• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.2
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• pp.77-81
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• 2016

The Dandy-Walker syndrome is a relatively rare congenital malformation occurring about one in 25,000-35,000 pregnancies. It is characterized by hydrocephalus, cystic dilatation of the fourth ventricle, complete or partial absence of the cerebellar vermis, and other various extra-CNS malformations. We report a dental caries treatment of a 4-year old girl with Dandy-Walker syndrome and partial trisomy 9q. The patient visited Seoul National University Dental Hospital due to multiple caries. The cause of multiple caries is mainly presumed as patient's eating habit caused by her general condition. She was still using milk bottle because she was having difficulties swallowing solid food due to breathing problem while eating. The treatment was performed under general anesthesia considering patient's condition; delayed development, very poor cooperation and respiratory problem. The dental procedure was successfully conducted and there were no postoperative complications. In consideration of her eating habit and oral hygiene problem, regular check appointment was recommended.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.2
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• pp.87-91
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• 2016

Pierre Robin syndrome (PRS) is characterized by the triad of congenital mandibular hypoplasia, glossoptosis and cleft palate. Infant PRS patients are frequently suffering from upper airway obstruction, gastroesophageal reflux and growth retardation caused by above mentioned problems. We report a dental caries treatment of 3-year old girl with Pierre Robin syndrome with multiple caries. The cause of multiple caries was mainly presumed as patient's eating habit caused by her general condition. She had some feeding problems and had history of gastric tube. She was still using milk bottle and took more than an hour to finish a meal. The treatment was performed under general anesthesia considering patient's condition; mild autism, poor cooperation and respiratory problem due to micrognathia. Severely affected upper incisors were treated with pulp treatment and restored with zirconia crown for esthetic purpose. Lower incisors were treated with pulp treatment and restored with composite resin. Upper right first primary molar was restored with stainless steel crown and other primary molars were treated with composite resin. There were no postoperative complications. According to her parents, the patient's compliance to oral hygiene management was greatly improved after the treatment since she was very pleased with the esthetic result and highly motivated by her looks. The treatment without sedation or general anesthesia would be possible once the airway is improved as the mandible grows.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.50-54
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• 2019

Muscular dystrophy (MD) is a heterogeneous group of inherited neuromuscular disorders, characterized by progressive muscle weakness. Severity of the disease ranges from mild to severe, and the disease is mostly caused by mutations in a number of genes. These genetic mutations cause lack of proteins which are essential for muscle cell stability. Muscle fibers are gradually replaced by fat and fibrous tissue. The muscles of the head and neck are affected in several types of MD that manifest as altered craniofacial morphology and dental malocclusion. A 3-year-10-month old, 15.0 kg boy with MD presented to Seoul National University Dental Hospital, Seoul, South Korea because of extensive carious teeth. A number of dental caries in primary dentition were identified during clinical oral examination. Due to dental anxiety and underlying systemic disease, general anesthesia was considered. General anesthesia was induced and maintained with intravenous anesthetics, propofol and remifentanil. Caries treatments - resin restoration, pulpectomy, zirconia crown restoration, stainless steel crown restoration - were performed. Under general anesthesia, successful dental procedure was done. Total intravenous anesthesia (TIVA) was performed instead of inhalation anesthesia in order to avoid risk of complications such as malignant hyperthermia and life-threatening rhabdomyolysis. With decreasing muscle function, plaque control becomes more difficult and leads to gingivitis. Especially, the open-mouth posture worsens gingivitis and can leads to malocclusions and problems in swallowing. Regular and periodic dental care is essential for maintaining oral health for patients with MD.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.1
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• pp.6-10
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• 2016

Esophageal atresia is the abnormal development of the esophagus that connects the mouth to the stomach. This birth defect results in the incomplete connection of the esophagus to the stomach causing an inability to swallow properly and breathing difficulties. Surgery is the only treatment for esophageal atresia. Patients undergone an esophageal reconstruction usually suffer from gastroesophageal reflex. A 5-year old boy with congenital esophageal atresia and Sciwitar syndrome visited Seoul National University Dental Hospital because generalized excessive loss of tooth structure. The patient had history of multiple operations for reconstruct esophagus. He had little food intake experience thought oral and had difficulty to swallowing. The patient was generalized severe erosive and decayed state. For patient, the dental procedure under general anesthesia was scheduled due to the multiple caries with erosion and poor cooperation. Under general anesthesia, pulpectomy and restoration as well as extraction was performed. Gastroesophageal reflex is potentially serious condition, with various extraesophageal adverse effects such as dental erosion. To prevent progression of dental caries, dietary counselling and oral hygiene instruction should be reinforced. Also, restorations to erosive teeth will maintain esthetics and function and preserve pulp vitality.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.65-69
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• 2019

