Background: Short term studies of exercise training have shown benefits in cystic fibrosis. Transferring exercise programmes to the community and sustaining them long term is a challenge for the patient. The effectiveness of an individualised unsupervised home based exercise programme was examined in adults with cystic fibrosis over a 1 year period. Methods: Subjects were randomised to undertak...

A t the basic level, we know the genetic cause of cystic fibrosis: it is an autosomal recessive disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR).1,2 At the clinical level, we know that chronic bacterial airway infection, prominent neutrophilic inflammation and mucus in airways, and progressive bronchiectasis characterize advanced cy...

Intestinal duplication in an adult is an uncommon congenital abnormality because only minority of cases present in adulthood. More than 80% of cases occur before the age of two years as an acute abdomen, bowel obstruction or other complications associated with it. Duplication has two types, either cystic or tubular. Here, we report a case of an adult who was diagnosed preoperatively on CT scan ...

BACKGROUND Mutational combinations of the cystic fibrosis transmembrane conductance regulator, CFTR, gene have different phenotypic manifestations at the molecular level with varying clinical consequences for individuals possessing such mutations. Reporting cystic fibrosis transmembrane conductance regulator mutations is important in understanding the genotype-phenotype correlations and associa...

Size and distensibility of large airways have important implications for flow limitation and the efficacy of coughing. From radiological and functional data, some authors have suggested an increased size and distensibility of the trachea in cystic fibrosis (CF). Using computed tomography (CT) we compared size and distensibility of the trachea in 5 cystic fibrosis patients and five age- and heig...