Cases reported "Dentin Dysplasia"

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1/6. Dysosteosclerosis: a case with unique dental findings and SEM evaluation of a hypoplastic tooth.

    A ten-year-old boy, who had the typical dental findings of dysosteosclerosis such as yellowish, hypoplastic teeth, retarded eruption, which upon eruption, decayed rapidly, is presented. To date this is the first known case reported with a congenital absence of the first permanent molars. Furthermore, SEM evaluation of the enamel and dentin was performed on a tooth from a patient with dysosteosclerosis for the first time. These studies showed weak ultrastructural compositions due to irregular calcification.
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2/6. Clinical, radiographic, and histological manifestations of dentin dysplasia, type I: Report of case.

    dentin dysplasia, type I, is a rare dental anomaly characterized by abnormal dentin formation affecting the roots of both primary and permanent teeth. Short, conical roots with occlusion of the pulp chamber and canal are produced. Periapical radiolucent areas are common, although no evidence of caries or trauma to the tooth may be seen. Coronal mantle dentin is unaffected, resulting in an apparently normal clinical crown. An abnormality may not be suspected until radiographs reveal pulp and root changes. Orthodontic treatment can be a successful variation of the usual treatment offered to patients, and is discussed in this case report.
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3/6. Dentinal dysplasia type I: a report of four cases.

    Dentinal dysplasia type I is a relatively uncommon condition and four cases are described. The radiographic features in these patients are somewhat different to those reported in other cases in that anterior teeth appeared to be less affected than posterior, and tooth loss was less prevalent. These findings support the proposal that the characteristics of dentinal dysplasia type I can be divided into two subgroups.
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4/6. fibrous dysplasia of bone and concomitant dysplastic changes in the dentin.

    fibrous dysplasia of bone and dentinal dysplasia have not been classified as related conditions. However, the present report describes a patient with both polyostotic fibrous dysplasia and dysplastic changes in the dentin. One bone lesion was first discovered on the right side of the mandible on a routine roentgenogram. There was an atypical radiolucency apical to a short-rooted molar with an obliterated pulp. Additional lesions were found on the left side of the mandible in the maxilla, in the frontal and occipital bones, in the ilium, in the proximal ulnae, and in the ribs. All lesions demonstrated an increased uptake of Tc99. A biopsy specimen of bone from the right side of the mandible showed small calcified islands in a cell-rich connective tissue. Microscopic analysis of the right first molar showed irregularly shaped dentin with the dentinal tubules arranged in a whorl-like fashion surrounding an almost completely obliterated pulp chamber and canal. Healing after tooth extraction and after the bone biopsy was unremarkable. After 6 months the alveolus was reorganized and the bone patterns appeared normal, although the radiolucent areas inferior to the teeth remained unchanged. The concomitant occurrence of dysplastic changes in bone and teeth may be a sign of a generalized defect in the mesenchymal hard-tissue-forming cells.
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5/6. Interradicular dentin dysplasia associated with amelogenesis imperfecta.

    Very few reports are available on the occurrence of dysplasia of both enamel and dentin. This report concerns a rare association of amelogenesis imperfecta with a dysplasia of dentin in the interradicular area in sisters of Japanese descent who have no other morphologic anomalies. Retarded tooth eruption was also a clinical feature in both sisters. Histologic examination of several teeth revealed that the anomalous interradicular dentin consisted of a mass of small, onion-like calcified bodies. The absence of any dental abnormalities in both parents, who are related as first cousins, supports the concept of autosomal recessive inheritance for this trait.
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6/6. dentin dysplasia type I: a scanning electron microscopic analysis of the primary dentition.

    dentin dysplasia type I (DD-I) is a rare autosomal dominant disorder which affects both the deciduous and permanent dentitions. The affected deciduous and permanent teeth have short conical roots with sharp, apical constrictions and frequently periapical radiolucencies in the absence of caries. Apical to a thin layer of normal coronal dentin are large, calcified, dentin masses which nearly obliterate the pulp chamber and canals. Presented here are light microscopic and scanning electron microscopic observations of deciduous teeth from three unrelated persons with the disorder. In general, the deciduous teeth had (1) normal enamel, (2) a thin layer of normal dentin adjacent to the dentinoenamel junction, (3) a crescent-shaped pulpal remnant below the normal dentin, (4) dysplastic dentin masses (ranging from atubular to a few small tubules) between which are spaces presumed to previously have contained smaller remnants of the original mesenchymal dental papilla, and (5) root dentin, which is dysplastic throughout. The SEM-defined phenotype, however, was noticeably variable among all three persons. Based on the current concepts of tooth morphogenesis, it is most likely that the abnormal root morphology of DD-I teeth is secondary to the abnormal differentiation and/or function of the ectomesenchymally derived odontoblasts.
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