Advances in cystic fibrosis

Cystic fibrosis is the most common lethal genetic disorder among whites, with an incidence of 1:1600 live white births in the United States. It is a clinical syndrome characterized by generalized exocrine dysfunction. Obstructive lesions are evident throughout multiple organ systems, and diverse disturbances are observed in mucous and electrolyte secretions. Pedigree analysis suggests that cystic fibrosis is inherited as an autosomal recessive trait, with heterozygotes having no recognizable clinical symptoms. Certain characteristics of the disease suggest a polygenic mode of inheritance, but no linkage with the HLA complex or other gene loci has been demonstrated.