• Journal of Oral Medicine and Pain
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• v.31 no.2
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• pp.121-126
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• 2006

Hypohidrotic ectodermal dysplasia refers to a group of disorders with the following common features : thin, sparse or absent hair, missing or peg-shaped teeth and inability to sweat adequately. Both the primary and secondary dentition are affected. Teeth may be absent (anodontia) or reduced in number (oligodontia) and abnormally shaped. In case, A 5-year-old man presented with hypohidrosis and dry skin. He had no teeth in mouth. We report a case of hypohidrotic ectodermal dysplasia with anodontia.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.1
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• pp.150-156
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• 2009

Objectives : The conventional removable appliance, composed of wires and acrylic resin, had unaesthetic results and poor retention. The flexible denture, as an alternative, presents improved aesthetics with the thin and strong resin retentive area. In addition, it also enhances patients' sensory function as a result of decreased volume of denture base. The flexibility of the flexible denture reduces the possibility of fracture and distributes the masticatory forces transmitted to the abutments and residual bone tissue. This report describes a 10-year-old girl and a 6-year-old boy with oligodontia treated with the flexible dentures as an alternative to conventional removable appliances. Methods : Impression was taken using alginate material and sent to a laboratory with the bite for fabrication of the flexible denture. Prior to try-in, the flexible denture was immersed in water at $90^{\circ}C$ for one minute and cooled. Impinging area of the denture was checked by $Fit-Checker^{(R)}$ and removed and the denture was delivered to the patient. Results : Both patients were satisfied with the flexible dentures, which presented improved retention and aesthetics. Conclusion : For patients with oligodontia, flexible dentures can be considered as a treatment of choice, which may replace the conventional denture.

• The Journal of the Korean dental association
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• v.17 no.2 s.117
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• pp.137-140
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• 1979

• The Journal of Korean Academy of Prosthodontics
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• v.59 no.2
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• pp.238-247
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• 2021

Congenital tooth agenesis is the most common developmental dental anomaly, of which oligodontia is defined as the absence of six or more permanent teeth, except the third molars. Tooth agenesis causes malocclusion, alveolar atrophy, aesthetic and psychosocial problems. This clinical report describes a multidisciplinary treatment for a patient diagnosed as oligodontia, who exhibited absence of 14 permanent teeth, atrophy of maxillary alveolar bone, and mandibular protrusion. Restoration space was secured and tooth axis was improved by the extraction of deciduous teeth and orthodontic treatment. However, edge-to-edge bite of posterior teeth and arch dimension discrepancy due to atrophic maxilla was remained. To restore the aesthetics and functionality, implant retained prosthesis was planned. Considering minimal bone grafts, location and number of dental implants and prostheses design were determined. Through the gradual adjustment of provisional restoration, the appropriate centric and eccentric occlusion was reflected into a definitive prosthesis. Currently, stable functional results were attained, however, regular follow up and maintenance care over lifetimes should be performed.

• Journal of the korean academy of Pediatric Dentistry
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• v.34 no.4
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• pp.658-665
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• 2007

Oligodontia is defined as the congenital absence of six or more teeth in dentition, excluding the third molars. The prevalence of congenital missing teeth is about 1.6 to 9.6% of population and the prevalence of oligidontia is about 0.08 to 1.1%. The mandibular second premolar is the most frequently absent after the third molar, followed by the maxillary lateral incisor and upper second premolar. Females seem to be affected slightly more than males. Oligodontia may occur either in isolation, or as a part of a syndrome such as ectodermal dysplasia. Different causes are possible for oligodontia: physical obstruction or distruction of the dental lamina, space limitation, functional abnormalities of the dental epithelium, failure of induction of the underlying mesenchyme, chemotherapy, radiotherapy or genetic factor. Because oligodontia would result in esthetic and functional problems, such as facial asymmetry or occlusal disharmony, early diagnosis from clinical and radiographic examination was necessary. And appropriate treatment plan should be followed. This case report was about oral conditions and treatment of the oligodontia patients who have no specific systemic disease.

• Journal of the korean academy of Pediatric Dentistry
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• v.34 no.3
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• pp.532-542
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• 2007