Wolf-Hirschhorn syndrome(WHS) is a congenital disorder caused by deletions of the short arm of chromosome 4. The most common characteristics are mental and growth retardation, dietary disorder and craniofacial features with a characteristic 'Greek warrior helmet' appearance. The dental characteristic of WHS includes delayed development, tooth agenesis, clefts, microdontia, taurodontism, and severely worn dentition. The purpose of this case report is to describe the dental treatment of a patient with WHS. 3-year-old boy with WHS visited the Seoul National University Dental Hospital for dental treatment. He had difficulty with nasotracheal intubation because of craniofacial anomalies and also had poor oral hygiene due to a limitation of mouth opening and dietary disorder. Due to his airway problem, behavior management and severity of dental conditions, dental treatment was performed under general anesthesia. This case suggests general anesthesia can be chosen with WolfHirschhorn syndrome patients to safely care for their dental problems.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.13 no.2
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• pp.86-90
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• 2017

Hydrocephalus is characterized by accumulation of cerebrospinal fluid in the ventricles of the brain causing progressive ventricular dilatation. Accumulation of cerebrospinal fluid typically causes increased pressure inside the skull, this may result in headaches, vomiting, nausea, seizures, sleepiness and mental impairment. Patients with hydrocephalus may have dental manifestations such as changes in the occlusion and greater accumulation of plaque. The prevalence of dental caries is high due to the difficulty of oral care. The purpose of this case report is to describe the dental care for an uncooperative child with hydrocephalus under general anesthesia.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.2
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• pp.96-100
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• 2016

Metachromatic leukodystrophy (MLD) is a progressive and degenerative neurological disease caused by a deficiency of the catabolic enzyme arylsulfatase A. Deficiency of arylsulfatase A results in accumulation of sulfatide in the white matter of the peripheral and central nervous system and it occurs demyelination as a result. The patient gradually goes through mental and motor failure. General symptoms of MLD include gait disturbance, mental deterioration, muscle rigidity and impaired swallowing. Inheritance of the disease is autosomal recessive. We report a dental caries treatment of a 3-year old boy with MLD. The patient underwent hematopoietic stem cell transplantation (HSCT) to slow the progression of the disease. He was suffered from difficulties of mastication and swallowing from the degenerative neurological symptom. He was ingesting food by both oral feeding and tubal feeding after he took percutaneous endoscopic gastrostomy (PEG). The cause of multiple caries was mainly presumed as patient's prolonged time of meal. The treatment was performed under general anesthesia considering patient's incompliance. Severely affected lower primary molars were treated with pulp treatment and restored with stainless steel crown. Others were restored with composite resin. There were no postoperative complications. MLD is life threatening progressive disease and also has an impact on unfavorable condition for oral health. Routine home oral care and periodic professional dental care should be emphasized to the caregiver of patient considering the susceptibility of dental caries. Not only the medical care, but periodic dental office visit would benefit the quality of life of the patient.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.74-78
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• 2019

Atypical teratoid rhabdoidal tumor(AT/RT) is a rare and fast-growing tumor usually diagnosed in childhood. Routine magnetic resonance imaging(MRI) assessment of AT/RT patients is done to detect metastatic tumors and recurrent lesions. The purpose of this case report is to present a case about caries treatment of a 3-year-8-month old female patient with AT/RT under general anesthesia. A 3-year-8-month old patient with AT/RT visited Seoul National University Dental Hospital for caries treatment. At the first dental visit, she was receiving chemotherapy. Multiple caries were observed from clinical and radiographic examination. The dental procedure was successfully performed under general anesthesia. Patients with brain tumor may take routine MRI to detect metastatic tumors and recurrent lesions. Dentists should take into consideration of possible dislodgement of restoration by ferromagnetic properties and artifact formation on MRI image. Ready-made zirconia crowns can be a good restoration option for such patients.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.2
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• pp.97-101
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• 2018

Lesch-Nyhan syndrome is a rare X-linked recessive genetic disorder. During purine metabolism, the hypoxanthine guanine phosphribosyl transferase (HGPRT) enzyme is deficient causing phosphoribosyl transferase to accumulate and resulting in excessive uric acid. Clinical symptoms include hypercalcemia, choreoathetosis, spasticity, mental retardation, and self-injury to lips, tongue or fingers. This results not only in pain caused by the self-injury but also secondary infection of the wound site and the esthetic damage of the soft tissue defect. Dental treatments include conservative methods using intraoral appliances such as soft mouthguards, fixed lip bumpers, and occlusal guards, and invasive methods such as extraction of all teeth or forming an artificial anterior open bite. We report two cases of Lesch-Nyhan syndrome patients with self-mutilation behavior; one was treated with a preservative method using a soft mouthguard, and the other was treated with extraction of all teeth.

• Journal of dental hygiene science
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• v.14 no.1
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• pp.67-73
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• 2014

This study analyzed 548 pieces of these, which were reported in the Journal of Dental Hygiene Science of having been published from 2001 to Vol. 12, No. 6 in 2012. In conclusion, as for analysis of research design, first, it was the largest in cross sectional research. Second, the research subjects of survey theses were higher in order of dental hygienist and dental hygiene student. Third, number of thesis authors was the largest in order of two persons and three persons. Fourth, statistical method was in order of descriptive statistics, t-test, and ANOVA. Research theme was in order of dental health behavioral science and clinical dental hygiene. Fifth, as for research-expense benefit, only 17.7% was supported research funds. As a result of this study, there should be a research on thesis of diverse designs in the future. There is a need of being performed actively a research on alienated classer or special subjects as well as a research on activity related to dental hygiene.