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        A Case Report on 30-Week Premature Twin Babies with Congenital Myotonic Dystrophy Conceived by In Vitro Fertilization

        Su Bin Son,Jung Mi Chun,Kyung Ah Kim,고선영,Yeon Kyung Lee,Son Moon Shin 대한의학회 2012 Journal of Korean medical science Vol.27 No.10

        Congenital myotonic dystrophy type 1 (DM1) presents severe generalized weakness,hypotonia, and respiratory compromise after delivery with high mortality and poor prognosis. We presented a congenital DM1 of premature twins in the 30th week of gestation. These twins were conceived by in vitro fertilization (IVF). Both babies presented apnea and hypotonia and had characteristic facial appearance. They were diagnosed DM1by genetic method. They were complicated by chylothorax and expired at 100 and 215days of age, respectively. Mother was diagnosed DM1 during the evaluation of babies. This is the first report on congenital DM1 which accompanied the chylothorax. More investigation on the association with chylothorax and congenital DM1 is recommended. With a case of severe neonatal hypotonia, congenital DM1 should be differentiated in any gestational age. Finally, since DM1 is a cause of infertility, we should consider DM1 in infertility clinic with detailed history and physical examination.

      • KCI등재후보

        호르몬 치료를 받은 한국 폐경 여성에서 발생한 유방암의 임상적 특성

        손정빈 ( Jung Bin Son ),정주은 ( Ju Eun Jeong ),주종길 ( Jong Kil Joo ),김기형 ( Ki Hyung Kim ),이규섭 ( Kyu Sup Lee ) 대한폐경학회 2012 대한폐경학회지 Vol.18 No.1

        연구목적: 국내 한 병원에서 호르몬 치료를 받은 여성들에게서 발생한 유방암의 위험인자의 비교 및 유방암의 조직학적, 임상적인 특징과 예후 등에 대한 연구를 시행하였다. 연구재료 및 방법: 부산대학교병원에서 폐경으로 인해 호르몬 치료를 받은 여성 중 악성 유방암이 발생한 환자 40명을 대상으로 후향적으로 의무기록지를 통해 조사하였다. 조사 항목으로는 환자군의 병력 및 기초 조사, 받았던 호르몬 치료의 종류와 기간, 호르몬 치료 시작 후 발병까지의 기간, 방사선학적인 특징, 환자의 병기, 조직학적인 진단, 종양의 크기, 조직의 등급, 임파선의 전이 상태, 그리고 에스트로겐 수용체와 프로게스테론 수용체의 유무, 5년 생존율의 여부 등에 대해 조사하였다. 결과: 유방암이 발생한 환자들의 위험요인 조사 항목으로 가족력을 가지고 있던 사람은 1명, 흡연력이 있는 사람은 없었고, 평균 BMI는 23.2 kg/m2로 나타났다. 유방암이 발생한 환자들의 이전 호르몬 치료 종류는 에스트로겐 단일 요일요법을 받은 여성이 12명으로 30%, 에스트로겐 프로게스테론 혼합 요법을 받은 여성이 13명으로 32.5%, 티볼론 요법을 받은 여성이 10명으로 25%였고 기타 환자들에게는 복합적으로 사용되었다. 호르몬의 치료기간은 평균 31 ± 27.9 (0.4-115)개월이었고 호르몬 치료 시작 후 유방암의 발생시간 분포는 1년 이내가 4건 (10%), 1-2년 사이가 5건 (12.5%), 2-3년 사이가 10건 (25%), 3-4년 사이가 4건 (10%), 4-5년 사이가 1건 (2.5%), 5년 이상 16건 (40%)으로 나타났다. 유방암의 평균 직경은 1.7 cm으로 작은 양상을 보이고, 조직학적으로 관암종 형태의 유형이 92.5%로 다수였으며, 병기는 0기와 I기가 66%로 나타났고, 등급으로는 1등급이 38%로 가장 높은 빈도를 보였으며, 호르몬 수용체가 양성인 유방암이 85%로 조사되었고, 임파절 전이는 70%에서 음성, 5년 생존률은 92%로 나타났다. 결론: 호르몬 치료를 받은 폐경기 여성에게서 발생한 유방암은 호르몬 수용체가 양성인 경우가 많으며 크기나 병기가 낮고 저등급의 분화도를 보여 재발이 흔하지 않고 5년 생존률에서 좋은 예후를 보이는 양상을 보였다. Objectives: This study was performed to assess the risk factors, histologic and clinical features of breast cancer in postmenopausal women receiving hormone therapy (HT). Methods: We evaluated 40 breast cancer patients who received HT due to postmenopausal symptoms by reviewing their medical charts at Pusan National University Hospital. Research variables, including patients` history, type and duration of received HT, moment of cancer debut after starting HT, radiological characteristics of breast cancer stage, histologic type, tumor size, grade, lymph node metastasis, estrogen and progesterone receptor status and 5-year survival were investigated. Results: In the risk factors of breast cancer patients, only one patient had familial history of breast cancer. No patient had smoking history. The average body mass index (BMI) was 23.2 kg/m2. Twelve patients (30%) had estrogen only therapy, 13 patients (32.5%) had combined estrogen and progesterone therapy, 10 patients (25%) had tibolone therapy and the others consecutively received combination therapy of the above regimens. The mean duration of treatment was 31 ± 27.9 months (range 0.4-115 months). In the distribution of the cancer debut after starting HT, in 4 cases (10%) was within 1 year, 5 cases (12.5%) within 1-2 years, 10 cases (25%) within 2-3 years, 4 cases (10%) within 3-4 years, 1 case (2.5%) within 4-5 years, and 16 cases (40%) within more than 5 years. The average diameter of tumor size was 1.7 cm. In 92.5% of cases, the tumor was of ductal type. Tumor stage 0 and 1 appeared in 66% and grade I was present in 38% of investigated cases. Hormone receptor-positive breast cancers were 85% and 70% of patients had negative lymph node metastases. The 5-year survival rate was 92%. Conclusion: The breast cancers which emerged during HT in postmenopausal women had hormone receptor-positive tendency. The size and stage of these breast cancers were shown as small and low, and represented low-grade differentiation. Recurrences of disease were uncommon and we found favorable 5-year survival rates and good prognosis.

