Ectodermal dysplasia with associated double tooth.

G Atar; M Uzamiş; S Olmez

PubMed · 9391716

Ectodermal dysplasia with associated double tooth.

Abstract

The case describes a double molar tooth in a seven-year-old girl who has ectodermal dysplasia. The most characteristic dental findings in ectodermal dysplasia are hypodontia and conically shaped crowns. In our case a double tooth was also present in the primary molar region, in addition to these characteristic findings.

Explore related subjects

Anodontia Child Ectodermal Dysplasia Female Humans Incisor Molar Tooth Crown Tooth, Deciduous Case Reports Journal Article

Keep this discovery

Explore connections, maps & timelines

BibTeX RIS

G Atar, M Uzamiş, S Olmez. Ectodermal dysplasia with associated double tooth.. https://pubmed.ncbi.nlm.nih.gov/9391716/

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

PubMed2007

Implants in children with ectodermal dysplasia: a case report and literature review.

The replacement of teeth by implants is usually restricted to patients with completed craniofacial growth. Implant insertions in children or adolescents are circumvented due to several unfavorable potential effects including trauma to tooth germs, tooth eruption disorders and multidimensional restrictions of skeletal craniofacial growth. Moreover, the functional and esthetic results of the oral rehabilitation are only temporary acceptable. However, to a small number of pediatric patients suffering congenitally from severe hypodontia caused by syndromes such as ectodermal dysplasia, conventional prosthodontic rehabilitations are insufficient. We report the case of a boy with ectodermal dysplasia who exhibited a severe hypodontia and who was treated with implants inserted into the anterior mandible at the age of 8 years. The implants were functionally loaded and resulted in a high patient satisfaction. We recommend the early insertion of dental implants in children with severe hypodontia. Reviewing the current literature, several aspects of syndromic hypodontia, patient selection and implant planning are discussed.

Anodontia↗

PubMed2007

Prosthodontic treatment of hypohidrotic ectodermal dysplasia with complete anodontia: case report.

The hereditary condition known as ectodermal dysplasia is characterized by the absence or defect of 2 or more ectodermally derived structures. A case of a 6-year-old child with hypohidrotic ectodermal dysplasia with complete anodontia is presented. Common dental, oral, and physical conditions were taken into consideration. Clinical management consisted of removable complete dentures to improve psychologic development and to promote better functioning of the stomatognathic system.

Anodontia↗

PubMed2006

A novel missense mutation in MSX1 underlies autosomal recessive oligodontia with associated dental anomalies in Pakistani families.

Tooth agenesis constitutes the most common anomaly of dental development in humans. In the majority of familial cases of hypodontia alone or in association with other anomalies, the mode of inheritance is autosomal dominant. In the present study, we have identified two distantly related consanguineous Pakistani kindreds with an autosomal recessive form of oligodontia with associated dental anomalies. Locus in this case has been mapped on chromosome 4p16.1-p16.3. The maximum two-point LOD score of 2.85 (theta=0.0) was obtained at markers D4S2925 and D4S2285. A maximum multipoint LOD score exceeding 4 was obtained at the same markers. Recombination events observed in affected individuals localized the disease locus between markers D4S412 and D4S2935, spanning a 9.24-cM region on chromosome 4p16.1-p16.3. Sequence analysis of candidate gene MSX1 revealed a novel recessive missense mutation resulting in substitution of alanine to threonine amino acid (p. A219T), located in the MSX1 homeodomain, which is important for DNA binding and protein-protein interaction. The mutation, p. A219T, is the first recessive mutation identified in MSX1.

Anodontia↗