Twenty years' use of expanded polytetrafluoroethylene sheet for an artificial cardiac pacemaker.

Physicians can prolongedly use expanded polytetrafluoroethylene sheets for fixation of artificial cardiac pacemakers to avoid pacemaker lead displacement. The sheets can also be used to prevent implant rejection in patients with metal allergies.

[Valve-sparing Aortic Root Replacement in Patient with Dissecting Ascending Aortic Aneurysm Complicated with Hemorrhagic Moyamoya Disease;Report of a Case].

A 62-year-old female with Moyamoya disease (MMD) had cardiomegaly pointed out by chest X-ray and was admitted to our hospital. Chest computed tomography (CT) scan and echocardiography revealed a large dissecting ascending aortic aneurysm 78 mm in diameter combined with severe aortic regurgitation and mild mitral regurgitation. She had a history of intracranial hemorrhage related with MMD twice. Considering her relatively young age and risk of intracranial hemorrhage, valve-sparing aortic root replacement was planned to avoid anticoagulant therapy. Operation was performed keeping intra-operative blood pressure, perfusion pressure, Paco2 and activating clotting time in appropriate ranges. The postoperative course was uneventful without cerebral complication. No clinical symptom related with MMD was observed in 4 years after the operation.

[Giant aortic arch aneurysm following ligation of patent ductus arteriosus in an adult patient with Down syndrome; report of a case].

A 45-year-old male with Down syndrome( DS) had abnormal findings pointed out by chest X-ray and admitted to our hospital. He had undergone ligation of the patent ductus arteriosus 33 years before. Computed tomography showed a giant aortic aneurysm at the aortopulmonary window. Aortic arch replacement was performed under cardiopulmonary bypass and circulatory arrest. The postoperative course was uneventful. Postoperative ductal aneurysm in an adult is relatively rare and needs early operation because of the high risk of rupture. In this case, considering the size of the aneurysm, the timing of diagnosis seemed to be late. As the life expectancy of patients with DS has been lengthning recently, their regular health examinations is mandatory to improve the life expectancy and quality of life.

[Left atrial myxoma who presented cardiac arrest immediately after the onset of coldness of a leg; report of a case].

A 55-year-old woman visited the emergency department of our hospital with complaint of coldness and pain in her right leg. Two hours after the onset, she suddenly felt dyspnea and temporarily suffered cardiac arrest. Echocardiography revealed a left atrial tumor prolapsing into the left ventricle through the mitral valve. By emergency operation, both the cardiac tumor and the embolus were diagnosed as myxomas. The postoperative course was uneventful. Cardiac failure and arterial embolism are major symptoms of a left atrial myxoma, but there have been no reports of both symptoms occurring at the same time. It is supposed that the changing form of the tumor because of embolus separation could have caused heart failure. We must consider the use of echocardiography to examine cardiac tumors in patients with arterial embolism who do not have cardiac arrhythmias.

Cloning and heterologous expression of the vibrioferrin biosynthetic gene cluster from a marine metagenomic library.

A biosynthetic gene cluster of siderophore consisting of five open reading frames (ORFs) was cloned by functional screening of a metagenomic library constructed from tidal-flat sediment. Expression of the cloned biosynthetic genes in Escherichia coli led to the production of vibrioferrin, a siderophore originally reported for the marine bacterium Vibrio parahaemolyticus. To the best of our knowledge, this is the first example of heterologous production of a siderophore by biosynthetic genes cloned from a metagenomic library. The cloned cluster was one of the largest of the clusters obtained by functional screening. In this study, we demonstrated and extended the possibility of function-based metagenomic research.

Clinical activity after 12 weeks of treatment with nonbiologics in early rheumatoid arthritis may predict articular destruction 2 years later.

OBJECTIVE: To investigate earlier prediction of future articular destruction in patients with early rheumatoid arthritis (RA). METHODS: We randomly allocated patients with RA with disease duration < 2 years to different nonbiologic disease modifying antirheumatic drug (DMARD) therapies in a double-blind trial. Progression of articular destruction over the 96-week treatment period was assessed using the modified Sharp method. RESULTS: Progression of articular destruction correlated more strongly with the American College of Rheumatology (ACR) core set measures after 12 weeks of treatment than with pretreatment values. Multiple regression analysis of data after 12 weeks yielded a correlation coefficient of 0.711. The sensitivity and specificity to predict articular destruction over the 75th percentile of the cohort were 78.6% and 84.6%, respectively. Patients who showed articular destruction over the 75th percentile of the cohort had low response to treatment at 12 weeks, and continued to have high clinical disease activity thereafter. Contrasting data were found in patients with slow progression of articular destruction. CONCLUSION: In patients with early RA, ACR core set measures after 12 weeks of nonbiologic DMARD treatment may predict articular destruction 2 years later. Low response to treatment at 12 weeks and continuing high disease activity thereafter were found in patients with rapid radiological progression. These data can be used to determine the appropriateness of treatment at 12 weeks and aid the decision to introduce biologic DMARD.

