It is uncommon for Fabry's disease (FD) patient to present with an isolated ischemic stroke without other typical symptoms or signs of FD. A 48-year-old woman presented with recurrent limb weakness and her brain magnetic resonance imaging revealed multiple ischemic brain lesions. Ten years ago, the patient had been diagnosed with heterozygote FD by the genetic test, but she had not shown any typical symptoms or sign of FD so far. Isolated organ involvement could occur in heterozygote FD.
Brain ; Brain Ischemia ; Extremities ; Fabry Disease ; Female ; Heterozygote ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Stroke

OBJECTIVE: The synovium in rheumatoid arthritis(RA) is characterized by an increase in the thickness of lining layer and infiltration of cells into the sublining area. Histomorphologic studies of RA have indicated that initial destruction is more closely related to the presence of transformed appearing proliferating synovial cells than to the presence of subsynovial or periarticular inflammation. Based on the fact that synovial lining cells have some properties of transformed appearing cells, we examined the expressions of Fos, Jun and Myc oncoproteins in the synovial tissue from patients with rheumatoid arthritis and osteoarthritis. METHODS: Synovial tissues from 15 patients with RA and 15 with osteoarthritis(OA) were studied by the immunohistochemical staining technique. Nine of 15 RA specimen were from arthroscopic synovectomy and the other 6 were from total knee replacement arthroplasty. RESULT: In all specimen studied, Myc and Fos were expressed in the synovial lining cells and Myc, Fos and Jun were expressed in the sublining cells, including lymphocytes, other inflammatory cells and blood vessels. Lymphocytes in the diffuse infiltrates showed increased expression of three oncoproteins compared to lymphocytes in the nodular aggregates. When oncoprotein expressions in RA were compared to OA, Fos and Myc expressions in the synovial lining cell layer were significantly higher in RA than in OA and Jun, Fos and Myc expressions in inflammatory cells were significantly higher in RA than in OA. The expressions of Fos and Myc were significantly correlated with the degree of synovial hypercellularity. In RA, the expressions of all three oncoproteins were increased in synovectomy group than joint replacement group. CONCLUSION: We observe that there are increased expressions of Myc, Fos and Jun in RA synovium than OA. These changes are more prominent in synovectomy group than joint replacement group, which suggest the differential expression of oncoproteins according to disease progression.
Arthritis, Rheumatoid ; Arthroplasty ; Arthroplasty, Replacement, Knee ; Blood Vessels ; Disease Progression ; Humans ; Inflammation ; Joints ; Lymphocytes ; Oncogene Proteins ; Oncogenes ; Osteoarthritis ; Synovial Membrane*

OBJECTIVE: Cardiovascular disease is the most frequent cause of death in rheumatoid arthritis(RA) patients and hyperhomocysteinemia is an independent risk factor for cardiovascular disease. We evaluated the status of homocysteine, vitamin B12 and folate in RA patients and the influence of the drugs used in RA on homocysteine and vitamin levels. METHODS: Fifty-six RA patients and 22 controls were studied. Plasma total homocysteine was measured by HPLC method and serum folate and vitamin B12 were measured by chemiluminescence immunoassay. In RA patients, age, sex, disease duration, medications and laboratory findings were analyzed. RESULTS: Serum vitamin B12 level was significantly lower in RA patients compared to controls(p=0.033). No significant difference in serum folate level was found between RA patients and controls but plasma total homocysteine level was significantly higher in RA patients. There was no difference in plasma total homocysteine level between patients taking MTX with folate and controls, but plasma total homocysteine level was significantly higher in patients not taking MTX compared with controls(p=0.028). In RA patients taking only hydroxychloroquine(HCQ) as a DMARD, there was significantly lower serum folate level(p=0.033) and higher plasma total homocysteine level(p=0.043) compared with controls. There was a significant negative correlation between plasma total homocysteine level and serum folate level in RA patients(r=-0.319, p=0.017). CONCLUSION: Plasma total homocysteine level was increased in RA patients but not in patients taking MTX and folate. These findings suggested that folate supplementation may be effective to prevent hyperhomocysteinemia in RA patients.
Antirheumatic Agents ; Arthritis, Rheumatoid* ; Cardiovascular Diseases ; Cause of Death ; Chromatography, High Pressure Liquid ; Folic Acid* ; Homocysteine* ; Humans ; Hyperhomocysteinemia ; Immunoassay ; Luminescence ; Plasma* ; Risk Factors ; Vitamin B 12* ; Vitamins*

Calcium pyrophosphate dihydrate (CPPD)crystal deposition disease is one of the most common causes of arthralgia in elderly.The acute form (pseudogout) may present as acute monoarticular or polyarticular arthritis.It is often accompanied by high fever and sometimes simulates acute infectious condition.The knee is the joint most frequently affected by pseudogout but other sites such as wrist,shoulder,ankle,elbow and hands may be affected.A few cases involving cervical and lumbar spine mistaken for CNS infection have also been described in foreign countries.We report here a case of CPPD crystal deposition disease mimicking meningitis.
Arthralgia ; Calcium Pyrophosphate* ; Calcium* ; Chondrocalcinosis ; Fever ; Hand ; Joints ; Knee ; Meningitis ; Spine

