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      • KCI등재후보

        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.

      • KCI등재후보

        항인지질증후군의 임상성

        이진학(Jin Hak Lee),김노경(Noe Kyeong Kim),김병국(Byoung Kook Kim),양성현(Sung Hyun Yang),신현춘(Hyun Choon Shin),계경채(Kyung Chae Kye),박선양(Seon Yang Park) 대한내과학회 1994 대한내과학회지 Vol.47 No.3

        N/A Background: Antiphospholipid syndrome is characterized by arterial or venous thrombosis, recurrent fetal loss, thrombocytopenia, and presence of antiphospholipid antibody. This study was done to know the clinical and serological characteristics of antiphospholipid syndrome in Korea and to evaluate the therapeutic efficacy of prednisolone and antithrombotic agents in antiphospholipid syndrome. Methods: Clinical and laboratory features of 11 patients with antiphospholipid antibodies and histories of spontaneous abortion and thrombosis were studied. Results: Of the 11 patients, 3 were male, and 8 were female, and the ages ranged from 19 to 54 years (median, 30). 3 patients had systemic lupus erythematosus, and the other 8 fulfilled criteia for the primary antiphospholipid syndrome. Initial presentations were deep vein thrombosis in 4 patients, spontaneous abortion in 4, pulmonary embolism in 1, splenic infarct in 1, and stroke in 1. Seven of 11 patents (63.6%) had episodes of venous thrombosis, and in 2 these were accompanied by pulmonary embolism. Arterial occlusions were present in 2 patients. These comprised splenic infarct and right middle cerebral arterial occlusion. Splenic infarct was accompanied by portal vein, splenic vein, and superior mesenteric vein thrombosis. Spontaneous abortion were present in 6 patients with wide range of episodes of fetal loss (range 1-6, median 2.5). Six episodes of spontaneous abortion occurred during the second trimester and 6 during the third. Combined diseases were idiopathic thrombocytopenic purpura, liver chirrosis, iron deficiency anemia, hypertension, and mitral regurgitation. Six patients had both the lupus anticoagulant and anticardiolipin antibodies; 2 had anticardiolipin antibodies alone; and 3 had only the lupus anitcoagulant. Antinuclear antibodies (>1:40) were present in 8 of the 11 patients and anti-ds-DNA (>25 units) in 3. False positive VDRLs were noted in 3 patients, and thrombocytopenia (<150,000/mm) in 6. Two of three pregnancy ended with normal live birth after adminstration of prednisolone and aspirin, and all thrombotic episodes were resolved with prednisolone plus heparine/warfarin or prednisolone plus aspirin or heparine/warfarin alone. Conclusions: The clinical and serological features of the antiphospholipid syndrome in korea were similar to those of previous reports. Prednisolone and antithrombotic agents were effective in these patients, especially in those who had been suffering from spontaneous abortions.

      • KCI등재후보

        한국인에 희귀한 β-Thalassemia Minor 가족 2 예

        조한익,이진학,김성록,김노경,김인후,김병국,박선양,양성현,신현춘 대한내과학회 1994 대한내과학회지 Vol.46 No.5

        β-Thalassemia is a hereditary hemolytic anemia characterized by decreased or absent synthesis of β globin chain. β-Thalassemia minor had been never reported in Korea until 1988 when the first case was reported. This syndrome should be differentiated from iron deficiency anemia since unnecessary clinical work up and treatment might be given. We experienced two familial cases of β-thalassemia minor: 47-year-old female referred to Seoul National University Hospital because of atypical hypochromic microcytic anemia and 31-year-old male with 7-year history of anemia and jaundice.

      • KCI등재후보

        급성 골수성 백혈병이 발생한 가족 1 예

        조한익,이진학,엄태현,안효섭,김병국,박선양,김노경,이혁표,신현춘,오경식,류명수 대한내과학회 1993 대한내과학회지 Vol.45 No.3

        Backgound: The genetic factors involved in the etiology of human leukmia have been discussed including the occurence of leukemia in twins, leukmia in relatives of leukemic patients, the relation of genotypes and phenotypes to leukemia, and the complication of leukemia in various congenital diseases associated with chromosomal abnormalties and/or immunodeficiencies, but there are many problems that remain to be clarified. Case: We experienced 2 cases of acute myelogenous leukemia within a family. Young brother and elderly sister whose father had been died of acute leukemia was diagnosed as AML (M4) and AML (M1), respectively. The sister aged 19 presented with dizziness and dyspnea was given Ara-c and Daunorubicin chemotheraphy but expired without response. Young brother aged 17 presented with fever and epistaxis was responded well to the same regimen chemotheraphy, in remission. But his leukemia was relapsed despite of following 2 cycles of consolidation chemotherapy, and died of 7 months after treatment. Known leukemogenic factor such as, radiation, drug, chemical exposure was excluded in these patient. Conclusion: Although about 130 cases of familial leukemia has been described to date in other countries, no case has been reported in Korea. So we report this case with brief review of the literiture.

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