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PubMed1987

Dental and bone abnormalities in patients with familial polyposis coli.

Dental and bone abnormalities of the maxilla and mandible are present in approximately 80% of patients with familial polyposis coli. The dental abnormalities include impacted teeth (other than third molars), supernumerary teeth, congenitally missing teeth, fused roots of first and second molars, and unusually long and tapered roots of posterior teeth. The bone lesions consist mostly of osteomas, either isolated or in clusters, in the maxilla and mandible or of exostoses with lateral and/or lingual extensions. Since dental and bone abnormalities are already present early in life there is a strong suggestion that they may be used as diagnostic features in the recognition of familial polyposis coli.

Adenomatous Polyposis Coli↗

PubMed1990

[Surgical and orthodontic treatment approach for cleidocranial dysostosis].

Developmental deformities of the permanent dentition are of primary concern in the treatment of patients suffering from cleidocranial dysostosis. The therapeutic approach should be based on interdisciplinary cooperation between orthodontists as well as oral and maxillofacial surgeons. Therapy may include surgical exposure of impacted teeth in combination with removal of supernumerary teeth. Already during the surgical procedure orthodontic bands and brackets may be attached. The next step in treatment should consist of orthodontic alignment of the teeth and correction of the jaw relations. In most cases, midfacial hypoplasia and corresponding abnormal jaw relations are also present. This deformity is best treated with midfacial osteotomy. Following this second surgical intervention, orthodontic treatment is completed in order to establish an optimal result regarding function and esthetics.

Adult↗

PubMed2001

Identification of a novel frameshift mutation (383insT) in the RUNX2 (PEBP2 alpha/CBFA1/AML3) gene in a Japanese patient with cleidocranial dysplasia.

Cleidocranial dysplasia (CCD) is an autosomal dominant disorder due to mutations in runt-related gene 2 (RUNX2)/polyomavirus enhancer-binding protein 2alphaA (PEBP2alphaA)/core-binding factor A1 (CBFA1)/acute myeloid leukemia 3 (AML3). To investigate the RUNX2 mutations in a Japanese patient with classic CCD, we analyzed the RUNX2 gene using polymerase chain reaction (PCR)-single-strand conformation polymorphism and PCR-restriction fragment length polymorphism. The patient had hypoplasia of the clavicles, patent fontanelles, short stature, supernumerary teeth, and retention of deciduous dentition. We identified a 1-bp insertion (383insT) at codon 128 of the RUNX2 gene. The 383T insertion affects the conserved residue in the runt domain and results in premature termination in the runt domain.

Adolescent↗

PubMed2005

Incontinentia pigmenti (IP2): familiar case report with affected men. Literature review.

UNLABELLED: Incontinentia pigmenti is a genodermatosis described by Garrod and in 1920 by Bloch, Sulzberger, Siemens y Bardach. It is an ectodermic disorder that affects skin, teeth, eyes and may also have neurological problems. The IP2 name describes the histological characteristics, the incontinence of melanin into the melanocytes cells in the epidermal basal layer and its presence in superficial dermis. IP2 is an x-linked dominant condition but genetic heterogeneity may exist. CASE REPORT: The patient was 4 yrs 5 months old when she came for the first time. In a physical exploration she presented sparse and thin hair, eyelashes and eyebrows, beaked nose, labial protrusion, the four central teeth have a conic crown and there was also a delayed eruption of other teeth, right eye strabismus, hipoacusia, language defects and a trunk, legs, feet, and face dermatosis characterized by grouped vesicles, hyperkeratotic and warty lesions and brownish-gray lesions in a lineal pattern. The patient s father had hypopigmented lesions in the posterior regions of both legs. The oral clinical and radiographic exams showed diverse anomalies. Both the patient's and the father's chromosomal studies were normal. DISCUSSION: In the present case we can see that the father has IP2 without supernumeraries X, with the antecedent that his mother had something similar. It is possible that the inheritance was autosomic dominant or it is a different mutation of NEMO (NF-kappa-B essential modulator) gene to a classical one, which was found in some affected men. It is necessary to carry out a molecular study of these patients.

