Biomaterial Database

Primary mucociliary transport failure. 1988

S Umeki

Department of Medicine, Kawasaki Medical School, Okayama, Japan.

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.

UI	MeSH Term	Description	Entries
D008297	Male		Males
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
D009626	Terminology as Topic	Works about the terms, expressions, designations, or symbols used in a particular science, discipline, or specialized subject area.	Etymology,Nomenclature as Topic,Etymologies
D009845	Oligospermia	A condition of suboptimal concentration of SPERMATOZOA in the ejaculated SEMEN to ensure successful FERTILIZATION of an OVUM. In humans, oligospermia is defined as a sperm count below 20 million per milliliter semen.	Cryptospermia,Cryptozoospermia,Low Sperm Count,Hypospermatogenesis,Oligoasthenoteratozoospermia,Oligozoospermia,Cryptospermias,Cryptozoospermias,Hypospermatogeneses,Low Sperm Counts,Oligoasthenoteratozoospermias,Sperm Count, Low,Sperm Counts, Low
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
D003550	Cystic Fibrosis	An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.	Mucoviscidosis,Cystic Fibrosis of Pancreas,Fibrocystic Disease of Pancreas,Pancreatic Cystic Fibrosis,Pulmonary Cystic Fibrosis,Cystic Fibrosis, Pancreatic,Cystic Fibrosis, Pulmonary,Fibrosis, Cystic,Pancreas Fibrocystic Disease,Pancreas Fibrocystic Diseases
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000818	Animals	Unicellular or multicellular, heterotrophic organisms, that have sensation and the power of voluntary movement. Under the older five kingdom paradigm, Animalia was one of the kingdoms. Under the modern three domain model, Animalia represents one of the many groups in the domain EUKARYOTA.	Animal,Metazoa,Animalia
D013577	Syndrome	A characteristic symptom complex.	Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes