• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.1
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• pp.36-40
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• 2018

CATCH22 syndrome or DiGeorge syndrome is a medical acronym of cardiac defects, abnormal facial appearances, thymic hypoplasia, cleft palate, and hypocalcemia. Patients with CATCH22 syndrome are susceptible to infection due to an absent or hypoplastic thymus and often have difficulties in maintaining good oral hygiene, which may require dental treatment. We present a case of dental treatment for the uncooperative child with CATCH22 syndrome under general anesthesia. A 4-year-old, 14.8 kg boy with CATCH22 syndrome visited Seoul National University Dental Hospital for dental check up. The patient had multiple caries requiring dental treatment. He experienced the corrective heart surgery due to Tetralogy of Fallot a few years ago. General anesthesia was planned because his heart rate and vital sign had shown unstable during the previous conscious sedation procedure. Dental restorative treatments were successfully performed and no complications were observed during and after the procedure. Safe and effective dental management of the patients with CATCH22 syndrome could be performed with the help of general anesthesia and careful monitoring.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.1
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• pp.22-25
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• 2018

Lennox-Gastaut syndrome (LGS) is one of the most severe epileptic encephalopathies of childhood onset. It is characterized by multiple types of generalized seizures, slowness of intellectual growth, and specific electroencephalogram pattern. It is one of the most difficult epilepsy syndromes to treat and is frequently resistant to treatment. In this case report, we report a case of dental treatment under general anesthesia in a child with LGS. A 4-year-6-month old boy with LGS visited Seoul National University Dental Hospital for dental treatment. Dental treatment was planned under general anesthesia because of his medical condition. Treatment was successfully done without any postoperative complications. If a LGS patient requires dental treatment, a treatment plan should be established to take into account the degree of seizure, the medication being used, and the cooperation of the patient. If the degree of seizure is severe and the patient's cooperation is poor, dental treatment under general anesthesia would be advantageous.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.79-83
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• 2019

Treacher Collins syndrome(TCS) is an autosomal craniofacial development disorder which results from mutations in the gene TCOF1. Major features include midface hypoplasia, micrognathia, microtia, conductive hearing loss. Oral manifestations are characterized by cleft palate, shortened soft palate, malocclusion, anterior open bite and enamel hypoplasia. The purpose of this presentation is to describe the interesting aspects of dental treatment of a patient with TCS. A 6-year-old boy with TCS visited Seoul National University Dental Hospital for dental caries. Multiple caries was observed from clinical and radiographic examination. Because of multiple caries and behavior management ploblem, dental treatment under general anesthesia was planned. Treatment of posterior teeth was performed and some primary teeth were extracted. General anesthesia was induced and maintained with sevoflurane, nitrous oxide and oxygen. Under general anesthesia, successful dental procedure was done. Considering behavior management problem and medical condition of patient with TCS, general anesthesia can be useful.

• Journal of the korean academy of Pediatric Dentistry
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• v.40 no.1
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• pp.66-71
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• 2013

Muscular dystrophy is a genetically heterogeneous group of disorders characterized by progressive muscle weakness of variable distribution and severity. Fukuyama type congenital muscular dystrophy (FCMD) is an unusual form of muscular dystrophy with autosomal recessive inheritance and is clinically characterized by an early age of onset, severe central nervous system involvement, facial muscle weakness, and multiple joint contractures. Muscular dystrophy is susceptible to perioperative respiratory, cardiac and other complications. Patients with FCMD have upper airway muscle weakness, therefore general anesthesia is preferred to sedation regarding maintaining the airway when treating these patients. The development of malignant hyperthermia in general anesthesia for patients with muscular dystrophy is a concern. Total intravenous anesthesia should be used instead of inhaled anesthetics because of the risk of malignant hyperthermia. A 3-year-9-month old, 13kg girl with Fukuyama type congenital muscular dystrophy was scheduled for dental treatment under general anesthesia. She had multiple caries and 14 primary teeth needed caries treatment. Prior to general anesthesia, oral premedication with 9 mg midazolam was given. General anesthesia was induced and maintained with target controlled infusion of propofol $3{\sim}3.5{\mu}g/mL$. The patient with progressive muscular dystrophy was successfully treated under total intravenous anesthesia with a target controlled infusion of propofol. There were no complications related to anesthesia and dental treatment during or after the operation. This case suggests that target controlled infusion of propofol is a safe and appropriate anesthetic technique in FCMD patients for dental treatment.

• Journal of The Korean Dental Society of Anesthesiology
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• v.12 no.4
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• pp.229-233
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• 2012

Hemophilia, the most common of the inherited bleeding disorder, is the result of a deficiency of clotting factor. Since bleeding after dental treatment may cause severe or even fatal complications, people with hemophilia must be given special dental care. We report on the diagnosis and treatment of a 9-year-old boy having severe hemophilia visited our department with the chief complaints of pus discharge on the left lower molar region. In the clinical and radiographic examination, periapical abscess and dental caries were diagnosed. Considering complexity of the treatment and complication in the coagulation, it was decided to carry on the treatment under general anesthesia. Clotting factor IX concentrates were intended to provide 50-70% plasma level. Pulpectomy, resin restoration and Stainless steel crown were given under general anesthesia. Several teeth were extracted and the sockets were packed with Surgicel$^{(R)}$ (Oxidized Regenerated Cellulose, Johnson and Johnson Co. Neuchatel, Switzerland) under general anesthesia. Transpalatal arch and lingual arch were given for maintaining the extracted space before discharged. For people with severe hemophilia, factor replacement is necessary before scaling, surgery or regional block injections. Therefore, if several extractions are needed, dental care under general anesthesia would be effective and efficient management.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.9 no.2
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• pp.107-110
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• 2013

