Cases reported "Osteosclerosis"

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1/7. 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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keywords = tooth, eruption
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2/7. Expansile skeletal hyperphosphatasia: a new familial metabolic bone disease.

    We describe a new familial metabolic bone disease characterized by expanding hyperostotic long bones, early onset deafness, premature tooth loss, and episodic hypercalcemia. The condition affects a mother and daughter studied at the age of 36 years and 11 years, respectively. Both individuals lost all hearing in early childhood and suffered premature shedding of teeth. Skeletal pains began just before puberty. Swelling and aching of most middle phalanges in the hands is an especially troublesome manifestation. The mother also had episodes of symptomatic hypercalcemia first documented in late childhood and subsequently during intercurrent illness and postpartum lactation. Radiographs show hyperostosis and/or osteosclerosis predominantly in the skull and appendicular skeleton. Long bones also are expanded considerably, especially the middle phalanges in the fingers. The mother's skeletal abnormalities are more severe. Biochemical parameters of bone turnover, including serum alkaline phosphatase (ALP) activity, are elevated substantially. In the proposita, dynamic histomorphometry of nondecalcified sections of iliac crest revealed rapid skeletal remodeling. In the mother, who had been treated with bisphosphonates, electron microscopy (EM) showed disorganized collagen bundles as well as necrotic and apoptotic bone cells but no osteocytic osteolysis. measles virus gene transcripts were not detected in peripheral blood monocytes. karyotyping was normal, 46,XX. Hyperphosphatasia with bone disease previously has been reported as either a sporadic or autosomal recessive condition. Expansile skeletal hyperphosphatasia (ESH) is probably inherited as an autosomal dominant trait with a high degree of penetrance.
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ranking = 0.19541925175737
keywords = tooth
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3/7. Garre's osteomyelitis: a case report.

    Garre's osteomyelitis is a distinctive type of chronic osteomyelitis associated with gross thickening of the periosteum of the bones and peripheral reactive bone formation resulting from mild irritation or infections. The condition is seen exclusively in children or young adults. mandible is more often affected than the maxilla. Clinically it manifests as bony hard, non-tender swelling which is slowly progressive and associated with painful carious tooth. In this article, a case report of a eight year old female is presented along with complete investigations and treatment.
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ranking = 0.19541925175737
keywords = tooth
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4/7. Cerebellar hypoplasia-endosteal sclerosis: a long term follow-up.

    Cerebellar hypoplasia with endosteal sclerosis is an infrequent entity that has been described in only four cases. Major clinical symptoms are cerebellar hypoplasia causing ataxia, hypotonia, mild to moderate developmental delay, microcephaly, growth retardation, endosteal sclerosis, tooth eruption disturbances, and hip dislocations. We report on a girl with this entity, whom we followed for 11 years. The endosteal sclerosis remained stationary over time, as were the clinical neurological symptoms, but neuroadiological symptoms were slowly progressive. We provide a short review of this probably autosomal recessively inherited disorder. (c) 2005 Wiley-Liss, Inc.
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ranking = 0.20687112236394
keywords = tooth, eruption
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5/7. periostitis ossificans (Garre's osteomyelitis) radiographic study of two cases.

    BACKGROUND: periostitis Ossificans (PO) is a non-suppurative type of osteomyelitis, commonly occurring in children and young adults, in mandible. The most common cause for PO is periapical infection of mandibular first molar. Radiographically PO is characterized by the presence of lamellae of newly formed periosteal bone outside the cortex, giving the characteristic appearance of "onion skin". case reports: Two male children 11 years of age reported to the Department of oral medicine with a painless and persistent bony hard swelling in the mandible, with a short duration (Figs 1, 5). Both the patients had grossly decayed mandibular permanent first molar tooth with periapical infection and buccal cortical plate expansion (Figs 2, 6). The radiographic study revealed different appearances, the Orthopantomograph of case I showed a single radiopaque lamella outside the lower cortical border, without altering original mandibular contour (Fig. 3) and in case II showed a newly formed bony enlargement on the outer aspect of the lower cortical border without altering the original mandibular contour (Fig. 7). Occlusal radiograph of both the patients showed two distinct radiopaque lamellae of periosteal bone outside the buccal cortex (Figs 4, 8). Kawai et al. classified PO of mandible into type I and type II, based on whether the original contour of mandible is preserved or not. Each type is further classified into two sub types (Table 1). In case I, the orthopantomographic appearance is characteristic of type I-1 (Fig. 3), but the appearance in occlusal radiograph is characteristic of type I-2 (Fig. 4). In case II, the appearances in both the radiographs are characteristic of type I-2 (Figs 7, 8). CONCLUSIONS: Apart from the typical onion skin appearance, PO shows various other radiographic appearances. The radiographic appearance of periostitis Ossificans may reflect the duration, progression and the mode of healing of the disease process. The radiographic classification of PO depends on the type of radiographs taken for evaluation.
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ranking = 0.19541925175737
keywords = tooth
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6/7. Tricho-dento-osseous syndrome: a scanning electron microscopic analysis.

    A large kindred of which multiple members have the Tricho-dento-osseous syndrome is presented. This is an autosomal dominant disorder characterized by defective enamel, taurodontia, unusually curly hair and occasionally mild to moderate skeletal osteosclerosis. Histologic investigation of teeth (by both LM and SEM) demonstrated that there is a uniformly thin enamel covering with randomly distributed depression and pits. The mineral content of this enamel is closer to that of the underlying dentin, which accounts for its lack of radiographic contrast. The dentin was normal. A bizarre finding is that of a periradicular sheath or membrane that enclosed the open apices and extended partway up the root. It was composed of collagen fiber bundles. The anatomical position of this membrane suggested that it may represent the developing peridontal ligament seen in early tooth formation. Recent embryologic evidence provides support for mesenchymal culpability for all reported features of the syndrome.
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ranking = 0.19541925175737
keywords = tooth
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7/7. Radiographic features of segmental odontomaxillary dysplasia: a study of 12 cases.

    The purpose of the study was to analyze the radiographic features of segmental odontomaxillary dysplasia by studying the radiographs of 12 new cases. With a standard data collection form, the radiographic characteristics were assessed by the two principal investigators who studied available radiographs of all cases. As in previously reported cases, abnormal findings in all patients were confined to the posterior segment of one maxilla. The most common findings in the cases studied were sclerosis and thickening of bony trabeculae, missing permanent premolar teeth, abnormal spacing of primary molars vertical orientation of bony trabeculae, a smaller maxillary sinus, and delayed eruption of permanent posterior teeth. Less commonly observed features were enlarged crowns, roots, and pulps of primary molars, irregular resorption of primary molar roots, mediolateral expansion of the alveolar process, splayed roots of primary molars, and an abnormal medial position of the teeth.
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ranking = 0.011451870606571
keywords = eruption
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