Cases reported "Cleidocranial Dysplasia"

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1/10. Combined surgical and orthodontic management of the oral abnormalities in children with cleidocranial dysplasia.

    Children with cleidocranial dysplasia have dental abnormalities which combine to prevent normal tooth eruption, and which if untreated may result in abnormal facial and jaw growth. A technique combining orthodontics and oral surgery has resulted in the establishment of excellent occlusion and facial appearance in these patients. Recent advances in direct enamel bonding techniques for orthondontic attachments have permitted a conservative surgical approach with minimal bone removal during surgery to expose unerupted teeth prior to orthodontic treatment.
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2/10. Anomalies of craniofacial skeleton and teeth in cleidocranial dysplasia.

    Mutations involving the transcription factor CBFA1 cause cleidocranial dysplasia (CCD) in man. Recently, a mouse model of CCD has been generated (Cbfal /-) [Komori et al., 1997], and disturbances of osteoclast differentiation have been documented. It has been shown that these animals exhibit hypoplastic clavicles and nasal bones, and retarded ossification of parietal, interparietal, and supraoccipital bones. humans with CCD show all these features, including severely retarded ossification of the cranial base, strongly suggesting that both intramembranous ossification and endochondral ossification are affected. In addition, CCD patients have multiple supernumerary teeth and delayed tooth eruption. The present report presents 3D reconstructions of computerised tomography (CT) scans of the craniofacial region of a CCD boy examined at both 1 and 7 years of age. The anomalies in craniofacial skeleton and teeth are analysed and compared to the findings of our previous clinical studies and to the findings in the animal model. Based on the available information, we suggest that osteoblast, osteoclast, and dentinoclast differentiation may be disturbed in CCD.
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3/10. A case of Japanese cleidocranial dysplasia with a CBFA1 frameshift mutation.

    cleidocranial dysplasia (CCD), which is caused by mutations of the core binding factor alpha 1 (CBFA1)/runt-related gene 2 (Runx2), is an autosomal, dominantly inherited disorder of high penetrance affecting skeletal ossification and tooth development. Recently, we found a novel frameshift mutation 383-T-insertion (S128F) in exon 3 in the CBFA1 gene of a Japanese classic CCD patient. We describe our detailed investigation of the patient with CCD associated with the CBFA1 mutation. The patient showed the characteristic expression of CCD, such as dysplasia of the clavicles, patent fontanelles, short stature, impacted supernumerary teeth, and delayed eruption of the permanent teeth. In addition to these characteristics, orthopantomography delayed ossification of the mandibular symphysis and a three-dimensional computed tomograph (3D-CT) analysis showed hypoplasia of the zygomatic arch. Furthermore, the acellular cementum of an impacted supernumerary tooth was absent in this patient. Thus, the CBFA1 mutation was critical for the pathogenesis of CCD in this patient.
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4/10. Histological and analytical studies of a tooth in a patient with cleidocranial dysostosis.

    A histopathological and analytical study of a permanent tooth from a patient with cleidocranial dysostosis (CCD) was performed. The patient was a 47-year-old woman, who had 10 erupted permanent teeth and 2 partially erupted and 19 completely impacted teeth, including supernumerary teeth. The erupted right upper premolar was extracted and observed using a light microscope and an electron probe X-ray microanalyzer (EPMA). Findings showed enamel hypoplasia, predominantly irregular globular dentin and Tomes' granular layer, and a complete lack of cellular cementum in the ground section. The incremental von Ebner and counter Owen lines were obscure. Comparative quantitative analysis using the EPMA showed that the quantities of calcium and phosphate were lower in the enamel and dentin than those of the control sample.
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5/10. cleidocranial dysplasia with decreased bone density and biochemical findings of hypophosphatasia.

