• Clinical and Experimental Pediatrics
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• 제61권2호
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• pp.37-42
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• 2018

Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is mainly plasmapheresis or plasma infusion; however, many children with aHUS will progress to chronic kidney disease despite plasma therapy. Eculizumab is a newly developed biologic that blocks the terminal complement pathway and has been successfully used in the treatment of aHUS. Currently, several guidelines for aHUS, including the Korean guideline, recommend eculizumab as the first-line therapy in children with aHUS. Moreover, life-long eculizumab therapy is generally recommended. Further studies on discontinuation of eculizumab are needed.

• 고려인삼학회:학술대회논문집
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• 고려인삼학회 1984년도 학술대회지
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• pp.89-95
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• 1984

Hydrocortisone과 ginseng 그리고 hydrocortisone과 ginseng의 혼합물이 phytohaemagglutinin (PHA-P)으로 유도한 모세혈관 림파구 변형에 미치는 영향을 검토하기 위하여 정상인 4명을 대상으로 연구하였다. 인삼의 농도를 0.16${\mu}g$/ml에서 1.60${\mu}g$/ml까지 증가시킴에 따라 PHA-P의 림파구 변형에 상관성이 있는 억제현상을 보였으며, hydrocortisone 500${\mu}g$/ml과 ginseng 0.8${\mu}/g$/ml을 동시 투여하였을 경우 각각 단일 물질 투여시 보다 상당한 PHA-P 변형의 억제효과를 나타냈다. 이는 인삼이 in vitro상에서 steroid와 유사한 효과를 갖고 있음을 시사해 주며, 면역에 hydrocortisone과 더불어 상당한 효과를 가지고 있다는 것을 보여주고 있다. in vivo상에서 6명의 정상인을 대상으로 PHA-P 변형에 대하여 인삼의 효과를 연구한 결과 4주에 이르러 상당한 억제효과가 관찰되었다.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• 제23권3호
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• pp.103-108
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• 2012

Objectives : The aim of this study is to investigate the effectiveness of treatment with osmotic-release oral system methylphenidate (OROS-MPH) on quality of life (QOL) in children with attention-deficit hyperactivity disorder (ADHD). Another aim is to assess the relationship between change in QOL and other factors including children's symptoms and academic performance or parents' depression and parenting stress. Methods : A total of 111 medication-naive children with ADHD in a multicenter, open-label, 12-week trial of OROS-MPH completed an evaluation using diverse rating scales at two time points; at baseline and after 12 weeks of treatment. Scales for investigation of children included the Parent Report Form-Children's Health and Illness Profile-Children's Edition (PRF-CHIP-CE) on QOL, the ADHD Rating Scale-IV on symptoms, and the Academic Performance Rating Scale (APRS). The Beck Depression Inventory and Parenting Stress Index were used for assessment of their parents. Results : Total scores for mean PRF-CHIP-CE increased from $207.9{\pm}26.7$ at baseline to $226.3{\pm}25.9$ after 12 weeks of treatment (p<.001). The change of APRS showed the strongest correlation with the increment of PRF-CHIP-CE scores (Pearson coefficient=0.561, p<.001), even after controlling for other factors (partial correlation coefficient=0.420, p<.001). Conclusion : Treatment with MPH-OROS results in improvement of QOL in children with ADHD in Korea. The advance in academic performance plays a key role in this change of QOL.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• 제28권4호
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• pp.213-219
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• 2017

Objectives: Early intensive interventions are very important for children with autism spectrum disorder. We examined the actual conditions of hospital-based early intensive interventions for autism spectrum disorder in Seoul, in order to help develop and implement an evidence-based early intensive intervention model for use in Korea. Methods: Nine hospital-based institutes running an early intensive intervention program for children with autism spectrum disorder responded to a questionnaire in September 2014. They provided a brief introduction to their program, explained its theoretical bases, and reported the number of children, their age, intervention time, duration and so on. Results: In the majority of the institutions, the intervention was provided for over 20 hours every week, and the theoretical bases included various applied behavioral analysis (ABA) methods and other therapies (language and occupational therapy). The therapist-child ratio ranged from 1:1 to 5:3. Various types of therapists were involved, including behavioral analysts, special education teachers and (or) language pathologists. There was only one clinic where the behavioral analyst was the main therapist. Usually, the intervention was terminated just before the child entered elementary school. The main merit of the hospital-based intervention in our survey was the effectiveness of the multi-disciplinary intervention plan and its other merits were the accuracy of the diagnosis, its ability to be combined with medicine, and so on. Conclusion: The current hospital-based early intensive intervention programs provide interventions for over 20 hours per week and employ multidisciplinary approaches. However, there are very few institutes for children with autism and very few intervention specialists and specialist education courses in the country. We need more educational programs for intervention therapists and have to try to develop policies which encourage the implementation of an evidence-based early intensive intervention program nationwide.

