Cystic fibrosis (CF) is an autosomal recessive genetic disorder resulting from defects in the cystic transmembrane conductance regulator (CFTR) protein, which turn results a multi-systemic disorder. There are numerous known CF alleles associated with different mutations of CFTR gene, most common allele being three-base-pair deletion as ΔF508. One manifestation glycemic dysregulation decreased i...

BACKGROUND Nutritional status is a prognostic factor in cystic fibrosis. Prevention of nutritional impairment and weigh loss are major clinical objectives because they are associated with worsening of lung function and increased mortality. OBJECTIVE To identify a potential relationship of clinical nutrition parameters, and their relative changes, with lung function (FEV1%) in a cohort of adol...

BACKGROUND Pulmonary infection is the main cause of death in cystic fibrosis (CF). Aspergillus fumigatus (AF) and Pseudomonas aeruginosa (PA) are the most prevalent fungal and bacterial pathogens isolated from the CF airway, respectively. Our aim was to determine the effect of different colonisation profiles of AF and PA on the clinical status of patients with CF. METHODS A retrospective anal...

To evaluate the effect of Kaftrio on units lipase/kg/day, faecal elastase level, HbA1c, insulin doses (primary outcome measures) and weight (secondary measure) after a 6 month period in adult patients with cystic fibrosis (CF). Data was collected for under St Barts CF service that had started Kaftrio, at baseline months compared using paired t-test (p < 0.05). Patients were excluded where confo...