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PubMed2001

Trisomy 17p10-p12 resulting from a supernumerary marker chromosome derived from chromosome 17: molecular analysis and delineation of the phenotype.

We report a 5-year-old boy with a small de novo marker chromosome derived from the proximal short arm of chromosome 17. His clinical features include hypotonia, global developmental delay, oval face with large nose and prominent ears, and ligamentous laxity of the fingers. Magnetic resonance imaging of the brain demonstrated mildly delayed myelination. G-band chromosome analysis revealed mosaicism for a small marker chromosome in 85% of the peripheral blood cells analyzed. Fluorescence in situ hybridization and microsatellite polymorphism studies showed that the der(17) was of maternal origin and included genetic material from the 17p10-p12 region, but did not contain the PMP22 gene. One breakpoint mapped within the centromere and the second breakpoint mapped adjacent to the Charcot-Marie-Tooth disease type 1A proximal low-copy repeat (CMT1A-REP). We compare the clinical characteristics of our patient with those previously reported to have a duplication involving the proximal short arm region of chromosome 17 to further delineate the phenotype of trisomy 17pl0-p12.

Charcot-Marie-Tooth Disease↗

PubMed1997

Heterozygous peripheral myelin protein 22-deficient mice are affected by a progressive demyelinating tomaculous neuropathy.

Hereditary neuropathy with liability to pressure palsy (HNPP) is associated with a heterozygous 1.5 megabase deletion on chromosome 17 that includes the peripheral myelin protein (PMP) gene PMP22. We show that heterozygous PMP22 knock-out mice, which carry only one functional pmp22 allele and thus genetically mimic HNPP closely, display similar morphological and electrophysiological features as observed in HNPP nerves. As reported previously, focal hypermyelinating structures called tomacula, the pathological hallmarks of HNPP, develop progressively in young PMP22(+/0) mice. By following the fate of tomacula during aging, we demonstrate now that these mutant animals are also interesting models for examining HNPP disease mechanisms. Subtle electrophysiological abnormalities are detected in PMP22(+/0) mice >1 year old, and a significant number of abnormally swollen and degenerating tomacula are present. Thinly myelinated axons and supernumerary Schwann cells forming onion bulbs as fingerprints of repeated cycles of demyelination and remyelination are also encountered frequently. Quantitative analyses using electron microscopy on cross sections and light microscopy on single teased nerve fibers suggest that tomacula are intrinsically unstable structures that are prone to degeneration; however, the severity of morphological and electrophysiological abnormalities in PMP22(+/0) mice is variable. These combined findings are reminiscent of the disease progression in HNPP and offer a possible explanation about why some HNPP patients develop a chronic motor and sensory neuropathy later in life that resembles demyelinating forms of Charcot-Marie-Tooth disease by both morphological and clinical criteria.

Aging↗

PubMed1998

Malocclusion, dental injuries and dental anomalies in the primary dentition of Belgian children.

AIM: To estimate the prevalence of malocclusion, dental injuries and dental anomalies in a sample of 3-5-year-old Belgian children. DESIGN: A cross-sectional study of 3-5-year-olds attending kindergartens in the municipality of Leuven, Belgium. METHODS: A total of 750 boys and girls participated in the study. The children were examined at the University School Health Centre in connection with their obligatory medical check-up. The clinical examination was performed by one examiner using generally accepted criteria for these oral conditions. RESULTS: 10.1% of the examined children had posterior cross-bite whereas over-bite was seen in only 2.0% of the sample. Open-bite was detected in 32.0% of the studied population. Boys showed a tendency for a higher frequency of malocclusions than girls. Traumatic injuries were identified in 18.0% of children. These were almost entirely restricted to maxillary incisors. Crown fractures were responsible for 42% of all injuries. The following dental anomalies were seen: six cases of supernumerary teeth, three cases of hypodontia, five cases of double teeth and one case of conical maxillary lateral incisor. CONCLUSION: Our findings emphasize the importance of early detection of these oral conditions in order to permit effective and long-term planning, according to the child's individual requirements.

Belgium↗

PubMed1990

How teeth erupt.

The cause of delayed eruption is usually obvious and treatable (as with lack of space or impeding supernumerary teeth). However, in some cases there is no obvious cause. Treatment of these cases might be easier if we fully understood the mechanism of eruption itself. Unfortunately, the precise process of generating the eruptive force for teeth has not yet been fully elucidated. Various hypotheses have been put forward. In this article the author reviews current theories, and describes some of the experimental difficulties in testing them.

Humans↗

PubMed2006

Occlusal/dental anomalies found in a random sample of Nigerian schoolchildren.

