• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.10-13
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• 2016

Chromosome 11q13 deletion syndrome has been previously reported as either otodental syndrome or oculo-oto-dental syndrome. The otodental syndrome is characterized by dental abnormalities and high-frequency sensorineural hearing loss, and by ocular coloboma in some cases. The underlying genetic defect causing otodental syndrome is a hemizygous microdeletion involving the FGF3 gene on chromosome 11q13.3. Recently, a new form of severe deafness, microtia (small ear) and small teeth, without the appearance of eye abnormalities, was also reported. In this report, we describe a 1-year-old girl presenting with ptosis of the left upper eyelid, right auricular deformity, high-arched palate, delayed dentition, simian line on the right hand, microcephaly, and developmental delay. In this patient, we identified a deletion in the chromosome 11q13.2-q13.3 (2.75 Mb) region by using an array-comparative genomic hybridization analysis. The deletion in chromosome 11q13 results in a syndrome characterized by variable clinical manifestations. Some of these manifestations involve craniofacial dysmorphology and require a functional workup for hearing, ophthalmic examinations, and long-term dental care.

• Journal of Korean Academy of Oral Health
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• 제42권4호
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• pp.238-241
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• 2018

Williams syndrome is a rare congenital disorder with various physical abnormalities and characterized by facial, oral, and dental features. Individuals with Williams syndrome typically have eating disorders in the early childhood, which lead to prolonged night feeding. Prolonged night feeding is a risk factor for rampant dental caries. Williams syndrome is caused by the microdeletion of chromosome 7, resulting in elastin deficiency. Elastin is integral to cardiovascular health. Many patients with Williams syndrome have complex cardiovascular abnormalities that must be considered a part of dental management. Complications related to cardiovascular diseases may induce adverse effects such as dangerously elevated blood pressure. This may occur in patients during stressful dental treatment. In addition, characteristics of auditory hyperalgesia and anxiety disorders among patients with William syndrome, complicate receiving routine dental management. Therefore, dental treatment under sedation or general anesthesia may be preferable for patients with Williams syndrome; in particular, patients who are very uncooperative and/or needs extensive dental treatment. A thorough evaluation of each patient's physical condition is required before making decisions regarding dental treatment. Careful monitoring and preparation for emergencies are very important during and shortly after dental treatment under general anesthesia or sedation. Monitoring is critical until vital signs have stabilized and return to normal. A 28-month-old man diagnosed as having Williams syndrome, visited the Dental Hospital of ${\bigcirc}{\bigcirc}$ University for the management of rampant dental caries. We reported on the management of this patient who had peripheral pulmonic stenosis, and received dental treatment under general anesthesia. We also reviewed the characteristics of Williams syndrome and discussed considerations for dental treatment under general anesthesia.

• Journal of Dental Anesthesia and Pain Medicine
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• 제20권1호
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• pp.49-53
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• 2020

Cri-du-chat syndrome (CdCS) is caused by the deletion of the short arm of chromosome 5. Most patients with CdCS develop intellectual disabilities. Therefore, they have poor oral hygiene and a high caries index. However, treating such patients is not an easy task, because of the difficulty in communication. General anesthesia may be a useful option in adult patients with CdCS and intellectual disability. General anesthesia should be administered very carefully, owing to the presence of comorbid diseases, which may need airway management. Infants with CdCS need general anesthesia if they have a concomitant cardiac anomaly. Intubation is reportedly difficult for such patients was, owing to the structural and functional abnormalities in the larynx and vocal cords. The purpose of this study was to report a case of difficult intubation while inducing general anesthesia in a patient with CdCS during dental treatment, due to a narrow larynx and trachea.

