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미숙아에서 말초 유도 중심정맥관 삽입과 연관된 흉막삼출과 횡격막마비
장미선 ( Mi Sun Chang ),백상훈 ( Sang Hun Baek ),이명숙 ( Myung Sook Lee ),한영미 ( Young Mi Han ),성세인 ( Se In Sung ),유혜수 ( Hye Soo Yoo ),안소윤 ( So Yoon Ahn ),김은선 ( Eun Sun Kim ),장윤실 ( Yoon Sil Chang ),박원순 ( Won S 대한주산의학회 2012 Perinatology Vol.23 No.3
Pleural effusion and diaphragmatic palsy secondary to fluid extravasation after central line insertion in the neonate are rare complications. Here we report a case of right pleural effusion and diaphragmatic palsy caused by fluid extravasation associated with peripherally inserted central venous catheter in the preterm infant.
Effect of Enzyme Replacement Therapy on Growth in Korean Patients with Mucopolysaccharidosis Type I
허림,조성윤,장미선,이지은,권영희,김수진,손영배,박성원,맹세현,권은경,한선주,정주연,진동규,Huh, Rimm,Cho, Sung Yoon,Chang, Mi Sun,Lee, Jieun,Kwun, Younghee,Kim, Su Jin,Sohn, Young Bae,Park, Sung Won,Maeng, Se Hyun,Kwon, Eun-Kyung,Han, Sun J The Korean Society of Inherited Metabolic Disease 2013 대한유전성대사질환학회지 Vol.13 No.2
목적: I형 뮤코다당증 (MPS I)은 ${\alpha}$-L-iduronidase 효소의 결핍으로 인하여 발생하는 리소좀 축적 질환으로, 광범위한 양상으로 다기관에 영향을 미친다. 저신장과 성장 속도의 감소는 MPS I의 중요한 특징이다. 본 연구에서는 효소 보충 요법이 MPS I 환자들의 성장에 미치는 효과에 대해 알아보기 위하여 단일 기관의 환자들을 대상으로 분석하였다. 방법: 2세에서 15세 사이에 효소 보충 요법을 시작하여 최소 3년 이상의 치료를 시행 받은 10명의 한국 MPS I 환자들의 키 측정치를 후향적으로 분석하였다. 효소 보충 요법 시작시의 평균 나이는 7년 7개월 이였으며, 남아는 6명, 여아는 4명 이였다. 키는 표준 편차(SDS)로 표현되었다. 효소 보충 요법 전과 후의 연간 성장 속도를 계산하였으며, 구분회귀모델을 이용하여 치료 전과 후의 키 z-score를 분석하였다. 표현형[(중증(Hurler) versus 경증(Hurler-Scheie, Scheie)]이 성장에 미치는 영향에 대해서는 개별 분석을 시행하였다. 결과: 효소 보충 요법 전 1년 동안의 연간 성장은 3.3 cm (z-score=-0.21) 였으며, 효소 보충 요법 후 1년, 2년, 3년에서는 각각 6.2 cm (z-score=0.17), 5.8 cm (z-score=0.07), 3.8 cm (z-score=-0.4)이였다. 회귀분석 결과, 효소 보충 요법 전에 비하여 치료 후 기울기에 유의한 호전을 보였다(기울기 차이=0.04; P=0.022). 중증과 경증 표현형 간의 치료 전(P=0.001)과 후(P<0.0001)의 기울기 차이는 통계적으로 유의하였으나, 표현형에 따라 분석하였을 때 통계적으로 유의한 차이는 보이지 않았다. 결론: MPS I 환자들의 키 성장에 있어 aldurazyme 효소 보충 요법이 긍정적인 효과를 미치는 것으로 보인다. Purpose: Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder caused by deficiency of the enzyme ${\alpha}$-L-iduronidase, which leads to a broad spectrum of multisystemic manifestations. Short stature and decreased growth velocity are prominent features of MPS I. The aim of the present study was to evaluate the effect of enzyme replacement therapy (ERT) on growth of Korean MPS I patients from a single center. Methods: Height data were obtained by retrospective chart review of 10 Korean patients with MPS I who had received ERT for a minimum of 3 years. Height was expressed as standard deviation scores (SDS) based on normative data. Annual growth rates were calculated before and during ERT. A piecewise regression model was used to analyze height z-scores before and after treatment. Individual analysis was performed for impact of phenotype [(severe (Hurler) versus attenuated (Hurler-Scheie, Scheie)] on growth. Results: Annual growth was 3.3 cm (z-score= -0.21) in the year before ERT and 6.2 cm (z-score= 0.17), 5.8 cm (z-score= 0.07), and 3.8 cm (z-score= -0.4) in the first, second, and third years of ERT, respectively. Regression analysis showed improvement in the slope after ERT (difference= 0.04; P=0.022). Estimated slope differences between severe and attenuated phenotypes were statistically significant before (P=0.001) and after treatment (P<0.0001), although no significant difference was noted when stratified by phenotype. Conclusion: ERT with aldurazyme appears to have a positive impact on linear growth in patients with MPS I.