• Journal of Acupuncture Research
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• 제19권4호
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• pp.89-100
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• 2002

Objective : We want to have a correct understanding and the distance about the Oriental Medical therapy on the CP(cerebral palsy) children. Methods : We made up a question of parents of CP children and analyzed it. Conclusion : 19 patients were taken physical treatment and 12 patients were taken occupational treatment in company with physical treatment(In our OPD CP patient) Parents complained about poor surroundings of our hospital for children and deficiency of physical treatment. The most satisfactory thing is acupuncture treatment, then the next thing is aroma therapy. On the satisfaction degree about the Oriental Medical therapy, very good is 27.3%, good 45.5%, ordinary 27.3% and unsatisfaction is none. The place Oriental medical therapy before our hospital was taken is local Oriental medical clinic, 9 cases out of 22.

• Parasites, Hosts and Diseases
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• 제52권5호
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• pp.545-549
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• 2014

Sparganosis is an infection with a parasitic tapeworm larva that occurs by eating infected foods or drinking contaminated water. The larvae can migrate to a tissue or muscle in the chest, abdominal wall, extremities, eyes, brain, urinary tract, pleura, pericardium, spinal canal, or scrotum. Herein, we report a 5-month old infant with scrotal sparganosis who was initially suspected to have a scrotal inflammatory mass with a history of applying raw frog meat into the umbilicus. Preoperative ultrasound examinations and computed tomography (CT) scanning misdiagnosed the mass as a scrotal teratoma. The scrotal mass was surgically removed, and the histopathology proved it to be scrotal sparganosis. This case displays the youngest patient ever reported with scrotal sparganosis, and the first description of CT characteristics of scrotal sparganosis. A detailed medical history is necessary for patients with scrotal masses suspected of sparganosis. In addition, ultrasound and CT examinations are helpful to rule out other causes of a scrotal mass.

• Pediatric Infection and Vaccine
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• 제30권1호
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• pp.20-32
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• 2023

목적: 국내 소아청소년에서 지역사회폐렴의 원인병원체 분포에 대해 잘 설계된 임상 연구가 부족한 실정이다. 코로나 19 판데믹 기간 동안 국내 소아청소년에서 진단된 입원을 요하는 지역사회폐렴의 역학과 위험인자를 파악하고 지역사회폐렴의 치료 방침 결정의 기초자료를 제공하고자 하였다. 방법: 2021년 1월 1일부터 2022년 2월 28일 사이에 서울대학교 어린이병원에 지역사회폐렴으로 입원한 소아청소년 및 2022년 1월과 2월 전국 7개 대학병원에 지역사회폐렴으로 입원한 소아청소년의 임상 정보를 후향적으로 수집하고 분석하였다. 해당 연구 기간 동안 참여 기관들에서는 폐렴 진단명을 임상 증상과 진찰 소견, 그리고 흉부방사선 소견에 대해 각각 엄격한 기준을 적용하고 이를 종합해서 선별적으로 넣도록 하였고, 이들에 대해 추후에 의무기록 분석을 통해 다시 한번 폐렴 진단의 적절성을 평가하여 최종적으로 연구 대상자 여부를 결정하였다. 결과: 코로나19 유행기의 14개월 동안 서울대학교 어린이병원에 지역사회폐렴으로 입원한 소아청소년은 총 91명이었다. 이 중 5세 이하가 68.1%로 높은 비중을 보였고, 79.1%는 기저질환을 가지고 있었다. 총 95건의 지역사회폐렴 중 호흡 보조가 필요했던 경우는 70.5%였으며, 인공호흡기는 20.0%에서 적용되었다. 최종적으로 5명의 사망자가 발생하였고 모두 면역저하 혹은 신경계 기저질환이 있었다. 위험인자분석에서도 신경계 기저질환과 면역저하상태는 각각 호흡 보조(P=0.003) 및 사망(P=0.014)과 유의한 상관관계가 있었다. 검출된 호흡기 병원체의 54.7%는 바이러스였고 그 중 리노바이러스의 비율이 35.9%로 가장 높았다. 2022년 초 2개월 간 8개 기관에서 지역사회폐렴 진단 하에 퇴원한 소아청소년은 총 169명이었고, 이들 중 92.3%에서 한 개 이상의 호흡기바이러스가 검출되었으며 대부분 respiratory syncytial virus (79.8%)였다. 결론: 코로나19 판데믹 기간에도 국내 소아청소년들이 주로 계절성 호흡기바이러스에 의해 발생한 지역사회폐렴으로 입원치료를 받았다. 주요 세균성 폐렴의 원인균들은 아직 유행이 없으나 지속적인 임상 감시와 연구가 필요하다.