• Imaging Science in Dentistry
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• 제49권3호
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• pp.191-199
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• 2019

Purpose: To compare the diagnostic ability of undergraduate dental students to detect maxillary sinus abnormalities in panoramic radiographs(PR) and cone-beam computed tomography (CBCT). Materials and Methods: This was a retrospective study based on the evaluation of PR and CBCT images. A pilot study was conducted to determine the number of students eligible to participate in the study. The images were evaluated by 2 students, and 280 maxillary sinuses were assessed using the following categories: normal, mucosal thickening, sinus polyp, antral pseudocyst, nonspecific opacification, periostitis, antrolith, and antrolith associated with mucosal thickening. The reference standard was established by the consensus of 2 oral radiologists based on the CBCT images. The kappa test, receiver operating characteristic curves, and 1-way analysis of variance with the Tukey-Kramer post-hoc test were employed. Results: Intraobserver and interobserver reliability showed agreement ranging from substantial (0.809) to almost perfect (0.922). The agreement between the students' evaluations and the reference standard was reasonable (0.258) for PR and substantial(0.692) for CBCT. Comparisons of values of sensitivity, specificity, and accuracy showed that CBCT was significantly better(P<0.05). Conclusion: CBCT was better than PR for the detection of maxillary sinus abnormalities by dental students. However, CBCT should only be requested after a careful analysis of PR by students and more experienced professionals.

• Imaging Science in Dentistry
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• 제40권1호
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• pp.25-32
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• 2010

Purpose : This study was designed to evaluate the efficacy and utility of panoramic radiograph for the improvement of the periodic oral examinations. Materials and Methods : Clinical examinations and panoramic examinations were done for the 242 subjects of oral examinations. The results of panoramic radiograph interpretation were compared with the clinical findings. Two questionnaires were created. One was carried out before the panoramic examination and the other done afterwards, to find out the subjects' cognition and satisfaction for the clinical and panoramic examinations. Results : 1. Panoramic findings showed a higher detection rate of 31.9% for periodontal diseases, and 23.1% for dental caries than clinical findings. 2. The additional abnormalities detected through panoramic examinations were impacted tooth in 81 subjects (33.6%), maxillary sinus abnormalities in 28 subjects (11.6%), condylar abnormalities in 5 subjects (2.1%), congenital and acquired dental anormalies in 59 subjects (24.5%), and other miscellaneous abnormalities in 34 subjects (14.1%). 3. 164 subjects (67.8%) were satisfied with the current periodic oral examination, and 75 subjects (31.1%) hoped for better accuracy. 4. In the first and second questionnaire, 154 subjects (67.0%) and 163 subjects (70.6%) responded respectively that panoramic examination was necessary, and 193 subjects (83.2%) responded that it actually helped. Conclusion : The panoramic examination was revealed to improve the effectiveness of the periodic oral examination and to increase the satisfaction of the subjects of examination.

• Imaging Science in Dentistry
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• 제41권4호
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• pp.177-181
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• 2011

Pyknodysostosis is a rare autosomal recessive disorder characterized by the post natal onset of short limbs, short stature, and generalized hyperostosis along with acro-osteolysis with sclerosis of the terminal phalanges, a feature that is considered essentially pathognomonic. Other features include persistence of fontanelles, delayed closure of sutures, wormian bones, absence of frontal sinuses, and obtuse mandibular gonial angle with relative mandibular prognathism. We report a case of 17-year-old girl who presented with a chief complaint of retention of deciduous teeth. General physical examination demonstrated short stature, frontal and parietal bossing, depressed nasal bridge, beaked nose, hypoplastic midface, wrinkled skin over the finger tips, and nail abnormalities. Radiographs showed multiple impacted permanent and supernumerary teeth, hypoplastic paranasal sinuses with acro-osteolysis of terminal phalanges, and open fontanelles, and sutures along with wormian bones in the lambdoidal region.

• Journal of Oral Medicine and Pain
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• 제47권3호
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• pp.126-134
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• 2022

Purpose: This study aimed to propose an efficient treatment approach for infection with different candida species. Methods: Fifty-three patients who presented with a chief complaint of oral mucosal pain and exhibited positive candida culture findings were divided into two groups (Candida albicans and non-albicans). Pain, mucosal manifestations, salivary flow rates, durations of disease and treatment, and responses to treatment (nystatin and clonazepam) were investigated in both groups. Results: Patients in the C. albicans group exhibited more prominent clinical characteristics (erythematous lesions, tongue coatings, and hyperalgesia) than those in the non-albicans group. In total, 70% of patients in the non-albicans group showed no abnormalities in the oral mucosa. Patients in the C. albicans group showed increased resistance to nystatin treatment compared to those in the non-albicans group, especially with longer disease durations. The patients resistant to nystatin treatment showed positive responses to clonazepam. Conclusions: Patients with oral mucosal pain should be tested for the presence of Candida, even in the absence of mucosal abnormalities, especially those infected with non-albicans species. If no response to antifungal therapy is observed, treatment with clonazepam should be initiated, especially in patients infected with C. albicans.

