• The Journal of Korea Assosiation for Disability and Oral Health
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• v.9 no.1
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• pp.51-55
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• 2013

Lennox-Gastaut syndrome (LGS) is a severe form of childhood epilepsy that is defined by generalized multiple type seizures, slowness of intellectual growth, and a specific EEG disturbance. Children affected might previously have infantile spasms or underlying brain disorder but etiology can be idiopathic. LGS seizures are often treatment resistant and the long term prognosis is poor. A 14-year-3-month old, 20.5 kg girl with LGS was scheduled for dental treatment under general anesthesia. The patient presented with multiple caries and heavy calculus. General anesthesia was induced and maintained with inhalation agent, Sevoflurane. And caries treatment, scaling, subgingival curretage were performed. LGS patients usually have poor oral hygiene due to mental retardation and have high risk of seizure during dental treatment. In dental treatment under general anesthesia for LGS, it must be considered of seizure control during peri-operative period. Also periodic recall check, instruction of oral hygiene must be performed.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.1
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• pp.1-5
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• 2016

Robinow syndrome is skeletal dysplasia with both autosomal dominant and recessive inheritance patterns. It is characterized by short-limbed dwarfism, abnormalities in the head and face, as well as vertebral segmentation. A 2-year-7-month old boy with Robinow syndrome had visited Seoul National University Dental Hospital, for the evaluation of tooth palatal eruption on maxilla. He had micrognathia, delayed tooth eruption, cleft lip with bifid uvula. He also had an erupted mesiodens on the palatal side of maxillary primary incisors, which was tuberculated and 8mm in major diameter. The patient was scheduled for mesiodens extraction under general anesthesia. He was a young child with delayed development, so general anesthesia was inevitable. General anesthesia was induced and maintained with inhalation agent, Sevoflurane. There were no postoperative complications related to anesthesia and dental treatment. Robinow syndrome patients have craniofacial dysmorphism and eruption disorders. Therefore, he requires regular check-ups as well as dental managements.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.60-64
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• 2019

Swyer-James syndrome (SJS), also known as Swyer-James-MacLeod syndrome and unilateral hyperlucent lung syndrome, is rare acquired pulmonary disorder develops secondary to infectious etiologies in early childhood. Viral respiratory infection such as adenoviruses or Mycoplasma pneumoniae in infancy or early childhood rarely cause Swyer-James syndrome. It is generally characterized on radiographs by a unilateral small lung with hyperlucency and air trapping on expiration. In many cases unaffected lung tissue functions normally, compensating for affected lung portion. Preoperative assessment is needed to determinate individual's pulmonary function. A 4-year-old boy with Swyer-James syndrome visited Seoul National University Dental Hospital Department of pediatric dentistry for caries treatment. Clinical and radiographic examinations revealed multiple carious lesions on deciduous teeth. Considering patient's underling disease, age, and level of cooperation, dental treatment under general anesthesia was scheduled. Dental treatment was done with composite resin and stainless-steel crown. Since ventilation of Swyer-James syndrome patients was diminished because of airway obstruction, close monitoring of ventilation is necessary during dental treatment. Considering pulmonary pathology, general anesthesia rather than sedation is recommended when special behavior management is required for dental treatment. Swyer-James syndrome patients can tolerate general anesthesia and surgery well, according to several reports.

• 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.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.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.1
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• pp.20-26
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• 2016

Sympathetic reaction to grotesque facial injuries with oral bleeding can trigger confusion and generate inefficiency among emergency room personnel. Regardless of the extent of the injury, or of the sympathy elicated, the victim must be evaluated and treated as a whole patient with multiple trauma. There must be no confusion as to order of importance in the steps of evaluation : 1. airway 2. hemorrhage 3. shock 4. associated injuries 5. local injury 6. triage of facial injuries. The most dangerous aspect of oral hemorrhage is the possibility of its obstructing the upper airway. Swallowing large amounts of blood will usually cause gastric irritation and lead to vomiting, thus further complicating the management of the patient. Once a clear airway is assured and hemorrhage have been controlled, consideration is given to possible associated injuries before undertaking treatment of the facial injuries themselves. Status of the cardiopulmonary, gastrointestinal and neuromuscular systems all have a bearing on decisions concerning facial injury treatment. In this light, treatment priority becomes exceeding important. On the other hand, the importance of facial injuries should never be minimized, especially in a mentally retarded patient with oral hemorrhage. This is a case report about oral hemorrhage control as treatment priority in a mentally retarded patient.

• 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.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.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.19 no.2
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• pp.497-507
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• 2018

Objectives: The purpose of this article was to evaluate the health related quality of life (HRQoL) of cancer survivors and to identify its predictors according to gender. Methods: The research was conducted with the data for 203 (cancer survivors?) taken from the 6th wave of the Korea National Health and Nutrition Examination Survey and the EQ-5D index score was used for the measurement of the health-related quality of life (HRQoL). The independent variables inluded socio-demographic data, health related factors (survival duration, disability, subjective health recognition), and psychosocial factors (stress, unmet medical needs). The data were analyzed by the t-test, ANOVA, and hierarchical multiple regression analysis. Results: the HRQoL of the female cancer survivors was significantly worse than that of the males in terms of their mobility, usual activities, pain/discomfort and anxiety/depression quality of life. The only statistically significant factor affecting the HRQoL of the male cancer survivors was their subjective health recognition. In the case of the female cancer survivors, the statistically significant factors were their age, subjective health recognition and unmet medical needs. Conclusions: the results of this study showed a different pattern of predictors according to the gender of the cancer survivors. Therefore, gender should be considered when assessing and addressing the individual care needs of cancer survivors, in order to obtain optimal treatment outcomes.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.9 no.1
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• pp.46-50
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• 2013

Developmental disorders are a group of psychiatric conditions originating in childhood that involve serious impairments in different areas. These disorders comprise language disorders, learning disorders, motor disorders and autism spectrum disorders. Midazolam is a short-acting drug in the benzodiazepine class developed by Hoffmann-La Roche in the 1970s. The drug is used for treatment of acute seizures, moderate to severe insomnia, and for inducing sedation and amnesia before medical procedures. It possesses profoundly potent anxiolytic, amnestic, hypnotic, anticonvulsant, skeletal muscle relaxant, and sedative properties. The anterograde amnesia property of midazolam is useful for premedication before surgery to inhibit unpleasant memories. This article presents a case report including caries treatment of a 8-year-old male patient with developmental disorders using oral midazolam premedication as an alternative method of behavior management prior to general anesthesia.