Biomaterial Database

Tricho-dento-osseous syndrome. Features of the hair and teeth. 1994

J T Wright, and M W Roberts, and A R Wilson, and R Kudhail

Department of Pediatric Dentistry, School of Dentistry, University of North Carolina, Chapel Hill.

Tricho-dento-osseous syndrome is a rare autosomal dominant hereditary disorder characterized by curly hair, enamel hypoplasia, taurodont teeth, and thickened cortical bone. A large kindred with this syndrome has been identified in North Carolina and characterized by pedigree analysis and clinical evaluation. Hair samples (N = 3) and an exfoliated primary tooth (N = 1) from persons with tricho-dento-osseous syndrome and hair and teeth from unaffected persons were examined. Scanning electron microscopy showed that the hair shaft morphology, diameter, and cuticular pattern and periodicity were similar for the tricho-dento-osseous syndrome and control samples. The affected tooth was examined using light microscopy and a scanning electron microscope that showed pitting hypoplasia of the enamel surface, an extremely large pulp chamber, and dentin that appeared structurally normal. The affected enamel was uniformly thin, only 10% the thickness of the control enamel. The bulk of this enamel showed no prism formation. This study indicates that although hair with tricho-dento-osseous syndrome often is clinically curly, it can be straight, and it does not appear to have unique ultrastructural features that are of diagnostic benefit. The dental characteristics of tricho-dento-osseous syndrome were the most consistent feature seen in this kindred.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D008855	Microscopy, Electron, Scanning	Microscopy in which the object is examined directly by an electron beam scanning the specimen point-by-point. The image is constructed by detecting the products of specimen interactions that are projected above the plane of the sample, such as backscattered electrons. Although SCANNING TRANSMISSION ELECTRON MICROSCOPY also scans the specimen point by point with the electron beam, the image is constructed by detecting the electrons, or their interaction products that are transmitted through the sample plane, so that is a form of TRANSMISSION ELECTRON MICROSCOPY.	Scanning Electron Microscopy,Electron Scanning Microscopy,Electron Microscopies, Scanning,Electron Microscopy, Scanning,Electron Scanning Microscopies,Microscopies, Electron Scanning,Microscopies, Scanning Electron,Microscopy, Electron Scanning,Microscopy, Scanning Electron,Scanning Electron Microscopies,Scanning Microscopies, Electron,Scanning Microscopy, Electron
D008963	Molar	The most posterior teeth on either side of the jaw, totaling eight in the deciduous dentition (2 on each side, upper and lower), and usually 12 in the permanent dentition (three on each side, upper and lower). They are grinding teeth, having large crowns and broad chewing surfaces. (Jablonski, Dictionary of Dentistry, 1992, p821)	Molars
D010375	Pedigree	The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition.	Family Tree,Genealogical Tree,Genealogic Tree,Genetic Identity,Identity, Genetic,Family Trees,Genealogic Trees,Genealogical Trees,Genetic Identities,Identities, Genetic,Tree, Family,Tree, Genealogic,Tree, Genealogical,Trees, Family,Trees, Genealogic,Trees, Genealogical
D001842	Bone and Bones	A specialized CONNECTIVE TISSUE that is the main constituent of the SKELETON. The principal cellular component of bone is comprised of OSTEOBLASTS; OSTEOCYTES; and OSTEOCLASTS, while FIBRILLAR COLLAGENS and hydroxyapatite crystals form the BONE MATRIX.	Bone Tissue,Bone and Bone,Bone,Bones,Bones and Bone,Bones and Bone Tissue,Bony Apophyses,Bony Apophysis,Condyle,Apophyses, Bony,Apophysis, Bony,Bone Tissues,Condyles,Tissue, Bone,Tissues, Bone
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D003743	Dental Enamel	A hard thin translucent layer of calcified substance which envelops and protects the dentin of the crown of the tooth. It is the hardest substance in the body and is almost entirely composed of calcium salts. Under the microscope, it is composed of thin rods (enamel prisms) held together by cementing substance, and surrounded by an enamel sheath. (From Jablonski, Dictionary of Dentistry, 1992, p286)	Enamel,Enamel Cuticle,Dental Enamels,Enamel, Dental,Enamels, Dental,Cuticle, Enamel,Cuticles, Enamel,Enamel Cuticles,Enamels
D003744	Dental Enamel Hypoplasia	An acquired or hereditary condition due to deficiency in the formation of tooth enamel (AMELOGENESIS). It is usually characterized by defective, thin, or malformed DENTAL ENAMEL. Risk factors for enamel hypoplasia include gene mutations, nutritional deficiencies, diseases, and environmental factors.	Enamel Hypoplasia,Enamel Agenesis,Enamel Hypoplasia, Dental,Hypoplasia, Dental Enamel,Hypoplastic Enamel,Agenesis, Enamel,Enamel Ageneses,Enamel Hypoplasias,Enamel, Hypoplastic,Hypoplasia, Enamel
D003786	Dental Pulp Cavity	The space in a tooth bounded by the dentin and containing the dental pulp. The portion of the cavity within the crown of the tooth is the pulp chamber; the portion within the root is the pulp canal or root canal.	Pulp Canal,Pulp Chamber,Root Canal,Cavities, Dental Pulp,Cavity, Dental Pulp,Dental Pulp Cavities,Pulp Cavities, Dental,Pulp Cavity, Dental,Canal, Pulp,Canal, Root,Canals, Pulp,Canals, Root,Chamber, Pulp,Chambers, Pulp,Pulp Canals,Pulp Chambers,Root Canals
D005260	Female		Females