Hypohidrotic ectodermal dysplasia: a unique approach to esthetic and prosthetic management: a case report.
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Hypohidrotic ectodermal dysplasia is a rare congenital disease that affects several ectodermal structures. The condition is usually transmitted as an x-linked recessive trait, in which gene is carried by the females and manifested in males. Manifestations of the disease differ in severity and involve teeth, skin, hair, nails and sweat and sebaceous gland. Ectodermal dysplasia is usually a difficult condition to manage. Prosthodontically, because of the typical oral deficiencies, and afflicted individuals are quite young to receive extensive prosthodontic treatment, which restores their appearance and helps them, for the development of positive self-image. This case report describes the management of upper jaw with over denture with copings on existing teeth i.e. two permanent peg shaped centrals as well as lateral incisors. However with adequate of retainer lower denture was provided with a new treatment modality. (+info)
Congenital absence of the permanent canines: a clinico-statistical study.
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We report here a clinico-statistical study of congenital absence of the permanent canines (CAPC). Sixty-five cases (22 men and 43 women) of CAPC were found in the files of 35,927 outpatients, an incidence of 0.18%. Thirty-seven cases had single absence of the canine and 28 cases had multiple absences. There were 42 cases in the maxilla, 17 in the mandible, and 6 in both the maxilla and the mandible. In the single absence cases, there was a tendency of occurrence at the left side in the maxilla, and the right side in the mandible. There were 39 CAPC cases without absence of other permanent teeth. Several complicated dental anomalies were seen such as persistence of deciduous teeth, congenital absence of other permanent teeth, microdontia, malposition, and complete or incomplete impaction (excluding the third molars). The complication rate of other permanent tooth absences was higher in the cases with CACP in the maxilla than in the mandible. (+info)
Hypodontia in orthodontically treated children.
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The frequency of hypodontia in orthodontically treated children, both male and female, and the association between tooth type, the upper or lower arch, the affected side and Angle's classification were studied using interviews, oral, study cast and panoramic radiographic examinations of 212 patients with a mean age of 12 years 7 months. A hypodontia frequency of 11.3 per cent was found for the total sample. This was higher than the incidence of hypodontia reported in other studies of orthodontically treated children. The most frequently missing teeth were the maxillary lateral incisors, and maxillary and mandibular second premolars. The missing teeth were more often absent on the right (54.2 per cent) than on the left (45.8 per cent) side, in both males and females. One tooth was absent in 29.2 per cent of patients, two in 58.5 per cent, but seldom three or more. Orthodontic space closure was the treatment of choice in 87.5 per cent of the subjects. (+info)
Maxillary canine anomalies and tooth agenesis.
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The aims of the study were to analyse the records of 26 subjects (18 females, eight males) with maxillary canine-first premolar transposition (Mx.C.P1) together with 160 subjects with a palatally displaced canine (PDC) to determine the pattern of tooth agenesis in these cases and to compare them with similar samples reported in the literature. A strong association between Mx.C.P1, lateral incisor and lower second premolar agenesis was found, with a 20 per cent prevalence of lateral incisor agenesis and a 24 per cent prevalence of lower second premolar agenesis. There was a lesser association with third molar (M.3) agenesis, with a prevalence of 52.2 per cent. Weaker associations were found for a PDC, with a prevalence of 5 per cent for lateral incisor agenesis. The prevalence of lower second premolar (5 per cent) and M.3 (27.5 per cent) agenesis approached reference values. Evidence for the implication of the MSX1 or PAX9 genes in the aetiology of PDC was weak. (+info)
Developmental absence of maxillary lateral incisors in the Portuguese population.
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The aim of this study was to evaluate the prevalence and clinical manifestation of developmental absence of maxillary permanent lateral incisors in the Portuguese population. The study group comprised 16 771 patients observed between 1993 and 2000 at the Faculty clinic. Two hundred and nineteen patients were found to have missing upper lateral incisors (131 females and 88 males, with ages ranging between 3 and 71 years), a prevalence of 1.3 per cent in this population. Absence of these teeth was bilateral in 44.7 per cent of the patients; of the unilateral cases, 33 per cent occurred on the right side and 21.9 per cent on the left side. In children under 8 years of age (n = 12), two (0.26 per cent) also had developmental absence of the primary lateral incisors, one unilateral and the other bilateral. Among the 121 subjects with unilateral developmental absence, the contralateral maxillary lateral incisor was found to be microdont in 57.1 per cent (right lateral 23 per cent, left lateral 34.1 per cent), suggesting the possibility that microdontia represents a variable expression of the same developmental defect that results in absence. (+info)
Clinical pearl In-treatment replacement of missing incisors.
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Four methods of in-treatment replacement of missing incisors are described. (+info)
Reduction of Pax9 gene dosage in an allelic series of mouse mutants causes hypodontia and oligodontia.
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Missing teeth (hypodontia and oligodontia) are a common developmental abnormality in humans and heterozygous mutations of PAX9 have recently been shown to underlie a number of familial, non-syndromic cases. Whereas PAX9 haploinsufficiency has been suggested as the underlying genetic mechanism, it is not known how this affects tooth development. Here we describe a novel, hypomorphic Pax9 mutant allele (Pax9neo) producing decreased levels of Pax9 wild-type mRNA and show that this causes oligodontia in mice. Homozygous Pax9neo mutants (Pax9neo/neo) exhibit hypoplastic or missing lower incisors and third molars, and when combined with the null allele Pax9lacZ, the compound mutants (Pax9neo/lacZ) develop severe forms of oligodontia. The missing molars are arrested at different developmental stages and posterior molars are consistently arrested at an earlier stage, suggesting that a reduction of Pax9 gene dosage affects the dental field as a whole. In addition, hypomorphic Pax9 mutants show defects in enamel formation of the continuously growing incisors, whereas molars exhibit increased attrition and reparative dentin formation. Together, we conclude that changes of Pax9 expression levels have a direct consequence for mammalian dental patterning and that a minimal Pax9 gene dosage is required for normal morphogenesis and differentiation throughout tooth development. (+info)
Gorlin syndrome: a case report.
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Gorlin syndrome is an autosomal dominant inherited condition that exhibits high penetrance and variable expressivity. It is characterized mainly by Basal cell carcinomas, Odontogenic keratocysts and skeletal anomalies. However, medical literature documents both common and lesser known manifestations of the disorder involving the skin, central nervous system, skeletal system etc. Diagnosis of the syndrome in childhood is basically through oral abnormalities. A case of Gorlin syndrome has been reported here, with review of literature. (+info)