Cases reported "Dental Enamel Hypoplasia"

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1/76. family with low-grade neuroendocrine carcinoma of salivary glands, severe sensorineural hearing loss, and enamel hypoplasia.

    Four sibs in a family on the Isle of Man, two brothers and two sisters ranging in age from 33 to 45 years, presented with low-grade malignant tumors of the submandibular gland in three cases and of the nasal cavities and maxillary sinuses in one. The neoplasms were all of the same histological type, apparently hitherto undescribed, showing well-differentiated neoplastic ducts, surrounded by neoplastic myoepithelial cells, together with sheets of epithelial cells expressing neuroendocrine markers by immunohistochemistry. Cervical neck node metastases have developed in all four cases. In the sib with a primary sinonasal neoplasm, widespread bloodstream metastases also became manifest and a single such metastasis in his brother. All four sibs have severe enamel hypoplasia and the same lesion is present in 5 of their 11 children. In the two male patients, severe sensorineural hearing loss has developed in adult life, unilateral in the left ear in one brother, bilateral in the other. In the brother with bilateral sensorineural hearing loss, magnetic resonance imaging revealed a vestibular schwannoma on the left side, which is currently under treatment. The inherited hearing loss is thought to be unilateral in this case also.
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2/76. Enamel staining and hypoplasia due to multiple causes in a Nigerian adolescent: report of a case.

    The aim of this report is to discuss the effects of childhood drug ingestion and metabolic disturbances of ill health in childhood on the coloration, structure and therefore the aesthetics of permanent dentition. To illustrate this, the report presents a case of a 15 year old Nigerian girl with combined tetracycline staining and chronological enamel hypoplastic defects on her teeth. The relationship between the clinical appearance of her teeth and her childhood medical and drug history are analysed. The various treatment modalities available for the management of the dental anomalies are discussed along with the rationale for the treatment given. Strategies for the prevention of these conditions are emphasised.
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3/76. Oral findings in digeorge syndrome: clinical features and histologic study of primary teeth.

    OBJECTIVE: For the purpose of supplementing the shortage of dental information about digeorge syndrome, we report two cases of the syndrome seen in Japanese boys. STUDY DESIGN: Two cases were compared with respect to orofacial and dental findings; one was a case of complete digeorge syndrome and the other a case of partial digeorge syndrome. Extracted deciduous teeth from the two boys underwent histologic study. RESULTS: Each patient showed systemic developmental delay, hypocalcemia, and slight mental retardation. In the orofacial area, hypertelorism, a short philtrum, thick and reflected lips, and hypoplasia of the nasopharynx were also observed. A dental examination showed delayed formation and eruption of permanent teeth, aplasia of the nasopharynx, and enamel hypoplasia along with enamel hypocalcification. Structural streaks with increased calcification were histologically detected in the deciduous tooth from the patient with complete digeorge syndrome. CONCLUSIONS: Common characteristic orofacial and dental findings were noted in the two digeorge syndrome cases. Furthermore, histologic study of the deciduous tooth from the boy with complete digeorge syndrome suggests that there was some relationship between transient relative hypercalcemia and dentinal hypermineralized streaking of the tooth.
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4/76. Adhesive solutions: report of a case using multiple adhesive techniques in the management of enamel hypoplasia.

    Enamel hypoplasia is a common condition that may present a severe aesthetic problem. Although the teeth affected may not be particularly susceptible to caries, patients may request cosmetic improvement. Adhesive techniques may be useful in such situations. This paper discusses the management of a patient with enamel hypoplasia using a combination of adhesive systems including enamel- and dentine-bonded veneers, dentine-bonded crowns, a cantilever resin-retained bridge, bonded amalgam restorations and chairside tin plating. Where adhesion was contraindicated, conventionally retained crowns were used.
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keywords = hypoplasia
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5/76. Abrasion, erosion, and abfraction combined with linear enamel hypoplasia: a case report.

    Linear enamel hypoplasia is a developmental disturbance of enamel resulting in clinically visible horizontal defects in enamel that are present on eruption of the tooth. Nondevelopmental lesions of the hard tissues of the tooth, including carious, abrasion, erosion, attrition, and abfraction lesions, require varying amounts of time after tooth eruption to develop. Because linear enamel hypoplasia lesions are present on eruption and are exposed to the factors responsible for abrasion, erosion, and abfraction, nondevelopmental lesions could occur within them in any combination. This report describes a patient with multiple teeth with linear enamel hypoplasia lesions containing nondevelopmental defects as well as nondevelopmental defects that occurred separately. Severe pain and a unique lesion morphology were associated with the linear enamel hypoplasia defects. Affected teeth were extracted because of advanced periodontitis and were sectioned to determine the nature of the enamel and dentin lesions.
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6/76. Malformation in the primary and permanent dentitions following trauma prior to tooth eruption: a case report.

    Dento-facial injuries that occur prior to the eruption of teeth in the primary dentition are rare, but can result in damage to the primary dentition. We report a rare case where an injury to the anterior maxilla and mandible of an infant prior to primary tooth eruption resulted in hypoplasia, displacement and impaction of the primary dentition and damage to a developing permanent tooth.
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7/76. Distinctive pitted enamel hypoplasia and short stature.

    We report a case of postnatal onset short stature and a distinctive pitted enamel hypoplasia in a 19-year-old woman. growth hormone deficiency and other endocrine deficiencies were excluded. Additional observations of similar cases might outline a newly recognized syndrome.
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8/76. epidermolysis bullosa acquisita: clinical manifestations, microscopic findings, and surgical periodontal therapy. A case report.

    BACKGROUND: epidermolysis bullosa acquisita (EBA) is an uncommon, acquired, chronic subepidermal bullous disease. This report describes a case of EBA with gingival involvement. A 43-year-old woman with EBA was referred to our clinic for periodontal therapy because of gingival tenderness and bleeding. She has been on cyclosporin A therapy for the last 2 years. methods: Clinical findings were analyzed. Anterior gingivectomy operations were performed in 2 stages. The samples obtained during the surgery were examined using histopathologic, immunohistologic, and electronmicroscopic methods. Long-term effects of the surgical periodontal treatment on gingiva were evaluated both clinically and microscopically. RESULTS: The dentition displayed minimal enamel hypoplasia. Decayed, missing, and filled surfaces score was found to be elevated. Periodontal examination showed generalized diffuse gingival inflammation and gingival enlargement localized mainly to the anterior region. Nikolsky's sign was positive. However, wound healing was uneventful after the operations. Microscopic findings were similar to those obtained from the skin. Twenty-one months after the operations, Nikolsky's sign was negative and no remarkable gingival inflammation was noted. Microscopic examination revealed that the blisters were fewer in number and smaller in size. CONCLUSIONS: These results indicate that gingival tissues may also be involved in EBA. Uneventful wound healing after periodontal surgery in this case suggests that periodontal surgery can be performed in patients with EBA. Moreover, both our clinical and histopathologic findings imply that gingivectomy proves useful in maintaining gingival integrity in these patients. Our data may also suggest that the patients with EBA are highly likely to develop dental caries.
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keywords = hypoplasia
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9/76. sotos syndrome with enamel hypoplasia: a case report.

    A rare case of sotos syndrome with enamel hypoplasia is described. Dental abnormalities include enamel hypoplasia, expansion of the pulp cavity, high arched palate, and absence of the bilateral pre-molar teeth of the mandible.
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keywords = hypoplasia
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10/76. 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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