[A case of polymyalgia rheumatica where a complication of large-vessel vasculitis was diagnosed by FDG-PET].

The patient was a 74-year-old female presenting with abrupt onset of fever and proximal muscle pains. She had been diagnosed with polymyalgia rheumatica (PMR). On physical examination, there was no tenderness or dilatation of the temporal artery and ocular fundi were normal. 18F-FDG-PET revealed accumulation of FDG in the aorta as well as in the bilateral subclavian arteries, which strongly suggested inflammation of the large blood vessels. Magnetic resonance angiography disclosed stenosis of the bilateral subclavian arteries, which was consistent with angitis. This case was considered to have developed PMR at an old age with positive HLA DR4, and to have a complication large-vessel giant cell arteritis (LV-GCA). Administration of prednisolone at a dose of 20 mg/day promptly relieved the fever and the myalgia as well. It is difficult to diagnose GCA in PMR if no tenderness or dilatation of the temporal artery is present. FDG-PET is considered useful, not only for exploration of tumors, but also for evaluation of inflammation of large vessels.

Efficacy of high-dose intravenous immunoglobulin therapy in Japanese patients with steroid-resistant polymyositis and dermatomyositis.

Intravenous immunoglobulin (IVIG) therapy was administered to 15 patients who were refractory to traditional steroid therapy [eight with polymyosis (PM), seven with dermamyosis (DM)] to evaluate its efficacy. Serum creatine kinase (CK) significantly decreased from week 1, and manual muscle test scores (MMT) and activities of daily living (ADL) significantly increased from week 2. Efficacy rates were 93.3% (14/15 patients) as assessed using the MMT score, 80.0% (12/15 patients) using the ADL score, and 100% (15/15 patients) using the serum CK level. When changes in the serum CK level over two four-week periods, one before IVIG therapy (from week -4 to week 0) and one after IVIG therapy (from week 0 to week 4), were transformed to natural logarithms, the four-week change after IVIG therapy was significantly greater than that before IVIG therapy. The estimated duration of the serum CK level remaining normal in 50% of the patients after IVIG therapy was 334.5 days. Adverse reactions were observed in seven of 16 patients (43.8%) during the study period, but none of the adverse reactions were considered to be serious or required emergency treatment. In conclusion, the present study indicates that IVIG therapy is effective for steroid-resistant PM/DM.

A multicenter, double-blind, randomized, placebo controlled trial of infliximab combined with low dose methotrexate in Japanese patients with rheumatoid arthritis.

OBJECTIVE: A placebo controlled, double-blind trial (DBT) was conducted for Japanese patients with active rheumatoid arthritis (RA) despite treatment with low dose methotrexate (MTX) to evaluate the efficacy and safety of infliximab. Extended treatment with infliximab was conducted in an open-label trial (OLT). METHODS: In the DBT, 147 patients were randomly assigned and treated with a placebo or 3 mg/kg or 10 mg/kg infliximab at Weeks 0, 2 and 6, combined with MTX. In the OLT, 129 patients from the DBT received 3 mg/kg infliximab every 8 weeks. RESULTS: The mean dose of MTX was 7.2 +/- 2.0 mg/week. Significantly more patients receiving 3 mg/kg (61.2%) and 10 mg/kg (52.9%)infliximab achieved a 20% improvement according to the American College of Rheumatology (ACR) criteria at Week 14, compared to placebo (23.4%) (p < 0.001). There was no significant difference in incidence of adverse events among the treatment groups. In patients receiving infliximab in the DBT, 11.6% of patients with serum infliximab just before the OLT developed antibodies to infliximab (ATI) in the OLT, whereas 62.2% of patients without serum infliximab did. In patients receiving placebo in the DBT, 43.9% developed ATI. CONCLUSION: The efficacy and safety of infliximab combined with low dose MTX were similar to those of the ATTRACT study. The data from the DBT and OLT also supported the importance of an induction treatment of infliximab, followed by a maintenance treatment without a long interval, giving stable serum concentrations in order to prevent formation of ATI.

Therapeutic effects of the combination of methotrexate and bucillamine in early rheumatoid arthritis: a multicenter, double-blind, randomized controlled study.

