title: Role of clotting factor(s) in the etiology of lung disease related to ciliostasis.
abstract: Mucociliary experiments conducted in vitro using avian tracheal rings show that serum from both normal and cystic fibrosis patients will cause ciliostasis in airways. The evidence suggests that endogenous mucus is not inhibitory to cilia and that inhibition observed with serum is due to blood clotting factors (probably fibrin), since a variety of anticoagulants and fibrin-destroying substances will either prevent or reverse the inhibitory effects of serum. Because fibrin and serum are normally found in the lung, we propose that an early event in the pathogenesis of many lung diseases (e.g., asthma, some forms of emphysema, cystic fibrosis), is an increase in the level of clotting factors in the respiratory tract. Based on these results, we provide a rationale for investigating the usefulness of a number of pharmacologic agents in the treatment of diseases related to mucostasis.
mesh_terms: D000818:Animals; D001779:Blood Coagulation Factors / Q000502:physiology*; D002645:Chickens; D002923:Cilia / Q000502:physiology*; D010856:Columbidae; D003550:Cystic Fibrosis / Q000097:blood*; D005337:Fibrin / Q000502:physiology; D006801:Humans; D008171:Lung Diseases / Q000503:physiopathology*; D009093:Mucus / Q000502:physiology; D012140:Respiratory Tract Diseases / Q000503:physiopathology
chemical_list: D001779:Blood Coagulation Factors; D005337:Fibrin