• Journal of Genetic Medicine
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• 제14권1호
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• pp.23-26
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• 2017

Isolated 3-methylcrotonyl-CoA carboxylase deficiency is an autosomal recessive disorder affecting leucine metabolism; it is one of the most common inborn metabolic diseases detected in newborn screening. Mutations in the genes MCCC1 or MCCC2 cause a defect in the enzyme 3-methylcrotonyl-CoA carboxylase, with MCCC2 mutations being the form predominantly reported in Korea. The majority of infants identified by neonatal screening usually appear to be asymptomatic and remain healthy; however, some patients have been reported to exhibit mild to severe metabolic decompensation and neurologic manifestations. Here we report the clinical features of a patient with asymptomatic 3-methylcrotonyl-CoA carboxylase deficiency and novel heterozygous MCCC1 mutations.

• Journal of Preventive Medicine and Public Health
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• 제19권2호
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• pp.281-292
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• 1986

Infection by hepatitis B virus is one of the major health problems of this nation. HBsAg positive rates of general population and school children were known to be as about 8 percent and 3.9 to 5.9 percent respectively. To study the incidence rate of hepatitis B infection in school children of rural area, author had examined 475 school children of relatively isolated agricultural area for baseline prevalence of hepatitis B virus serologic markers and followed up 415 school children during 10 months to determined the frequency of serologic conversion. The major results are summarized as followings: 1) Among the 278 susceptible children who were followed up, 26 had seroconversion for HBsAg or Anti-HBs. Therefore, the cumulative incidence rate during 10 months is estimated 9.4%. 2) The incidence rate of hepatitis B infection tends to increase with age (6-9yrs: 3.2%, 10-14yrs: 9.5%, 15-17yrs: 18.9%), and the incidence rate in male (13.0%) was higher than in female (5.7%). 3) The incidence rates of hepatitis B virus infection were not different statistically among three economic classes (The rates of lower, middle and higher class were 11.8%, 7.1% and 10.5%.). 4) The incidence rates of hepatitis B virus infection were not different statistically between visitors and non-visitors of clinic or hospital, dental clinic, persons received IV and not received IV, and persons with familial history and without familial history of liver diseases. Therefore all of these factors were not identified as risk factor of hepatitis B virus infection. And the transmissibility within the class of school was not recognized, too. 5) Among the 25 children who were HBsAg positive when enrolled, 15 (60%) were still HBsAg positive, who were identified as chronic carrier 15 of 415 school children were chronic carriers, then chronic carrier rate was estimated 3.6%, and there was no difference between sexes. 6) Of 38 children who had been Anti-HBs positive when enrolled, 5 (13.2%) lost Anti-HBs. Therefore, the loss rate of Anti-HBs per year is estimated to be 15.8%.

• 한국환경보건학회:학술대회논문집
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• 한국환경보건학회 2004년도 International Conference Global Environmental Problems and their Health Consequences
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• pp.213-216
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• 2004

This work was based on the hypotheses that fingernail clippings can be used as a biomarker for the subchronic exposure to fluoride. This study was conducted to prove the hypotheses and fingernail clippings were collected form 120 preschool children aged $3{\sim}6$ residing in Jumunjin(community water fluoridation area) and Kangnung(non-fluoridation area). The acid-diffusible fluoride in the fingernail clipping was isolated by the acid-diffusion technique and measured with a fluoride electrode. The mean fingernail fluoride as the children's living area were 4.49ug/g(SD 2.43) to Jumunjin and 2.68ug/g(SD 1.13) to Kangnung. As would be expected, the fluoride concentration in fingernail clippings were significantly different between the two geographical groups(p<0.001) determined by t-test.

• Clinical and Experimental Pediatrics
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• 제49권1호
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• pp.82-86
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• 2006