Disease-modifying antirheumatic drug (DMARD) combination therapies are used widely, but there have been few reports clearly demonstrating that combination therapy is more effective than DMARD monotherapy. We conducted a multicenter, double-blind controlled trial in order to clarify that the combination of methotrexate and bucillamine is more effective than either alone. The subjects of this study were 71 patients with active rheumatoid arthritis within 2 years of onset. Dosages were 8 mg methotrexate with 5 mg folic acid per week (MTX group), 200 mg bucillamine per day (BUC group), or both MTX and BUC (combination group). Clinical effects and adverse reactions were observed for 96 weeks. The ACR 20 response rate was 79.2% in the combination group, significantly higher than the rates of 43.5% for the MTX group (P = 0.008) and 45.8% for the BUC group (P = 0.0178). The cumulative survival curve of maintaining the ACR 20 response was significantly higher in the combination group than in the MTX and BUC groups (P = 0.0123 and P = 0.0088, respectively). The mean increase in the total Sharp score over 96 weeks was 12.6 +/- 9.0 in the combination group, significantly lower (P = 0.0468) than the value of 28.0 +/- 28.3 for the single DMARD (combined MTX and BUC) group. The incidence of adverse reactions did not differ significantly between the three groups. It was concluded that the combination therapy with MTX and BUC showed significantly higher clinical efficacy than either of the single DMARD therapies.

Prospective study of high-dose intravenous immunoglobulin for the treatment of steroid-resistant polymyositis and dermatomyositis.

Abstract High-dose intravenous immunoglobulin (IVIG) therapy has been effective in treating many autoimmune and systemic inflammatory diseases. In the present prospective study, we evaluated the efficacy of IVIG for patients with polymyositis (PM) and dermatomyositis (DM) refractory to treatment with high-dose corticosteroids. PM/DM was defined as steroid-resistant when the muscle strength of a patient did not improve despite the administration of more than 50 mg prednisolone per day for more than 4 weeks. A total of 12 patients with biopsy-proven, steroid-resistant PM/DM received one infusion of polyethylene glycol-treated human IgG at a dose of 0.4 g per kg per day for five successive days. Three of the patients received a second infusion. All patients were followed for up to 3 months after the infusion. Finally, 8 patients (6 PM and 2 DM; 5 men and 3 women) aged 29-67 years (mean 48 years) were analyzed. Their clinical response was assessed by changes in (a) subjective signs, i.e., fatigue (visual analog scale, VAS), muscle pain (VAS), activities of daily living (ADL), (b) objective signs, i.e., manual muscle strength (MMT) and serum level of creatine kinase (CK). At 12 weeks after the infusion, the patients showed significant improvement in their scores of muscle strength (from a mean of 67.0 to 81.0) and their ADL scores (from a mean of 27.1 to 39.1). The mean serum CK level decreased significantly from 1287.4 to 612.6 IU/l. In addition, the mean VAS of fatigue decreased significantly from 5.5 to 1.3 cm. The physicians' assessment showed that 87.5% of patients had improved. The average reduced dose of prednisolone was 47.1 mg/day at 12 weeks after infusion in 7 patients who exhibited improvement. Adverse effects, i.e., asymptomatic myocardial infarction and increased blood urea nitrogen (BUN), were noted with two of the 15 infusion (13%). Overall, IVIG was found to be safe and effective for refractory PM and DM.

An atmospheric dispersion model for the environmental impact assessment of thermal power plants in Japan--a method for evaluating topographical effects.

An atmospheric dispersion model was developed for the environmental impact assessment of thermal power plants in Japan, and a method for evaluating topographical effects using this model was proposed. The atmospheric dispersion model consists of an airflow model with a turbulence closure model based on the algebraic Reynolds stress model and a Lagrangian particle dispersion model (LPDM). The evaluation of the maximum concentration of air pollutants such as SO2, NOx, and suspended particulate matter is usually considered of primary importance for environmental impact assessment. Three indices were therefore estimated by the atmospheric dispersion model: the ratios (alpha and beta, respectively) of the maximum concentration and the distance of the point of the maximum concentration from the source over topography to the respective values over a flat plane, and the relative concentration distribution [gamma(x)] along the ground surface projection of the plume axis normalized by the maximum concentration over a flat plane. The atmospheric dispersion model was applied to the topography around a power plant with a maximum elevation of more than 1,000 m. The values of alpha and beta evaluated by the atmospheric dispersion model varied between 1 and 3 and between 1 and 0.4, respectively, depending on the topographical features. These results and the calculated distributions of y(x) were highly similar to the results of the wind tunnel experiment. Therefore, when the slope of a hill or mountain is similar to the topography considered in this study, it is possible to evaluate topographical effects on exhaust gas dispersion with reasonable accuracy using the atmospheric dispersion model as well as wind tunnel experiments.