Adrenoleukodystrophy (ALD) is a rare, inherited disorder that leads to progressive brain damage and failure of the adrenal glands. It is passed down from parents to their children as an X-linked genetic trait. Therefore, it affects primarily males especially under the age of 10. People with ALD have excessive accumulation of very long chain fatty acids (VLCFAs) in their brain because they do not have the enzyme which break down these fatty acids. Special method for treatment of ALD is not available. Eating a diet low in VLCFAs and taking special oils, called Lorenzo's oil, can lower the blood levels of VLCFAs. But this oil cannot stop the destruction of nerve cell. This article presents a case report: Dental treatment of a 11-year-old male patient with ALD under general anesthesia. Careful management must be required during treatment procedure because of difficulty in cooperation, seizure disorders, gastroesophageal reflux, pulmonary aspiration and airway complications.

• Journal of The Korean Dental Society of Anesthesiology
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• v.13 no.4
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• pp.215-220
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• 2013

The co-occurrence of congenital central hypoventilation syndrome (CCHS) and Hirschsprung's disease (HD) is termed Haddad syndrome, which is an extremely rare discorder. It was reported first by Haddad in 1978 and there are approximately 60 cases reported in the worldwide literature. Recent studies described that congenital central hypoventilation syndrome had deep relation to the mutation of the PHOX2B gene in its diagnosis and phenotype. This article presents a case report: Dental treatment of a 3-year-old male patient with Haddad syndrome under outpatient general anesthesia. The special considerations of dental care, especially caries theatment of the patient with Haddad syndrome are discussed.

• Journal of The Korean Dental Society of Anesthesiology
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• v.13 no.4
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• pp.203-207
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• 2013

Aplastic anemia (AA) is a serious hematologic disease characterized by hypocellular bone marrow and deficient production of erythrocytes, granulocytes and platelets. Serious complications such as uncontrolled bleeding and bacteremia can occur. A case of severe AA are presented with dental considerations. A 4-year-old boy had been referred from Seoul National University Hospital for dental examination before the hematopoietic stem cell transplantation (HSCT). Treatments were planned under general anesthesia, due to the poor compliance. Following medical consult, dental treatments were performed after platelet transfusion and antibiotic prophylaxis. Postoperatively, neither significant bleeding nor complictation was observed. On the time of the treatment planning. the anesthesiologist and dentist should perform a complete hematological assessment. It is imperative not only platelet counts but also other leukocyte counts are under safe boundaries. It is mandatory to follow strict aseptic precautios for all anesthetic and surgical maneuvers. In severe thrombocytopenic patients, platelet transfusion should be considered. Also, it is recommended to establish a good oral hygiene.

• Journal of the korean academy of Pediatric Dentistry
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• v.42 no.2
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• pp.158-163
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• 2015

General anesthesia (GA) for dental care in handicapped patients is necessary to facilitate the provision of safe, efficient, and effective quality treatment. The aims of this study were to determine the anesthetic characteristics of handicapped patients in need of dental treatment in these day care units, and to establish for plan to provide better services. 325 patients who had outpatient general anesthesia from January 2005 to March 2014 were assessed for this study. Patients' distribution and treatment patterns were examined. The proportion of male patients (202, 62.2%) was higher than female patients (123, 37.8%) and the largest group of patients were 5 to 10 years old (85, 26.2%). The reasons for general anesthesia included mental and physical disabilities (207, 63.7%), behavior management (84, 25.8%), parent needs (14, 4.3%), and so on. Restorative treatment was the most common procedure with the average of 4.2 teeth treated per one patient and 43 (13.2%) patients underwent general anesthesia for dental treatment more than once. To expand and improve access to the dental care of the disabled, improvement of the health care system, enhancement of their training for dental care by professionals, and enlarging caregivers'understanding of the importance of oral care in the early stages are required.

• Journal of the korean academy of Pediatric Dentistry
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• v.47 no.2
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• pp.99-108
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• 2020

This study was performed to evaluate the effectiveness of the 2-step needle insertion technique to reduce pain during local anesthesia in children. Local anesthesia was performed bilaterally using conventional technique and a 2-step technique, and to compare the degree of pain using the 2-step with that using the conventional technique during local anesthesia using a pain scale. The pain scores were significantly different between the conventional technique and the 2-step insertion technique at 5.82 ± 2.14 and 2.57 ± 2.09, respectively (p < 0.001). The subjective degree of pain based on gender, age, and Frankl's behavior rating scale was significantly lower in the 2-step insertion technique (p < 0.001). Subjective pain levels in the area of maxilla and mandible, anterior and posterior parts were lower in the group treated using the 2-step insertion technique (p < 0.001). The 2-step insertion technique is a simple and effective way to reduce pain during local anesthesia not only in children with a positive attitude to dental treatment, but also in children with negative attitudes, and is thought to be more effective if combined with application of topical anesthesia or distraction.