regulate this exaggerated proinflammatory response following LPS/ATP stimulation.

Cytomegalovirus-associated pulmonary exacerbation in patients with cystic fibrosis.

http://ow.ly/Rdds30hlnjV.

When Women with Cystic Fibrosis Become Mothers: Psychosocial Impact and Adjustments.

Advances in the treatment and life expectancy of cystic fibrosis (CF) patients mean that motherhood is now a realistic option for many women with CF. This qualitative study explored the psychosocial impact and adjustments made when women with CF become mothers. Women with CF ( = 11) were recruited via an online forum and participated in semistructured telephone interviews about their experiences of becoming a mother.

Bloodstream infections in cystic fibrosis: nine years of experience in both adults and children.

Background: We report the aetiology and outcome of bloodstream infections (BSI) occurring at two regional cystic fibrosis (CF) centres (one adult, one paediatric) between 1998 and 2006.

Methods: A retrospective analysis of all positive blood cultures during the study period was performed.

Results: During the study period 1691 blood culture sets were taken.

Multicentre trial of weekly risedronate on bone density in adults with cystic fibrosis.

Background: The aim of this study was to assess the efficacy, tolerability and safety of risedronate in adults with CF.

Methods: Patients with a lumbar spine (LS), total hip (TH) or femoral neck (FN) bone mineral density (BMD) Z-score of -1 or less were randomised to receive risedronate 35 mg weekly or placebo, and calcium (1g)+vitamin D(3) (800IU).

Results: At baseline, BMD Z-scores in the risedronate (n=17) and placebo (n=19) groups were similar.

Sequential bronchoscopy in the management of lobar atelectasis secondary to allergic bronchopulmonary aspergillosis.

Lobar atelectasis is a common complication in patients with cystic fibrosis. Failure to reexpand the lung isassociated with poorer outcomes. We report sequential weekly flexible bronchoscopy, with instillation of recombinant human DNase, in 5 patients with cystic fibrosis who had lobar collapse secondary to allergic bronchopulmonary aspergillosis.

Clinical impact of reducing routine susceptibility testing in chronic Pseudomonas aeruginosa infections in cystic fibrosis.

BACKGROUND Susceptibility testing results are not predictive of clinical response to antibiotic therapy in chronic Pseudomonas aeruginosa infections in cystic fibrosis (CF). We assessed the impact of reducing the number of routine susceptibility tests performed on clinical outcome in these cases. METHODS In June 2006, we introduced a protocol whereby susceptibility tests of P.

Nutritional status, perceived body image and eating behaviours in adults with cystic fibrosis.

Background & Aims: Achieving and maintaining an ideal nutritional status is the primary aim of the nutritional management of cystic fibrosis (CF). It is unclear how nutritional interventions impact on patients' perceptions and behaviours concerning body image and eating. This work aimed to provide a psychosocial profile and compare CF patients receiving (a) enteral tube feeding, (b) nutritional supplements, (c) no nutritional interventions, and (d) healthy controls.

Associations between clinical variables and quality of life in adults with cystic fibrosis.

Background: The disease progression of cystic fibrosis (CF) is marked by an increase in clinical conditions and therapeutic interventions, which have the potential to affect health-related quality of life (HRQoL). This cross-sectional study explored associations between clinical variables and HRQoL.

Methods: HRQoL was measured using the Cystic Fibrosis Quality of Life (CFQoL) questionnaire, which consists of nine domains: physical, social, treatment, chest symptoms, emotional functioning, concerns for the future, relationships, body image, and career concerns.

Pharmacokinetics and safety of itraconazole in patients with cystic fibrosis.

Objective: To assess the pharmacokinetics of itraconazole and hydroxy-itraconazole in patients with cystic fibrosis.

Methods: Patients were divided into those <16 and >/=16 years of age. All received itraconazole oral solution 2.

Serum and sputum concentrations following the oral administration of linezolid in adult patients with cystic fibrosis.

Objectives: Linezolid is a new oxazolidinone antibiotic with efficacy against a broad range of Gram-positive bacteria, including methicillin-resistant Staphylococcus aureus (MRSA). In this study, we have determined the serum and sputum linezolid concentrations in adults with cystic fibrosis (CF) following oral drug administration.

Methods: Eleven adult patients with CF were recruited.

Steady-state pharmacokinetics of intravenous colistin methanesulphonate in patients with cystic fibrosis.

Objectives: To define the steady-state pharmacokinetics of colistin methanesulphonate and colistin in patients with cystic fibrosis (CF) following intravenous administration of the former.

Materials And Methods: The study was conducted in 12 patients with CF following intravenous administration of colistin methanesulphonate (1.63-3.

Alcaligenes infection in cystic fibrosis.

The aim of this study was to investigate the effect of chronic Alcaligenes species infection of the respiratory tract on the clinical status of patients with cystic fibrosis. We conducted a retrospective case-controlled study. The microbiological records of all patients attending the Leeds Regional Pediatric and Adult Cystic Fibrosis Units from 1992-1999 were examined.