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[Ichthyosis vulgaris, growth retardation, hair dysplasia, tooth abnormalities, immunologic deficiencies, psychomotor retardation and resorption disorders. Case report of 2 siblings].

Two siblings (4 and 10 years old) with ichthyosis vulgaris, growth and mental retardation, hair dysplasia, teeth abnormalities, recurrent infections and malabsorption are presented. The elder sister of the children as well as the rest of the family are unremarkable. The complex of symptoms does not fit into one of the known Ichthyosis syndromes. Microscopically the dystrophic hairs show changes in diameters, nodules, lack of the medulla, rough cuticle and splits vertical to the axis, sometimes resembling trichorrhexis nodosa. In the scanning electron-microscope there is a clear difference to Netherton's syndrome. The plasma concentrations of the vitamines A, B12 as well as of beta-carotines are low in spite of normal nutrition. During parenteral therapy with vitamin A and B12 symptoms ameliorate slightly.

Abnormalities, Multiple

Abnormal tooth tissue in human odontodysplasia.

The affected teeth in this case of odontodysplasia exhibited abnormal hypoplastic enamel, abnormal dentin containing extensive interglobular regions and completely lacking a peritubular matrix, and an abnormal irregular tissue consisting of highly calcified and partially fused granules located within the dentin of the tooth tips. Electron micrographs of the irregular tissue showed that the granules consisted of crystallites densely and radically packed in a noncollagenous amorphous matrix. Granules were surrounded by a slightly calcified, irregularly arranged, collagenous matrix. The irregular tissue formed in the pulp of the tooth tip befofe and independently of the dentin. It was at least partially formed by pulpal calcification. Abnormal dentin matrix was formed by odontoblasts which were less differentiated than normal. Odontodysplastic teeth showed abnormal differentiation of odontoblasts and ameloblasts resulting in defective enamel and dentin and, in extreme cases, in extensive pulpal calcification.

Child

Osteomatous changes and tooth abnormalities found in the jaw of patients with adenomatosis coli.

Oral findings on the panoramic tomograms of fifty-two patients with adenomatosis coli (AC) are presented. Osteomatous lesions, embedded teeth, and supernumerary teeth were found more frequently in AC patients than in a normal population. The osteomatous lesions were found in more than 80 percent of the AC patients, and the average number of such lesions per patient was 4.7. In a normal population, 15 percent had the osteomatous lesions, and the average number per patient was 1.1. The panoramic tomograms of twelve AC patients who had more than six osteomatous lesions showed very characteristic images. The possibility of early detection of AC through oral radiographic findings is suggested.

Adenoma

[An analysis of the prevalence of isolated dental anomalies and of those associated with hereditary syndromes: a model for evaluating the genetic control of the dentition characteristics].

Tooth ontogenesis, morphogenesis, and eruption are under a multifactorial (genetic, epigenetic, environmental) control. The aim of this study has been a genetic appraisal of tooth developmental characteristics by means of the prevalence and patterns of association of tooth anomalies in a large sample of hereditary syndromes. MATERIALS AND METHODS. A sample of 169 hereditary syndromes exhibiting tooth abnormalities was collected from the literature: original articles were controlled in order to achieve uniformity of judgement about the definition of the dental defects. Tooth anomalies were classified according to anatomoclinical criteria: number (hypodontia/hyperdontia), shape, position, structure (enamel/dentin/cementum), size (micro-/macrodontism), eruption (precocious/delayed including unerupted teeth). Prevalence data were calculated for: 1) modes of syndrome inheritance; 2) syndromes exhibiting a single dental anomaly vs. syndromes exhibiting associated tooth abnormalities; 3) different types of tooth abnormalities; 4) isolated and associated tooth abnormalities.

Abnormalities, Multiple

Abnormalities of tooth development in pituitary dwarfism.

Roentgenographic studies of the jaws and teeth in a group of forty-eight pituitary dwarfs showed the following abnormalities in the development of the teeth: 1. Delayed shedding of the deciduous teeth. 2. Absence of resorption of the roots of the deciduous teeth at the usual time. 3. Marked delay in eruption of the permanent teeth. 4. Retention of permanent teeth in the maxillary and mandibular shafts. 5. Development of the apical parts of roots of the retained permanent teeth and their growth toward the lower mandibular edge. 6. Displacement of the first molars from the mandibular shaft to rami. 7. Tilting of some of the retained teeth. 8. Small size of the maxilla and mandible with overcrowding of the teeth in these bones. 9. Complete absence of buds of the wisdom teeth, even in patients in the fourth decade of life. 10. Stimulation of development and eruption of the teeth after administration of anabolic drugs. These abnormalities when present in combination depend on growth hormone deficiency since they do not occur in other types of dwarfism.

Adolescent

Associated dental anomalies in an Etruscan adolescent.

Three fragments of the upper jaw of an Etruscan adolescent of the 6th century B.C. discovered at the necropolis of Cancellone 1 (Magliano in Tuscany, Grosseto, Italy) were examined. A triad of associated dental anomalies was found: congenitally missing second premolars, "peg-shaped" permanent lateral incisors, and ectopic (palatal) eruption of a permanent canine. These findings provided the opportunity to discuss etiopathogenetic aspects of the associations among different types of tooth abnormalities.

Adolescent