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      • SCOPUSKCI등재

        71세 여성에게 진단한 Liddle 증후군

        백운희 ( Baeg Un Hui ),김영욱 ( Kim Yeong Ug ),홍순창 ( Hong Sun Chang ),박상준 ( Park Sang Jun ),김윤권 ( Kim Yun Gwon ),김소연 ( Kim So Yeon ),김영중 ( Kim Yeong Jung ),조민구 ( Jo Min Gu ),이권전 ( Lee Gwon Jeon ) 대한신장학회 2003 Kidney Research and Clinical Practice Vol.22 No.4

        Liddle`s syndrome is a rare inferited disease with characteristic clinical manifestations of hytertension an hypokalemic metabolic alkalosis. Markedly suppressed serum aldosterone and renin levels are important laboratory findings to differentiate this disorder from primary hyperaldosterionism. When Liddle et al. reported the disorder in 1963, they proposed aggressive Na+ absorption and increased excretion of K+ as the pathogenesis of the syndrome. Since then, specific mutaion in the epithelial Na+ channel located in the collecting duct of the kidney has been dlucidated as a disease mechanism. Liddle`s syndrome is inherited by an autosomal dominant trait and generally the onset of the syndrome is before the age of 20 with increased risk of premature death due to stroke or heart failure. Recently, however, a few cases of late onset and genetically proven non-familial cases with de novo mutation of β or γ Na+ channel have been reported. We report a case of seventy-one year old woman who had hypertension with hypokalemic metabolic alkalosis and was diagnosed as Liddle`s syndrome. Further evaluation revealed low renin and aldosterone levels. Primary aldosteronism, Cushing`s syndrome, glucocorticoid remediable aldosteronism and deficiency of 11 β-OHase and 17α-OHase were ruled out based on her laboratory data and history. Her hypertension and hypokalemia responded to amiloride treatment but not to spironolactone. (Korean J Nephrol 2003;22(4):464-468)

      • KCI등재후보

        각종 간질환에서 혈청 Antithrombin III 활성도 측정의 임상적 의의

        신요식,백운희,임수진,전규락,김영욱,김준형,박상준,김윤권,김소연,김영중,조민구,이권전 대한내과학회 2002 대한내과학회지 Vol.63 No.4

        Background : Antithrombin III (AT-III) produced from hepatocytes and endothelial cells is a coagulation inhibitor. The authors investigated the activity levels of AT-III in patients with liver disease and attempt to elucidate the clinical significance of activity levels of AT-III in relation to various liver disease. Methods : This study includes 158 patients with liver disease, who visited the National Police Hospital between October 1997 and March 2002. We performed laboratory tests such as LFT, AFP and either abdominal sonography or abdominal CT. At the same time, AT-III activity levels was measured by chromogenic method using ACL 3000 (IL, Lexington, USA). AT-III activity level of 70~120% was regarded as normal. Results : AT-III activity level of liver cirrhosis patients was decreased along with severity of the disease evaluated by Child-Pugh Classification. AT-III activity level of liver cirrhosis patients and hepatocellular carcinoma patients with liver cirrhosis, whose serum AFP were within normal limits, were 50.11±2.86% and 75.58±6.61%, respectively. The difference between the two groups was statistically significant (p<0.001). Conclusion : Considering the results of the decrease of AT-III activity level in liver cirrhosis patients and the increase in hepatocellular carcinoma patients with liver cirrhosis, further evaluation for the possibility of hepatocellular carcinoma in liver cirrhosis patients without decrease of AT-III level or increase of AFP, may be in need.(Korean J Med 63:379-385, 2002) 서론 : Antithrombin III (AT-III)는 간세포 및 혈관 내피 세포에서 생성되는 항응고물질로 각종 간질환에 따라 다양한 변화를 보인다는 외국보고가 있으나 우리나라에서는 각종 간질환에서 AT-III의 측정 및 임상적 의의에 대한 국내문헌을 참조하기가 곤란하다. 이에 저자들은 각종 간질환에서 혈청 AT-III를 측정하고, 혈청 AT-III 활성도 측정이 각종 간 질환에 따른 임상적 의의를 밝히고자 이 연구를 시행하였다.방법 : 본 연구에서는 1997년 10월부터 2002년 3월까지 본원을 방문한 간질환 환자를 대상으로 간기능 검사와 AFP, 복부초음파, 혹은 복부 컴퓨터 단층촬영을 시행하였고, 동시에 ACL 3000(IL, USA)을 이용한 chromogenic법으로 혈장 AT-III 활성도를 측정하였다. 단, AT-III 활성도의 정상 범위는 70∼120%로 정하였다.결과 : 지방간 환자 15예, 급성 B형 간염 환자 5예, 만성 B형 간염 환자 45예에서 AT-III 활성도는 각각 95.70±8.22%, 83.60±14.36%, 84.98±16.39%로 정상 범위 내에 있었다. 간경변증 환자 53예에서 AT-III 활성도는 50.26±18.78%였고, 정상 범위 보다 낮게 측정되었다.간경변증에 병발한 간세포암 환자 36예에서 AT-III 활성도는 72.31±24.18%로 정상 범위내에 있었으나, 간경변증에서의 AT-III 활성도 보다는 증가된 양상을 보였다(p<0.001). 간경변증이 동반되지 않은 간세포암 4예에서 AT-III 활성도는 82.25±20.04%로 정상 범위 내에 있었으나, 간경변증에 병발한 간세포암의 AT-III 활성도 보다는 증가된 양상을 보였다(p=0.013). 만성 B형 간염 환자에서 AT-III 활성도는 혈청 albumin이 감소될수록 의미있게 감소하였다(R=0.4649, p=0.0013). 간경변증 환자에서 AT-III 활성도는 혈청 albumin이 감소될수록 의미있게 감소하였고(R=0.3038, p=0.0270), 혈청 bilirubin이 증가될수록 의미있게 감소하였다(R=-0.3384, p= 0.0132). 간경변증에서 진행된 간세포암 환자에서 AT- III 활성도는 혈청 cholesterol이 증가될수록 의미있게 증가하였다(R=0.6335, p=0.0001). 간경변증에서 Child-Pugh Classification에 따른 AT-III 활성도는 장애가 심할수록 감소되는 양상을 보였다. AFP가 정상범위인 간경변증 환자와 간경변증에 병발한 간세포암 환자에서 AT-III의 활성도는 각각 50.11±2.86%, 75.58±6.61%로 유의한 차이를 보였다(p<0.001).

