• Journal of Chest Surgery
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• v.31 no.8
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• pp.763-769
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• 1998

Background: Supravalvular aortic stenosis is a rare form of congenital cardiac anomaly involving ascending aorta distal to coronary orifice. Materials and methods: We operated 12 cases of supravalvular aortic stenosis between July 1986 and March 1997. Age ranged from 4 to 17(mean 10.2) years and 11 of them were male. Nine patients had clinical features of Williams syndrome. We experienced two types of supravalvular aortic stenosis, including 10 hour glass type and 2 diffuse type. Results: Preoperative transaortic pressure gradient ranged from 40 to 180(mean 92) mmHg by cardiac catheterization. Pulmonary stenosis was associated in 5 and 2 of them required angioplasty. Operative techniques included 6 standard aortoplasty with elliptical patch, 4 extended aortoplasty with inverted Y shaped patch, and 2 modified Brom's repair. There were no operative deaths. Postoperative echocardiographic evaluation was done at a mean interval of 12 months. Grade I or II aortic regurgitation was found in 3 cases. Postoperative cardiac catheterization revealed a mean transaortic pressure gradient of 26 (range 0 to 75) mmHg. A mean pressure drop was 78(range 30 to 114) mmHg. All patients were followed up for a mean of 40(range 1 to 67) months with uneventful clinical course. Conclusions: Our data proved the low mortality and excellent hemodynamic improvement after surgical relief of supravalvular aortic stenosis in children.

• Advances in pediatric surgery
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• v.19 no.2
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• pp.108-121
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• 2013

목적 Pheochromocytoma of the Adrenal gland Scaled Score (PASS) 시스템과 면역화학염색 등을 통한 갈색세포종 및 부교감신경절종의 악성화 예측인자가 제시되고 있으나 명확한 병리학적 또는 분자생물학적 예측인자는 밝혀진 바 없다. 본 연구에서는 임상적, 병리학적 분석을 통해 갈색세포종 및 복강내 부교감신경절종의 악성화 예측인자를 확인하고자 하였다. 대상 및 방법 1990년 1월부터 2010년 12월까지 서울대학교어린이병원에서 수술적 절제 후 병리학적으로 갈색세포종 및 복강내 부교감신경절종으로 확진된 20명의 18세 이하 소아 환자를 대상으로 임상적 특징을 분석하였고, PASS 시스템에 따른 병리 슬라이드 판독하였다. 세포활성도를 반영한다고 알려진 유전자에 대한 항체 중 Ki-67, p53, bcl-2, mdm-2, cycline D1, p21, p27을 이용해 면역 화학검사를 한 후 결과를 확인하였다. 결과 20명의 환자 중 갈색세포종은 14명, 복강내 부교감신경절종은 6명이었다. 악성화는 각각 4명, 3명에서 관찰되었다. 혈관 침범, 주변부 지방조직 침습, 세포분열 증가가 통계적으로 유의한 악성화 예측인자였으며(각각 p=.007, .031, .031), 갈색세포종만 분석하였을 때도 통계적으로 유의하였다(각각 p=.033, .003, .019). PASS 시스템은 악성화를 예측하는데 있어 통계적으로 유의하지 않았으며, 혈관 침범, 주변부 지방조직 침습, 세포분열 증가를 항목으로 하여 새롭게 만든 병리 스코어 시스템은 악성 환자군과 양성 환자군 사이에 통계적으로 유의한 차이를 보였다(p< .001). 악성과 양성 질환 사이의 면역화학염색 결과에서 유의한 차이는 없었다. 결론 소아에서 갈색세포종 및 복강내 부교감신경절종의 악성화 예측인자로 혈관 침범, 주변부 지방조직 침습, 세포분열 증가를 이용할 수 있다. 소아에서 PASS시스템으로 악성화를 예측할 수 없었으나, 새로운 병리스코어 시스템으로 악성 환자군을 예측할 수 있었다. 면역화학검사 결과 세포 활성도를 반영하는 인자들은 악성화를 예측할 수 없었다.

• Pediatric Infection and Vaccine
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• v.22 no.1
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• pp.23-28
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• 2015

Purpose: The aim of this study was to investigate the clinical manifestations and laboratory characteristics of eosinophilic meningitis in Severance Children's Hospital. Methods: We examined 6,335 children under the age of 18 years old who had visited the tertiary hospital in Seoul, Korea, and had received cerebrospinal study results between January 2007 and July 2012. The medical records of the patients identified as eosinophilic meningitis were retrospectively reviewed. Results: Eosinophilic meningitis was diagnosed in 39 patients (0.6%). The mean age was 6 years (range 0-18 years) and the sex ratio was 1.3:1 (22 males and 17 females). The underlying diseases and past history were neurologic disease (n=36, 92%). Eosinophilic meningitis was diagnosed in thirty-five patients who had undergone postoperation neurosurgery (90%). The most common symptoms were fever (50%), headache (20%), vomiting (15%), seizure (10%), and dizziness (5%). The average duration for recovery was five days, and intravenous antibiotics or steroids were used. Conclusions: Manifestations of eosinophilic meningitis are similar to other types of meningitis. The most common cause of eosinophilic meningitis in children was neurosurgery. Eosinophilic meningitis should be considered for patients showing fever and headache after neurosurgery. Through careful investigation, use of improper antibiotics could be avoided.

