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Development of the dentition in cleidocranial dysplasia.

The purpose of the present investigation was to describe the formation, maturation and eruption of the dentition, including supernumerary teeth in a sample of patients with cleidocranial dysplasia. The dentition was evaluated from orthopantomograms, intraoral radiographs, cephalometric films, surgically removed teeth and intraoral photographs in 19 patients (9 men, 10 women), aged 3.5 to 34 years. Formation of primary teeth was normal, whereas all patients but one had supernumerary permanent teeth. Frequency of supernumerary teeth ranged from 22% in the maxillary incisor region to 5% in the molar regions. Supernumerary teeth were formed lingually and occlusally to the normal teeth. Maturation of the primary dentition was normal, while permanent teeth were delayed from 1 to 4 yr. Supernumerary teeth were delayed about 4 years in relation to normal permanent teeth. Eruption of primary teeth was normal, whereas all patients had severe eruption problems of permanent teeth. It was hypothesized that the dental lamina for both primary and permanent dentition is normal, but does not resolve completely and therefore may form supernumerary teeth. Abnormalities of tooth morphology is related to inadequate space and arrested eruption. Delayed or arrested eruption is probably caused by diminished resorption of bone and of primary teeth and to the presence of multiple supernumerary teeth.

Adolescent

The blood-vessel thrust theory of tooth eruption and migration.

The Blood-Vessel Thrust Theory is a new hypothesis regarding the forces which produce the normal eruption of teeth, and the movement of 'nonerupted' teeth through bone away from their normal position in the jaws. It points out that the flow of blood through the vessels of the dental pulp, and of the tissues surrounding the tooth, must produce hydrodynamic and hydrostatic forces within the blood vessels, and that these forces have a resultant towards the tooth crown, thus causing the tooth to move, crown first, through the bone during normal eruption or abnormal tooth migration.

Humans

Dental phenotypes associated with novel PHEX variants in X-linked hypophosphatemia.

OBJECTIVES: X-linked hypophosphatemia (XLH) is a genetic disorder related to bone, mainly due to the mutations in PHEX gene. Previous studies have reported that XLH patients had various tooth phenotypes. It is unclear whether there are any rules about these abnormal tooth phenotypes, especially in those XLH cases with PHEX mutations. The objectives of this study were to find the most representative dental characteristics of XLH and the possible phenotype-genotype correlation. DESIGN: Two unrelated patients with XLH underwent clinical, radiographic, biochemical, and genetic evaluation. Whole-exome sequencing and whole-genome sequencing were used to identify pathogenic variants. The ultrastructure of extracted teeth was analyzed using a stereomicroscope, micro-CT, and scanning electron microscopy. In addition, a PubMed search (up to January 2026) identified 22 articles involving 366 patients for descriptive phenotype comparison. RESULTS: Two novel PHEX variants were identified: a novel complex structural variant (NC_000023.11, g.22035649-22041668delins) and a novel heterozygous splice-site variant (NM_000444.6, c.850-1 G>A). Radiographic examination showed enlarged pulp chambers and irregular pulp morphology. Ultrastructural analysis revealed dentin defects, including globular dentin, irregular interglobular dentin, disrupted dentinal tubules, and exposed collagen fibrils. Literature-based analysis indicated prevalent clinical manifestations (pulp necrosis, tooth loss, periodontitis) and radiographic findings (enlarged pulp chamber, and prominent pulp horn). CONCLUSION: In these two patients, novel PHEX variants were associated with a recurrent dentin-pulp phenotype. Integrated clinical, radiographic, ultrastructural, and literature evidence supports dentin defects as a central component of the dental phenotype in XLH and underscores the importance of early dental assessment.

Humans

[Oral rehabilitation in dentinogenesis imperfecta. Report of a case].

We present a case of Amelogenesis imperfecta associated with Dentinogenesis imperfecta, affecting the primary dentition which is rehabilitated under general anesthesia. Dentinogenesis imperfecta is a tooth abnormality which presents clinical, radiological and histological characteristics, they should be recognized by the dentist who will determine the treatment depending on age and grade of affection. In the primary dentition we recommend the use of stainless steel crowns do to it's resistance and easy adaptation which will remain in the mouth until it's normal exfoliation.

Child, Preschool

New cases of dermoodontodysplasia?

We report on 2 sisters and one brother with severe dental anomalies, trichodysplasia, onychodysplasia, and slight skin alterations. Four other relatives have only mild dental anomalies. Differential diagnosis includes 3 other ectodermal dysplasias: hypodontia and nail dysgenesis, dermoodontodysplasia, and trichodermodysplasia with dental alterations. Cause is unknown.

Abnormalities, Multiple

The relationship of buccal pits to caries formation and tooth loss.

It is demonstrated that in mandibular molars there is a statistically significant tendency for teeth with buccal pits to be lost premortem more frequently than teeth without buccal pits. The mandibles of a large ossuary population (ca. 1600 A.D.) are examined with regard to buccal pitting, caries formation and premortem tooth loss. A log likelihood ratio test is used to test the relationship between age and frequency of buccal pits. A G-value of 20.84 (p less than 0.025) indicates that the frequency of pits is significantly higher among individuals under ca. 18 years. It is argued that caries formation is the mechanism through which the molars are lost, given high caries frequencies that approximate pitting frequencies in their distribution.

Age Factors

Non-carious interproximal grooves in Arikara Indian dentitions.

The dentitions of adult Arikara Indians from the Larson site (39WW2) were examined to determine the frequency and etiology of noncarious interproximal grooves. The observations included groove morphology, loci and association with dental pathology. Approximately 30% of the individuals exhibit one or more pronounced grooves. The use of dental probes in conjunction with dietary grit is the likely responsible for interproximal grooves in this population sample.

Anthropology, Physical

Health and differential survival in prehistoric populations: prenatal dental defects.

Linear hypoplasia of the deciduous teeth is rare in most human populations, but common where nutritional status is poor. Deciduous enamel hypoplasia, hypocalcification, and hypoplasia-related caries are described in Middle and Late Woodland skeletal series from the Lower Illinois Valley. Gross enamel defects that can be referred to pre-natal development are found in 83 of 170 children under six years of age at death. Circular caries secondary to hypoplasia is significantly more common in the Late Woodland series, reflecting the apparent higher cariogenicity of Late Woodland diets. There is a significant association between prenatal dental defects and bony evidence for anemia and infectious disease. Children with enamel defects show relatively higher weaning age mortality than those without. These relationships suggest that at least moderate levels of malnutrition existed in Illinois Woodland populations.

Adult

[The surgical-orthodontic classification of retained and dystopic teeth of the second dentition in dysostosis cleidocranialis].

Dysostosis cleidocranialis concerns both teeth and jaw and is characterized by supernumerary teeth, dentitio tarda, tooth impaction and eventually follicular cysts. The concept of treatment can be divided in two stages: 1. At the beginning of the delayed secondary dentition (dentitio tarda) the operative removal of the supernumerary tooth germs and 2. corresponding to the belated morphological development (dentitio tarda) the operative exposure of the impacted teeth of the secondary dentition. The first operation facilitates a morphological development and especially a vertical drift without any obstacles. The second becomes necessary when only the morphological development, but not the vertical drift of the teeth of the secondary dentition takes place. The operative exposure is done using a self-developed technique [13, 18]. At the same time as the exposed teeth undergo a spontaneous vertical drift, orthopedic treatment for anomaly is started.

Adolescent