• Maxillofacial Plastic and Reconstructive Surgery
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• v.23 no.3
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• pp.205-211
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• 2001

Cleft palate is one of the most serious congenital anomalies in human that causes a sucking problem in newborn babies and morphologic deformity that usually leads to death in newborn mouse offspring due to an insufficient ability to suck milk. Therefore cleft palate had been researched with epidemiologic and molecular methods, and many etiologic factors were examined closely. Among of the research methods, biologic molecule researches have been more important method for cleft palate formation study. The $TGF-{\beta}$ had an important role in the cell migration, epithelial-mesenchymal transdifferentiation, extracellular matrix synthesis and deposition. But there was a little research which was study about correlation cleft palate induced by beta-aminonitroproprionitrile(BAPN) with $TGF-{\beta}$ expression. A purpose of this presented study was examed how $TGF-{\beta}$ expression in cleft palate mice. At gestation days 13, BAPN-monofumarate salts($(C_3H_6N_2)_2$ ${\cdot}$ $C_4H_4O_4$, Sigma Co.) was single oral administered to 4 pregnant rats according to 1g/kg body weight. And pregnant rats were sacrificed on day 20 post coitus(p.c.), The $TGF-{\beta}$ expression patterns of cleft formed fetus mice was followed that; 1.Osteoblast, mesenchymal cell and epithelial cell of cleft mice were low expression compare to control mice. 2.There was no $TGF-{\beta}$ difference expression pattern of osteocyte of cleft mice compare to control mice. 3. In western blot analysis, thickness of band of $TGF-{\beta}$ in cleft mice was thin and dilute compare to control mice.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.33 no.6
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• pp.681-683
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• 2007

Kabuki(Niikawa-Kuroki) syndrome was first reported by Niikawa et al(1981). The faces of the patients are similar to the make-up of traditional Japanese Kabuki actors: long palpebral fissures, an ectropium of the lateral third of the eyelids, and arching eyebrows with sparse lateral halves. Craniofacial findings include a depressed nasal tip, short nasal septum, large and prominent ears, and micrognathia. Other main features area mild to moderate mental deficiency, short stature, skeletal and dermatoglyphic abnormalities, including prominent finger tip pads. Oral anomalies are common in KS(over 60%) and include abnormal dentition, widely spaced teeth, cleft palate or lip, high vault of palate, hypodontia, conical incisors, screw driver-shaped incisors and ectopic upper 6-year molars. The increased occurrence of cleft lip and palate or the development of a high vault of palate has been described by a number of authors. This condition is believed to be common in Japan, but has been reported from other parts of the world. The objective of this presentation is to report a case of this syndrome in six-year-old girl, with characteristic findings.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.19-24
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• 2016

Constitutional interstitial deletions of the long arm of chromosome 5 (5q) are quite rare, and the corresponding phenotype is not yet clearly delineated. Severe mental retardation has been described in most patients who present 5q deletions. Specifically, the interstitial deletion of chromosome 5q33.3q35.1, an extremely rare chromosomal aberration, is characterized by mental retardation, developmental delay, and facial dysmorphism. Although the severity of mental retardation varies across cases, it is the most common feature described in patients who present the 5q33.3q35.1 deletion. Here, we report a case of a de novo deletion of 5q33.3q35.1, 46,XY,del(5)(q33.3q35.1) in an 11-year-old boy with mental retardation; to the best of our knowledge this is the first case in Korea to be reported. He was diagnosed with severe mental retardation, developmental delay, facial dysmorphisms, dental anomalies, and epilepsy. Chromosomal microarray analysis using the comparative genomic hybridization array method revealed a 16-Mb-long deletion of 5q33. 3q35.1(156,409,412-172,584,708)x1. Understanding this deletion may help draw a rough phenotypic map of 5q and correlate the phenotypes with specific chromosomal regions. The 5q33.3q35.1 deletion is a rare condition; however, accurate diagnosis of the associated mental retardation is important to ensure proper genetic counseling and to guide patients as part of long-term management.

• 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.24 no.4
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• pp.795-804
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• 1997

Developmental dental morphological anomalies are fusion, gemination, twinning, concrescence and etc. They may cause many problems in conservative, periodontal and esthetic aspects. Fusion is a condition where two separate tooth buds unite at some stage in their development to form a bifid crown. If tooth contact occurs early, at least before the start of calcification, the two teeth may be completely unites to form a single large tooth. If tooth contact occurs after the time when a portion of the tooth crown has completed its formation, there may be union of the roots only. In fusion the dentin is always confluent. Fusion teeth is more common in the deciduous than in the permanent dentition. Fused teeth are relatively rare, and are mostly mandibular anterior teeth. Fusion of normal and supernumerary teeth or between normal teeth may occurs. The exact etiology factor of fusion is unknown, but genetic and environmental factors seems to be related. A variety of complications and subsequent treatments have been suggested on this teeth. Periodontal conditions may arise due to a groove formed at the line of fusion of the two teeth. Crowding or Protrusion with potential for malocclusion or delayed eruption of adjacent teeth. Fusion teeth appear in the anterior region, they usually cause esthetic problems. Treatments vary depending on the problem, the location, and the extent of fusion. Treatment of fused teeth has been reported from endodontic, orthodontic, periodontic, surgical and multidisciplinary. This report presents the esthetic improvements by separation of two clinical crowns. Bone reduction or endodontic treatments are not required.