• Pediatric Infection and Vaccine
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• v.15 no.2
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• pp.188-194
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• 2008

Purpose : The purpose of this study is to investigate the clinical characteristics of recurrent Kawasaki disease (KD). Methods : From January 2004 to December 2007, the medical records of 20 children with recurrent KD in Severance Children's Hospital were retrospectively reviewed. The clinical characteristics, laboratory findings, treatment and complications of these patients were compared between the initial episode and the second episode. Results : At the initial episode of the recurrent KD group, the gender ratio was 1.2:1 (male:female) and the mean age was $37.2{\pm}19.9$ months. The interval between the two episodes in the recurrent KD group was 3.3 months. The febrile period before admission was shorter for the second episode (P=0.034). The skin rash was less developed in the second episode. But there were no differences in the laboratory results and complications between the initial episode and the second episode. Three patients (15%) among those with a second episode failed to respond to the initial intravenous immunoglubulin treatment. On comparison between the initial episodes of the recurrent group and the nonrecurrent group, the erythrocyte sedimentation rate was higher in the first episode of the recurrent KD group. Conclusions : For recurrent KD, it tends to present more atypical features than the KD that occurs for the first time. Physicians should consider these characteristics when making the diagnosis and treating recurrent KD.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.2
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• pp.122-129
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• 2008

Purpose: Patients with chronic gastrointestinal disease are at risk for trace element deficiency due to impaired absorption and gastrointestinal loss. The aim of this study was to evaluate the trace element status of patients with gastrointestinal disease by blood and hair analysis, and to determine the usefulness of hair mineral analysis for diagnosing trace element deficiency not detected by a blood test. Methods: An analysis of hair minerals was performed and compared with blood mineral analysis in 13 patients with chronic gastrointestinal disease. The concentration of each element in the hair and blood was compared in the subgroups based on parenteral nutritional support or clinical symptoms. Results: Almost all patients had trace element deficiency. The trace elements deficient in the blood or hair analysis included zinc, selenium and copper. The hair zinc concentration was significantly lower in the group receiving parenteral nutritional support. The hair selenium concentration was statistically associated with the clinical symptoms of hair loss, brittle hair and loss of hair pigmentation. Conclusion: The results of this study suggest that patients with chronic gastrointestinal disease should receive adequate zinc and selenium replacement to avoid trace element deficiency especially when treated with long-term parenteral nutrition. Hair mineral analysis is useful as a complementary tool for the detection of a trace element deficiency.

• Pediatric Infection and Vaccine
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• v.23 no.3
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• pp.217-222
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• 2016

Purpose: This study aimed to identify the differential clinical, laboratory, and imaging characteristics between patients with cervical lymphadenitis as first presentation of Kawasaki disease (CLKD) and those with acute unilateral cervical lymphadenitis (AUCL). Methods: We surveyed 372 patients who visited Pusan National University Children's Hospital because of fever and cervical lymph node enlargement, and underwent neck computed tomography (CT) from January 2010 to December 2014. We compared 28 confirmed cases of Kawasaki disease and 28 cases of AUCL based on a retrospective review of the medical records of the patients. Results: Patients with CLKD and AUCL showed no differential clinical characteristics in terms of the duration of fever, antibiotic use, or the size of lymph nodes. Patients with CLKD had higher white blood cell count, absolute neutrophil count, erythrocyte sedimentation rate, and C-reactive protein levels (P<0.05) than those of patients with AUCL. The presence of retropharyngeal edema on neck CT was similar between the groups (64% vs. 33%, P=0.686). Conclusions: CLKD and AUCL showed no differentiating clinical and radiological characteristics; hence, Kawasaki disease should be the presumptive diagnosis in patients with fever and cervical lymph node enlargements who fail to respond to antibiotic treatment.

