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Dental findings in the Schimke immuno-osseous dysplasia.

Schimke immuno-osseous dysplasia is a rare autosomal recessive disorder that affects primarily bone, T lymphocytes, kidneys, and skin. The patients have a triangular face, broad nasal bridge, bulbous nose tip, small palpebral fissures, short neck, long upper lip, and low hairline. Dental abnormalities of affected patients have not been discussed in detail. The patient described in this clinical report presented with clinical and radiographic abnormalities that may constitute a diagnostic characteristic in this condition.

Child↗

[Periodontal and esthetic aspects of orthodontic frontal space closure--results of a long-term study].

In this study follow-up results of patients are presented, who have been treated in cases of missing incisors by orthodontic space closure. The statements of clinical examinations, casts, intra- and extraoral photographies, x-rays, periodontal examinations and questionnaires pointed out, that the abnormal position of the moved teeth do not interfere with the periodontal tissues. Should the occasion arise of correction by crowns or by grinding, the patients consider the abnormal positioned teeth as esthetical.

Adult↗

A TERATOGENIC EFFECT OF A SULPHONAMIDE IN EXPERIMENTAL ANIMALS.

Sulphamoprine, when given in the diet at levels from 0.025% upwards to pregnant rats and mice, produced abnormalities of eruption of the incisor teeth and of the skull of the offspring. Rabbits are not susceptible to this teratogenic action.

Abnormalities, Drug-Induced↗

Dental characteristics of the Wolf-Hirschhorn syndrome: a case report.

Little attention in the dental literature has been given to the dental characteristics of patients with the Wolf-Hirschhorn Syndrome (WHS). The syndrome is caused by deletions of the terminal portion of the short arm of chromosome 4. This case report provides information on dental anomalies noted in a child with WHS. The dental findings include agenesis of multiple permanent teeth, particularly premolars and molars, taurodontism, and over-retained primary teeth. This syndrome exhibits variable clinical expressivity, possibly due to the extent and the specific locus of the chromosomal deletion. Further studies are required to obtain a clearer view of the clinical oral/dental manifestations of this syndrome.

Abnormalities, Multiple↗

Image accuracy of plain film radiography and computerized tomography in assessing morphological abnormality of impacted teeth.

This study compares the image accuracy of computerized tomography (CT) with that of plain film radiography (PFR) in analyzing the 3-dimensional shape of impacted teeth suspected of being malformed. Eighteen patients with 22 malformed teeth were studied by PFR and CT. Crown shape, root shape, crown/root relationship, and tooth inclination and structure were assessed by PFR and CT. CT was found to be superior to PFR in showing the multiplanar shape of tooth crown and root, crown/root relationship, and tooth inclination (P <.001). No difference was found in the representation of tooth structures. The findings demonstrate that CT imaging is useful to diagnose and plan treatment for patients with unerupted and malformed teeth.

Adolescent↗

Hypodontia, impacted permanent teeth, spinal defects, and cardiomegaly in a previously diagnosed case of the Yunis-Varon syndrome.

The Yunis-Varon syndrome is a rare hereditary disorder with ectodermal and skeletal anomalies that include agenesis or hypoplasia of the clavicles, craniofacial disproportion with severe micrognathia, digital anomalies, prenatal and postnatal growth deficiency, and neonatal death. Only 12 cases have been reported in the literature. Although neonatal death is a significant feature of this syndrome, two case reports describe children, both males, who were 30 months and 3 years of age at the time of investigation. The 30-month-old child was reexamined at 11 years of age, and had further clinical and radiologic features that included hypodontia, impacted permanent teeth, spinal defects, cardiomegaly, bilateral hearing loss, and metatarsus adductus. Children who survive the neonatal period and continue to thrive with many of the features of the Yunis-Varon syndrome as well as the new features described in this article may not have a distinct yet related syndrome.