김지연 ( Ji Yeon Kim ),장미선 ( Mi Sun Jang ),고바라다 ( Ba Ra Da Koh ),지태경 ( Tea Kyung Ji ),성창민 ( Chang Min Sung ),박다해 ( Da Hae Park ),김현중 ( Hyun Joong Kim ),김은선 ( Eun Sun Kim ),김용환 ( Yong Hwan Kim ) 한국동물위생학회 2013 韓國家畜衛生學會誌 Vol.36 No.3
A total of 301 samples of bovine liver, spleen and omasum were collected from butchers and restaurants in Gwangju, Korea during 2012 and all samples were subjected to bacteriological examination and antibiotic residues. Also, this study was performed to survey the consciousness for hygiene of livestock workers who are handling bovine by-products in Gwangju. The detection rate of aerobic plate count (APC) was higher in summer than in other seasons in all by-products (P=0.000). The detection rate of E. coli count was lower in the liver than the spleen and omasum (P=0.000). Twenty four of the samples (8.0%) were contaminated with S. aureus while one spleen sample (0.3%) was contaminated with L. monocytogenes and finally 10 (3.3%) of the liver and omasum samples were contaminated with Cl. perfringens. Five of the twenty-four S. aureus isolates harbored enterotoxin gene. However, the cpe gene of Cl. perfingens was not detected among any of the 10 isolates. Antibiotic residues were not detected in the liver samples. The consciousness survey`s results showed that most of them (58.8%) were safe.
한국인 집단에서 알츠하이머 치매의 임상적 진단을 위한 ApoE 유전자와 BchE-k 변이형과의 상승관계에 관한 연구
신은심 ( Eun Sim Shin ),정호진 ( Ho Jin Choung ),장미선 ( Mi Sun Chang ),윤송로 ( Song Ro Yoon ),임용빈 ( Yong Bin Eym ),이기철 ( Gi Chul Lee ),이강희 ( Kang Hee Lee ),김재종 ( Jae Jong Kim ),최수경 ( Soo Kyung Choi ) 대한임상검사과학회 1999 대한임상검사과학회지(KJCLS) Vol.31 No.2
The Apolipoprotein E type 4 allele (ApoE e4) is genetically associated with the common late onset familial and sporadic forms of Alzheimer``s disease. ApoE gene is located on chromosome 19, and the three common alleles are designated e 2, e 3, and e 4. The e 4 gene is a major risk factor for late onset Alzheimer``s disease(LOAD). It has been reported that the polymorphic k variant of butyrylcholinesterase (BchE-k) has an elevated frequency in AD patients carrying the e 4 allele of ApoE when compared with a nonnal hea1thy group. BchE activiη in the brain increases around age 60 and is elevated in AD. The BchE-k variant, which is the common variant of the BchE gene, has been reported to show allelic association with AD in subjects who are also carriers of the e 4 allele of ApoE, especilly in subjects over the age of 75. This study was performed to evaluate the distribution of ApoE and BchE genotype in healthy and AD group and to evaluate the synergy between BchE-k variant and ApoE з 4 in AD. ApoE and BchE genotype were determined in DNA samples from 610 healthy group and 60 LOAD patients by using ARMS a11ele specific oligonucleotides (ASOs) amplification by standard agarose gel electrophoresis. πle efIect of ApoE E з4 was significant1y associated with AD(p<0.05). A comparison between AD patients and the hea1thy individua1s, both with the E 4 a11ele, indicated an interaction between BchE-k and ApoE E з4(p<0.05). The association of BchE-k with AD was limited to carriers of ApoE E 4 a11ele, among whom the presence of BchE-k gave an odds ratio of AD of 3.48 (95% C.I. 1.3-9.2). Together these resu1ts suggested that further evidence of an association between ApoE E 4 and LOAD, and BchE-k acts in synergy with ApoE E з4 as a susceptibility gene for AD.
광주지역 소 생고기 선호도 및 유통단계별 세균학적 분석
나호명 ( Ho Myung Na ),배성열 ( Seong Yeol Bae ),고바라다 ( Ba Ra Da Koh ),장미선 ( Mi Sun Jang ),성창민 ( Chang Min Sung ),김지연 ( Ji Yeon Kim ),박헌규 ( Heon Gyu Park ),문용운 ( Yong Un Mun ),김용환 ( Yong Hwan Kim ) 한국동물위생학회 2012 韓國家畜衛生學會誌 Vol.35 No.4
Consumer`s preference and microbial inspections on fresh raw beef were carried out to understand the actual market status in Gwanju, Korea. Over 15 questions on questionnaire by 1,111 randomly selected respondents between April and May in 2011, results showed 65.5% positive on eating fresh raw beef, 63.8% negative on good hygiene condition of fresh raw beef, and 72.5% positive on the secure of the hygiene-safety for priority program, respectively. For microbial inspections, a total of 302 samples were collected from fresh raw beef purchased from slaughterhouse (n=122), transport (n=69) and consumer (n=81) stage, from lettuce (n=30) at consumer stage. The aerobic plate count (APC), E. coli count and food borne bacteria such as Salmonella spp., Listeria monocytogenes, Staphylococ-cus( S.) aureus and E. coli O157:H7 were tested in the samples. As results, the level of count on APC of fresh raw beef ranged 6×10(1)∼1.8×10(5) CFU/g from slaughterhouse, 2×10(2)∼8.3×10(5) CFU/g from transport stage and 1×10(2)∼4×10(5) CFU/g from consumer stage. The level of count on E. coli of fresh raw beef ranged 1∼9×10(1) CFU/g from slaughterhouse, 1∼7×10 CFU/g from transport stage and 1∼5.5×10 CFU/g from consumer stage. In total, 26 S. aureus were isolated, 10 (14.5%) from fresh raw beef at transport stage, 12 (14.8%) from fresh raw beef and 4 (13.3%) from lettuce at consumer stage. Enterotoxin of S. aureus was not detected among 26 isolates. All S. aureus isolates were typed using a DiversiLab(TM) rep-PCR system for genetic similarity test, showing over 95% of genetic relationship amon isolates.