      • KCI등재

        ABA signal transduction from ABA receptors to ion channels

        임채우,백운희,임소희,이성철 한국유전학회 2012 Genes & Genomics Vol.34 No.4

        The plant hormone abscisic acid (ABA) is involved in regulating a number of major processes such as seed dormancy, seedling development, and biotic and abiotic stress responses. The function and effect of ABA on pathogens are still unclear,but the roles of ABA in seed germination and abiotic stress responses have been well characterized.Abiotic stresses elevate ABA levels and activate ABA signaling; thus, inducing a variety of responses, including the expression of stress-related genes and stomatal closure. The past decade has witnessed many significant advances in our understanding of ABA signal transduction due to application of a combination of approaches including genetics, biochemistry, electrophysiology, and chemical genetics. A number of proteins associated with the ABA signal transduction pathway such as PYR/PYL/RCAR family of START proteins, have been identified. These ABA receptors bind to ABA and positively regulate ABA signaling via inactivation of PP2C phosphatase activity, which inhibits SnRK2-type kinases by direct interaction and dephosphorylation. Additionally, SnRK2-type kinases and PP2Cs interact with one another and with other components of ABA signaling and function as positive and negative ABA regulators,respectively. In this review, we focus on ABA function to abiotic stresses and highlight each component in relation to ABA and its interactions.

      • KCI등재

        비부비동 반정성 유두종의 전산화 단층촬영상과 자기공명영상의 분석

        배창훈,서영중,이석춘,천승민,백운희,정은채,송시연,김용대 영남대학교 의과대학 2005 Yeungnam University Journal of Medicine Vol.22 No.2

        Background: Computed tomography (CT) is commonly used to evaluate the degree of sinus involvement in case of inverted papilloma (IP). However, CT cannot differentiate tumor from adjacent inflammatory mucosa or retained secretions. By contrast, magnetic resonance imaging (MRI) has been reported to be useful in distinguishing IP from paranasal sinusitis. This study investigated whether preoperative assessment with MRI and CT accurately predict the extent of IP. Materials and methods: CT and MRI were retrospectively reviewed in 9 case of IP. Patients were categorized into staged based on CT and MRI findings according to the staging system proposed by Krouse. The involvement of IP in each sinus was also assessed. Results: Differentiation of IP from inflammatory disease may be more successful in routine case where the inflammatory mucosa has low signal intensity on T1-weighted images and very high signal intensity on T2-weighted images. CT imaging could not differentiate tumor from adjacent inflammatory mucosa or retained secretions. Conclusion: Preoperative MRI of IP can predict the location and extent of the tumor involvement in the paranasal sinuses and sometimes predicts malignant changes.

