• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.4
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• pp.467-471
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• 2010

Amelogenesis imperfecta, one of the dental genetic disease, is clinically and genetically complex disease. Amelogenesis imperfecta can be classified into three major categories according to clinical phenotype; hypoplastic, hypomaturation, and hypocalcification. Recently a novel gene, Fam83h, was identified to cause autosomal dominant hypocalcification amelogenesis imperfecta, however its functional role in the pathogenesis of enamel defect is not known yet. So this study was aimed to identify the knockdown effect of Fam83h gene on the amelogenin mRNA expression via shRNA transfection into immortalized ameloblast cell line. The result showed that the knockdown of Fam83h did not influence the amelogenin expression. Further study of the functional role of Fam83h gene should be performed to understand the complex nature of amelogenesis as well as molecular pathogenesis of amelogenesis imperfecta.

• The Journal of Korean Academy of Prosthodontics
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• v.52 no.4
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• pp.317-323
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• 2014

Dentinogenesis Imperfecta, with a high incidence rate of 1 : 6 - 8000, is inherited by autosomal dominant genetic transmission. This dental disorder causes discoloration of the teeth and the enamel and dentin show hypoplastic or hypocalcified defects which lead to frequent fractures and rapid attrition. Therefore, timely treatment is necessary for the preservation of the remaining teeth. In this particular case, a 19-year-old patient suffering from Type 1 dentinogenesis imperfecta showed signs of brownish hued teeth with multiple fractures, a loss of vertical dimension, excessive interdental space in the maxillary anterior teeth, and a lack of 5 posterior teeth. To improve the esthetic appearance of the anterior teeth, the vertical dimension was increased. Resin caps were used to alleviate the difficulty of taking an impression of multiple teeth at once. Monolithic zirconia materials used in this case showed high fracture strength and the ability to mask the discoloration of the teeth and therefore, functionally and esthetically favorable results were achieved.

• Journal of the korean academy of Pediatric Dentistry
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• v.47 no.3
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• pp.344-351
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• 2020

Amelogenesis imperfecta (AI) occurs either in isolation or in association with other dental abnormalities and systemic disorder. A rare syndrome associating AI with nephrocalcinosis was named as Enamel Renal Syndrome (ERS; OMIM #204690). This syndrome is characterized by severe enamel hypoplasia, failed tooth eruption, intra pulpal calcifications, enlarged gingiva, and nephrocalcinosis. Nephrocalcinosis is a condition where calcium salts are deposited in renal tissue, and this may lead to critical kidney complications. This rare syndrome shows pathognomonic oral characteristics that are easily detectable at an early age, which proceeds the onset of renal involvement. Pediatric dentists are the first oral health practitioners whom ERS patients will meet at early age. The role of pediatric dentists is critically important for early diagnosis and referral of patients to both nephrologists for renal assessment and geneticists for identification of causative mutation and diagnosis. Early detection of renal involvement may provide chances to prevent further undesired renal complications.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.3
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• pp.400-405
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• 2004

Biliary atresia is defined as a complete obstruction of bile flow owing to destruction or absence of all or part of the extrahepatic bile ducts. This disease is occurring in approximately 1:10,000 live births and moderate predominance of female is noted. The etiology of biliary atresia remained unsolved. The signs and symptoms are hyperbilirubinemla, jaundice, clay-colored stools, steatorrhea, dark yellow urine and hepatomegaly. Currently biliary atresia is best managed by hepatic portoenterostomy with or without liver transplantation. Biliary atresia patients with these cases showed staining of the teeth. The stains ranged in color from yellowish-brown to deep green. Enamel hypoplasia was all erupted teeth present. Patients had poor oral hygiene and rampant caries.

• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.3
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• pp.504-508
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• 2008

Delayed eruption and abnormal morphology of the primary incisors following intubation may be due to follicular displacement and localized trauma caused by the process of intubation. Result of such damage included palatal groove formation, defective incisors and acquired clefts. This clinical report presents effect of intubation on the primary dentition of premature low-birth-weight children prior to tooth eruption.