OBJECTIVE: The aim of this study was to assess the occlusal/dental anomalies needing early treatment for maximal occlusal development among 11-12-year-olds in Ibadan, Nigeria. MATERIALS AND METHODS: An epidemiological survey of 361 school students, 171 (47.4%) males and 190 (52.6%) females, was carried out in Ibadan, Nigeria. Subjects were randomly selected from different schools in the city. One examiner, under natural illumination in the school premises, examined all the children. RESULTS: Prolonged retention of primary teeth with displacement of the permanent series was observed in 4.2% while dental anterior cross bite accounted for 5.5%. Oral habits with their deleterious effects on the occlusion were noted in 5.0%. Other findings were: clinically missing permanent teeth, 3.6%; supernumerary teeth, 1.4%; double teeth, 1.9%; carious lesions, 6.9%; conically shaped lateral incisors, 1.4%; and transposition, 0.6%. Proclination of the upper incisors with increased overjets of more than 5mm, as well as some fractures of the incisors, accounted for 22.4%. In all, 51.8% had one form of occlusal/dental need or another. No statistically significant gender differences were observed for all the various needs (p > 0.05) except the prevalence of supernumerary teeth, which was statistically higher in males (p < 0.05). CONCLUSION: Over half of the children could benefit from one interceptive need or the other for proper occlusal development. Routine dental check ups are very much encouraged in developing countries like Nigeria, as in other developed parts of the world.

Anodontia↗

PubMed2004

Cleido cranial dysplasia: report of a family.

A family case of Cleidocranial Dysplasia is presented. A mother and two adolescent girls were examined. In all three cases, a radiological series was performed over the entire body. Generalized dysplasia in bones, prolonged retention of primary teeth, and delayed eruption of permanent, as well as supernumerary teeth was diagnosed. The citogenetic study with GTG band showed normal 46, XX. Bilateral audiometry in the mother demonstrated a mild to moderate hypoacustic condition. Radiological findings are presented and the importance of early diagnosis is discussed.

Adolescent↗

PubMed1992

Dens evaginatus in the Vietnamese.

Dens evaginatus (supernumerary cusps in premolars) were detected in 50 of 4677 Vietnamese patients examined. A systematic study including morphologic, histologic, pathologic aspects of dens evaginatus was carried out and data were compared with those given in previous reports pertaining to dens evaginatus in other Asian countries. It is expected that a better knowledge concerning this anomaly will lead to appropriate measures for prophylactic treatment of dens evaginatus.

Adolescent↗

PubMed1984

Natal and neonatal teeth: a clinical study.

In a survey of 18,155 infants, 7,155 of whom were examined personally, the prevalence of natal teeth was one in 716 for the infants examined and one in 3,667 for those surveyed. Another group of 38 infants and children with 61 natal and neonatal teeth were examined prospectively. All were mandibular central incisors. Sixty-one percent of the subjects had a pair of natal or neonatal teeth (or both). Ninety-five percent of the natal and neonatal teeth were normal primary central incisors, and 5% were supernumerary primary central incisors. Initially, in 31% of the teeth, enamel dysplasia was seen, the occurrence of which correlated with the duration of the gingival covering. It is recommended that natal and neonatal teeth be left in place, if possible, and removed only if they are extremely mobile, such that reattachment is unlikely.

Age Factors↗

PubMed2003

Odontoma: a clinicopathologic study of 81 cases.

BACKGROUND AND PURPOSE: Odontoma is the most common odontogenic tumor. It includes 2 types, the compound and complex odontomas. There has not been a series study of the clinical and histologic features of odontomas from Taiwan. This study evaluated the clinicopathologic features of odontoma in Taiwanese. METHODS: Cases of odontoma treated from 1998 to 2002 identified from medical records were included. The microscopic features, radiographic features, and clinical history of the patients were reviewed and analyzed. RESULTS: A total of 81 odontomas in 81 patients (36 males and 45 females) were included. There were 62 compound and 19 complex odontomas. The mean age of the patients was 18 years with the majority of odontomas occurring in the first (32%) and second decade (38%) of life. Odontomas had a marked predilection for the maxilla (70%) and for the anterior region of the jaw (83%), particularly for the anterior maxilla (62%). Sixty four (79%) of the 81 odontomas were associated with 80 impacted teeth, including 71 permanent teeth, 2 deciduous teeth, and 7 supernumerary teeth. Of the 71 impacted permanent teeth, the maxillary central incisor (27%) was most commonly affected, followed by the maxillary canine (26%) and mandibular canine (24%). Histologic examination revealed enamel matrix in 90%, dentin in 100%, cementum in 88%, pulp tissue in 96%, fibrous capsule in 93%, ghost cells in 83%, reduced enamel epithelium in 86%, and nests of odontogenic epithelium in 58% of odontomas. Dentigerous cyst was associated with 9% of odontomas. CONCLUSIONS: In this series, odontomas occurred most often in the first and second decade of life. Although complex odontomas are usually found in the posterior jaw, in this Taiwanese series they were most commonly found in the anterior maxilla. Odontoma is frequently associated with an impacted tooth and occasionally with a dentigerous cyst. No recurrence of odontomas was found after surgical excision with follow-up of 1 to 15 years.