      • KCI등재후보

        각종 간질환에서 혈청 Antithrombin 3 활성도 측정의 임상적 의의

        신요식(Yo Sig Shin),백운희(Won Hee Baek),임수진(Su Jin Im),전규락(Gyu Rak Chon),김영욱(Young Wook Kim),김준형(Jun Hyoung Kim),박상준(Sang Joon Park),김윤권(Yun Kwon Kim),김소연(So Yon Kim),김영중(Young Jung Kim),조민구(Min Koo Cho), 대한내과학회 2002 대한내과학회지 Vol.63 No.4

        서론 : Antithrombin III (AT-III)는 간세포 및 혈관 내피 세포에서 생성되는 항응고물질로 각종 간질환에 따라 다양한 변화를 보인다는 외국보고가 있으나 우리나라에서는 각종 간질환에서 AT-III의 측정 및 임상적 의의에 대한 국내문헌을 참조하기가 곤란하다. 이에 저자들은 각종 간질환에서 혈청 AT-III를 측정하고, 혈청 AT-III 활성도 측정이 각종 간 질환에 따른 임상적 의의를 밝히고자 이 연구를 시행하였다. 방법 : 본 연구에서는 1997년 10월부터 2002년 3월까지 본원을 방문한 간질환 환자를 대상으로 간기능 검사와 AFP, 복부초음파, 혹은 복부 컴퓨터 단층촬영을 시행하였고, 동시에 ACL 3000(IL, USA)을 이용한 chromogenic법으로 혈장 AT-III 활성도를 측정하였다. 단, AT-III 활성도의 정상 범위는 70∼120%로 정하였다. 결과 : 지방간 환자 15예, 급성 B형 간염 환자 5예, 만성 B형 간염 환자 45예에서 AT-III 활성도는 각각 95.70±8.22%, 83.60±14.36%, 84.98±16.39%로 정상 범위 내에 있었다. 간경변증 환자 53예에서 AT-III 활성도는 50.26±18.78%였고, 정상 범위 보다 낮게 측정되었다. 간경변증에 병발한 간세포암 환자 36예에서 AT-III 활성도는 72.31±24.18%로 정상 범위내에 있었으나, 간경변증에서의 AT-III 활성도 보다는 증가된 양상을 보였다(p<0.001). 간경변증이 동반되지 않은 간세포암 4예에서 AT-III 활성도는 82.25±20.04%로 정상 범위 내에 있었으나, 간경변증에 병발한 간세포암의 AT-III 활성도 보다는 증가된 양상을 보였다(p=0.013). 만성 B형 간염 환자에서 AT-III 활성도는 혈청 albumin이 감소될수록 의미있게 감소하였다(R=0.4649, p=0.0013). 간경변증 환자에서 AT-III 활성도는 혈청 albumin이 감소될수록 의미있게 감소하였고(R=0.3038, p=0.0270), 혈청 bilirubin이 증가될수록 의미있게 감소하였다(R=-0.3384, p=0.0132). 간경변증에서 진행된 간세포암 환자에서 AT-III 활성도는 혈청 cholesterol이 증가될수록 의미있게 증가하였다(R=0.6335, p=0.0001). 간경변증에서 Child-Pugh Classification에 따른 AT-III 활성도는 장애가 심할수록 감소되는 양상을 보였다. AFP가 정상범위인 간경변증 환자와 간경변증에 병발한 간세포암 환자에서 AT-III의 활성도는 각각 50.11±2.86%, 75.58±6.61%로 유의한 차이를 보였다(p<0.001). 결론 : AT-III 활성도가 간경변증에서는 저하되고 간경변증에 병발한 간세포암 환자에서는 상승하는 경향을 보여 특히, AT-III 활성도가 감소되지 않은 간경변증 환자에서는 AFP의 상승이 없더라도 간암에 대한 정밀 검사를 해야 할 것으로 보인다. Background : Antithrombin III (AT-III) produced from hepatocytes and endothelial cells is a coagulation inhibitor. The authors investigated the activity levels of AT-III in patients with liver disease and attempt to elucidate the clinical significance of activity levels of AT-III in relation to various liver disease. Methods : This study includes 158 patients with liver disease, who visited the National Police Hospital between October 1997 and March 2002. We performed laboratory tests such as LFT, AFP and either abdominal sonography or abdominal CT. At the same time, AT-III activity levels was measured by chromogenic method using ACL 3000 (IL, Lexington, USA). AT-III activity level of 70∼120% was regarded as normal. Results : AT-III activity level of liver cirrhosis patients was decreased along with severity of the disease evaluated by Child-Pugh Classification. AT-III activity level of liver cirrhosis patients and hepatocellular carcinoma patients with liver cirrhosis, whose serum AFP were within normal limits, were 50.11±2.86% and 75.58±6.61%, respectively. The difference between the two groups was statistically significant (p<0.001). Conclusion : Considering the results of the decrease of AT-III activity level in liver cirrhosis patients and the increase in hepatocellular carcinoma patients with liver cirrhosis, further evaluation for the possibility of hepatocellular carcinoma in liver cirrhosis patients without decrease of AT-III level or increase of AFP, may be in need.(Korean J Med 63:379-385, 2002)

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