Editorial
Secondary genetic factors in cystic fibrosis lung disease
| The first 150 words of the full text of this article appear below. |
Cystic fibrosis (CF) is the most common autosomal recessive
genetic disorder amongst populations of northern European descent. Cloning of the cystic fibrosis transmembrane regulator (CFTR) gene in
19891 has allowed the assessment of clinical phenotype in
patients with a specific genetic abnormality. Genotype/phenotype analysis showed a good correlation between the common CF mutation (deletion of phenylalanine at position 508 in the CFTR gene) and pancreatic insufficiency. This correlation does not hold for pulmonary disease which can vary markedly between patients with the same 
F508
CFTR mutation.2 Many explanations have been advanced to
account for this finding. Pulmonary disease is influenced by environmental factors such as the patient's age, pancreatic status, treatment regime, social class, smoking history, nutritional status, and colonisation with strains of Pseudomonas
aeruginosa or Burkholderia cepacia.3-8 Moreover, those patients who receive
care in a dedicated CF centre have better lung function when all the
known environmental factors are
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