Atypical bronchial cilia in children with recurrent respiratory tract infections. A comparative ultrastructural study. 1984

F J Cornillie, and J M Lauweryns, and L Corbeel

Ultrastructurally atypical bronchial cilia are studied and semiquantitatively analysed in 24 children suffering from recurrent respiratory tract infections with or without bronchiectasis. In patients with Kartagener's syndrome normal-looking and shortened dynein arms are present at some axonemal microtubular doublets. This finding suggests that the polymerization or assemblage of dynein molecules on microtubules only is defective but not totally lacking. Bilateral, local and partial absence of dynein arms is demonstrated in some of the patients with acquired unilateral bronchiectases. These patients also reveal anomalies of the "9 + 2" microtubular axonemal pattern. It is suggested that these abnormalities of the tubulin-dynein system are local and acquired defects that may impair bronchial mucociliary clearance. None of the patients with pneumonia and asthma or with cystic fibrosis studied show any anomalies of the dynein arms. However aberrant axonemal microtubular patterns and other ciliopathies such as naked axonemes and megacilia are present at times in these patients. We postulate that these atypical cilia are secondary acquired abnormalities. Only some patients with bacterial or viral pneumonia demonstrate a partial lack of dynein arms in bronchial cilia. Other ciliopathies such as megacilia, naked and intracytoplasmic axonemes and apical blebs are more frequent and more common in these patients. We suppose they manifest a secondary and rather aspecific pathogenic influence upon the bronchial ciliary substructure.

UI MeSH Term Description Entries
D007619 Kartagener Syndrome An autosomal recessive disorder characterized by a triad of DEXTROCARDIA; INFERTILITY; and SINUSITIS. The syndrome is caused by mutations of DYNEIN genes encoding motility proteins which are components of sperm tails, and CILIA in the respiratory and the reproductive tracts. Kartagener Triad,Ciliary Dyskinesia, Primary, 1,Ciliary Dyskinesia, Primary, 1, With Or Without Situs Inversus,Dextrocardia, Bronchiectasis, and Sinusitis,Kartagener's Syndrome,Kartagener's Triad,Polynesian Bronchiectasis,Siewert Syndrome,Bronchiectasis, Polynesian,Kartageners Syndrome,Kartageners Triad,Polynesian Bronchiectases,Syndrome, Kartagener,Syndrome, Kartagener's,Syndrome, Siewert
D008854 Microscopy, Electron Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen. Electron Microscopy
D008870 Microtubules Slender, cylindrical filaments found in the cytoskeleton of plant and animal cells. They are composed of the protein TUBULIN and are influenced by TUBULIN MODULATORS. Microtubule
D009068 Movement The act, process, or result of passing from one place or position to another. It differs from LOCOMOTION in that locomotion is restricted to the passing of the whole body from one place to another, while movement encompasses both locomotion but also a change of the position of the whole body or any of its parts. Movement may be used with reference to humans, vertebrate and invertebrate animals, and microorganisms. Differentiate also from MOTOR ACTIVITY, movement associated with behavior. Movements
D011014 Pneumonia Infection of the lung often accompanied by inflammation. Experimental Lung Inflammation,Lobar Pneumonia,Lung Inflammation,Pneumonia, Lobar,Pneumonitis,Pulmonary Inflammation,Experimental Lung Inflammations,Inflammation, Experimental Lung,Inflammation, Lung,Inflammation, Pulmonary,Inflammations, Lung,Inflammations, Pulmonary,Lobar Pneumonias,Lung Inflammation, Experimental,Lung Inflammations,Lung Inflammations, Experimental,Pneumonias,Pneumonias, Lobar,Pneumonitides,Pulmonary Inflammations
D012008 Recurrence The return of a sign, symptom, or disease after a remission. Recrudescence,Relapse,Recrudescences,Recurrences,Relapses
D012141 Respiratory Tract Infections Invasion of the host RESPIRATORY SYSTEM by microorganisms, usually leading to pathological processes or diseases. Respiratory System Infections,Upper Respiratory Tract Infection,Upper Respiratory Tract Infections,Infections, Respiratory,Infections, Respiratory Tract,Infections, Upper Respiratory,Infections, Upper Respiratory Tract,Respiratory Infections,Upper Respiratory Infections,Infection, Respiratory System,Infection, Respiratory Tract,Respiratory Infection, Upper,Respiratory System Infection,Respiratory Tract Infection
D001980 Bronchi The larger air passages of the lungs arising from the terminal bifurcation of the TRACHEA. They include the largest two primary bronchi which branch out into secondary bronchi, and tertiary bronchi which extend into BRONCHIOLES and PULMONARY ALVEOLI. Primary Bronchi,Primary Bronchus,Secondary Bronchi,Secondary Bronchus,Tertiary Bronchi,Tertiary Bronchus,Bronchi, Primary,Bronchi, Secondary,Bronchi, Tertiary,Bronchus,Bronchus, Primary,Bronchus, Secondary,Bronchus, Tertiary
D001987 Bronchiectasis Persistent abnormal dilatation of the bronchi. Cylindrical Bronchiectasis,Cystic Bronchiectasis,Saccular Bronchiectasis,Varicose Bronchiectasis,Bronchiectases,Bronchiectasis, Cylindrical,Bronchiectasis, Cystic,Bronchiectasis, Saccular,Bronchiectasis, Varicose,Cylindrical Bronchiectases,Cystic Bronchiectases,Saccular Bronchiectases,Varicose Bronchiectases
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children

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