Child, Preschool↗

PubMed1974

Congenital X-linked cataract, dental anomalies and brachymetacarpalia.

An investigation of a family with congenital X-linked cataracts, microcorneas, supernumerary incisors, anteverted pinnae and shortened metacarpals is presented. The carriers had posterior sutural opacities and cone-shaped teeth. There have been four previously reported families with X-linked cataracts, one of which also presented microcorneas. Four other families have been reported with X-linked cataracts, microphthalmia and somatic and mental anomalies. Linkage studies could be used to demonstrate whether these diseases result from allelic or nonallelic genes but were uniformative in the present family.

Cataract↗

PubMed1986

Gardner's dento-maxillary stigmas in patients with familial adenomatosis coli.

Fifty patients with familial adenomatosis coli (FAC) studied by panoramic tomography (PTG) showed osteomatous jaw changes in 82% as compared with 10% in matched controls. Supernumerary teeth, compound odontomas and/or impacted teeth were seen in 30% of the patients compared with 4% of the controls. A correlation was found between dental abnormalities and the large number of osteomas. The results support the view that both osteomas of the jaws and dental abnormalities are features of FAC. PTG of the jaws may serve as a valuable tool for early detection of FAC by oral surgeons, at least in the case of the most striking changes (about 20%).

Adenomatous Polyposis Coli↗

PubMed1994

Dental and craniofacial findings in hypohidrotic ectodermal dysplasia during the primary dentition phase.

A more detailed knowledge of dental and craniofacial features in hypohidrotic ectodermal dysplasia (HED) during childhood is needed in order to include these characteristics among diagnostic criteria. The present study comprised 5 HED children, 4 males and 1 female, during the primary dentition phase. Clinical and radiological dental findings consisted of multiple congenitally missing primary teeth, conoid primary incisors, moderate to severe taurodontic second primary molars. Supernumerary cusps and diastema were found as well. A pattern of symmetry was assessed for hypodontia in the primary dentition. The cephalometric study compared the HED sample to a matched non-syndrome sample and revealed abnormally short maxillary depth (p < 0.05), strongly reduced lower facial height (p < 0.01) and a reduction in facial soft tissue thickness (p < 0.05 - p < 0.01) in HED children. The importance of an early diagnosis and treatment of HED dento-facial malformations so as to improve esthetics and function is stressed.

Anodontia↗

PubMedDate not supplied

Hallerman-Streiff syndrome: case report and recommendations for dental care.

Hallerman-Streiff syndrome is a rare congenital anomaly characterized by a peculiar bird facies, mandibular and maxillary hypoplasia, dyscephaly, cataracts, microphtalmia, hypotrichosis, skin atrophy, and short stature. Dental abnormalities are present in 80 percent of the cases and include malocclusion, crowding, severe caries, supernumerary and neonatal teeth, enamel hypoplasia, hypodontia, premature eruption of primary dentition, agenesis of permanent teeth, and anterior displacement or absence of condyles. Very few cases have been described in the dental literature. The predisposition to severe caries, together with other problems, makes it imperative that young patients be started in a strong prevention program as early as possible. This is a case report of a five-year-eleven-month-old white male who presented for a dental examination at The Children's Hospital in Denver, CO. The findings and recommendations for treatment are discussed.

Anodontia↗

PubMed2003

Stimulation of ectodermal organ development by Ectodysplasin-A1.

