• Journal of Chest Surgery
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• v.44 no.6
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• pp.423-426
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• 2011

We report on two women who underwent myocardial revascularization associated with antiphospholipid syndrome (APS) with different pathogenic patterns. The first woman presented with acute myocardial infarction, and preoperative angiograms demonstrated rapidly progressing coronary lesions, presumptive unstable plaque, and dissection. Operative findings, however, showed fresh thrombi in the coronary arteries, and she was diagnosed postoperatively as having APS. Her one-year angiogram demonstrated improved coronary lesions and a competitive flow pattern in the grafts. The second woman presented with unstable angina and had been treated for systemic lupus erythematosus and secondary APS for more than 14 years. She underwent myocardial revascularization due to accelerated coronary atherosclerosis. Her one-year angiogram demonstrated patent grafts.

• Clinical and Experimental Reproductive Medicine
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• v.26 no.2
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• pp.271-274
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• 1999

Activated factor XII (FXIIc: Hageman factor) is a central component of the contact activation system of blood coagulation, fibrinolysis, and kinin formation. Although patients deficient in FXIIc (up to 50% of normal) do not show increased bleeding tendency, thrombotic complications were reported in 8% to 10% among these patients. The reduced generation of bradykinin resulting in diminished release of tissue plasminogen activator is proposed as a cause of thrombosis in factor XII (FXII)-deficient patients. Similarly, in patients with elevated levels of circulating antiphospholipid antibodies, hemostasis may be impaired resulting in excessive thrombophilia. Both vascular and placental thromboses because of antiphospholipid antibodies or FXIIc deficiency have been reported to be associated with recurrent fetal loss. We have experienced a case of factor XII deficiency in woman with recurrent spontaneous abortion. So we report this case with a brief review of literatures.

• Journal of Yeungnam Medical Science
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• v.23 no.1
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• pp.113-117
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• 2006

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that may affect many organ systems including the nervous system. The immune response in patients with SLE can cause inflammation and other damage that can cause significant injury to the arteries and tissues. A 48-year-old woman was admitted to the hospital because of transient monocular blindness. Magnetic resonance imaging and conventional angiography showed severe stenosis of the distal intracranial internal carotid artery. The patient was diagnosed as having SLE but the antiphospholipid antibodies were negative. Amaurosis fugax has not been previously reported as an initial manifestation of SLE in Korea. We report a patient with a retinal transient ischemic attack as the first manifestation of SLE.

• The Journal of the Korean life insurance medical association
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• v.31 no.1
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• pp.19-28
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• 2012

Nowadays, Rheumatic diseases are increasing more and more. So, it's important knowing the pathophysiology and extra-risk of each rheumatic disease so as to do sound underwriting. Here is brief review and long-term prognosis of some rheumatic diseases such as rheumatoid arthritis, systemic lupus erythematosus, Sj$\ddot{o}$gren syndrome, antiphospholipid syndrome, systemic sclerosis, ankylosing spondylitis, Takayasu's arteritis, and Behcet syndrome.

• Clinical and Experimental Reproductive Medicine
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• v.27 no.1
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• pp.83-90
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• 2000

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.146-152
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• 2009

Purpose : We performed this study to evaluate the incidence and clinical significance of antiphospholipid antibodies (aPL Ab) in Korean children with Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP). Methods : The medical records of 62 patients (31 boys and 31 girls) aged $46.0{\pm}3.1$ (1-16) years with a clinical diagnosis of HSP based on the EULAR/PReS criteria were reviewed retrospectively. From the years 2007 to 2009, the sera from children with acute HSP were tested for aPL Ab such as LA, anti-cardiolipin antibody and anti-${\beta}_2$ glycoprotein I antibody. Results : LA was positive in 18 (29%) of the 62 patients with HSP and We divided the patients into the two groups LA positive group (N=18) and LA negative group (N=44). There were no significant differences between the two groups with regard to abdominal pain, arthralgia and renal involvement, but LA positive group had significantly higher C-reactive protein ($4.3{\pm}7.2$ mg/dL vs. $1.3{\pm}1.8$ mg/dL, P=0.035), erythrocyte sedimentation rate ($37.5{\pm}26.2$ mm/hr vs. $25.1{\pm}22.6$ mm/hr, P= 0.039), IgM ($148.1{\pm}48.4$ mg/dL vs. $114.9{\pm}41.5$ mg/dL, P=0.024), C3 ($143.1{\pm}21.9$ mg/dL vs. $129.7{\pm}24.5$ mg/dL, P=0.048) and C4 levels ($30.9{\pm}6.3$ mg/dL vs. $24.9{\pm}7.8$ mg/dL, P=0.002) compared with LA negative group. Conclusion : We found that the incidence of positive aPL Ab tests was relatively higher in Korean children with HSP and the presence of aPL Ab was associated with acute inflammatory process of HSP. These results suggest that the aPL Ab are involved in the pathogenesis of HSP in children.