• Journal of the korean academy of Pediatric Dentistry
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• v.29 no.4
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• pp.489-497
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• 2002

The purpose of this study was to compare the clinical sedation effect of chloral hydrate and hydroxyzine combination with and without nitrous oxide/oxygen inhalation when young children were sedated for dental treatment. The uncooperative 20 children aged, 21 to 47 months of age(ASA Class I), participated in the study. The author examined 20 children(male 12, female 8). Each patient was assigned to receive chloral hydrate(50mg/kg) and hydroxyzine(25mg). Next appointment, each patient was assigned to receive $N_2O-O_2$, choral hydrate and hydroxyzine. Sleep, crying, movement, and overall behavior response were evaluated, and the sedative effects were evaluated by Houpt's rating scale. Pulse rate and peripheral oxygen saturation were also measured for monitoring the sedated patients during treatment period by pulse oximeter. The result were as follows : 1. In the evaluation of sleep scores, crying scores, and movement scores, chloral $hydrate/hydroxyzine/N_2O-O_2$ combination group was significantly rated high(p<0.05). 2. In the evaluation of overall behavior scores, chloral hydrate/hydroxyzine/$N_2O-O_2$ combination group was significantly rated high(p<0.05). 3. In the evaluation of overall behavior evaluation scores(by Houpt), 93.3% in chloral $hydrate/hydroxyzine/N_2O-O_2$ combination group and 63.3% in chloral hydrate/hydroxyzine combination group were rated "good" or "very good". 4. There was no adverse side effect(i.e. respiratory depression) in both group.

• Journal of the korean academy of Pediatric Dentistry
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• v.40 no.4
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• pp.274-282
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• 2013

The goal of this study was to analyze prevalence and distribution of congenitally missing teeth in 3302 patients from 7 to 15 years old, who had visited and taken panoramic radiographs at the Department of Pediatric Dentistry, from January 2008 to December 2012. The prevalence of hypodontia in the males (5.9%) was slightly higher than in the females (4.7%). In the affected patients, the mean number of congenitally missing teeth in the mandible was statistically higher than in the maxilla (p < 0.05). However, there was no significant difference in the number of congenitally missing teeth between left and right dentitions (p > 0.05). Excluding the Mn. third molars, the most frequent congenitally missing teeth site was the Mn. second premolars. In contrast, the most infrequent congenitally missing teeth sites were the Mx. and Mn. first premolars, and Mx. central incisors. The bilateral combination or antagonistic quadrants combination of congenitally missing teeth in hypodontia patients was 80.3%. The result of this study will contribute to clinicians to make accurate diagnosis and establish favorable treatment plans, by offering invaluable data about the number and distribution of congenitally missing teeth.

• Pediatric Infection and Vaccine
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• v.19 no.2
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• pp.71-78
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• 2012

Purpose: Human bocavirus (hBoV), a recently discovered virus, has been detected in children with respiratory tract infections worldwide. The aim of this study was to analyze the frequency and molecular phylogeny of hBoV in the respiratory samples of children with acute respiratory tract infections in 2010. Methods: Nasopharyngeal samples were collected from 953 children with lower respiratory tract infections at Severance children's hospital in Korea from January 2010 to December 2010. We applied the multiplex PCR technique for the identification of 12 respiratory viruses from the samples. Among the total specimens, hBoV positive samples were subjected to phylogenetic analysis by sequencing a fragment of the VP1/VP2 gene junction. Results: hBoV was detected in 141 (14.8%) among 953 patients. The 61.7% of hBoV-positive samples were found to co-exist with other respiratory viruses. The results of phylogenetic analysis showed that all 141 hBoV-positive isolates were identified as hBoV 1, revealing a high similarity among the isolates (>98%). Conclusion: hBoV 1 with minimal sequence variations circulated in children with acute respiratory infections during 2010. More research is needed to determine the clinical severity and outcomes of the minimal sequence variations.

• Pediatric Infection and Vaccine
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• v.16 no.2
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• pp.199-204
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• 2009

Purpose : Various proteins encoded in the early region 3 (E3) of adenoviruses protect cells from being killed by cytotoxic T cells and death-inducing cytokines. We sought to find out whether the genetic heterogeneity of the E3 gene might contribute to the molecular diversity of adenoviruses. Methods : Sequences in the E3 region were analyzed for 14 adenovirus type 3 (Ad3) strains that were isolated from children with lower respiratory tract infections in the Seoul National University Children's Hospital during the period 1991-2000. Full-length adenoviral DNA was purified from the infected A549 cell lysates using a modified Hirt procedure. Results : There was 98% homology between 14 Korean Ad3 strains with a reference strain (M15952). Homology within the Korean Ad3 strains was 98.7%. Variation was found in the region of transcripts 20.1 kDa, 20.6 kDa, truncated 7.7 kDa, 10.3 kDa, 14.9 kDa, and 15.3 kDa. In particular, all 14 Korean strains showed a missense single point mutation at the start codon of the truncated 7.7 kDa. In addition, a deletion was found in the truncated 7.7 kDa region by 58 base pairs in 10 strains and 94 base pairs in 4 strains. Variations in amino acids were observed in the receptor internalization and degradation complex (10.3 kDa/14.9 kDa) which stimulates the clearance from the cell surface and subsequent degradation of the receptors for the Fas ligand and TRAIL, while no variations were observed in another immunoregulatory transcript, 19 kDa. Conclusion : Sequence analysis of the immunoregulatory region of adenovirus E3 shows that genetic heterogeneities are related to genome type patterns.