Hallermann-Streiff syndrome: those are not supernumerary teeth.
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The Nager syndrome is a rare condition associated with craniofacial malformations such as micrognathia, zygomatic hypoplasia, cleft palate, and preaxial limb deformities. This report features a case of the Nager syndrome occurring in a 4-year-old boy showing microdontia, thumb duplication and radioulnar synostosis, and ventricular septum defect, characteristics not usually encountered in the published cases.
Atypical dentofacial structures may be the first indicator of other anomalies linked to a syndrome. This case describes the management of a 9-year-old girl referred for the routine management of supernumerary teeth. The anomalous form of her teeth, together with multiple supernumerary units and a history of congenital cataracts, were suggestive of a diagnosis of Nance-Horan syndrome. This is an X-linked disorder, in which females usually demonstrate mild expression; this case was unusual in respect to the marked phenotype expressed. Unusually, the girl developed 2 spontaneous abscesses of her noncarious upper incisor teeth; a feature never previously described in this syndrome. This report details the patient's dental management and discusses the possible pathogenesis of the dental abscesses, together with the genetic implications of this syndrome.
OBJECTIVE: To examine the prevalence of dental anomalies in the deciduous and permanent teeth, ectopic eruption of permanent first molars, and associated oral malformations in individuals with Treacher Collins syndrome. STUDY DESIGN: Clinical and radiographic examination of 15 patients with Treacher Collins syndrome, from the Hospital for Rehabilitation of Craniofacial Anomalies of the University of São Paulo, not submitted to orthodontic or orthognathic treatment. RESULTS: Dental anomalies were present in 60% of the sample, with 1-8 anomalies per individual. Hypoplastic dental anomalies were the most common, followed by heterotopic and hyperplastic dental alterations. The most frequent anomalies were tooth agenesis (33.3%), mainly affecting the mandibular second premolars, and enamel opacities (20% of patients). Ectopic eruption of the maxillary first molars was found in 13.3% of subjects. Associated oral malformations, primarily isolated cleft palate, followed by complete cleft lip and palate and macrostomia, were observed in 66.7% of patients. CONCLUSIONS: Presence of cleft palate associated with the syndrome is in agreement with the literature. The high prevalence of dental anomalies suggests a possible etiologic relationship, not described in the literature so far, between such alterations and the Treacher Collins syndrome. Further investigations on larger samples are required to confirm these results.
OBJECTIVE: To determine the prevalence and distribution of dilaceration in all tooth groups by using radiographs. STUDY DESIGN: The sample included 953 periapical intraoral radiographs and 488 panoramic radiographs from different caucasian patients. The ages of the patients ranged from 18-65 years. Dilaceration of the root was detected by measuring the degree of deviation from the long axis (deviation ł 90 degrees), and evaluating the "bull's eye" appearance. The prevalence of root dilaceration for each tooth-type was expressed in percentages. RESULTS: The teeth showing the highest prevalence of root dilaceration were mandibular third molars (24.1%), maxillary first molars (15.3%), second molars (11.4%) and third molars (8.1%). In the mandible, dilacerations were less common than in the maxilla. CONCLUSIONS: In the adult population examined in Croatia, dilaceration was most frequently found to affect posterior teeth.
Macrodontia associated with multituberculism, central cusps, and pulpal invaginations is reported in a 7-year-old Hungarian girl. Over 5 years' follow-up the problems of the diagnosis, the similarity with and relationship to the Ekman-Westborg and Julin trait, and the difficulties of the treatment are discussed.
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CSF-1 and MCP-1, released by dental follicle cells, stimulate the influx of monocytes into the follicle sac and enhance the formation of osteoclasts that, in turn, resorb alveolar bone for the eruption pathway. PDGF and bFGF, released by cells adjacent to the follicle or by activated monocytes, are prime candidates that may regulate CSF-1 and MCP-1 gene expression. The present study demonstrates that PDGF and bFGF are mitogens for dental follicle cells and stimulate CSF-1 and MCP-1 mRNA, but with different time course kinetics. Peak induction of CSF-1 mRNA was observed at 6-8h, while maximal MCP-1 induction was observed at 2h. These findings suggest that MCP-1 is an early chemotactic signal for monocytes and that subsequent release of CSF-1 may act synergistically with MCP-1 to enhance monocyte influx. Further understanding of the molecular mechanisms by which cytokines regulate CSF-1 and MCP-1 may lead to more effective treatment regimens for disorders associated with abnormal tooth eruption.
A study was conducted to identify the major candidate chromosome and to detect the region which included the candidate gene causing gutter-shaped root (GSR) in an inbred strain of mice. The candidate chromosomal analysis was performed on genetic crosses of C57L/J strain mice, which have GSR, and C57BL/6J strain mice, which have normal roots. Linkage analysis suggested that mouse chromosome 5 was one of the major candidates and therefore this chromosome was investigated in detail by individual genotyping of all backcross mice. The highest linkage was found at D5Mit161, and other high linkages were evident at D5Mit29, 321 and 427. Based on these findings, it is suggested that the gene causing GSR formation in mice maps close to these microsatellite loci.
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Wistar rats given a single intraperitoneal injection of 75 mg cyclophosphamide revealed dental anomalies in the form of shortening or lengthening of incisors, and development of supernumerary teeth. Rats given additionally 5 mg/kg b. w. of a nitrosomethylurea solution over a period of four months showed the same dental anomalies, but with relatively shorter latency times. In a supplementary investigation examining the effects of benzo(a)pyrene and nitrosomethylurea applied locally to the organon dentale, neoplastic lesions and dental dysplasia were seen.
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Oral health and systemic health are intimately related, and a thorough evaluation of the oral health of children is critical in providing appropriate health care. By understanding the normal sequence and patterns of tooth development, clinicians can readily identify children who deviate from normal dental development and provide appropriate interventions or make appropriate referrals. Developmental defects of the human dentition are not uncommon and can severely adversely affect the physical and psychological health of children. Despite the severity of some developmental defects of the dentition, the ability to diagnose and manage these conditions, in most cases, allows children the benefit of optimal oral health.
A case report is presented demonstrating a two-rooted mandibular incisor. Because of prosthetic considerations, endodontic therapy was not performed and the tooth was extracted. However, this case reinforces the importance of proper radiographic techniques and interpretation in detecting radicular anomalies.
Clinical records and radiographs were reviewed for 15 patients who had endodontic treatment performed on 16 maxillary molars with two palatal roots. These cases, plus six extracted teeth or slides, were evaluated. From the morphology of these roots, a classification of three types is proposed.
This article presents a case of a two-rooted maxillary central incisor with previous endodontic therapy. One root canal was well filled and the other partially filled. A post was present in each root and the tooth had a crown. Surgery was performed and an amalgam retrograde filling was placed. A 6-month postoperative radiograph shows repair.
A case report of a developmental anomaly known as a radicular groove is presented. This case is unusual in that it is believed to be the first reported case of complex involvement of the entire facial aspect of a tooth root. An alveolar crest to apex facial root defect in a maxillary right lateral incisor of a 12-yr-old black female led to early pulpal necrosis and periapical rarefaction. Clinical and histologic findings as well as morphologic and treatment ramifications are discussed.