• 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.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.

• Journal of Music and Human Behavior
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• v.5 no.2
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• pp.67-92
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• 2008

The purpose of this study is to give an overview of the clinical process of songwriting with pediatric patients undergoing cancer treatment. Many literatures have proved that music therapy aids patients during their cancer treatment to relax, reduce anxiety and feelings of isolation, and promote self-esteem and quality of life and so on. However, among music therapy methods, I found songwriting proved to be very effective method. By using songwriting as therapy with pediatric patients undergoing cancer treatment, songwriting helped patients express difficulties of dealing emotionally with their illness and ongoing aggressive and invasive treatments. Also the process of songwriting established trust and relationship between therapist and patient which promoted normalization of hospital environment. In my case study, patient N has expressed her feelings and thoughts through writing songs about her current life which revolved around treatment of her brain tumor. Moreover, patient N found her creativity and developed ways of getting touch with her inner strength.

• Journal of the korean academy of Pediatric Dentistry
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• v.25 no.3
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• pp.506-512
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• 1998

The dentists often encouter a child who can not be treated with traditional behavior management technique. Mentally or physically handicapped child could receive proper dental care under general anesthesia. General anesthesia for dental care can be done in an outpatient surgery or inpatient surgery depending on the patient. In outpatient surgery, the patient is admitted and discharged the same day. Postoperative care and discharge procedure are abbreviated. Advantages of outpatient surgery are much less emotional disturbance of patients, reduced risk of nosocomial infection and less cost of treatment. A case of a physically handicapped patient treated under general anesthesia at Day Surgery Center is presented and the procedure and patient selection of outpatient surgery as well as its limitation is reviewed in this paper.

• Advances in pediatric surgery
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• v.18 no.1
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• pp.1-11
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• 2012

담관 낭종은 흔하지는 않지만, 외과적 절제가 필요한 주요 질환이다. 절제하지 않고 남겨두면 다른 질환으로의 이환이나 다양한 합병증에 의한 사망까지도 야기할 수 있기 때문에 적절한 수술적 치료가 필수적이다. 최근 수술에 따른 다양한 문제들이 계속 보고되고 있으며, 출생 전이나 건강 검진 시 발견되는 무증상의 담관 낭종의 수술 시기에 대해서도 아직 논란이 되고 있다. 저자들은 1995년부터 2009년까지 담관 낭종으로 수술 받은 환자 중 수술 받을 당시의 연령이 18세 이하인 32명 환자의 임상 양상과 수술 기록을 후향적으로 분석하여, 수술 성적에 영향을 주는 요인들을 알아보고자 하였다. 전체 32명 중 남자 10명, 여자 22명이었으며, 수술 당시의 평균 연령은 5.4세였다. 췌담관 합류이상은 9명(28.1%)이었으며, 평균 추적 관찰 기간은 34.6개월이었다. 술 전 증상을 호소한 환자는 30명(93.8%)이었으며, 복통(63.3%), 황달(40.0%), 구토(23.3%), 복부종물(16.7%), 발열(16.7%)등의 순서였다. 술 전 혈액 검사에서 AST/ALT의 상승이 18명(56.3%), 고빌리루빈혈증이 8명, 백혈구 증가증이 7명(21.9%)이었다. 술 후 합병증은 5명(15.6%)에서 나타났는데, 2명은 담관염이었고, 나머지 3명은 문합부 협착, 술 후 출혈, 장 폐쇄가 각각 1명 있었다. 술 후 평균 재원 기간은 12.2일 이었으며, 추적 관찰 기간 중 3명(9.0%)이 재입원 하였다. 수술 성적에 영향을 미치는 요인으로, 수술 당시의 나이가 많을수록 평균 수술 시간과 재원 기간이 증가하였다(p=0.004, p=0.028). 증상이 있었던 환자군에서 무증상 환자군보다 재원 기간이 더 길었다(p=0.001). 또한 췌담관 합류이상이 있었던 환자군에서 재입원율이 유의하게 높았다(p=0.005). 소아의 담관 낭종은 진단이 되면 증상 발현 전이라도 조기에 수술하는 것이 수술 시간 및 재원 기간을 단축시킬 수 있으며, 췌담관 합류이상이 동반된 경우에는 술 후에 재입원할 가능성이 높으므로 주의깊게 추적 관찰하는 것이 바람직할 것이다.