Adolescent↗

PubMed1989

Dental abnormalities and bone lesions associated with familial adenomatous polyposis: report of cases.

Dental abnormalities and bone lesions of the maxilla and mandible are found in more than 70% of patients with familial adenomatous polyposis (FAP). Dental abnormalities include impacted teeth (other than third molars), supernumerary teeth, congenitally missing teeth, and fused roots of molars. Bone lesions consist of osteomas--isolated or in clusters--in the maxilla and mandible. In this study of 24 patients, 75% had demonstrable dental abnormalities, and 79% had osteomas of the maxilla and mandible. Because dental abnormalities and bone lesions are present early in life, these features may be helpful in diagnosing this syndrome.

Adenomatous Polyposis Coli↗

PubMed2006

Cleidocranial dysplasia: 2 generations of management.

Patients with cleidocranial dysplasia (CCD) commonly present with significant dental problems, such as retention of multiple deciduous teeth, impaction or delay in eruption of permanent teeth and, often, the presence of supernumerary teeth. Several approaches have been described for the management of such patients. We report 2 cases illustrating the shift in the management paradigm from edentulation and prosthetic replacement to orthodontically assisted forced eruption and fixed appliance orthodontic treatment combined with orthognathic surgery.

Adult↗

PubMed1993

A panoramatomographic study of the teeth and jaws of Finnish university students.

A random sample of panoramic radiographs was taken of 1027 (386 male and 641 female) university students. All the students were born between 1965 and 1971. The following were examined: caries, apical radiolucencies, endodontically treated teeth, marginal and vertical bone loss, third molars, supernumerary, missing, and persisting teeth, bone structure, condyles, maxillary sinuses and soft tissue calcifications. In the inter- (of two examiners) and intra-examiner variation Kappa indices varied between 0.15 and 1.00, and agreement percentages from 52, to 100. Kappa index was poor (0.15) in evaluation of the bone structure. The mean DMFT index for the students was 8.4. A sound dentition was found in 6.6%, and 48.4% of the students were free of caries. The mean number of carious lesions was 1.1. There were 50 retained roots (49 from primary teeth). 76.6% of the students had one or more unerupted wisdom teeth and 22.7% had radiologic changes typical of pericoronitis in the region of the mandibular third molars. 16.2% of the students had at least one missing wisdom tooth. Bone structure was normal in 92.4% of the students, idiopathic osteosclerosis being the most common divergence (3.5%). Arthrotic changes in the condyles were found in 9% of the students. In eight cases there was erosion of the condyles, which was diagnosed as arthritis. Styloid ligament calcifications were found in 21.5% of the students. In the maxillary sinuses, male students exhibited changes 1.7 times more often than female students (26.3% in males and 15.1% in females).

Adult↗

PubMed1989

Screening panoramic radiographs in children: prevalence data and implications.

The purpose of this paper was to review the rationale for the radiographic screening of asymptomatic pediatric patients and to report the prevalence of selected pathologic and developmental conditions using panoramic radiographs. Three observers participated in this retrospective study that utilized panoramic radiographs from 849 subjects, aged 3-9 years, chosen randomly from the School of Dentistry treatment records of the University of North Carolina at Chapel Hill. Findings indicated that 2.4% of the subjects had supernumerary teeth, 7.8% were missing permanent teeth, 9.1% had ectopic eruption, 0.1% had radiolucencies of the jaws, and 0.1% had radiopacities of the jaws. These prevalences are discussed in light of recent evidence concerning the risk/benefit ratio of the panoramic radiograph. We conclude that the panoramic radiograph is a poor projection for screening the dental needs of asymptomatic healthy children; alternative screening protocols should be examined.

Child↗

PubMed1977

Prevalence of delayed emergence of permanent teeth as a result of local factors.