목 적: 1990년대 초반부터 시행된 학교집단소변검사에서는 단독혈뇨가 가장 많이 발견되고 있으나, 이러한 환아들의 장기적 예후나 추가적검사의 필요성에 대해서는 아직 논란이 있다. 이에 저자들은 이들 환아의 추적 관찰결과를 통해 예후와 필요한 추적검사의 정도를 알아보고자 하였다. 방 법: 1990년 8월부터 2004년 2월까지 학교신체검사에서 무증상적 혈뇨가 발견되어 서울아산병원 소아과에 의뢰된 환아 중 단백뇨(>250 mg/일)나 다른 신염의 증거, 선천성신기형, 신기능의 저하(크레아티닌청소율 <85 mL/분/$1.73m^2$)가 없는 단독 혈뇨 환아들을 대상으로 경과를 관찰하였다. 추적 관찰은 6개월마다 요검사와 혈청 크레아티닌, 단백, 알부민을 측정하였고 요검사 상 단백뇨가 나온 환아에서 24시간 요단백을 측정하여 500mg/일 이상인 경우 신생검을 시행하였다. 결 과: 총 331명의 경과를 관찰하였으며 남자는 157명, 여자는 174명이었다. 평균 나이는 $9.9{\pm}2.3$세(7-15세)였고, 평균 추적 관찰기간은 $2.2{\pm}1.6$년(1-10년)이었다. 경과관찰 중 혈뇨가 소실된 환아는 75명(22.7%)으로 평균 소실 기간은 $2.6{\pm}1.7$년(1-8년)이었으며, 나머지 256명(77.3%)에서는 혈뇨가 지속되었고 그 중 8명(2.4%)에서 유의한 단백뇨 증가를 보였다. 이들 8명 중 4명은 경과관찰 중 단백뇨가 다시 감소하여 추가적인 검사 없이 경과관찰 중이며, 나머지 4명에서는 단백뇨가 지속되어 신생검을 시행하였다. 이중 2명에서는 특이 소견이 발견되지 않았고, 나머지 2명은 경도의 IgA신병증으로 진단되었다. 경과관찰 중 신기능 저하나 고혈압을 보인 환아는 없었다. 결 론: 학교집단소변검사에서 발견된 단독 혈뇨 환아의 임상 경과는 양호하므로 신생검 등의 침습적검사는 불필요하며 정기적인 요검사와 혈액검사로 환아의 경과를 관찰하여도 충분할 것으로 판단된다.

• Journal of Chest Surgery
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• 제17권2호
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• pp.212-222
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• 1984

A total of and consecutive 291 patients underwent isolated mitral valve replacement using the Ionescu-Shiley bovine pericardial xenograft valve during the 5-year period between October 1978 and June 1983. Thirty-two patients were the children under 15 years of age. There were 15 deaths within 30 days after surgery [operative mortality, 5.2%]. All early survivors except 6 children were placed on the long-term oral anticoagulation longer than postoperative 3 months. A total follow-up period extended for 398.2 patient-years, and 12 patients died [late mortality, 4.1%, or 3.0%/patient- year]. Ten patients experienced the thromboembolic complication [2.51%/patient-year], occurring in 8 patients within the first 3 postoperative months, and 4 died. Three patients had the late prosthetic valve endocarditis [0.75%/patient-year] and 2 died. The incidence of overall valve failure according to the criteria was 3.01%/patient-year, or 12 patients, and 2 had replacement of the failed bioprostheses [primary tissue failure, 0.5%/patient-year]. The long-term survival rate was 87.8%\ulcorner2.6% at 5 years postoperatively, and 84% of the late survivors were in NYHA Class I at the end of the follow- up. The probability remaining free from thromboembolism and overall valve failure was 89.8%\ulcorner6.3% and 81.2%\ulcorner.8% at 5 years respectively. These clinical results confirm the safety of mitral valve replacement. The only remaining clinical problem is the structural and functional durability of the bovine pericardial xenograft valve, and its use in young patients may be stopped in preference to the mechanical prosthetic valves.

• 한국임상약학회지
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• 제23권1호
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• pp.20-25
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• 2013

Background & Objectives: Burn injury mortality and septic complication are frequent and well-known in burned pediatric patients. The overuse of antibiotics is the base for development of wound infection by resistant microorganisms as well as opportunist agents. Methods: We have carried out a study of the bacterial profile and antimicrobial resistance clinically important bacteria isolated from burn wound infections in children patients. The most common isolate from burn wound cultures was Pseudomonas aeruginosa (26.8%), followed by Staphylococcus aureus (25.4%), Acinetobacter baumannii (12.7%), coagulase negative staphylococcus (12.0%), Enterococcus faecium (7.7%), Escherichia coli (4.9%), Enterococcus faecalis (3.7%), Burkholderia cepacia (3.0%), Enterobacter cloacae (2.3%) and Klebsiella pneumonia (2.3%). Colistin was very significantly effective drug in gram negative organism, such as Pseudomonas aeruginosa and Acinetobacter baumannii. Results & Conclusion: The resistance rates were 65% and 98% to piperacillin, 63% and 97% to ceftazidime, 28% and 50% to levofloxacin. The most effective antibiotic in gram positive organism, such as Staphylococcus aureus, coagulase negative staphylococcus were moxifloxacin. The resistance rates were 83% and 64% to ciprofloxacin, 80% and 17% to clindamycin.

• 한국산학기술학회논문지
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• 제10권7호
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• pp.1766-1772
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• 2009

본 연구에서는 한국인 아동의 우식치아로부터 S. mutans를 분리하고, acid stress하에서 분리한 S. mutans의 내산성 능력과 관련된 단백질을 규명하고자 하였다. 2D gel electrophoresis를 수행한 결과, acid stress동안 elongation factor Ts, hypothetical protein, putative amino acid ABC transporter, adenylate kinase, fructokinase, Putative 40K cell well protein precursor, peptide deformylase, shikimate 5-dehydrogenase, mannose-6-phosphate isomerase, threonine synthase, putative dTDP-glucose-4,6-dehydratase의 발현량이 뚜렷이 증가하였으며 이들 단백질은 acid stress에 관여하는 단백질들로 추정된다.