Abnormalities, Multiple↗

Odontodysplasia, gingival manifestations, and accompanying abnormalities.

Regional odontodysplasia is an uncommon developmental dental disorder that may occasionally be accompanied by other abnormalities. A case is described in which the chief report was of a gingival enlargement arising in a female patient who also had dolichocephaly, thin calvarium, clinodactyly and transverse grooving of her fingernails, and a history of abnormal hair. Previously suggested etiologic factors and cases reported in association with other abnormalities are reviewed.

Abnormalities, Multiple↗

Relationship of abnormalities in dental and skeletal development in the osteopetrotic (os) rabbit.

Osteopetrosis is a metabolic bone disease characterized by reduced bone resorption of heterogenous cause. The rabbit mutation is lethal and exhibits ultrastructural aberrations in osteoclasts and osteoblasts together with hypocalcemia, hypophosphatemia and failure to be cured by bone marrow transplantation. We have studied dental abnormalities in mutants from birth to 3 wk using radiographic, cytologic and autoradiographic methods. Radiographs show hypoplasia of most teeth in mutants. The maxillary incisor is smaller and more curved and the mandibular incisor thin and straight compared to normal littermates. The first 3 molars in both arches are unerupted and of distorted shape while the last 2 are less affected. Microscopically areas of ankylosis of mutant incisors and the first 3 molars were commonly encountered even at birth. Osteoclasts were numerous. Autoradiograms of 3H-proline incorporation showed strong periosteal and weak endosteal labeling of bone in both mutants and normal littermates. Dentin labeling in mutant incisors and molars was not less than that in normal rabbits and sites of ankylosis in mutants exhibited labeling. These data indicate that osteopetrotic rabbits exhibit major aberrations in shape and eruption of incisors and most molars and that these effects are not due to lack of dentin formation. Early ankylosis, perhaps secondary to congenital reduction of bone resorption, appears to be the major cause of the dental abnormalities which are less severe in the youngest (posterior) teeth.

Animals↗

Dental abnormalities in rats after a single large dose of cyclophosphamide.

Delayed drug-related mortality in rats treated with a single high dose (75 mg/kg) of cyclophosphamide complicated experiments using this drug treatment. We observed that this delayed mortality was due to dental abnormalities including broken teeth, absent teeth, extra long teeth, and/or supernumerary teeth. These dental abnormalities developed about 140 days after treatment and, if left untreated, interfered with eating. Eventually, the untreated rats starved. Clipping their long teeth and feeding the rats powdered chow eliminated the deaths. Researchers should be aware that high doses of cyclophosphamide may result in dental abnormalities several months after the treatment.

Animals↗

Dens evaginatus--how does it present and how should it be managed?

Dens evaginatus is a developmental dental anomaly predominantly affecting premolars in people of Mongolian origin. It is likely to become more prevalent in New Zealand due to the increase in immigration of Asians. The formation of dens evaginatus is postulated to be an abnormal evagination of the internal enamel epithelium and dental papilla into the stellate reticulum during morphodifferentiation. An important complication resulting from damage to the evagination is periapical abscess. When considering treatment options, the long-term prognosis of the tooth, its root length, the general alignment of the teeth, and overall arch length should be reviewed. Management options include composite resin placed at the base of the tubercle, excision of the tubercle followed by direct or indirect calcium-hydroxide pulp capping and composite-resin restoration, and apexification procedures followed by conventional endodontic therapy. Grinding of the intact tubercles of newly erupted evaginated premolars may contribute to secondary infection of the pulp, and is not recommended.

Adolescent↗

Dental findings in patients with Aarskog syndrome.

Dental manifestations of the Aarskog syndrome were studied in 10 individuals. There was retarded development and eruption of the permanent teeth in six boys. Dental age was less retarded than height age and bone age. The prevalence of hypodontia and the prevalence and degree of orthodontic anomalies were higher than in the general population. Caries prevalence was high.

Abnormalities, Multiple↗