      • KCI등재

        주박과 누룩의 추출물에 의한 지방세포형성억제, 항염증 및 항성장 활성

        손정빈(Jung-Bin Son),이승훈(Seung Hoon Lee),손호용(Ho-Yong Sohn),신우창(Woo-Chang Shin),김종식(Jong-Sik Kim) 한국생명과학회 2015 생명과학회지 Vol.25 No.7

        본 연구에서는 5종의 주박과 누룩의 추출물 및 유기용매 분획물을 제조하고, 이들에 의한 지방세포형성억제, 항염증 및 항성장 활성을 연구하였다. 지방세포형성억제 활성 연구를 위하여 마우스 전지방 세포인 3T3-L1 세포주에 지방세포형성을 유도한 후 추출물 및 분획물 5종을 처리하였다. 처리한 시료 중 W-Ju의 에틸아세테이트 분획물(WPAc)이 가장 뚜렷한 지방세포형성 억제 활성을 보여 주었다. 이것은 oil red O 염색과 pro-adipogenic 유전자의 발현 감소에 의해 증명되었다. 또한, WPAc의 처리는 시간 의존적으로 PPAR-gamma 유전자의 발현을 감소시켰다. 항염증 연구를 위하여 RAW 264.7 세포주에서 5종의 시료에 의한 nitric oxide (NO) 생산에 미치는 영향을 연구하였다. 5종의 시료 중 B-Ju의 에틸아세테이트 분획물(PAc)의 처리에 의해 LPS로 활성화된 RAW 264.7 세포에서 NO 생산이 가장 높게 저해되었고, 또한 농도 의존적인 저해 양상을 보여 주었다. 게다가, PAc는 인간 대장암 세포주인 HCT116의 세포 생존율을 현저하게 감소시켰으며, 또한 농도의존적인 생존율 저해 양상을 보여 주었다. 또한, PAc는 NAG-1과 ATF3 유전자의 발현도 농도 의존적으로 감소 시켰다. 종합적으로, 이러한 연구결과는 주박과 누룩이 지방세포형성억제 활성, 항염증 활성 그리고 항성장 활성 등 다양한 생리활성을 가지고 있음을 시사한다. This study examined extracts from five different kinds of lees and nuruk and their organic solvent fractions in terms of several biological functions, such as anti-adipogenic, anti-inflammatory, and anti-proliferative activities. The anti-adipogenic activity was investigated by treating mouse pre-adipocyte 3T3-L1 cells with one extract (YE) and four organic solvent fractions (YAc, PAc, RAc, and WPAc) during adipogenesis. Among the treated samples, the ethyl acetate fraction of W-Ju lees (WPAc) showed the strongest anti-adipogenic effect, which was confirmed with oil red O staining and down-regulation of pro-adipogenic genes such as PPAR-gamma and SCD-1. Treatment with WPAc also reduced the expression of PPAR-gamma in a time-dependent manner. The effects of five different extracts were examined on nitric oxide (NO) production in mouse RAW 264.7 cells to determine anti-inflammatory activity. The ethyl acetate fraction of B-Ju lees (PAc) significantly decreased NO production in LPS-stimulated RAW 264.7 cells and it also inhibited NO production in a dose-dependent manner. The PAc fraction also dramatically decreased the viability of human colorectal cancer HCT116 cells in a dose-dependent manner. In addition, PAc increased the expression of NAG-1 and ATF3 genes in a dose dependent manner. Overall, these results indicate that lees and nuruk have several biological functions, including anti-adipogenic, anti-inflammatory, and anti-proliferative activities.

