The incidence of systemic lupus erythematosus (SLE) is known to be affected by sex hormone. Patients with Klinefelter's syndrome were reported to have abnormal sex hormonal metabolism and their chronic estrogenic stimulation seems to affect the pathogenesis of SLE. Therefore, association of SLE and Klinefelter's syndrome has been considered as a clue of the effect of sex hormone on SLE. We report the first case of Klinefelter's syndrome in a patient with SLE in Korea and discuss the association of SLE with Klinefelter's syndrome.
Estrogens ; Humans ; Incidence ; Klinefelter Syndrome* ; Korea ; Lupus Erythematosus, Systemic* ; Metabolism

OBJECTIVE: The predominant histopathologic lesion in Behcet's disease is vasculitis. Thrombotic complications have been reported in approximately 10-40% of patients with Behcet's disease, but the precise mechanisms are not known. To investigate the coagualtion abnormalities in patients with Behcet's disease, coagulation and fibrinolytic activities were examined. METHODS: Thirty-two patients with Behcet's disease and thirty-two healthy individuals as a control group were included in the study. The presence of thrombosis and risk factors for hypercoagulability, and blood components concerning coagulation and fibrinolytic activites were evaluated. RESULTS: Of thirty-two patients with Behcet's disease, thrombosis was found in four patients(13%). No patient had risk factors for hypercoagulability except one with lymphoma. Levels of white blood cell count(mean+/-SD 8,362+/-2,893 vs 5,934+/-1,755/mm2, p<0.001), erythrocyte sedimentation rate(40.5+/-37.6 vs 3.3+/-2.73mm/hr, p<0.001), C reactive protein(2.26+/-3.99 vs 1.20+/-0.26mg/dl, p=0.008), fibrinogen(387.7+/-128.5 vs 240.6+/-49.5mg/dl, p<0.001) and von Willebrand factor antigen(131.9+/-46.6 vs 105.2+/-1.75%, p=0.008) were significantly higher in patients with Behcet's disease compared with controls. The level of fibrinogen correlated with erythrocyte sedimentation rate(r=0.721, p<0.001) and C reactive protein(r=0.454, p=0.018). High density lipoprotein(HDL) cholesterol(46.6+/-12.7 vs 65.5+/-16.1mg/dl, p<0.001), apolipoprotein A-1(118.8+/-24.7 vs 134.6+/-18.5mg/dl, p=0.018) and antithrombin III(92.8+/-16.7 vs 106.3+/-14.7%, p=0.004) were significantly lower in patients with Behcet's disease. No differences were observed in lipoprotein(a), plasminogen, protein C, and protein S activities. Activated protein C(APC) resistance was not observed in any patients with Behcet's disease. Lupus anticoagulant was positive in four patients(13%), one of whom had deep vein thrombosis. Antiphospholipid antibody was found in one patient(3%), but thrombosis was not found. CONCLUSIONS: Significantly higher level of von Willebrand factor antigen was observed in Behcet's disease, which suggested injury of vascular endothelium. Levels of HDL cholesterol, apolipoprotein A-1 and antithrombin III were decreased in Behcet's disease. APC resistance was not found.
Activated Protein C Resistance ; Antibodies, Antiphospholipid ; Antithrombin III ; Apolipoprotein A-I ; Apolipoproteins ; Blood Sedimentation ; Cholesterol, HDL ; Endothelium, Vascular ; Fibrinogen ; Fibrinolysis ; Humans ; Leukocytes ; Lipoprotein(a) ; Lupus Coagulation Inhibitor ; Lymphoma ; Plasminogen ; Protein C ; Protein S ; Risk Factors ; Thrombophilia ; Thrombosis ; Vasculitis ; Venous Thrombosis ; von Willebrand Factor