BACKGROUND: The prevalence of gout in Korea seems to be increasing but previous studies were outdated and small in number of cases. We enrolled relatively large number of patients and compared our data with previous studies to provide a better understanding of clinical manifestations of gout in Korean. METHODS: From 1994 to 2000, 372 patients, visited Samsung medical center for gout, were reviewed retrospectively. Age, sex, duration of disease, body mass index, 24 hour urine uric acid level, involved joints, frequency of tophi, bone changes on simple X-ray, associated diseases and complications of treatments were investigated. RESULTS: In 372 patients reviewed, 361 patients were male (97%) and only 11 patients (3%) were female. Age of onset was 46.4 +/- 13.3 years. Most frequently affected joint in first attack was 1st metatarsophalangeal joint (62.2%). Tophi were observed in 20.4% and bony erosions were detected in 34.6% of patients. The most commonly accompanied disease was hypertension (41.1%) and overweight and obesity were noted in 60.7% of patients. Liver function abnormalities associated with allopurinol were noted in 37.7% of patients and after cessation of drug, most of them showed complete recovery. CONCLUSION: Clinical features were similar to those of western countries. It may be due to changes in life style and usage of drugs that affect serum level of uric acid. So more emphasis on life style modification and control of chronic illness will be needed.
Age of Onset ; Allopurinol ; Body Mass Index ; Chronic Disease ; Female ; Gout* ; Humans ; Hypertension ; Joints ; Korea* ; Life Style ; Liver ; Male ; Metatarsophalangeal Joint ; Obesity ; Overweight ; Prevalence ; Retrospective Studies ; Uric Acid

Pulmonary alveolar hemorrhage (PAH) is a rare and often fetal presenting feature of systemic lupus erythematosus (SLE) and enters the differential diagnosis of diffuse lung disease in patients with SLE. Reported mortality rats are extremely high, between 70 and 90 percents. Because death frequently occurs within the first several days of the hemorrhage, the diagnosis needs to be established promptly and treatment should be initiated immediately. Treatment of alveolar hemorrhage has included various combinations of corticosteroids, cytotoxic agents, and plasmapheresis, but survival rates have been extremely low despite aggressive therapy. We experienced a case of diffuse alveolar hemorrhage in a 29 year-old SLE male patient. PAH was diagnosed by hemoptysis, anemia, infiltration on chest X-ray and hemosiderin-laden macrophages in bronchoalveolar lavage. After high dose intravenous steroid, cyclophosphamide intravenous therapy and plasmapheresis, the condition of patient was markedly improved. He was discharged and received monthly intravenous pulse cyclophosphamide. He has done well since, showing no further pulmonary hemorrhage with steroid tapering.
Adrenal Cortex Hormones ; Adult ; Anemia ; Animals ; Bronchoalveolar Lavage ; Cyclophosphamide ; Cytotoxins ; Diagnosis ; Diagnosis, Differential ; Hemoptysis ; Hemorrhage* ; Humans ; Lung Diseases ; Lupus Erythematosus, Systemic* ; Macrophages ; Male ; Mortality ; Plasmapheresis* ; Rats ; Survival Rate ; Thorax

OBJECTIVE: To investigate the clinical usefulness of serial measurement of erythrocyte sediment rate(ESR) and C-reactive protein(CRP) for monitoring the radiological progression in early rheumatoid arthritis(RA). METHODS: Thirty one patients with RA according to the 1987 ACR criteria, with joint symptoms for less than one year, and who had not previously received disease modifying antirheumatic drugs(DMARDs) were assessed from Oct. 1994 to Aug. 1998 in retrospective study. Area under curve(AUC) of ESR and CRP were calculated in these patients. Joing damage in the hands was assessed by modified Sharp's method. RESULTS: Male to female ratio was 1: 3.4. Median age of this group was 45.8+/-13.2 years. Patients who had positive rheumatoid factor were 82%. Mean duration until first visit was 271.9+/-145.8 days. At the first clinic visit, mean ESR and CRP were 50mm/hr and 2.0mg/dL. Mean erosions and joint space narrowing score by modified Sharp's method were 55.2 and 12.4, respectively. Correlation analysis showed that ESR AUC and CRP AUC were significantly associated with radiologic progression. CONCLUSIONS: Elevated over-time ESR and CRP provide a convenient short term correlation with radiologic outcome(i.e. erosions). Elevated over-time ESR and CRP can be used as a guide for therapy.
Ambulatory Care ; Area Under Curve ; Arthritis, Rheumatoid* ; Erythrocytes ; Female ; Hand ; Humans ; Joints ; Male ; Retrospective Studies ; Rheumatoid Factor

