Abnormal viscosity of mucus produced at the epithelial surfaces causing recurrent infections and bronchiectasis in lungs

Patients also have pancreatic insufficiency amongst other secretory problems

Autosomal recessive defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene located on the long arm of chromosome 7.

CFTR controls chloride channel behavior causing defective chloride secretion and increased sodium absorption altering the composition of mucus secretions

As those with bronchiectasis, usually presenting in the first years of life, malnourishment, males are infertile due to the absence of the vas deferens

As those of bronchiectasis , failure to thrive, and steatorrhoea secondary to pancreatic insufficiency

Sweat test to look for excess sweat sodium, DNA analysis, Chest X-Ray, CT Thorax and pulmonary function tests

Physiotherapy for sputum clearance, nutrition and supplemental pancreatic enzymes

Antibiotics, mucolytics, nebulised antibiotics, lung/heart-lung transplants