• The Journal of the Korean dental association
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• v.22 no.4 s.179
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• pp.329-332
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• 1984

The author observed a patient of ectodermal dysplasia with oligodontia. The treatments were done as follows: Vital pulpotomy and restorative treatment were done as usual method. Removable type space maintainer was set on the area of primary teeth removed. Follow-up checking were required for the observation of growth and development of the permanent teeth and the jaw.

• Journal of the korean academy of Pediatric Dentistry
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• v.50 no.4
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• pp.483-494
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• 2023

This report presents two cases concerning strategies for improving denture retention in pediatric patients with oligodontia caused by hypohidrotic ectodermal dysplasia (HED). Both patients presented with multiple missing teeth, conical canines, alveolar bone atrophy, and a skeletal Class III tendency. In the first case, a modified form of conical-crown-retained denture was used to cover the canines. This approach was carried out without tooth extraction or coping. In the second case of severe alveolar bone resorption and a distally tilted lower left canine, the tooth was restored with a hybrid ceramic crown. It was subsequently converted into a clasp-retained removable partial denture, utilizing a suction mechanism. Both patients are currently receiving regular check-ups for the maintenance of their prosthetic appliances and the evaluation of their growth patterns. This study presents innovative prosthetic treatment methods for pediatric patients with HED who have inadequate denture retention.

• Journal of the korean academy of Pediatric Dentistry
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• v.21 no.2
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• pp.570-576
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• 1994

Ectodermal dysplasia is characterized by a partial or complete lack of primary and permanent teeth, other ectodermal structures that may be affected include the skin, hair, and sweat glands. The patient with the so-called anhidrotic type of ectodermal dysplasia exhibits dry skin, lack of sweat glands, sparse eyebrows, body hair, saddle nose, and everted lips. Genetic basis of anhidrotic ectodermal dysplasia is recessive and sex-linked, being manifested chiefly in males, but this is debatable. A 6-year-old boy, with typical signs of anhidrotic ectodermal dysplasia, was presented. Prosthetic restoratoins are of great value to these patients, both from the standpoint of function and for psychologic reasons. The need for complete denture is critical during preschool periods and continues into adulthood. The following case report is an approach to the management of a patient with anhidrotic ectodermal dysplasia.

• Journal of the korean academy of Pediatric Dentistry
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• v.42 no.3
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• pp.270-274
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• 2015

Although there are a number of studies on the treatment planning and the prognosis of dental implants, limited studies provide the information on the implant placement on growing adolescents. The aim of this review is to discuss the use of dental implants in growing patients and the impact of skeletal and dentoalveolar growth on the long-term stability of implants. The general information regarding skeletal growth of maxilla and mandible would be briefly reviewed and the general treatment options would be discussed.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.4
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• pp.636-643
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• 1999

Ectodermal dysplasia is a genetic birth defect in which at least abnormally develop two structures derived from the ectoderm. It is usually inherited in autosomal dominant or autosomal recessive pattern. Oral manifestations are oligodontia, anodontia, dysmorphic teeth(conical shape), decreased occlusal vertical dimension and alveolar bone. Extraoral signs may include decreased or absent sweat glands, sparse and fine hair, saddle nose, hearing loss and decreased production of body fluids including saliva. Most affected children require extensive dental treatment to restore their appearance and help the development of a positive self image. The patient's overclosed profile was due to a decreased vertical dimension. The use of overdenture is to preserve erupted teeth, to accomodate the newly constructed occlusal plane, to improve retention and stability of denture and to maintain the remaining alveolar bone. The restoration of vertical dimension improved the child's speech, swallowing, and eating. Growth continue until the age of approximately 18. As child grows, replacement dentures will have to be fabricated primarily to accomodate increasing vertical dimension and changing dentition. Implants may be indicated later if the alveolar bone is adequate. Periodic recall visits are advised, to monitor the dentures during periods of growth and development, and eruption of the permanent teeth.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.4
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• pp.701-704
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• 2004