Delayed emergence of permanent teeth as a result of local etiologic factors was found in 45 of 1,032(4.3%) persons ages 8 through 18 in a dentally indigent population. In 36 persons (3.4%) delayed emergence resulted from causes other than those associated with premature primary molar extraction. This statistic probably approximates the potential for this problem in a treated population group. Teeth most commonly involved were mandibular second premolars, maxillary canines, and maxillary central incisors. The most common causes, respectively, were space loss, palatal position, and mesiodens. Supernumerary, malformed, and congenitally missing teeth were more frequent in persons with delayed emergence than in the rest of the population sampled. The difference was statistically significant. No correlation was found between delayed emergence and sex of the patient. In this study, teeth delayed in emergence behaved as other teeth in that they exhibited normal root development and did not contribute to resorption of adjacent roots. Except for maxillary canines, enlarged follicular spaces did not develop after eruption

Adolescent↗

PubMed1989

Caries, gingivitis, and dental abnormalities in preschool children with cleft lip and/or palate.

Oral health was studied in 49 children aged 5 or 6 years old with clefts of the lip and/or palate (CL(P] and 49 healthy controls matched for sex and age. The results showed a statistically significant increase in the prevalence and activity of caries in CL(P) children. The mean number of decayed and filled surfaces in the CL(P) group was 7.0 compared with 3.9 in the control group (p less than 0.05). The most evident difference between the two groups was found in the number of decayed proximal surfaces. The mean number of decayed proximal surfaces in the CL(P) group was 2.5, as compared with 0.9 in the control group (p less than 0.001). There were no significant differences in the caries prevalence and activity in children with clefts that involves the alveolus compared with those of children with isolated clefts of the lip or palate. The CL(P) children also exhibited a significant increase (p greater than 0.01) in the number of gingival units with gingivitis. Other dental abnormalities included an increased frequency of enamel hypomineralization (p less than 0.05), supernumerary teeth (p less than 0.01), unilateral crossbite (p less than 0.001), mesial terminal plane (p less than 0.01), and crowding (p less than 0.001). The results show that the CL(P) children must be considered as a group with an increased caries risk and should therefore be subjected to an additional preventive program.

Child↗

PubMed1999

The diagnostic value of routine intra-oral premaxillary radiographs in orthodontic assessment.

OBJECTIVES: The purpose of this study was to determine whether significant dental abnormalities are likely to be overlooked in the anterior region of the maxilla if, in the absence of any clinical indication for further views, the only radiograph used during an initial orthodontic assessment is a good-quality orthopantomogram (OPT). SAMPLE AND METHODS: Two orthodontists examined, retrospectively, the written and radiographic dental hospital records of 1169 consecutive, new, young patients who on their first visit to a dental teaching hospital underwent radiographic examination comprising an OPT and one or more supplementary radiographs of the anterior maxilla. RESULTS: In five cases (0.43%) significant findings would have been overlooked if the intra-oral views had not been taken. These included periapical lesions and supernumerary teeth, but in three of the cases the image quality of the OPT was poor. CONCLUSIONS: If the OPT is not routinely supplemented by intra-oral views, the chances of completely missing significant findings in the anterior maxilla are small, provided a thorough history and clinical examination have been completed and the image quality of the OPT is good.

Adolescent↗

PubMed1976

[General anesthesia for multiple dental extractions].

Between 1970 and 1975, 528 sessions of teeth extractions were carried out under general anaesthesia at the Ambroise Paré Hospital in Boulogne. The distribution was as follows: -70 multiple dental extractions in cardiac patients, mostly sent up by cardiology units outside the establishment. -458 multiple dental extractions for orthodontic treatment, germectomies, removal of included or supernumerary teeth, contra-indication or refusal of local anaesthetic. Since 1972, all patients for whom a surgical decision was considered were examined at the Anaesthetic out-patients and the advantages appear obvious. The type of premedication, the choice of the anesthetic technique were classical in the vast majority of cases and, in particular, intubation was carried out as a routine.

Adolescent↗

PubMed1992

Alteration of murine odontogenic patterning and prolongation of expression of epidermal growth factor mRNA by retinol in vitro.

Retinoids alter the patterning of murine odontogenesis in vivo and stimulate epithelial proliferation. Because odontogenesis is dependent on proliferation of mandibular epithelium, the effects of retinol on the patterning of odontogenic epithelium were studied. These experiments control for developmental stage, applied retinoid concentration and duration of exposure. Explants exposed for 24 h to 0.1 micrograms/ml of retinol exhibited enhanced odontogenesis. Day-9 mandibles exposed to retinol at 1-5 micrograms/ml had altered epithelial patterns consistent with those in previous in vivo experiments, including supernumerary epithelial buds in regions associated with supernumerary incisors in vivo. These changes were associated with a dose-dependent increase in epithelial proliferation and a prolonged expression of epidermal growth factor (EGF) mRNA. Altered expression of EGF mRNA may be responsible for the disrupted pattern of the dental lamina. This is the first report of a retinoid-induced alteration in EGF mRNA expression.

Animals↗