• IMMUNE NETWORK
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• v.2 no.4
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• pp.227-232
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• 2002

Background: Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by diverse clinical manifestations and autoantibody production, which is known to be strongly influenced by genetic factors. Previous studies have revealed the associations of SLE with HLA class II alleles and antiphospholipid antibody system (anticardiolipin antibody (aCL) and anti-${\beta}_2$ glycoprotein I antibody (anti-${\beta}_2$ GPI)). Therefore, we studied the associations of HLA class II alleles with SLE and antiphospholipid antibody system. Methods: The genotyping for HLA-DRB1 and DQB1 alleles were performed in 61 SLE patients and 100 controls by the polymerase chain reaction (PCR)-sequence specific oligonucleotide probe method. ELISA tests for aCL and anti-${\beta}_2$ GPI were performed in 39 of the 61 SLE patients. The results were evaluated statistically by Chi-square test. Results: The frequencies of the HLA-$DRB1^*15$ and $DQB1^*06$ in SLE patients were significantly higher than those in controls. HLA-$DRB1^*12$ was significantly lower in SLE patients than controls. Nine of 39 patients were positive for aCL (IgG) and three were positive for aCL (IgM). One of 39 patients were positive for anti-${\beta}_2$ GPI (IgG) and none of them positive for anti-${\beta}_2$ GPI (IgM). Association of aCL with HLA class II alleles was not observed in our study. Conclusion: According to our results, it was found that HLA-$DRB1^*15$ and $DQB1^*06$ were associated with genetic susceptiblility and $DRB1^*12$ was associated with resistance to SLE in Korean population. No Association of aCL with HLA class II alleles was observed and the positive rate for anti-${\beta}_2$ GPI was very low.

• Clinical and Experimental Pediatrics
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• v.56 no.12
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• pp.545-549
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• 2013

We report the case of a 17-year-old Korean girl with systemic lupus erythematosus (SLE) who presented with sudden weakness of the right-sided extremities and dysarthria. Oral prednisolone was being taken to control SLE. Results of clinical and laboratory examinations did not show any evidence of antiphospholipid syndrome or thromboembolic disease nor SLE activity. Cerebral angiography showed stenosis of the left internal carotid artery and right anterior cerebral artery with accompanying collateral circulation (moyamoya vessels). After the patient underwent bypass surgery on the left side, she recovered from the neurological problems and did not experience any additional ischemic attack during the 14-month follow-up period. This case represents an unusual association between moyamoya syndrome and inactive SLE (inactive for a relatively long interval of 2 years) in a young girl.

• Childhood Kidney Diseases
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• v.28 no.2
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• pp.80-85
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• 2024

Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) is a rare entity characterized by the presence of lupus anticoagulant (LA) and prothrombin (factor II) deficiency. It may cause severe bleeding contrary to classical antiphospholipid syndrome. Here, we report a case of LAHPS presenting with a hemorrhagic ovarian cyst in a 17-year-old girl with systemic lupus erythematosus (SLE) nephritis. She had been followed up for 8 years. Her first manifestation of SLE was prolonged gingival bleeding after tooth extraction at 9 years of age. During the follow-up period, she had neither severe bleeding nor thrombotic complications despite a positive LA and a prolonged activated partial thromboplastin time (aPTT). At this visit, the patient presented with colicky abdominal pain, a hemorrhagic ovarian cyst, a prolonged prothrombin time, a prolonged aPTT, a low factor II level, and a positive LA, leading to the diagnosis of LAHPS. While a hemorrhagic ovarian cyst resolved completely in 3 months, she received oral pill, transfusions of red blood cells and plasma, and intravenous cyclophosphamide pulse therapy in combination with glucocorticoids due to persistent menorrhagia, anemia, prolonged aPTT, and lupus flaring. Thus, LAHPS needs to be considered in SLE patients with positive LA and prolonged aPTT.

• IMMUNE NETWORK
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• v.4 no.3
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• pp.161-165
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• 2004

Background: Anti-cardiolipin antibody (Anti-CL Ab) is one of the various antiphospholipid antibodies (Anti-PL Abs) and found in the plasma of patients with systemic lupus erythematosus (SLE), atherosclerosis, and other infectious diseases. While anti-PL Abs found in the sera of patients with infectious diseases bind directly to CL, binding of anti-PL Abs to CL circulating in the sera of patients with autoimmune diseases is mediated by $\beta_2-$glycoprotein 1 ($\beta_2-GP1$). The purpose of this study is to investigate the effect of <$\beta_2-GP1$ on the antigen binding assay of anti-CL Abs present in the sera of patients with atherosclerosis, which has been known as one of autoimmune diseases. Methods: ELISA was performed with sera containing anti-CL Abs from three patients with atherosclerosis in the presence or absence of $\beta_2-GP1$ or FBS. Results: Reactivity of anti-CL Abs to CL was increased in the presence of $\beta_2-GP1$ or FBS in a dose dependent manner. Conclusion: <$\beta_2-GP1$ or FBS could be used as co-factor in CL ELISA with anti-CL Abs present in the sera of patients with atherosclerosis. It is suggested that anti-CL Abs found in atherosclerosis patients are similar in terms of antigen binding property to those circulating in the patients with autoimmune diseases, not to infectious diseases.