• Pediatric Infection and Vaccine
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• v.19 no.2
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• pp.61-70
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• 2012

Purpose: This study was performed to compare the clinical characteristics of 2009 pandemic influenza A(H1N1) [A(H1N1) pdm09] and seasonal influenza A infection in the pediatric cancer patients. Methods: A retrospective review was performed in the pediatric cancer patients who had confirmed A(H1N1)pdm09 infection at Samsung Medical Center from August 2009 to February 2010. For the comparison, the medical records of pediatric cancer patients with seasonal influenza A from January 2000 to May 2009 were reviewed retrospectively. Results: Eighty-two A(H1N1)pdm09 infections were confirmed in the pediatric cancer patients. Ten patients (12.2%) developed complicated clinical course by lower respiratory infections or extrapulmonary infections; 4 pneumonia, 1 bronchitis, 1 pericarditis with pneumonia, 1 encephalitis with pneumonia, 2 meningitis and 1 pericarditis. Three patients received mechanical ventilator and ICU care. Three pediatric cancer patients (3.7%) died. The risk factors related to complicated A(H1N1)pdm09 infections were date of infection (44-45th week 2009) and nosocomial infection. When comparing with previous seasonal influenza A infections, more prompt and aggressive antiviral therapy was given in A(H1N1)pdm09 infections. Conclusion: The A(H1N1)pdm09 infections caused a various clinical manifestations including fatal cases in pediatric cancer patient during pandemic season. There was no significant difference in clinical course between influenza A(H1N1)pdm09 and seasonal influenza A infections except the antiviral treatment strategy.

• Journal of the Korean Child Neurology Society
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• v.26 no.4
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• pp.246-250
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• 2018

Purpose: Meningitis is an acute childhood infection caused by viral or bacterial infection. The purpose of the present study is to analyze the differences between enteroviral meningitis and non-enteroviral aseptic meningitis. Methods: From January 2013 to December 2016, we retrospectively reviewed the medical records of a total of 303 aseptic meningitis patients who visited Wonju Severance Christian Hospital. We examined demographics of all patients and analyzed serologic and cerebrospinal fluid tests, clinical symptoms, and outcomes. Results: Of a total of 303 patients, 197(65.0%) were male, and the most cases occurred from June to November (91.8%). The most common pathogen of meningitis was found to be enterovirus (65.0%). According to the etiology, the enteroviral meningitis group had significantly more headache and enteric symptoms (P=0.0003 and P=0.0013, respectively). Furthermore, the non-enteroviral meningitis group showed pleocytosis in the cerebrospinal fluid and a significantly higher rate of seizure at 1 to 4 years (P=0.0360 and P=0.0002, respectively). Conclusion: In this study, enteroviral meningitis was the most common and the prognosis was good. When compared between two groups, neurological symptoms were frequent in patients with non-enteroviral meningitis in groups 1 to 4 years.

• Journal of the Korean Child Neurology Society
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• v.26 no.4
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• pp.197-204
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• 2018