    cleidocranial dysplasia (CCD; MIM 119600) is an autosomal dominant skeletal dysplasia characterised by hypoplastic clavicles, patent fontanelles, short stature, tooth anomalies and other variable skeletal changes. Different mutations of the RUNX2/CBFA1 gene (MIM 600211) have been detected in patients with CCD. We investigated a mother and daughter with features of CCD presenting with reduced plasma alkaline phosphatase activity, increased urinary phosphoethanolamine excretion and decreased bone density. The latter findings were suggestive of hypophophatasia but mutation analysis showed no mutation in the tissue-nonspecific alkaline phosphatase gene (TNSALP; MIM 171760). However, a heterozygous mutation (Arg169Pro caused by nucleotide change 506G > C) was detected in the RUNX2 gene. Metabolic alterations gradually improved in both mother and daughter but bone-specific alkaline phosphatase remained low (less than 30% of normal) and mild phosphoethanolaminuria persisted. Recent studies in the Cbfa1 knock-out mouse showed decreased expression of alkaline phosphatase in differentiating bone. CONCLUSION: we suggest that the observed metabolic alterations are secondary to the RUNX2 gene mutation affecting early bone maturation and turnover. This is the first description of biochemical findings of hypophosphatasia in patients with cleidocranial dysplasia.
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6/10. cleidocranial dysplasia: a light microscope, electron microscope, and crystallographic study.

    An unusual case of cleidocranial dysplasia associated with more than 60 unerupted teeth is presented and examined with light and scanning electron microscopes and crystallographic techniques. The present case revealed pseudoprognathism with facial asymmetry, the right side being larger than the left. The extracted teeth showed enamel hypoplasia in light and scanning electron microscopy, yet the crystalline composition did not differ from that of the normal teeth. The potential causes of lack of eruption and supernumerary tooth formation in cleidocranial dysplasia are discussed.
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7/10. histology of alveolar bone and primary tooth roots in a case of cleidocranial dysplasia.

    cleidocranial dysplasia is commonly reported as an autosomal dominant inherited condition with defective formation of clavicles, malformation of the craniofacial bones, very slow exfoliation of the primary teeth and failure of the eruption of the permanent dentition. Lack of clinical resorption of the roots of the deciduous teeth and/or surrounding bone, lead to eruption failure of permanent teeth. Histopathological study (light and scanning electron microscopy), in a case of cleidocranial dysplasia, gives prominence to the hypothesis of abnormal remodelling of bone and cementum.
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8/10. Sibs with cleidocranial dysplasia born to normal parents: germ line mosaicism?

    cleidocranial dysplasia (CCD) is classically an autosomal dominant disorder. However, the possibility of an autosomal recessive form of CCD has been suggested based on a report of 2 consanguineous families, one with a single affected child, the second with affected sibs, born to normal parents. We present a family with sibs with CCD born to normal parents, and suggest germ line mosaicism as the more likely mechanism for this occurrence.
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9/10. Mandibular infected buccal cyst and paradental cyst: the same or separate entities?

    The WHO classification of odontogenic cysts includes the radicular (residual) and paradental (inflammatory collateral; mandibular infected buccal) cysts as inflammatory odontogenic cysts. Because the paradental group of inflammatory cysts may clinically cause diagnostic and therapeutic problems when they are associated with first and second molar teeth it was decided to review the literature and present an additional case. The aetiology and histological features of the inflammatory collateral cyst, the paradental cyst and the mandibular infected buccal cyst are identical and the differences that exist in their clinical and radiological presentation can be related to the different teeth that are involved and the difference in the ages at which these teeth erupt. These cysts represent the same entity and their treatment is dependent on the tooth involved. With the aid of magnetic resonance imaging (MRI) the distinction between a paradental cyst on the buccal aspect of a molar tooth and a periodontitis can be made.
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10/10. Use of selectively colored stereolithography for diagnosis of impacted supernumerary teeth for a patient with cleidocranial dysplasia.

    The objective of the present study was to develop a three-dimensional method of locating the position of impacted supernumerary teeth in a patient with cleidocranial dysplasia. Stereolithographies of the patient's teeth and maxillofacial structure were prepared, coloring the teeth red. This model established accurate tooth malposition and provided support for selection of surgical exposure for extraction and traction of the impacted teeth. Selectively colored stereolithography is expected to be useful in a number of diverse situations.
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