      • KCI등재
      • SCISCIESCOPUS

        Lipase and Protease Double-Deletion Mutant of Pseudomonas fluorescens Suitable for Extracellular Protein Production

        Son, Myunghan,Moon, Yuseok,Oh, Mi Jin,Han, Sang Bin,Park, Ki Hyun,Kim, Jung-Gon,Ahn, Jung Hoon American Society for Microbiology 2012 Applied and environmental microbiology Vol.78 No.23

        <B>ABSTRACT</B><P>Pseudomonas fluorescens, a widespread Gram-negative bacterium, is an ideal protein manufacturing factory (PMF) because of its safety, robust growth, and high protein production.P. fluorescenspossesses a type I secretion system (T1SS), which mediates secretion of a thermostable lipase (TliA) and a protease (PrtA) through its ATP-binding cassette (ABC) transporter. Recombinant proteins inP. fluorescensare attached to the C-terminal signal region of TliA for transport as fusion proteins to the extracellular medium. However, intrinsic TliA from theP. fluorescensgenome interferes with detection of the recombinant protein and the secreted recombinant protein is hydrolyzed, due to intrinsic PrtA, resulting in decreased efficiency of the PMF. In this research, the lipase and protease genes ofP. fluorescensSIK W1 were deleted using the targeted gene knockout method. Deletion mutantP. fluorescensΔ<I>tliA</I>Δ<I>prtA</I>secreted fusion proteins without TliA or protein degradation. Using wild-typeP. fluorescensas an expression host, degradation of the recombinant protein varied depending on the type of culture media and aeration; however, degradation did not occur with theP. fluorescensΔ<I>tliA</I>Δ<I>prtA</I>double mutant irrespective of growth conditions. By homologous expression of<I>tliA</I>and the ABC transporter in a plasmid, TliA secreted fromP. fluorescensΔ<I>prtA</I>andP. fluorescensΔ<I>tliA</I>Δ<I>prtA</I>cells was found to be intact, whereas that secreted from the wild-typeP. fluorescensandP. fluorescensΔ<I>tliA</I>cells was found to be hydrolyzed. Our results demonstrate that theP. fluorescensΔ<I>tliA</I>Δ<I>prtA</I>deletion mutant is a promising T1SS-mediated PMF that enhances production and detection of recombinant proteins in extracellular media.</P>

      • SCOPUSSCIEKCI등재

        Treatment of Scalp Arteriovenous Malformation

        Jung, Sung-Hoon,Yim, Man-Bin,Lee, Chang-Young,Song, Dal-Won,Kim, Il-Man,Son, Eun-Ik The Korean Neurosurgical Society 2005 Journal of Korean neurosurgical society Vol.38 No.4

        Objective : The object of this study is to present the treatment experience of the 6cases of scalp arteriovenous malformations[AVMs] focus on treatment strategy. Methods : Six patients with scalp AVM were treated during past 12years. We analysis the clinical characteristics of the lesions, treatment methods and management outcomes. Results : The lesions were located on temporal in 2patients, parietal in 2patients, frontal and occipital area in each one. Four of six patients had a trauma history on scalp. The presenting symptoms were progressive enlarged pulsating mass with or without bruit. Four of the six lesions had the large fistula in the lesion. Two patients were treated with surgical resection alone, three patients with proximal feeding artery balloon[s] occlusion followed by surgical resection, and one patient with coil embolization through trans-venous route alone. We obtained good results in all patients. Conclusion : Most of scalp AVM can be completely cured by Judicious selection and a combination of treatment modalities, i.e., surgery only, or embolization only, or embolization plus surgical therapy. Although embolization became a primary therapy for this sort of scalp AVM recently, the selection of treatment modality should be chose based on the size, angioarchitecture, and clinical presentations of the lesion.

      • KCI등재후보

        고혈압성 뇌실질내혈종의 뇌정위적제거술에 대한 결과분석 : Analysis of Factors Affecting Outcome

        손은익,임만빈,이정교,김인흥,김동원 啓明大學校 醫科大學 1990 계명의대학술지 Vol.9 No.2

        The ultimate purpose of treatment in hypertensive intracerebral hematoma(HICH) is effective evacuation of the hematoma with minimal damage to normal brain tissue and prevention of increasing intracranial pressure and progression of an ICH into neighbouring tissue. According to this viewpoint, stereotactic surgery in patients of HICH is recent trend for its safety and precision. An analysis of 50 consecutive patients from Jun. 1988 to Mar. 1989 undergoing stereotactic surgery with urkinase irrigation in HICH was performed. We excluded the HICH below 15cc of volume, poor clinical grade as coma with herniation sign, intraventricular hemorrhages only, infratentorial hematoma and atypical spontaneous intracerebral hematomas as angiographically verified aneurysms, AVM and Moyamoya diseases. Procedures were accomplished utilizing the CT-guided BRW stereotactic frame, then urokinase irrigation was done every 6 hours through a stereotactically inserted catheter until a sufficient volume was drained, and then the patient was checked by a follow-up CT scan. The results were analysed as follows: The incidence was highest in 6th decade with male predominence(62%). Mean dose of urokinase was 6000 IU ×18.73(4.6 days). The statistical analysis of the factors affecting outcome showed that initial neurological grading was highly significant (P<0.001); volume of hematoma was significant also (p<0.05). Associated intraventricular hemorrhage especially ventricular tamponade in HICH was highly poor prognostic factor. Overall good result was 54% and mortality rate was 12% All computations were performed by means of the commonly used SPSS statistical package.

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