Purpose: West syndrome is a severe form of age-specific epilepsy that typically affects infants younger than 2 years of age with mitochondrial disease. We aimed to examine age-specific characteristics of the syndrome in these patients. Methods: We retrospectively analyzed 54 patients with West syndrome diagnosed with mitochondrial disease between March 2006 and March 2016. We compared treatment strategies and diagnostic and clinical variables between patients with early-onset (<6 months of age) and late-onset (${\geq}6$ months of age) seizures. Results: Seizure was the first symptom in 30 (90.9%) and 13 (65%) patients of the early-onset and late-onset groups, respectively (P=0.046). Delayed development was observed in 3 (9.1%) and 7 (35%) patients of the early-onset and late-onset groups, respectively (P=0.023). Lactate levels were normal in 17 patients (55%) of the early-onset group and 5 (25%) of the late-onset group (P=0.036), while initial brain magnetic resonance imaging (MRI) findings were normal in 23 (67.6%) and 8 (40%) patients of the early-onset and late-onset groups, respectively. Final MRI findings were abnormal in 32 patients (94.1%) of the early-onset group and 18 (90%) of the late-onset group (P=0.036). Although ketogenic diets reduced seizure frequency in both groups, the difference was not significant. Conclusion: There is no significant difference in epilepsy-related variables when patients are divided based on a cut-off age of 6 months. However, differences in the first symptom at onset and MRI findings were observed. Although lactate levels were not of significant diagnostic value in the early-onset group, they may be in the late-onset group.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.9 no.1
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• pp.54-66
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• 1998

Objectives：This study was to investigate the impact of parental psychiatric disorder on offspring's depression, anxiety, self concept, perception of familial relationship compared with offspring of normal control. In offsprings of parents with psychiatric disorder, this study explored whether their psychopathology, self concept, and perception of familial relationship were influenced by parent’s sex, onset time of parent’s psychiatric disorder and parent’s psychiatric diagnosis. Methods：52 offsprings aged 10-18 years of 39 psychiatric outpatient were surveyed from June, 1997 to April, 1998 and completed several questionnaaire, including Korean from of the Family Environment Scale, Korean form of the State-Trait Anxiety Inventory for Children, Korean form of Kovac’s Children’s Depression Inventory, and Korean form of Piers-Harris Children’s Self-Concept Scale. Their score was compared with offsprings’ of normals. In offsprings of parents with psychiatric disorder, they were compared according to parent’s sex, onset time of parent's psychiatric disorder and parent’s psychiatric diagnosis. Results：The results were as follows：1) Offsprings of parents with psychiatric disorder reported higher level of state anxiety and lower level of the FES expressive subscale than offsprings of normals(p<0.05). But they reported higher level of PHCSCS intellectual & school status subscale and popularity subscale than normals(p<0.05). 2) There were no differences in anxiety, depression, self concept, and perception of familial relationship between patient’s sex. 3) Offsprings less than 3 years old when parent’s psychiatric disorder had developed showed higher level of trait anxiety and lower level of FES control subscale than offsprings more than 3 years old (p<0.05). 4) There were no diferences in anxiety, depression, self concept, and perception of familial relationship between patient’ diagnostic groups(schizophrenia spectrum disorder-mood disoderneurosis). Conclusion：The finding indicated that self reported scale of anxiety and depression showed no significant difference between offsprings of psychiatric patients and offsprings of normals. In offsprings of parents with psychiatric disorder, parent’s sex and psychiatiric diagnosis had no influence on offspring’s psychopathology. But the offspring’s age(before 3 years old) when the parent’s psychiatriric disorder developed had influence on higher level of offspring’s trait anxiety. For further high risk group study, direct interview and evaluation of parent-child agreement or teacher-child agreement will be needed in longitudinal study.

• Pediatric Infection and Vaccine
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• v.30 no.3
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• pp.180-187
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• 2023

In Korea, >90% of children and adolescents aged <19 years have been infected with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) since 2020. Among confirmed cases of pediatric coronavirus disease 2019 (COVID-19), 40-60% of patients developed neurologic symptoms such as seizures, headache, and encephalitis. Herein, we report the case of a 3-year-old female patient with SARS-CoV-2 infection who presented with seizures and altered consciousness and was diagnosed with COVID-19 encephalitis. The patient recovered after treatment with intravenous immunoglobulin, high-dose steroids, anti-seizure drugs, and an anti-viral agent. She was discharged after regaining the ability to speak words and walk alone on hospital day 39. Complete recovery was observed at the 1-year follow-up. The findings in this case suggest that early detection and active intervention is associated with better outcomes in patients with COVID-19 encephalitis.