Organs developing as ectodermal appendages share similar early morphogenesis and molecular mechanisms. Ectodysplasin, a signaling molecule belonging to the tumor necrosis factor family, and its receptor Edar are required for normal development of several ectodermal organs in humans and mice. We have overexpressed two splice forms of ectodysplasin, Eda-A1 and Eda-A2, binding to Edar and another TNF receptor, Xedar, respectively, under the keratin 14 (K14) promoter in the ectoderm of transgenic mice. Eda-A2 overexpression did not cause a detectable phenotype. On the contrary, overexpression of Eda-A1 resulted in alterations in a variety of ectodermal organs, most notably in extra organs. Hair development was initiated continuously from E14 until birth, and in addition, the transgenic mice had supernumerary teeth and mammary glands, phenotypes not reported previously in transgenic mice. Also, hair composition and structure was abnormal, and the cycling of hairs was altered so that the growth phase (anagen) was prolonged. Both hairs and nails grew longer than normal. Molar teeth were of abnormal shape, and enamel formation was severely disturbed in incisors. Furthermore, sweat gland function was stimulated and sebaceous glands were enlarged. We conclude that ectodysplasin-Edar signaling has several roles in ectodermal organ development controlling their initiation, as well as morphogenesis and differentiation.

Animals↗

PubMed1995

Spatial distribution of epidermal growth-factor transcripts and effects of exogenous epidermal growth factor on the pattern of the mouse dental lamina.

The initiation of odontogenesis is characterized by the site-specific proliferation of mandibular epithelium in the formation of the dental lamina. The epidermal growth factor (EGF) gene is expressed in the developing mandible immediately before the appearance of the dental lamina. This expression is necessary for the formation of the dental lamina and subsequent development of teeth. Previous work has demonstrated that retinoids and EGF may interact in the establishment of the pattern of the dentition. In the present study explanted mandibles that were treated with exogenous EGF (40 ng/ml of medium) contained supernumerary buds of mandibular epithelium in the diastema region. These pattern changes were the same as in previous retinoid-treated explants. These results, in addition to the previously reported effects of retinoids on the expression of the EGF gene, support the hypothesis that retinoids and EGF interact in controlling, at least in part, the pattern of the dentition by affecting the pattern of the dental lamina. The spatial distribution of EGF transcripts was also characterized. The location of EGF transcripts in the mesenchyme adjacent to the mandibular epithelium suggests a paracrine mechanism in the stimulation of epithelial proliferation in the formation of the dental lamina.

Animals↗

PubMed1999

Mutation analysis of core binding factor A1 in patients with cleidocranial dysplasia.

Cleidocranial dysplasia (CCD) is a dominantly inherited disorder characterized by patent fontanelles, wide cranial sutures, hypoplasia of clavicles, short stature, supernumerary teeth, and other skeletal anomalies. We recently demonstrated that mutations in the transcription factor CBFA1, on chromosome 6p21, are associated with CCD. We have now analyzed the CBFA1 gene in 42 unrelated patients with CCD. In 18 patients, mutations were detected in the coding region of the CBFA1 gene, including 8 frameshift, 2 nonsense, and 9 missense mutations, as well as 2 novel polymorphisms. A cluster of missense mutations at arginine 225 (R225) identifies this residue as crucial for CBFA1 function. In vitro green fluorescent protein fusion studies show that R225 mutations interfere with nuclear accumulation of CBFA1 protein. There is no phenotypic difference between patients with deletions or frameshifts and those with other intragenic mutations, suggesting that CCD is generally caused by haploinsufficiency. However, we were able to extend the CCD phenotypic spectrum. A missense mutation identified in one family with supernumerary teeth and a radiologically normal skeleton indicates that mutations in CBFA1 can be associated exclusively with a dental phenotype. In addition, one patient with severe CCD and a frameshift mutation in codon 402 had osteoporosis leading to recurrent bone fractures and scoliosis, providing first evidence that CBFA1 may help maintain adult bone, in addition to its function in bone development.

Bone and Bones↗

PubMedDate not supplied

[Gardner syndrome].