• Clinical and Experimental Pediatrics
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• 제53권6호
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• pp.705-710
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• 2010

Purpose: To determine the clinical manifestations and outcomes of patients with tracheoesophageal fistula (TEF) and esophageal atresia (EA) born at a single neonatal intensive care unit. Methods: A retrospective analysis was conducted for 97 patients with confirmed TEF and EA who were admitted to the neonatal intensive care unit between 1990 and 2007. Results: The rate of prenatal diagnosis was 12%. The average gestational age and birth weight were $37^{+2}$ weeks and $2.5{\pm}0.7kg$, respectively. Thirty-one infants were born prematurely (32%). Type C was the most common. The mean gap between the proximal and distal esophagus was 2 cm. Esophago-esophagostomy was performed in 72 patients at a mean age of 4 days after birth; gastrostomy or duodenostomy were performed in 8 patients. Forty patients exhibited vertebral, anorectal, cardiac, tracheoesophageal, renal, limb (VACTERL) association with at least 2 combined anomalies, and cardiac anomaly was the most common. The most common post-operative complications were esophageal stricture followed by gastroesophageal reflux. Balloon dilatation was performed for 1.3 times in 26 patients at a mean age of 3 months. The mortality and morbidity rates were 24% and 67%, respectively, and the most common cause of death was sepsis. The weight of approximately 40% patients was below the 10th percentile at 2 years of age. Conclusion: Mortality and morbidity rates of patients with TEF and EA are high as compared to those of infants with other neonatal surgical diseases. Further efforts must be taken to reduce mortality and morbidity and improve growth retardation.

• Clinical and Experimental Pediatrics
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• 제53권1호
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• pp.106-110
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• 2010

해마경화증은 난치성 측두엽간질의 가장 흔한 원인들 중 하나이다. 일반적으로 해마경화증은 뇌 자기공명영상에서 높은 민감도와 특이도로 규명될 수 있다. 이 병변의 뇌 자기공명영상 소견은 해마에 국한된 조영증가와 이와 연관된 위축된 해마 소견이라 할 수 있다. 반면에 국소성 겉질 형성이상증은 회백질-백질 경계부의 불분명함, 백질 부분의 이상 조영증강 소견을 보인다. 저자들은 수술적 치료 이전에 뇌 자기공명 영상에서 좌측 측두엽의 겉질 형성이상증의 소견을 보였으나, 수술 후 병리검사에서 해마경화증 외에 정상소견을 보였던 1례를 경험하였기에 보고하고자 한다.

• Journal of Genetic Medicine
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• 제14권2호
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• pp.56-61
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• 2017

Purpose: Neurofibromatosis type 2 (NF2) is characterized by multiple tumors, including vestibular schwannoma (VS) and others affecting cranial and peripheral nerves. NF2 is caused by mutation of the NF2 gene. The mutation spectrum of NF2 has not been characterized in Korean patients. In the current study, the clinical and genetic characteristics of Korean NF2 patients were analyzed. Materials and Methods: Twenty-five unrelated Korean families were enrolled according to the Manchester criteria. Genetic analysis was performed by direct sequencing and multiplex ligation-dependent probe amplification methods using genomic DNA from peripheral lymphocytes or tumor tissues. Results: All patients had bilateral/unilateral VS and/or other cranial and peripheral nerve tumors. Two patients were familial cases and the other 24 patients were sporadic. Germline NF2 mutations were detected in peripheral lymphocytes from both familial cases, but only in 26.1% of the 23 sporadic families. Somatic mutations were also found in tumor tissues from two of the sporadic families. These somatic mutations were not found in peripheral lymphocytes. A total of 10 different mutations including 2 novel mutations were found in 40.0% of studied families. Five mutations (50.0%) were located in exon 6 of NF2, the FERM domain coding region. Conclusion: Family history was an important factor in identifying germline NF2 mutations. Further study is required to investigate whether exon 6 is a mutation hotspot in Korean NF2 patients and its correlation to phenotypic severity.