This study was designed for the investigation of the effectiveness and limitation of the endovascular coil embolization of intracranial aneurysms. From July 1995 to October 1997, the authors treated 25 patients of intracranial aneurysm with endovascular coil embolization. There were 15 cases of ruptured aneurysms and 10 cases of unruptured aneurysms. The main reasons for the coil embolization were difficult anatomical location and poor neurological condition. Total 28 aneurysms were tried to embolize with coil. Among these, 12 aneurysms were occluded completely, 12 aneurysms partially, and 1 vertebral artery fusiform aneurysm was treated by coil occlusion of the proximal vertebral artery. The complete occlusion was possible in 10 cases of 15 small aneurysms(66.7%), and in 2 cases of 6 large aneurysms(33.3%). Only partial occlusion was achieved in all 3 giant aneurysms. Of 18 small neck aneurysms(4mm) could be occluded completely. There were 3 cases of failure, 2 cases being catherizatioin failure because of the tortuous vessel and 1 case due to local vasospasm. The complications related to the coil embolization were the perforation of the aneurysm caused by the catheterization in 2 cases, rebleeding after partial embolization of the aneurysm in 2 patients of Hunt-Hess grade 4 and 5, and infarction caused by parent vessel occlusion by coil protrusion in 2 cases. In conclusion, the coil embolization can be an alternative modality in the treatment of intracranial aneurysms. But the complete occlusion is not always possible in all cases, and the risk of the rebleeding is still remained in the partially occluded aneurysms. The long-term study is needed to confirm the definite effectiveness of the coil embolization in the treatment of intracranial aneurysms.
Aneurysm ; Aneurysm, Ruptured ; Catheterization ; Catheters ; Embolization, Therapeutic* ; Humans ; Infarction ; Intracranial Aneurysm* ; Neck ; Parents ; Vertebral Artery

To assess the degree of malignancy in cerebral gliomas at the time of diagnosis, we compared the metabolic ratio using 18F-fluorodeoxyglucose(FDG)-Positron Emission Tomography(PET) with histologic grading and proliferative index(Ki-67) of cerebral gliomas. Materials for this study were histologically-examined 21 gliomas and they were divided into glioblastomas as group 1, anaplastic gliomas as group 2, and low-grade gliomas as group 3. The visual analysis of FDG-PET images showed hypermetabolic lesions in 14(87.5%) out of 16 high-grade gliomas (glioblastomas and anaplastic gliomas), and hypometabolic lesions in 4(80%) out of 5 low-grade gliomas. Tumor to cerebellum ratio(T/Cbll) in FDG-PET was used as metabolic ratio and the values of T/Cbll in each group were 1.30+/-0.10, 0.73+/-0.07, 0.70+/-0.07, respectively. In comparision of T/Cbll between group 1 with remaining two groups, differences were statistically significant(p=0.0002, p=0.0002, respectively), however, there was no statistical difference between group 2 and group 3. The values of Ki-67 were 24.16+/-5.66 in group 1, 8.10+/-2.70 in group 2, 5.46+/-1.23 in group 3, and differences were statistically significant between group 1 and group 2, 3(p=0.015, p=0.015, respectively), but there was no statistical difference between group 2 and group 3. The correlation between T/Cbll and Ki-67 was good and statistically significant(p=0.0047). In conclusion, the visual and semiquantitative analysis of FDG-PET would be helpful in determining the degree of malignancy in cerebral gliomas.
Cerebellum ; Diagnosis ; Glioblastoma ; Glioma*

OBJECTIVE: For lumbar spondylolisthesis, various spinal operation methods have been used to relieve clinical symptoms and to stabilize the spine. To determine the effectiveness of the lumbar interbody metal cages and pedicle screw fixation in patients with spondylolisthesis, the authors retrospectively reviewed 70 patients under-going surgery, and also analyzed the relationship between post-operative clinical improvement and bony fusion, postoperative reduction, as well as transitional vertebrae. METHODS: The authors performed the lumbar interbody metal cages and pedicle screw fixation in 70 consecutive patients suffering from spondylolisthesis between November 1994 and December 1997. Follow-up averaged 18 months(range, 3 to 39 months). The post-operative clinical outcome was expressed as good, fair, or poor. The bony fusion was determined by post-operative simple X-ray films. RESULTS: Of 70 patients with clinical, radiologically unstable spondylolisthesis, the clinical results were good and fair in 92% of the patients, and radiological bony fusion were occurred in 86%. There was the significant relationship between clinical improvement and bony fusion. But there was no significant relationship between clinical improvement and post-operative reduction as well as transitional vertebrae. The post-operative complications occurred in 4% of patients and reoperation was required in 1 patient. CONCLUSION: This study demonstrates that the lumbar interbody cage with pedicle screw fixation is the effective operation method in the treatment of spondylolisthesis, though it demands more difficulties technically.
Follow-Up Studies ; Humans ; Reoperation ; Retrospective Studies ; Spine ; Spondylolisthesis* ; X-Ray Film