Cases reported "Dentin Dysplasia"

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1/11. 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.
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2/11. 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.
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3/11. dentin dysplasia type I: report of atypical cases in the permanent and mixed dentitions.

    dentin dysplasia type I is a rare hereditary disturbance of dentin formation characterized clinically by nearly normal appearing crowns and severe hypermobility of teeth. Radiographic analysis shows obliteration of all pulp chambers, short, blunted, and malformed roots, and periapical radiolucencies of noncarious teeth. This paper presents 2 cases demonstrating both classic and atypical features of type I dentin dysplasia in the mixed and permanent dentitions. The clinical, radiographic, and histopathologicfindings of this condition and treatment are described.
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4/11. Endodontic therapy on a dentition exhibiting multiple periapical radiolucencies associated with dentinal dysplasia Type 1.

    Dentinal dysplasia (DD) Type I, is a hereditary disturbance in dentine formation. In this anomaly, teeth in both primary and secondary dentitions are affected, and radiographically show short and blunted roots with obliterated root canals and periapical pathosis. Management of patients with DD has presented dentists with problems. Extraction has been suggested as a treatment alternative for teeth with pulp necrosis and periapical abscess. Follow-up and routine conservative treatment is another choice of treatment plan in DD. Another approach for the treatment of teeth with DD has included periapical surgery and retrograde filling, which is recommended in the teeth with long roots. The purpose of this report is to present an unusual case of dentinal dysplasia Type I in a 22-year-old woman showing upper and lower teeth with obliterated root canals and periapical radiolucencies. In this case, conventional endodontic treatment was performed. Postoperative radiographs and clinical evaluation demonstrated periapical healing and successful results. Based on the results of this case report, conventional endodontic treatment for cases with pulp necrosis and periapical radiolucencies in dentinal dysplasia is highly recommended.
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5/11. Multiple pulp stones and shortened roots of unknown etiology.

    An unusual case of generalised pulpal calcifications (pulp stones) with normal clinical crowns is reported in a 13-year-old boy. Radiographic examination revealed pulp stones in the single rooted and premolar teeth, situated at the midroot level, with the roots bulging around them. The apical portion of the roots, periodontal ligament space, and surrounding bone had a normal radiographic appearance, apart from the upper premolars, and no periapical pathology was discernible. The upper premolars exhibited considerably shortened roots. No medical, dental, or family history was found to be contributory. Reviewing the literature revealed similar cases, but with differing diagnoses including dentine dysplasia (DD) or idiopathic cases. This report suggests either a variation of DD or possibly a new nonsyndromic dentine defect, and highlights the difficulties in establishing a definitive diagnosis by traditional methods. The recent discovery that mutation of the bicistronic dentine sialophosphoprotein gene (DSPP) is involved in DD may provide solutions to this problem.
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6/11. Dentinal dysplasia, Type I. Report of a case with endodontic therapy.

    The first reported case of endodontic therapy in dentinal dysplasia, Type I, is described. The first histologic diagnosis of periapical granuloma instead of periapical cyst in dentinal dysplasia, Type I, is also reported.
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7/11. 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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8/11. dentin dysplasia type I: a clinical report.

    A case of dentin dysplasia type I (radicular) is described. The practitioner should be aware that several periapical radiolucent areas may occur. If periodontal communication occurs, infection follows. Because of the abnormal anatomic configuration of pulp chambers and root canals, endodontic treatment may not be feasible. The shortened roots may also allow hypermobility and spontaneous exfoliation at an early age. Such patients may be doomed to prosthetic replacements. Well-fitting, esthetic appliances are essential for appearance and function.
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9/11. Spectrum of dentin dysplasia in a family: case report and literature review.

    The dentin dysplasias (DD), which may be classified as type 1 (DD1) or type 2 (DD2), form a group of rare, inherited dentin abnormalities that are clinically distinct from dentinogenesis imperfecta. Studies of affected families may help to distinguish different types of DD and provide further insight into their etiology and clinical management. This report describes a family that showed characteristic dental features of DD1, including clinically normal crowns in both primary and permanent dentitions, and mobile teeth that may be associated with premature exfoliation. Radiographic features included calcification of the pulp with crescent-shaped, radiolucent pulp remnants, short, tapering, taurodontic roots, and many periapical pathoses that may be cysts or granulomas. A spectrum of dentin dysplasia was noted within the family. Strategies to prevent pulp and periapical infections and early exfoliation of the teeth include meticulous oral hygiene and effective caries-preventive measures.
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10/11. Dentinal dysplasia type I: review of the literature and report of a family.

    A family is reported with dentinal dysplasia type I affecting both dentitions. Presenting features included unusual mobility of the teeth, followed by early exfoliation; normal clinical shape of the crowns of the teeth, but with an amber color without any sign of attrition or abnormal loss of enamel. Radiographic findings showed pulp-chamber and root-canal obliteration, poor root formation, radiolucent linear appearance of the pulp chamber parallel to the cementoenamel junction and frequent periapical radiolucencies. Histological studies have reported large masses of calcified tubular dentin, atypical osteodentin, and also true denticle.
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