In this case, we would like to discuss about the single rooted deciduous mandibular first molar. The deciduous mandibular molar of a five years old boy was shown to have a single root, bilaterally. Ordinarily, the maxilary molars have three roots and the mandibular molars have two roots. However, when the hertwig's epithelial root sheath do not invaginate properly during tooth development, root fusion can occur from the absence of root separation. Molars with fused roots not only have unfavorable crown to root ratio, but also according to many reports, have higher probability of having multiple congenitally missing teeth or dens invaginatus in the maxillary incisors, consequently requiring preventive dental treatment In addition, disorders such as ectodermal dysplasia, syndactyly, clinodactyly, bluish sclera can also be related to this condition. Root fusion is known to be of autosomal recessive inheritance. Up to date, single rooted molars have been reported several times in permanent dentitions but hardly in deciduous dentition, which is the motive for this paper.

• 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.33 no.1
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• pp.122-130
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• 2006

Congenital Missing Teeth(hypodontia, oligodontia) is the developmental absence of one or more teeth. It has been reported as being the most common anomaly of dental development in human, relatively common in the permanent dentition. In a recent review, Vastardis has quoted incidence ranges of $1.6%{\sim}9.6%$ in the permanent dentition. Brook has quoted a prevalence of $3.5%{\sim}6.5%$ in most populations, with severe hypodontia, defined as the absence of six or more teeth, having a prevalence of $0.3{\sim}0.4%$. The most commonly affected teeth are third molars, followed by maxillary lateral incisor, and second premolars. The etiology is unknown, several hypotheses include trauma, nutritional deficiency, infection, metabolic abnormalities, systemic disease and genetic influence. The multiple congenital missing is commonly associated with specific syndrome or severe systemic abnormalities such as cleft lip & palate and Down's syndrome. These cases present that children have multiple congenital missing teeth in the permanent dentition, without any systemic disease. Management of this condition must be considered orthodontic and prosthodontic treatment comprehensively. In these cases, children were treated by space maintainer or orthodontic appliance and follow-up checked.

• Clinical and Experimental Pediatrics
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• v.46 no.2
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• pp.128-136
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• 2003

Purpose : Hyper IgM syndrome(HIGM) is characterized by severe recurrent bacterial infections with decreased serum levels of IgG, IgA, and IgE but elevated IgM levels. Recently, it has been classified into three groups; HIGM1, HIGM2 and a rare form of HIGM. HIGM1 is a X-linked form of HIGM and has now been identified as a T-cell deficiency in which mutations occur in the gene that encodes the CD40 ligand molecule. HIGM2 is an autosomal recessive form of HIGM. Molecular studies have shown that the mutation of HIGM2 is in the gene that encodes activation-induced cytidine deaminase(AID). Recently, another rare form of X-linked HIGM syndrome associated with hypohydrotic ectodermal dysplasia has been identified. We encountered a patient with a varient form of HIGM2. To clarify the cause of this form of HIGM, we evaluated the peripheral B cells of this patient. Methods : The lymphocytes of the patient were prepared from peripheral blood. B cells were immortalized with the infection of EBV. Cell cycle analysis was done with the immortalized B cells of the patient. Peripheral mononuclear cells were stained with monoclonal anti-CD40L antibody. Total RNA was extracted from the peripheral mononuclear cells. After RT-PCR, direct sequencing for CD40L gene and HuAID gene were done. Immunostainings of a lymph node for CD3, CD23, CD40, Fas-L, bcl-2, BAX were done. Results : The peripheral B cells of this patient showed normal expression of CD40L molecule and normal sequencing of CD40L gene, and also normal sequencing of AID gene. Interestingly, the peripheral B cells of this patient showed a decreased population of G2/mitosis phase in cell cycles which recovered to normal with the stimulation of IL-4. Conclusion : We suspect that the cause of increased serum IgM in this patient may be from a decrease of G2/mitosis phase of the peripheral B cells, which may be from the decreased production or secretion of IL-4. Therefore, this may be a new form of HIGM.