Antibiotic exposure and interpersonal variance mask the effect of ivacaftor on respiratory microbiota composition
G551D is a class III mutation of the cystic fibrosis transmembrane regulator (CFTR) that results in impaired chloride channel function in cystic fibrosis (CF). Ivacaftor, a CFTR-potentiating agent improves sweat chloride, weight, lung function, and pulmonary exacerbation rate in CF patients with G551D mutations, but its effect on the airway microbiome remains poorly characterised.
The prevalence of cystic fibrosis-related diabetes is increasing. This condition is potentially responsible for respiratory decline.
Exercise is a cornerstone of the therapeutic management of individuals with cystic fibrosis (CF). Regular exercise and physical activity participation, irrespective of age or disease severity, together with routine assessment of exercise capacity are recommended in guideline based care [1,2].
Corrigendum to “Does current reporting of lung function by the UK cystic fibrosis registry allow a fair comparison of adult centres?” [J Cyst Fibros (2017) 585–591]
The authors regret that the methods section of the manuscript describing the study population contained a factual error. The authors mistakenly believed that from 2012 onwards the UK registry stipulated that the annual review data be recorded as the best in year value. It has been bought to our attention that the annual review data used throughout our analysis represents the value of FEV1 on the date of the annual review. In addition to recording this data, since 2012 the UK registry has also requested that a best in year value be entered as a separate variable for all patients.
IgG avidity to Pseudomonas aeruginosa over the course of chronic lung biofilm infection in cystic fibrosis
The mechanisms leading to low effectiveness of the humoral immune response against P. aeruginosa in cystic fibrosis (CF) are poorly understood. The aim of the present study was to assess the avidity maturation of specific antipseudomonal IgG before and during the development of chronic lung infection in a cohort of Danish CF patients.
Comparing the management of constipation and distal intestinal obstruction syndrome between paediatricians and adult physicians
Intestinal complications are common in children and adults with cystic fibrosis (CF). Constipation affects up to half of all patients and DIOS affects around 5% of patients in any one year . Although these two conditions share symptomatology and treatments, the pathogenesis is different. DIOS is acute complete or incomplete faecal obstruction in the ileocaecum whereas constipation is gradual faecal impaction of the total colon . We wanted to assess and compare the treatment regimens used by paediatricians and adult physicians for the management of constipation, incomplete DIOS and complete DIOS.
Significant progress has been achieved in developing precision therapies for cystic fibrosis; however, highly effective treatments that target the ion channel, CFTR, are not yet available for many patients. As numerous CFTR therapeutics are currently in the clinical pipeline, reliable screening tools capable of predicting drug efficacy to support individualized treatment plans and translational research are essential. The utilization of bronchial, nasal, and rectal tissues from individual cystic fibrosis patients for drug testing using in vitro assays such as electrophysiological measurements of CFTR activity and evaluation of fluid movement in spheroid cultures, has advanced the prediction of patient-specific responses.