Three patients, sisters of B, 12 and 14 years of age with a diagnosis of Gardner's Syndrome are reported. The family history indicated that their mother died of a carcinoma of the intestine at the age of 30. This Syndrome, which is of dominant autosomic inheritance, can be seen in aur city, with so heterogeneous hereditary features. Dentists must know the oral cavity characteristics of this disease (odontomas, cementomas, cysts, supernumerary teeth) that accompany gastric, intestinal, skeletal and tissular disturbances an of other organs (polyps, osteomas, cysts, adenomas), being intestinal poliposis of the colon the most important feature that becomes an "Adenocarcinoma", in the third ald fourth decade in a 100% of the cases. Its incidence in our city is real. The proper diagnosis is made when the clinical study is completed and the dentist may be the first one to watch this syndrome's feature in the oral cavity. A proper medical diagnosis would allow a preventive procedure and a patient's control until the "total surgery of the colon", which is the appropriate therapeutic procedure, is achieved; although there are controversies as regards the time to be carried out.

Adolescent↗

PubMedDate not supplied

Dental development and molar root length in children with cleidocranial dysplasia.

Cleidocranial dysplasia (CD) is an autosomal dominant skeletal disorder with characteristic dental findings of numerous supernumerary teeth and noneruption of permanent teeth. This investigation compared the dental development and root lengths of the mandibular first permanent molar in 11 CD patients with those of 22 healthy, normal children matched for race, age and sex. The results showed that children with CD experienced a delay in dental development of approximately 3 years compared with normal children (P < 0.05). In addition, the root lengths of the mandibular first permanent molar were significantly longer than those of the comparison children (17.8 +/- 1.6 mm vs 13.6 +/- 1.2 mm (P < 0.001). This study thus revealed two significant clinical features of CD: 1) severe delay in dental development, and 2) excessive root lengths of mandibular permanent first molars. These features may be important in the pathogenesis of delayed dental eruption observed in this disorder.

Adolescent↗

PubMed2003

Dentomaxillofacial variability of cleidocranial dysplasia: clinicoradiological presentation and systematic review.

OBJECTIVES: The aim of this study was to determine the clinical and radiological presentation of cleidocranial dysplasia (CCD) in our patient group and to compare them with other reported cases by a systematic review (SR) of the literature. METHODS: The study comprises two elements, a complete series of all diagnosed patients at the Center for Craniofacial Genetics at the University of Regensburg, Germany, and a SR. Relevant literature was identified by electronic databases, review of citation lists and hand searching of key journals. The principal selection criterion was that the study should contain as many pertinent cases as possible. The presented signs and symptoms were assigned to the following categories: "supernumerary teeth", "failure of eruption", "hypoplastic maxilla" and "clavicular sign". Additionally, the family history was taken into account. RESULTS: From the 410 English, German or French articles, 40 single case presentations and 17 multiple case studies remained that met the selection criteria. This report reviews the data of 283 patients with CCD including our own patient cohort of 24 individuals. Dental signs such as supernumerary teeth and eruption failure were expressed in over 93.5%. Skeletal symptoms such as hypoplastic maxilla and the clavicular sign were exhibited in over 84.3%. The prevalence of spontaneous mutations differs slightly when comparing the single case studies (72.0%) with our patient data (58.3%). The fraction of spontaneous mutations in multiple case studies was 5.0%. CONCLUSION: The diagnosis of CCD can be difficult when typical features are not clearly expressed. Since the multiple case studies concentrated on specific clinical aspects, an overall ranking including all associated findings was not possible. Owing to their prevalence, we recommend referencing to the described list of clinical signs as major symptoms for the pathognomy in CCD, since they are infrequent in other conditions and in the general population. To categorize the expression of CCD, more interdisciplinary studies are necessary. Nevertheless, a subjective classification is possible according to the related restrictions in the patients' quality of life.

Cleidocranial Dysplasia↗

PubMed1993

Continuous c-fos expression precedes programmed cell death in vivo.

The development of a multicellular organism involves a delicate balance among the processes of proliferation, differentiation and death. Naturally occurring cell death aids tissue remodelling, eliminates supernumerary cell populations and provides structural elements such as hair and skin. In the nervous system, selective cell death contributes to the formation and organization of the spinal cord and sympathetic ganglia, retina and corpus callosum. But cell death also occurs in several neuropathological conditions, such as amyelotrophic lateral sclerosis and Alzheimer's disease. Therefore an elucidation of the mechanisms responsible for cell death is critical for an appreciation of both normal development and neuropathological disorders. Using a fos-lacZ transgenic mouse, we provide evidence showing that the continuous expression of Fos, beginning hours or days before the morphological demise of the cell, appears to be a hallmark of terminal differentiation and a harbinger of death.