• The korean journal of orthodontics
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• v.51 no.1
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• pp.55-74
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• 2021

Objective: To systematically review studies on canine agenesis prevalence in different populations and continents, based on the jaw, sex, location, and associated dental anomalies. Methods: Electronic and hand searches of English literature in PubMed, Web of Science, Scopus, OpenGrey, and Science Direct were conducted, and the authors were contacted when necessary. Observational studies (population-based, hospital/clinic-based, and cross-sectional) were included. For study appraisal and synthesis, duplicate selection was performed independently by two reviewers. Study quality was assessed using a modified Strengthening the Reporting of Observational Studies in Epidemiology checklist, with main outcome of prevalence of canine agenesis. Results: The global population prevalence of canine agenesis was 0.30% (0.0-4.7%), highest in Asia (0.54%), followed by Africa (0.33%), and the least in Europe and South America (0.19% in both continents). Canine agenesis was more common in the maxilla (88.57%), followed by both maxilla and mandible (8.57%), and the least common was mandible-only presentation (2.86%). The condition was more common in females (female:male ratio = 1.23), except in Asia (female:male ratio = 0.88) and Africa (female:male ratio = 1). In Asia, unilateral agenesis was almost twice as prevalent as bilateral, but in Europe, the bilateral form was more common. Conclusions: The overall prevalence of canine agenesis is 0.30%, with the highest prevalence in Asia, followed by Africa, Europe, and South America. The condition is more common in the maxilla than the mandible, and in females than males (except in Asia and Africa), with unilateral agenesis being more common in Asia and the bilateral form showing a greater prevalence in Europe.

• Journal of the korean academy of Pediatric Dentistry
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• v.50 no.1
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• pp.35-46
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• 2023

Congenitally missing teeth are one of the most prevalent dental anomalies in the oral cavity. In this study, the prevalence, distribution, and symmetry of congenitally missing permanent teeth among 1,865 patients aged 7 to 15 years who visited the Pediatric Dentistry Department of Wonju Severance Christian Hospital from March 2011 to May 2021 and took panoramic radiographs were investigated and analyzed. Most of the patients had one or two congenitally missing teeth, mainly in the second premolars and lateral incisors. Congenitally missing teeth occurred more in the mandible than in the maxilla, and there was no significant difference in prevalence between the left and right sides. Congenitally missing teeth tend to occur symmetrically on the left and right sides and in the maxilla and mandible, depending on the tooth. Early oral examination and radiological examination are required to prevent complications due to congenitally missing teeth, and appropriate interdisciplinary treatment is required.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.9 no.1
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• pp.42-45
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• 2013

De Grouchy syndrome or Distal 18q- is a genetic condition caused by a deletion of genetic material within chromosome 18, and the deletion involves the distal section of 18q. It causes a wide range of medical and developmental concerns. Congenital orthopedic anomalies, cleft lip and palate are relatively common. People with distal 18q- are often small for their age. Most individuals with distal 18q- fall in the mild to moderate range of intellectual disability. Strabismus and nystagmus, changes in the optic nerve as well as colobomas are also fairly common. People with distal 18q- frequently have conductive and/or sensorineural hearing loss. At present, treatment for distal 18q- is only symptomatic. This article presents a case report: Caries treatment of a 4-year-old female patient with de Grouchy syndrome under general anesthesia. The special considerations of dental care, especially caries treatment for the patient with de Grouchy syndrome are discussed.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.2
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• pp.262-266
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• 2004

The parathyroid hormone plays a major role in the metabolism of calcium and phosphorus. In hypoparathyroidism the parathyroid glands are atrophied or absent associated with autoantibodies against parathyroid tissue. Pseudohypoparathyroidism is a metabolic disease caused by the disturbance in peripheral action of parathormone, but parathormone level is normal. In general, patients with pseudohypoparathyroidism have short stature, round face, brachydactylia, obesity, mental retardation, cataracts & ectopic calcifications on soft tissues. Dental manifestations are enamel hypoplasia, delayed eruption, blunting of root apex, hypodontia, pulp calcification, thickened lamina dura, excessive caries & malocclusion. In this case, intraoral examination showed enamel hypoplasia on the erupted permanent teeth & hypermobility on the remaining deciduous teeth. From the radiographic view severe dental anomalies were observed on canines and shortening and blunting of root apex was observed on mandibular incisors. Pathologic root resorption was also observed on deciduous teeth.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.3
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• pp.473-478
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• 1999

Osteopetrosis is an uncommon hereditary bone condition characterized by a generalized symmetric increase in skeletal density and abnormalities of bone resorption remodeling. In 1904, the first case of generalized sclerosis of the skeleton was reported by $Albers-Sch\ddot{o}nberg$. Osteopetrosis is generally divided into two main type. The infantile(malignant, congenita) type is the most severe form of the disease; It is characterized by skeletal and hematologic abnormalities. The adult(benign, tarda) type which is usually diagnosed in the third or fourth decade of life is limited predominantly to skeletal anomalies and it carries a more favorable prognosis. The recently recognized intermediate form with its mild and variable clinical recessive trait. There is no reported gender or racial predilection. The characteristic feature of osteopetrosis which is an abscence of physiologic bone resorption results in accumulation of bone mass and mainfests skeletal disturbance. Dental finding of osteopetrosis includes delayed eruption, congenitally absent teeth, unerupted and malformed teeth, and enamel hypoplasia. Our report involves a patient with a chief complaint of tooth mobility and delayed eruption. After clinical and radiologic examination, this patient was referred to dept. of pediatrics under the suspicion of osteopetrosis and it was confirmed.