The purpose of study was to review the transition of dentition according to the evolution of man to know the background of the dental problems like hypodontia and malocclusion. Man is Kingdom Animalia, Phylum Chordata, Class Mammalia, Order Primates, Suborder Haplorrhini, Superfamily Hominoidea, Family Hominidae, Genus Homo, Species Sapiens by taxonomy. The first hominid was Australopithecus which appeared c. 4 millions of years ago and showed bipedalism and distinct dentition. Homos began with H. habilis who appeared c. 2.5 millions of years ago and made stone tools, and then H. erectus and H. neanderthalensis appeared and disappeared until H. sapiens came. The dental formula of primitive mammalians which was I3 C1 P4 M3 changed to I2 C1 P4 M3 of primitive primates, to I2 C1 P3 M3 of Haplorrhini, and to I2 C1 P2 M3 of hominoids. That of H. sapiens is changing to I2 C1 P2 M2.The box type dentition of hominoids changed to the omega type dentition of Australopithecus, and to the parabolic type of H. sapiens. The size of teeth decreased continually, especially the canine and sexual dimorphism. The dentition moved backward and downward to the cranial crown according to the increase of the brain and decrease of the jaws. It was suggested that the change of diet to the starchy foods, food processing, and the development of cooking reduced the necessity of mastication and caused the change of dentition. The future of H. sapiens who is quite a new species in the earth histroy and is now causing the mass extinction of other species is hard to see. It seems that hypodontia and malocclusion are related to the dentition change according to the evolution of man and is likely to increase.

• Journal of the korean academy of Pediatric Dentistry
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• v.43 no.3
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• pp.299-305
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• 2016

The aim of this study was to identify the causative genetic mutation in a family with non-syndromic oligodontia. The 7-year-old female proband and her mother underwent oral examination, panoramic radiographs were obtained and blood samples were collected. All exons of the PAX9 gene were amplified by polymerase chain reaction and sequenced. The sequencing results were compared with the standard human gene sequence. The proband lacked 11 permanent teeth, and her mother lacked 19 permanent teeth. No other birth defects were observed. As a result of gene analysis, there was a novel heterozygous nonsense mutation (c.184G>T, $p.Glu62^*$) in exon 2 in both affected subjects. It is suspected that the nonsense mutation leads premature termination of translation, yields a truncated protein 280 amino acids shorter than the wild-type protein. These defects include parts of the paired box domain, a DNA-binding site that plays an essential role in protein function. Otherwise, more likely the mutant transcript would be degraded by nonsense-mediated decay system, resulting haploinsufficiency to cause oligodontia in this family.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.4
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• pp.631-639
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• 2009

Ectodermal dysplasia is a genetic disease which shows various congenital dysplasias in tissues differentiated from the ectoderm. As the most common type of the ectodermal dysplasia, hypohidrotic ectodermal dysplasia(HED) shows dysplasia mainly in the hair, fingernails, teeth and the skin. Symptoms are more severe in males than in females and heterozygous females are usually normal showing no symptom. The treatment for these patients differ according to individuals, but since patients can easily become depressed socially and emotionally due to a decrease in mastication and speech function caused by multiple loss of teeth as well as some aesthetic problems, an early treatment is required. In a case, with a 10 years-old boy diagnosed with HED which shows partial edentia of the maxilla, and the edentia of the mandible in the pediatric dentistry department of the Chosun University Dental Hospital, a fabrication of denture resulted in the recovery of mastication and speech function and aesthetic improvement due to an increase of the face height.

• The korean journal of orthodontics
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• v.30 no.6 s.83
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• pp.739-745
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• 2000

Ectodermal dysplasia is a hereditary disease characterized by a congenital dysplasia of one or mote ectodermal structures. Characteristic manifestations Include scanty hair and eyebrows, pigmented and hyperkeratinized skin around the eyes and mouth, frontal bossing with prominent supraorbital ridges, nasal bridge depression and dental anomalies. Hyperthermia or unexplained high fever as a result of the deficiency of sweat glands is common medical history. Findings of intraoral structures are anodontia or oligodontia with conical crowns. Consequently, generalized spacing and loss of vertical dimension of occlusion. Interdisciplinary approach has been performed to treat a 10-year old boy with ectodermal dysplasia. Orthodontists and a prosthodontist worked together on this case, and the result was satisfactory.

• Journal of the korean academy of Pediatric Dentistry
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• v.28 no.4
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• pp.626-632
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• 2001

Ectodermal dysplasia is a hereditary disease characterized by congenital dysplasia of one or more ectodermal structures. Intraorally, common findings are anodontia or oligodontia, conical teeth, and, consequently, generalized spacing. This case presented the oral rehabilitation of a child with hypohidrotic ectodermal dysplasia. Oral rehabilitation is important from functional, esthetic, and psychologic perspectives. Due to the absence of teeth, the volume of alveolar bone and its growth are decreased, resulting in a loss of vertical dimension and protuberant lips. The treatment involved increasing the patient's vertical dimension of occlusion, fabricating a maxillary partial denture, and using magnets to help retain the mandibular partial denture. A 5-year 7-month old Korean boy was referred to the pediatric department for examination, evaluation and treatment of his disorder. we used magnets on '73 and '83 for enhanced retention of a mandibular overdenture. The magnet used in this case was the Magfit system(GC Co., Japan).