Cases reported "Dentin Dysplasia"

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1/40. dentin dysplasia type I--a case report.

    A case report on dentin dysplasia type I, a congenital disease (autosomal dominant gene defect) affecting deciduous and permanent teeth, is depicted including representations of clinical and histological features, X-ray and CT-findings. Therapy includes extraction of all teeth, ectomization of cystic alteration, revision of paranasal sinus. Aesthetic and functional rehabilitation by means of insertion of a complete denture was achieved. ( info)

2/40. Dentinal dysplasia type I: report of a case.

    A case of dentinal dysplasia type I is presented. This rare hereditary disturbance of dentine is characterized by short-rooted teeth with sharp conical apical constrictions, aberrant growth of dentine in the pulp chamber leading to reduced pulp space in permanent teeth and total pulpal obliteration in the primary dentition. Clinical, radiographic and histopathological material from a 7-year-old boy, showing the typical features of this disorder in which teeth are prematurely lost through periapical abscesses, cysts or spontaneous exfoliation, is described. A review of the theories of pathogenesis of this condition is included. Management of patients with dentinal dysplasia is difficult and a discussion of the shortcomings of various treatment strategies, including conventional endodontic therapy, periapical curettage and retrograde root filling, and a preventive regimen, are discussed. In this case, despite diagnosis being made at an early age and the provision of regular dental care, the patient is now losing teeth because of spontaneous abscess formation. ( info)

3/40. Localized deficient root development associated with taurodontism: case report.

    Dentinal dysplasia type I (DDI) is a rare disturbance in dentin formation. This case report illustrates different radiographic features from other reported DDI cases in that only one quadrant (lower right posterior teeth) has the characteristic of DDI and both right and left upper molars exhibit taurodontism. This finding might be a variation of DDI. However, it is possible that this type of developmental defect could occur because of regionalized abnormalities in cellular function and proliferation as occurs in regional odontodysplasia. ( info)

4/40. Pulp stones throughout the dentition of monozygotic twins: a case report.

    Pulpal calcifications are relatively common. However, their occurrence in the entire dentition is relatively infrequent. The presence of such calcification arouses suspicion of systemic or hereditary origin. This case report describes twin sisters with pulpal calcifications in their entire dentitions. No systemic cause was detected. The pattern of calcification was partially consistent with the hereditary condition of dentinal dysplasia. ( info)

5/40. dentin dysplasia, type II: report of 2 new families and review of the literature.

    dentin dysplasia, type II, is an inherited autosomal dominant disorder in which primary teeth are amber and translucent, with pulp chambers obliterated by abnormal dentin. The permanent teeth have a normal coronal morphologic character and coloration but exhibit "thistle tube"-shaped pulp chambers as well as numerous pulpal calcifications. The disorder has traditionally been thought to be somewhat rare; however, this article presents 2 new families in which several generations with the disorder were reported to the authors within a 1-year period. It also includes a review of the literature documenting a total of 17 previously reported families. ( info)

6/40. 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. ( info)

7/40. dentin dysplasia type I. Clinical, morphologic, and genetic studies of a case.

    This report documents a case of dentin dysplasia Type I in a 17-year-old boy and two members of his family. The clinical, radiographic, histologic, and ultrastructural findings indicate that this condition is distinct from other heritable defects of dentin. The entity is transmitted as an autosomal dominant trait and is characterized by teeth which have a normal color and exhibit pulpal obliteration, short roots, periapical radiolucencies, and spontaneous exfoliation. Our ultrastructural findings in agreement with those reported by Sauk and associates. ( info)

8/40. Seckel syndrome: report of a case.

    An interesting case of a seven years old boy with a combination of clinical, genetic, radiological, pathologic and dental findings is presented in view of Seckel syndrome literature. General appearance of the patient was characterized by small forehead, posteriorly slanted ears, slightly beaked nose, midfacial hypoplasia very stunted stature with microcephaly. He had borderline mental retardation with normal motor development. Class II dentoskeletal pattern with mild overjet and open bite, congenitally missing permanent teeth, microdontia, enamel hypoplasia, taurodontism and dentinal dysplasia was observed according to the clinical and radiographic examination. In conclusion, Seckel syndrome is not encountered routinely in dental clinics, this case illustrates the importance of dental care in such a rare condition. ( info)

9/40. Hereditary pattern for dentinal dysplasia type id: a case report.

    Generalized pulpal calcifications arouse suspicion of diseases or conditions of systemic or hereditary origin. This case report describes a 45-year-old patient with generalized pulpal calcifications and bulging of the roots in areas corresponding to the pulp chambers in otherwise normal teeth. Similar findings were present in the patient's daughters and brother. This pattern of pulpal calcifications is consistent with the hereditary condition of dentinal dysplasia type id. ( info)

10/40. Rough hypoplastic amelogenesis imperfecta with follicular hyperplasia.

    This report documents a unique case of rough hypoplastic amelogenesis imperfecta with apparent anterior oligodontia and multiple anomalies of the associated mesenchymally derived tissues. Multiple unerupted teeth showed hypercementosis, distorted roots with aberrant dentin formation, and marked follicular hyperplasia. The hyperplastic follicles had a complex histopathologic appearance that recapitulated some features of the WHO-type odontogenic fibroma. The features of these teeth, the nature of the associated follicular lesions, and their relationship to the unerupted teeth are discussed. ( info)
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