• Journal of Genetic Medicine
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• v.14 no.1
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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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• v.19 no.2 s.20
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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%.

• Proceedings of the Korean Environmental Health Society Conference
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• 2004.06a
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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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• v.49 no.1
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• pp.82-86
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• 2006

Purpose : The isolated microscopic hematuria is the most common abnormality detected by school urinary screening, but there is no consensus about the range of investigations and long-term outcomes of isolated hematuria in children yet. This study aims to elucidate the prognosis of hematuria and the range of diagnostic studies by follow-up results. Methods : Students with isolated hematuria who were referred to the Department of Pediatrics, Asan Medical Center from Aug. 1990 to Feb. 2004 were analysed retrospectively. Cases that presented Through significant proteinuria(>250 mg/day), other symptoms of nephritis or renal dysfunction (creatinine clearance <85 mL/min/$1.73m^2$) were excluded. Follow-up was done every six months with checking urinalysis, serum creatinine, protein and albumin. When albuminuria was detected, 24 hour urine protein was checked. Renal biopsy was done when urine protein was over 500 mg/day. Results : A total of 331 students were enrolled in this study. There were 157 males and 174 females. The mean age at presentation was $9.9{\pm}2.3$ years(7-15 years) and mean follow-up period was $2.2{\pm}1.6$ years(1-10 years). Seventy five(22.7 percent) patients showed the resolution of microscopic hematuria. The mean resolution period was $2.6{\pm}1.7$ years(1-8 years). Eight(2.4 percent) patients developed significant proteinuria and renal biopsy was done in four of them. Two cases of mild IgA nephropathy and two of minimal change were detected. None of them developed hypertension. At the end of the follow-up, renal function had remained stable in all subsets of patients. Conclusion : The prognosis of isolated microscopic hematuria was good. This study suggests that invasive studies including renal biopsy are not necessary and a regular follow-up of urinalysis is enough for children with isolated microscopic hematuria.

• Journal of Chest Surgery
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• v.17 no.2
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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.

• Korean Journal of Clinical Pharmacy
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• v.23 no.1
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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.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.10 no.7
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• pp.1766-1772
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• 2009

In this study, we are interested in comparing the protein profiles of acid-shocked and control cells of S. mutans isolated from Korean children with caries. The results of 2D gel electrophoresis showed that twelve proteins are up-regulated when the cells were grown under 20 mM lactic acid stress in the exponential phase. Up-proteins under acid stress were estimated a major key of the survival and proliferation of S. mutans in low pH environments. These proteins are estimated generally associated with three biochemical pathways: glycolysis, alternative acid production and branched-chain amino acid biosynthesis.

• Clinical and Experimental Pediatrics
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• v.57 no.1
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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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• v.22 no.3
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• pp.178-185
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• 2015

Purpose: We investigated trends in antibiotic pressure and the antibiotic susceptibility of gram negative bacteria isolated from Korean children over 10 consecutive years. Methods: From January 2004 to December 2013, the antibiotic susceptibility of Klebsiella pneumoniae, Escherichia coli, Pseudomonas aeruginosa, and Acinetobacter baumannii blood isolates obtained from children <18 years of age was determined according to the 2009 Clinical and Laboratory Standards Institute guidelines. Antibiotic consumption data were also analyzed. Results: The prevalence of K. pneumoniae, E. coli, P. aeruginosa, and A. baumannii bacteremia was 4.6, 3.5, 3.4, and 2.2 cases/1,000 blood cultures/year, respectively. In K. pneumoniae, resistance to the third and fourth cephalosporin did not increase significantly. However, carbapenem-resistant K. pneumoniae first appeared in 2010, and the resistance rate increased to 9% between 2012 and 2013. Resistance to 3rd and 4th cephalosporin increased from 10% to 50% in E. coli, and resistance to carbapenem rose abruptly from 11% to 71% in A. baumannii (P for trend <0.01). However, such an increase of resistance was not observed in P. aeruginosa. There is a positive correlation between the resistance rate of cefepime in E. coli and the consumption of cefepime (r=0.900, P=0.037). Conclusion: The significant burden of antibiotic consumption and the high prevalence of antibiotic resistance to gram negative pathogen isolated from bacteremic children were observed. Empirical antibiotics should be wisely selected, and continued efforts to decrease the overall antibiotic pressure are mandatory, especially in highly resistant situations.

• Clinical and Experimental Pediatrics
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• v.46 no.11
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• pp.1085-1088
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• 2003

Purpose : This study was performed to assess the difference of organisms causing bacterial meningitis according to time. Methods : We analyzed retrospectively 40 medical records of bacteriologically proven meningitis from 1992 to 2002. We divided them into two groups; neonate's group(14 cases), and children's group(26 cases). The results of the neonate's group were compared with those of previously reported articles in Korea, in 1970s-1980s. The causative agents of the children's group were analyzed according to the stage before and after the introduction of H. influenza type b(Hib) vaccine. Results : In neonates, Group B streptococci(GBS) was the most common cause of bacterial meningitis. There was a trend in Korea that major causative agents of neonatal bacterial meningitis have changed from gram negative bacteria including E. coli to gram positive bacteria including GBS. In children, H. influenzae was isolated in six out of 11 cases(55%) in 1992-95, before the introduction of Hib vaccine, while two out of seven(29%) were isolated in 1999-2002, after the introduction of the Hib vaccine. Conclusion : Our study showed that the most common agent of neonatal bacterial meningitis was GBS. There was a trend that after the introduction of Hib vaccine, the incidence of H. influenza meningitis decreased in children.