3T3 Cells↗

PubMed2003

Dental anomalies as a microsymptom of palatal canine displacement.

AIM: A retrospective study was performed to investigate whether palatal canine displacement is associated with other dental features permitting early clinical diagnosis of the eruption disturbance. PATIENTS AND METHODS: The study was based on the complete records of 235/8556 patients at the Department of Orthodontics, School of Dental Medicine, University of Freiburg i.Br. (mean age = 14.11 years) with at least one palatally displaced permanent canine. These patients were examined for ten different morphologic parameters, e.g. impaction and congenital absence of further teeth, hypoplastic, peg-shaped, rotated and congenitally missing upper lateral incisors, supernumerary teeth, Angle classification, and cover-bite (= "Deckbiss"). The data were compared with those of a control sample of the same size with physiologic upper canine eruption (mean-age = 10.0 years). Furthermore, in a right/left comparison the local influence of anomalies of the upper lateral incisors on palatal canine displacement was evaluated by multiple linear regression analysis. RESULTS: The statistical analyses revealed that the risk of palatal canine displacement was significantly higher in patients with hypoplasia, peg shape or congenital aplasia of upper lateral incisors, further impacted and congenitally missing teeth and cover-bite. The intergroup differences in terms of gender, rotation of upper lateral incisors and Angle classification were not statistically significant. The right/left comparison revealed a significantly higher risk of palatal canine displacement in association with an adjacent hypoplastic or peg-shaped lateral incisor and with aplasia of the contralateral upper incisor. CONCLUSION: The clinical significance of the study is that the occurrence of palatally displaced canines is often closely linked with other dental anomalies. In this context, anomalies of upper lateral incisors, aplasia or impaction of further teeth, and the anomaly of cover-bite may serve as indicators of palatal canine displacement. A retarded development of the upper lateral incisor seems to be more disturbing for physiologic canine eruption than aplasia. In patients exhibiting the stated microsymptoms, close clinical follow-up of the maxillary permanent canine eruption during the late exfoliation period is strongly recommended.

Adolescent↗

PubMed1996

YAC and cosmid FISH mapping of an unbalanced chromosomal translocation causing partial trisomy 21 and Down syndrome.

Most cases of Down syndrome (DS) result from a supernumerary chromosome 21; however, there are rare cases in which DS is due to partial trisomy of chromosome 21, involving various segments of the chromosome. The characterization of cases of DS that are due to partial trisomy 21 allows the phenotype to be correlated with the genotype. We present a case with features of DS and a partial trisomy of chromosome 21 inherited from a paternal balanced translocation involving chromosomes 13 and 21. Fluorescence in situ hybridization analysis using yeast artificial chromosome (YAC) probes mapped the breakpoint to 21q22.1, within YAC 230E8, which contains markers CBR, D21S333 and D21S334. Further mapping using cosmids positioned the breakpoint proximal to CBR. The patient was also monosomic for the distal portion of chromosome 13 (q33-qter). Many phenotypic features of DS were present including hypotonia, flat occiput, flat facies, up-slanted palpebral fissures, epicanthic folds, flat nasal bridge, macroglossia, open mouth, small ears and a heart murmur. This case further supports the contention that the majority of the phenotypic features of DS map to 21q22-qter and further refines the location of some of them. In addition to the DS phenotype, the patient had a prominent upper maxilla with protruding upper incisors, and low levels of the coagulation factors VII and X, consistent with a syndrome resulting from monosomy 13q33-qter. Since some features overlap between the two syndromes, including severe mental retardation, it is unclear to what extent monosmy for 13q33-qter, trisomy for 21q22.1-qter, or a combination of both, contributed to the common features of the phenotype.

Adult↗