• Journal of the korean academy of Pediatric Dentistry
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• v.27 no.1
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• pp.108-112
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• 2000

Hypophosphatemic rickets is lack of reponse to physiologic doses of vitamin D, different from the vitamin D-dependent rickets. It is inherited in an X-linked dominant fashion. The prime features of this disorder are lowered serum phosphate levels, elevated serum alkaline phosphatase and normal serum calcium levels. The dental manifestation often include apical radiolucencies, abscess and fistulas of clinically sound teeth. Dental radiographs show ricketic bone trabeculations, abscent or abnormal lamina dura and abnormal cementum. This case which was diagnosed to hypophosphatemic rickets, showed multiple spontaneous periapical abscess and gingival fistula enlarged pulp chambers, extension of the pulp horns into the cusp tips and delayed eruption.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.1
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• pp.124-129
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• 2010

Seckel syndrome is an autosomal recessive disorder characterized by intrauterine growth retardation and post-natally by dwarfism, severe microcephaly, bird-headed profile with receding chin, prominent nose, joint defects, clubfoot, sparse hair, malformation of genitourinary tract and rectum, mental retardation and hematological disorders. There is also a reduction in the number of blood cells. Dental anomalies of Seckel syndrome are crowded teeth with malocclusion, enamel hypoplasia, absence of some teeth and taurodontism. This report described the oral and maxillofacial manifestations of children associated with Seckel syndrome. Children with Seckel syndrome have several dental and skeletal irregularities. The purpose of this study was to report the dental and medical characteristics of the patient and review the literatures of Seckel syndrome.

• Journal of the korean academy of Pediatric Dentistry
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• v.28 no.2
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• pp.255-260
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• 2001

Trauma to the primary incisors may cause developmental or eruptional disturbance of the permanent successors. Two cases illustrate developmental disturbances of arrested root formation, dilaceration, and eruptional disturbances of impaction, ectopic eruption of permanent successors caused by traumatic injury to deciduous incisors. The patient of the first case suffered trauma at the age of 4 years 7 months, causing alveolar bone fracture including the maxillary right primary central and lateral incisors that were immedi-ately extracted. The second patient had trauma episodes at the age of 3 years. Avulsion of the maxillary primary right central and lateral incisors were occurred due to trauma. After such trauma, regular follow-up including radiographs is necessary to detect early any possible interference with normal eruption of permanent successors.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.2
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• pp.251-255
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• 2005

Radiotherapy for head and neck tumors is a viable treatment modality. However, a wide range of potentially debilitating dental complications may be accompanied by this treatment. We report two cases of developmental disturbance of permanent tooth germs after radiotherapy. The one was that of a seven-year-old girl, who had congenitally missing teeth, and microdontia of permanent tooth germs. she had received radiotherapy for acute myelocytic leukemia at the age of 19 months. The other was that of a nine-year-old boy, in which congenitally missing teeth, microdontia, root hypoplasia, and enamel hypoplasia of permanent teeth were observed. He had undergone a course of radiotherapy for bilateral retinoblastoma at the age of 13 months.

• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.1
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• pp.144-150
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• 2008

Multimodal cancer therapy including surgery, chemotherapy, and radiotherapy could not only improve the prognosis of malignancy but also reduce the dosage and toxicity of cancer drug for treatment of malignant tumor. The effects of radiotherapy are generally localized, additive, and accumulative, and depend on dosage, site and cell sensitivity. However, in growing individuals, the dental and skeletal sequelae to radiotherapy result in dental or facial abnormalities that are irreversible : arrested root development, disturbances in enamel formation, microdontia, anodontia, altered tooth eruption and mandibular or maxillary hypoplasia. Especially, the teeth which are developing is affected according to the stage. We report three cases of developmental disturbance of permanent teeth after radiotherapy. These children had received radiotherapy for malignant tumor at the age of 3 to 4 years, in which root hypoplasia, short tapered root and early apex closure were observed. For the management of radiation caries and radiotherapy-related teeth, periodic recall check and oral hygiene instruction are required.