• Clinical and Experimental Pediatrics
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• 제57권1호
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• pp.29-34
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• 2014

Purpose: In this study, we aimed to investigate the prevalence of year-round respiratory viral infection in children with lower respiratory tract infection (LRTI) and the relationship between respiratory viral infection and allergen sensitization in exacerbating asthma. Methods: We investigated the sources for acute LRTIs in children admitted to our hospital from May 2010 to April 2011. The 6 most common respiratory viruses were isolated from nasopharyngeal aspirate using multiplex reverse transcription-polymerase chain reaction in 309 children; respiratory syncytial virus (RSV), adenovirus (AV), parainfluenza virus (PIV), influenza virus (IFV), human metapneumovirus (hMPV), rhinovirus (RV). Atopic sensitization was defined if more than 1 serum specific Immunoglobulin E level measured using UniCAP (Pharmacia) was over 0.35 IU/mL. Results: RSV was the most common pathogen of bronchiolitis in hospitalized children through the year. RV or IFV infection was more prevalent in asthma exacerbations compared to other LRTIs. AV and hMPV were more likely to cause pneumonia. RV and IFV were associated with asthma exacerbations in children with atopic sensitization, but not in nonatopic children. Conclusion: RV and IFV are associated with hospitalization for asthma exacerbation in children with atopic sensitization.

• Pediatric Infection and Vaccine
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• 제22권3호
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• pp.178-185
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• 2015

목적: 국내 소아 그람음성균혈증에서 항생제 내성변화 및 항생제 사용량과의 관계를 분석하였다. 방법: 최근 10년간 18세 이하 입원 환자의 혈액에서 분리된 그람음성균의 항생제 내성률 변화 및 항생제 사용량과의 관계를 분석하였다. 결과: 폐렴막대균, 대장균, 녹농균, 아시네토박터 바우마니의 분리율은 연간 혈액배양 1,000건당 각각 4.6례, 3.5례, 3.4례 및 2.2례였다. 폐렴막대균에서 광범위 세팔로스포린에 대한 내성변화는 없었으나 2010년부터 카바페넴 내성 폐렴막대균이 동정된 후 점차 빈도가 증가하였다. 대장균의 광범위 세팔로스포린 내성이 10%에서 50%로 아시네토박터 바우마니의 카바페넴 내성이 11%에서 71%로 크게 증가하였다(P for trend <0.01). 녹농균은 여러 항생제에 높은 내성을 보였으나 유의한 내성변화를 보이지 않았다. 대장균의 cefepime 내성과 cefepime 사용량 사이에 유의한 양의 상관관계를 보였다(r=0.900, P=0.037). 결론: 국내 소아에서 발생한 그람음성균 균혈증 분석시 카바페넴 및 광범위 세팔로스포린 내성이 증가하였고 일부에서 항생제 사용량과의 관련성을 확인하였다. 이는 원내 경험적 항생제 결정시 중요한 고려 사항이며 추후에도 지속적인 원내 항생제 사용량 및 내성률에 대한 감시가 필요하겠다.

• Clinical and Experimental Pediatrics
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• 제46권11호
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• pp.1085-1088
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• 2003

목 적 : 국내 소아 세균성 수막염의 원인 균주가 시대적으로 차이가 있는지 알아보고자 하였다. 방 법 : 1992년 1월부터 2002년 12월까지 가톨릭대학교 대전성모병원에 세균성 수막염으로 입원한 40례(신생아군 14례, 소아군 26례)를 대상으로 하여 신생아군은 1970-80년대의 국내 보고와 비교하였고, 소아군은 Hib 백신이 도입되기 전후 시기의 H. influenzae 수막염의 발생 빈도를 비교하였다. 결 과 : 신생아군에서 GBS(33.3%)가 가장 흔한 원인 균주였으며 국내에서도 그람 음성균에서 GBS를 주로 하는 그람 양성 균주로의 변화 양상을 보였다. 소아군에서는 Hib 백신이 도입되기 이전인 1992년-1995년 사이에는 11례의 세균성 수막염 중 6례(55%)가 H. influenzae에 의한 수막염이었다. Hib 백신이 일반화된 시기인 1999-2002년에 7명의 세균성 수막염 환아 중 2명(29%)으로 H. influenzae 수막염이 감소하는 추세를 보였지만 통계학적으로 유의하지는 않았다(P=0.36). 결 론 : 신생아 수막염에서 GBS가 주된 원인 균주로 나타났으며, 이후 연령에서는 Hib 백신이 도입된 이후 H. influenzae에 의한 수막염은 감소하는 경향을 보였다.