• 구강회복응용과학지
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• 제25권3호
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• pp.211-224
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• 2009

본 연구의 목적은 연령과 성별 및 CT 촬영 목적에 따른 하악 과두의 골 이상에 대한 양상과 발병률 그리고 호발 부위를 살펴보고, 촬영 목적에 있어서 TMD 진단을 위한 그룹과 그 외의 그룹간의 발병 양상을 비교해 보기 위함이다. 원광대학교 산본 치과 병원으로부터 $i-CAT^{TM}$ Cone Beam Computed Tomography를 이용하여 촬영된 199명의 3차원 CT 영상을 수집하고, 촬영된 MPR 이미지는 하악 과두의 연속된 시상 이미지와 관상 이미지를 얻기 위하여 TMJ 모드로 전환되었다. 3명의 관찰자는 이를 토대로 골 이상의 양상 및 발병률, 호발 부위, 그리고 하악 과두의 전반적인 외형에 대해 각기 독립적으로 판독한 결과 type F가 type N보다도 훨씬 흔하게 관찰 되었다. 시상 이미지에서의 호발 부위는 type D-C를 제외하고는 하악 과두의 전상방 및 상방 부위였으며, type D-C에서는 후상방 부위가 가장 흔하였다. 관상 이미지에서는 type E를 제외하고는 외상방 및 상방 부위에 호발하였으나, type E에서는 내상방 및 상방 부위에서 빈발 하였다. 하악 과두의 골 이상 소견은 type D-C를 제외하고는 TMD 진단을 위한 그룹에서 보다 높게 나타났으며, 그 외의 그룹에서는 type N 및 type D-C가 보다 높게 나타났다. 본 연구에서는, 하악 과두의 골 이상 소견을 6가지 분류해 보았으며, 이러한 골 이상 소견은 각기 빈발하는 부위가 있는 것으로 나타났다. 그리고 TMD 진단 그룹이 type D-C를 제외하고는 골 이상 소견이 더 높게 나타나는 경향을 보였다.

• 대한구순구개열학회지
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• 제10권2호
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• pp.81-88
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• 2007

Cleft lip and palate (CLP) is one of the most prevalent congenital craniofacial anomalies. It has a significantly greater incidence of dental abnormalities in number, size, shape, and eruption of the teeth. Knout-out mouse model can identify several genes which play an important role in tooth agenesis. Since disruption of these genes has been confirmed to result in tooth agenesis in humans, CLP associated with hypodontia may be the best models for isolated tooth agenesis. According to the studies of dental abnormalities in CLP, the severity of dental defect is known to be influenced by the CLP phenotype. The cumulative data obtained from mouse and human genetic studies indicated that MSX1, PAX9 and AXIN2 are considered as candidate genes in non-syndromic hypodontia, while Shh, Pitx2, Irf6, p63 and EDA pathway genes are involved in syndromic one. We expect that genetic approach of CLP can offer the basis for tooth regeneration and be a new target in hypodontia therapy.

• 치과방사선
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• 제15권1호
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• pp.67-74
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• 1985

The incidence and associated dental abnormalities of taurodontism were studied radiographically (panoramic view) in 1895 patients of deciduous dentition and 2167 patients of permanent dentition who had visited to the Department of Oral Radiology, Kyung Hee University in Korea. The obtained results were as follows: 1. The prevalence of taurodontism was 3.59% in the deciduous dentition group, and 1.8% in the permanent dentition group. 2. There was no definite sex difference in the deciduous dentition, and the permanent dentition group. The type of mesotaurodontism was predominent in the deciduous dentition group, and hypotaurodontism in the permanent dentition group. 4. The vast majority of this abnormalities had occurred in the mandibular 1st deciduous molar in the deciduous dentition group, and mandibular 2nd permanent molar in the permanent dentition group. 5. Of the cases with taurodontism, the deciduous dentition group revealed 95.6% bilaterally, and 82% bilaterally in the permanent dentition group. 6. Multiple teeth occurance, not single tooth only, were involved in 98.5% of the cases in the deciduous dentition group, and 82% in the permanent dentition group.