Immotile cilia syndrome: nasal mucociliary function and nasal ciliary abnormalities. 1994

M Armengot, and G Juan, and R Barona, and L Garin, and J Basterra
ENT Department, General and University Hospital, Medical School, Valencia, Spain.

We present 17 patients with a typical symptomatology of immotile cilia syndrome, seven of them with complete situs inversus. Firstly, a study of the nasal mucociliary transport was made by means of the radioisotopic technique with serum albumin-Tc99m. In all cases there was absence of transport. Secondly, we studied the ultrastructure of the nasal cilia. Defects in the dynein arms were frequently found (65%). In two cases (11%) there were no cilia; in two other cases the cilia were normal, and in another two cases alterations of the central pair of microtubules were seen. Ciliary complexes were detected in all cases. We conclude that in patients with chronic or recurrent infections of the airways without known cause we must initiate a study of the nasal mucociliary transport. If this is absent or decreased, study of ciliary ultrastructure should be carried out. If mucociliary transport is normal, immotile cilia syndrome is ruled out and ultrastructural study of the cilia is not required.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009079 Mucociliary Clearance A non-specific host defense mechanism that removes MUCUS and other material from the LUNGS by ciliary and secretory activity of the tracheobronchial submucosal glands. It is measured in vivo as mucus transfer, ciliary beat frequency, and clearance of radioactive tracers. Mucociliary Transport,Clearance, Mucociliary,Clearances, Mucociliary,Mucociliary Clearances,Mucociliary Transports,Transport, Mucociliary,Transports, Mucociliary
D009297 Nasal Mucosa The mucous lining of the NASAL CAVITY, including lining of the nostril (vestibule) and the OLFACTORY MUCOSA. Nasal mucosa consists of ciliated cells, GOBLET CELLS, brush cells, small granule cells, basal cells (STEM CELLS) and glands containing both mucous and serous cells. Nasal Epithelium,Schneiderian Membrane,Epithelium, Nasal,Membrane, Schneiderian,Mucosa, Nasal
D011877 Radionuclide Imaging The production of an image obtained by cameras that detect the radioactive emissions of an injected radionuclide as it has distributed differentially throughout tissues in the body. The image obtained from a moving detector is called a scan, while the image obtained from a stationary camera device is called a scintiphotograph. Gamma Camera Imaging,Radioisotope Scanning,Scanning, Radioisotope,Scintigraphy,Scintiphotography,Imaging, Gamma Camera,Imaging, Radionuclide
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D002923 Cilia Populations of thin, motile processes found covering the surface of ciliates (CILIOPHORA) or the free surface of the cells making up ciliated EPITHELIUM. Each cilium arises from a basic granule in the superficial layer of CYTOPLASM. The movement of cilia propels ciliates through the liquid in which they live. The movement of cilia on a ciliated epithelium serves to propel a surface layer of mucus or fluid. (King & Stansfield, A Dictionary of Genetics, 4th ed) Motile Cilia,Motile Cilium,Nodal Cilia,Nodal Cilium,Primary Cilia,Primary Cilium,Cilium,Cilia, Motile,Cilia, Nodal,Cilia, Primary,Cilium, Motile,Cilium, Nodal,Cilium, Primary
D002925 Ciliary Motility Disorders Conditions caused by abnormal CILIA movement in the body, usually causing KARTAGENER SYNDROME, chronic respiratory disorders, chronic SINUSITIS, and chronic OTITIS. Abnormal ciliary beating is likely due to defects in any of the 200 plus ciliary proteins, such as missing motor enzyme DYNEIN arms. Ciliary Dyskinesia,Ciliary Dyskinesia, Primary,Immotile Cilia Syndrome,Primary Ciliary Dyskinesia,Ciliary Dyskinesias,Ciliary Motility Disorder,Disorder, Ciliary Motility,Dyskinesia, Ciliary,Dyskinesia, Primary Ciliary,Immotile Cilia Syndromes
D005260 Female Females

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