Primary mucociliary transport failure.
Among the disorders associated with male infertility and chronic sinopulmonary infections, primary ciliary dyskinesia or cystic fibrosis is characterized by ciliary dysfunction or abnormality of mucus secretion. In addition, Young's syndrome differs from the former because of the absence of ultrastructural cilia disorders and from the latter because of normal sweat and pancreatic functions. However, a number of manifestations seen in these disorders appear to overlap each other, e.g., male infertility and chronic sinopulmonary infections which often develop bronchiectasis. Therefore, I would like to propose that the term 'muco(secretion)ciliary transport failure' is used for illnesses in patients with primary impairment of mucosecretion and/or ciliary transport in organs containing the mucociliary transport system. Primary mucociliary transport failure encompasses three hereditary disorders, that is, primary ciliary dyskinesia, cystic fibrosis and Young's syndrome. Ciliary activity is closely associated with mucus production. For a better understanding of the relationship between ciliary activity and mucus production, further basic and clinical studies should be attempted.
Umeki
S
S
Department of Medicine, Kawasaki Medical School, Okayama, Japan.
D000818:Animals; D002923:Cilia / Q000502:physiology; D002925:Ciliary Motility Disorders / Q000503:physiopathology; D003550:Cystic Fibrosis / Q000503:physiopathology; D006801:Humans; D008297:Male; D009079:Mucociliary Clearance; D009845:Oligospermia / Q000503:physiopathology; D013577:Syndrome; D009626:Terminology as Topic