BACKGROUND: It is known that prolonged QRS duration (QRSd) in a 12-lead ECG is associated with decreased left ventricular (LV) systolic function in patients with dilated cardiomyopathy. Development of LV systolic dysfunction and prolongation of paced QRSd are often observed in patients with permanent pacemakers (PPM). However, the significance of prolonged paced QRSd in patients with PPM is not determined. METHODS: We studied sixty-four patients (male:female=27:37, mean age=57.6+/-15.4 years) who had been on PPM (DDD: 15, VDD: 18, VVI: 31) for more than one year (mean: 68.2+/-44.0 months). LV function was normal before implantation of PPM. The 12-lead ECG and echocardiography were recorded prior to implantation, immediately after implantation and at the last follow-up. RESULTS: Paced QRSd did not significantly increase during the follow-up period. LVEF at the last follow-up (LVEF-FU) was significantly lower than that prior to implantation (59.3+/-11.5% vs. 64.9+/-10.1%, p<0.001). Eleven (17.2%) patients developed LV systolic dysfunction (LVEF<50%) during the follow-up period. Paced QRSd at the last follow-up and diabetes mellitus were correlated with LV systolic dysfunction (R2=0.302, p<0.001). There was a significant negative correlation between the paced QRSd and LVEF-FU (r=-0.451, p<0.01) and between change of paced QRSd and that of LVEF during follow-up (r=-0.419, p<0.01). LVEF-FU was reduced more in patients with paced QRSd >or=180 ms than in patients without (44.4+/-12.0% vs. 61.7+/-9.5%, p<0.001). In predicting LV systolic dysfunction with the paced QRSd (cut-off value: 180 ms), sensitivity, specificity, positive and negative predictive values were 60.0%, 88.7%, 50.0% and 99.2%, respectively. The paced QRSd at the last follow-up was significantly correlated with paced QRSd immediately after implantation (r=0.542, p<0.01). CONCLUSION: LV systolic dysfunction after long-term right ventricular apical pacing may develop. Prolongation of paced QRSd 180 ms during follow-up may suggest development of LV systolic dysfunction. New technologies to minimize prolongation of paced QRSd should be investigated to prevent LV systolic dysfunction after permanent ventricular pacing.
Cardiomyopathy, Dilated ; Diabetes Mellitus ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Humans ; Sensitivity and Specificity ; Ventricular Dysfunction, Left* ; Ventricular Function

There are limited data on outcomes after implantation of everolimus-eluting stents (EES) in East Asian patients with small vessel coronary lesions. A total of 1,600 patients treated with XIENCE EES (Abbott Vascular, CA, USA) were divided into the small vessel group treated with one ≤2.5 mm stent (n=119) and the non-small vessel group treated with one ≥2.75 mm stent (n=933). The primary end point was a patient-oriented composite outcome (POCO), a composite of all-cause death, myocardial infarction (MI), and any repeat revascularization at 12 months. The key secondary end point was a device-oriented composite outcome (DOCO), a composite of cardiovascular death, target-vessel MI, and target lesion revascularization at 12 months. The small vessel group was more often female, hypertensive, less likely to present with ST-elevation MI, and more often treated for the left circumflex artery, whereas the non-small vessel group more often had type B2/C lesions, underwent intravascular ultrasound, and received unfractionated heparin. In the propensity matched cohort, the mean stent diameter was 2.5±0.0 mm and 3.1±0.4 mm in the small and non-small vessel groups, respectively. Propensity-adjusted POCO at 12 months was 6.0% in the small vessel group and 4.3% in the non-small vessel group (p=0.558). There was no significant difference in DOCO at 12 months (small vessel group: 4.3% and non-small vessel group: 1.7%, p=0.270).Outcomes of XIENCE EES for small vessel disease were comparable to those for non-small vessel disease at 12-month clinical follow-up in real-world Korean patients.

BACKGROUND AND OBJECTIVES: Diffuse long coronary artery disease (DLCAD) still has unfavorable clinical outcomes after successful percutaneous coronary intervention (PCI). Therefore, we aimed to evaluate the effectiveness and safety of Resolute™ zotarolimus-eluting stent (R-ZES; Resolute™ Integrity) for patients with DLCAD. METHODS: From December 2011 to December 2014, 1,011 patients who underwent PCI using R-ZES for CAD with longer than 25 mm lesion were prospectively enrolled from 21 hospitals in Korea. We assessed the clinical outcome of major adverse cardiac events (MACE) defined as the composite of cardiac death, non-fatal myocardial infarction (MI), and clinically-driven target vessel revascularization at 12 months. RESULTS: Mean age was 63.8±10.8 years, 701 (69.3%) patients were male, 572 (87.0%) patients had hypertension, 339 (33.8%) patients had diabetes, 549 (54.3%) patients diagnosed with acute MI and 545 (53.9%) patients had multi-vessel disease (MVD). A total of 1,697 stents were implanted into a total of 1,472 lesions. The mean diameter was 3.07±0.38 mm and the length was 28.27±6.97 mm. Multiple overlapping stents were performed in 205 (13.8%) lesions. A 12-month clinical follow-up was available in 1,004 patients (99.3%). The incidences of MACE and definite stent thrombosis at 12-month were 3.0% and 0.3% respectively. On multivariate Cox-regression analysis, multiple overlapping stents implantation, previous congestive heart failure, MVD, and age ≥75 years were independent predictors of one-year MACE. CONCLUSIONS Our study shows that R-ZES has an excellent 1-year clinical outcome in Korean patients with DLCAD.