Cases reported "Amelogenesis Imperfecta"

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1/9. Dental development after successful treatment of infantile osteopetrosis with bone marrow transplantation.

    A 3-week-old boy was diagnosed with congenital osteopetrosis. He underwent a bone marrow transplant at 6 weeks of age. At 3 years of age the primary teeth had all erupted, but the canines and the first molars totally lacked root development. The teeth were smaller in size and had evidence of both enamel hypomineralization and hypoplasia. In the permanent dentition, multiple missing teeth were found. The incisors were conical and the mandibular laterals were extremely small. All permanent teeth had normal eruption. This case shows that dental development and eruption of teeth can be reconstituted in a child with congenital osteopetrosis. bone marrow transplantation induces normalization of osteoclast function, which is a prerequisite for normal dental development and eruption of teeth.
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keywords = eruption
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2/9. amelogenesis imperfecta: diagnosis and resolution of a case with hypoplasia and hypocalcification of enamel, dental agenesis, and skeletal open bite.

    A case of amelogenesis imperfecta with hypoplasia, hypocalcification of the enamel, congenital absence of teeth 12 and 22, delayed eruption of tooth 23, edge-to-edge incisal relationship, open bite, and bilateral posterior cross bite at the level of the first and second premolars is presented. Lateral skull teleradiography indicated a Class III skeletal pattern of maxillary origin associated with a dolichofacial pattern with multiple indicators of facial hyperdivergence. The patient presented a major esthetic abnormality of the face and required orthodontic treatment prior to a prosthetic solution with full-coverage metal-ceramic crowns in both the maxilla and the mandible. The diagnosis of cases such as this one and the therapeutic implications from an orthodontic and prosthetic standpoint are reported.
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ranking = 0.33333333333333
keywords = eruption
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3/9. amelogenesis imperfecta and nephrocalcinosis: a new case of this rare syndrome.

    This article describes a new case of a rare syndrome including enamel agenesis of the primary and permanent dentition, delayed or absent eruption of the permanent dentition, coronal intra-alveolar resorption and gingival enlargement. Renal symptoms include medullary nephrocalcinosis without any apparent cause, and evolution to a renal failure. The early diagnosis provided by the oral symptoms leads to a better renal prognosis. As a consequence, pediatric dentists should be aware of this pathology.
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ranking = 0.33333333333333
keywords = eruption
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4/9. Unusual manifestations in X-linked amelogenesis imperfecta.

    This paper describes a female with X-linked amelogenesis imperfecta (XAI). This case is unusual in having taurodontism, pulpal calcifications, coronal defects prior to tooth eruption and unerupted teeth. These findings have been reported in some cases of autosomal dominant and autosomal recessive AI but have not previously been documented in XAI.
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ranking = 68.919590156472
keywords = tooth eruption, eruption
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5/9. amelogenesis imperfecta--multidisciplinary management from eruption to adulthood. review and case report.

    amelogenesis imperfecta (AI) is a group of hereditary conditions that affect enamel formation. It is associated with a high morbidity for the patients and may present major restorative and sometimes orthodontic challenges for the dental team. Early recognition followed by appropriate preventive and restorative care is essential in the successful management of AI. A multidisciplinary approach with careful planning from early childhood will maximise the treatment options available for the permanent dentition and optimise the final outcome. In this case, a team consisting of two paediatric dentists, an orthodontist, a restorative dentist, and an oral and maxillofacial surgeon were involved in the management of the patient over a 12-year period. Treatment included preventive advice, interim composite restorations, two phases of orthodontic treatment, orthognathic surgery and placement of cast crowns. The patient is extremely happy with the result.
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ranking = 1.3333333333333
keywords = eruption
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6/9. Case report of a rare syndrome associating amelogenesis imperfecta and nephrocalcinosis in a consanguineous family.

    A rare syndrome associating amelogenesis imperfecta (AI) with nephrocalcinosis has been reported. The purpose of this study is to characterise the phenotype of a consanguineous family presenting amelogenesis imperfecta, delayed permanent teeth eruption and nephrocalcinosis. Six family members were examined. Ground sections of the case index deciduous teeth and biopsies of enlarged dental follicles were analysed. The patients's parents were first cousins. The case index had yellow discoloration and altered teeth shapes, retention of deciduous teeth, and delayed eruption. Panoramic radiographs revealed multiple enlarged pericoronal follicles in unerupted teeth and generalised intrapulpal calcifications. Renal ultrasound showed the presence of nephrocalcinosis. No other family members presented enamel defects or nephrocalcinosis. Histologically, the enamel appeared hypoplastic, and dental follicles indicated pericoronal hamartoma. The consanguineous marriage suggests an autosomal recessive mode of inheritance. Further studies are necessary to clarify the genetic defect behind this syndrome that associates AI, nephrocalcinosis and impaired tooth eruption.
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ranking = 69.586256823139
keywords = tooth eruption, eruption
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7/9. amelogenesis imperfecta: multiple impactions associated with odontogenic fibromas (WHO) type.

    Three types of amelogenesis imperfecta (AI) are recognised, namely hypoplastic, hypomature and hypocalcified varieties. We report on two cases of hypoplastic AI, the type which occurs most frequently. Both patients presented with multiple impacted permanent teeth. Odontogenic fibromas of the WHO type were found to be associated with the crowns of all the impacted teeth and are considered to have prevented normal eruption. Dentinal dysplasia found only in the furcation area of the multirooted impacted teeth was evident. The macroscopic, microscopic and radiological appearance of the affected teeth, pericoronal lesions and interradicular dentinal dysplasia are described, and the most likely origins of the odontogenic fibromas and calcifications observed, are discussed.
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keywords = eruption
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8/9. Autosomal recessive rough hypoplastic amelogenesis imperfecta. A case report with clinical, light microscopic, radiographic, and electron microscopic observations.

    A case is presented of a 12-year-old Japanese girl with nearly complete lack of enamel in the deciduous and the permanent dentitions, coupled with a gross abnormality in the pattern of eruption. There was no family history of a similar condition. Deciduous molars were extracted, and hyperplastic gingival tissue was resected. On the basis of clinical, radiographic, and microscopic findings, a diagnosis of autosomal recessive rough hypoplastic amelogenesis imperfecta was made. The configuration of the abnormal enamel was examined with scanning and transmission electron microscopy as well as with light microscopy. Prismatic structure was virtually absent, and the scant enamel showed globular protrusions superficially. Two different surface structures were identified as covering parts of the enamel. At the ultrastructural level, calcified bodies located in the gingival tissue appeared to be composed, in part, of a dense enamel-like substance and, in part, of a tissue with features of afibrillar cementum.
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ranking = 0.33333333333333
keywords = eruption
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9/9. 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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ranking = 68.919590156472
keywords = tooth eruption, eruption
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