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Henoch - Shonein 자반증 환자의 혈중 제 13 혈액응고인자의 변동
신현춘(Hyun Choon Shin),박선양(Seon Yang Park),이진학(Jin Hak Lee),김병국(Byung Kook Kim),김노경(Neo Kyoung Kim),최지영(Jee Young Choi),박상철(Sang Chul Park),손영우(Young Woo Son),이홍복(Hong Bock Lee) 대한내과학회 1994 대한내과학회지 Vol.46 No.1
N/A Objectives: Factor X III is activated by thrombin to have transglutaminase activity that catalyzes the formation of ε-(Υ-glutamyl)lysine cross-links Between fibrin monomers rendering fibrin more elastie and less amenable to resolution by fibrinolytic agents, therefore diminished Factor X III activity leads to serious defect in hemostasis. Henoch-Sh5nlein purpura is different from other primary hemorrhagic diseases in that the disease connot be detected by routine tests in coagulation lab. But several reports have shown that hemorrhagic tendency of Henoch-Sh6nlein purpura is related to decreased level of the Factor X III. Method: Twenty one patients with Henoch-Sh5nlein purpura were collected from october 1988 and, Factor X III activity in the patients were compared to 10normal controls. The patients were diagnosed as Henoch- Shonlein purpura by typical clinical symptoms and giving mormal value in routine coagulation tests, The Factor XUI activity was assayed by isotope method. We also tried to find out the Factor X III activity variation according to clinical symptoms such as purpura and hematuria. Result: 1) The Factor XIII activity in normal controls and Henoch-Shonlein purpura were 3344±1126.7cpm and 2550.8±831.3cpm respectively. The degree of decreased Factor X III activity in patients compared to normal controls was statistically significant (p<0.05). 2) The Factor XIII activity during purpuric period was lower than that of non-purpuric period without statistical significance (p=0.1729). 3) Comparing hematuric period with non-hematuric period, the Factor X III activity during hematuric period was decreased without statistical significance (p=0, 2049). 4) There was more frequent hematuric symptom in purpuric period than non-purpuric period (p<0.05). Conclusion: We confirmed that the Factor XIII activity of patients with Henoch-Shonlein purpura was diminished, and the degree of reduction was correlated to the clinical symptoms such as prupura and hematuria. We consider that Factor X III activity monitoring would be helpful in diagnosis and determination of prognosis of Henoch-Shonlein purpura, furthermore replacement fo Factor X III concentrate could alter the course of the disease.
비골수억제성 조혈모세포 이식술 후 발생한 이식편대 숙주반응
나임일,신현춘,송은기,이근욱,윤탁,김지현,윤성수,이종석,박선양,김병국 대한혈액학회 2006 Blood Research Vol.41 No.2
배경: 비골수억제성 조혈모세포이식술(non-myeoloablative hematopoietic stem cell transplantation, NST)은 전통적인 조혈모세포 이식술에 적합하지 않은 환자에서 최근 많이 시행되는 추세를 보이고 있다. 이식편대 숙주반응은 조혈모세포 이식술 후 발생하는 심각한 합병증 중의 하나이지만, NST시행 후 발생한 이식편대 숙주반응은 임상상 및 예후가 잘 알려져 있지 않다. 본 연구는 NST 후 발생한 이식편대 숙주반응의 임상상 및 예후에 관한 후향적 연구이다.방법: 2000년 10월부터 2004년 10월까지 NST를 시행한 61명에 대해서 급성 및 만성 GVHD의 발생률 및 치료효과와 생존율에 대해서 후향성 조사를 하였다. 발생률 및 생존율은 카플란-마이어 생존 곡선으로 구하였다.결과: 전체 환자의 중앙 생존 기간은 6.5개월이었다. II도 이상의 급성 및 만성 이식편대 숙주반응의 발생률은 각각 33% (18/53)와 78% (29/37)였으며, 치료율은 각각 33% (6/18)와 89% (26/29)였다. 급성 및 만성 이식편대 숙주반응으로 치료를 받은 환자의 생존율은 27%와 89%였다.