Macrophage Activation Syndrome in Juvenile Systemic Lupus Erythematosus A Multinational Multicenter Study of Thirty-Eight Patients

Objective. To describe the clinical and laboratory features of macrophage activation syndrome as a complication of juvenile systemic lupus erythematosus (SLE).Methods. Cases of juvenile SLE-associated macrophage activation syndrome were provided by investigators belonging to 3 pediatric rheumatology networks or were found in the literature. Patients who had evidence of macrophage hemophagocytosis on bone marrow aspiration were considered to have definite macrophage activation syndrome, and those who did not have such evidence were considered to have probable macrophage activation syndrome. Clinical and laboratory findings in patients with macrophage activation syndrome were contrasted with those of 2 control groups composed of patients with active juvenile SLE without macrophage activation syndrome. The ability of each feature to discriminate macrophage activation syndrome from active disease was evaluated by calculating sensitivity, specificity, and area under the receiver operating characteristic curve.Results. The study included 38 patients (20 with definite macrophage activation syndrome and 18 with probable macrophage activation syndrome). Patients with definite and probable macrophage activation syndrome were comparable with regard to all clinical and laboratory features of the syndrome, except for a greater frequency of lymphadenopathy, leukopenia, and thrombocytopenia in patients with definite macrophage activation syndrome. Overall, clinical features had better specificity than sensitivity, except for fever, which was highly sensitive but had low specificity. Among laboratory features, the best sensitivity and specificity was achieved using hyperferritinemia, followed by increased levels of lactate dehydrogenase, hypertriglyceridemia, and hypofibrinogenemia. Based on the results of statistical analysis, preliminary diagnostic guidelines for macrophage activation syndrome in juvenile SLE were developed.Conclusion. Our findings indicate that the occurrence of unexplained fever and cytopenia, when associated with hyperferritinemia, in a patient with juvenile SLE should raise the suspicion of macrophage activation syndrome. We propose preliminary guidelines for this syndrome in juvenile SLE to facilitate timely diagnosis and correct classification of patients.

Proxy-reported health-related quality of life of patients with juvenile idiopathic arthritis: Pediatric rheumatology international trials organization multinational quality of life cohort study

Objective. To investigate the proxy-reported health-related quality of life (HRQOL) and its determinants in patients with juvenile idiopathic arthritis (JIA).Methods. In this multinational, multicenter, cross-sectional study, HRQOL of patients with JIA was assessed through the Child Health Questionnaire (CHQ) and was compared with that of healthy children of similar age from the same geographic area. of joint inflammation, Childhood Health Assessment Questionnaire (CHAQ), and erythrocyte sedimentation rate.Results. A total of 6,639 participants (3,324 with JIA and 3,315 healthy) were enrolled from 32 countries. The mean SD physical and psychosocial summary scores of the CHQ were significantly lower in patients with JIA than in healthy children (physical: 44.5 +/- 10.6 versus 54.6 +/- 4.0, P < 0.0001; psychosocial: 47.6 +/- 8.7 versus 51.9 +/- 7.59 P < 0.0001), with the physical well-being domain being most impaired. Patients with persistent oligoarthritis had better HRQOL compared with other subtypes, whereas HRQOL was similar across patients with systemic arthritis, polyarthritis, and extended oligoarthritis. A CHAQ score > 1 and a pain intensity rating > 3.4 cm on a 10-cm visual analog scale were the strongest determinants of poorer HRQOL in the physical and psychosocial domains, respectively.Conclusion. We found that patients with JIA have a significant impairment of their HRQOL compared with healthy peers, particularly in the physical domain. Physical well-being was mostly affected by the level of functional impairment, whereas the intensity of pain had the greatest influence on psychosocial health.

Long-Term Outcome and Prognostic Factors of Juvenile Dermatomyositis: A Multinational, Multicenter Study of 490 Patients

Objective. To investigate the long-term outcome and prognostic factors of juvenile dermatomyositis (DM) through a multinational, multicenter study.Methods. Patients consisted of inception cohorts seen between 1980 and 2004 in 27 centers in Europe and Latin America. Predictor variables were sex, continent, ethnicity, onset year, onset age, onset type, onset manifestations, course type, disease duration, and active disease duration. Outcomes were muscle strength/endurance, continued disease activity, cumulative damage, muscle damage, cutaneous damage, calcinosis, lipodystrophy, physical function, and health-related quality of life (HRQOL).Results. A total of 490 patients with a mean disease duration of 7.7 years were included. At the cross-sectional visit, 41.2-52.8% of patients, depending on the instrument used, had reduced muscle strength/endurance, but less than 10% had severe impairment. Persistently active disease was recorded in 41.2-60.5% of the patients, depending on the activity measure used. Sixty-nine percent of the patients had cumulative damage. The frequency of calcinosis and lipodystrophy was 23.6% and 9.7%, respectively. A total of 40.7% of the patients had decreased functional ability, but only 6.5% had major impairment. Only a small fraction had decreased HRQOL. A chronic course, either polycyclic or continuous, consistently predicted a poorer outcome. Mortality rate was 3.1%.Conclusion. This study confirms the marked improvement in functional outcome of juvenile DM when compared with earlier literature. However, many patients had continued disease activity and cumulative damage at followup. A chronic course was the strongest predictor of poor prognosis. These findings highlight the need for treatment strategies that enable a better control of disease activity over time and the reduction of nonreversible damage.

Green supply chain management in local and multinational high-tech companies located in Brazil

The objective of this article is to analyse how green supply chain management (GSCM) practices are being adopted by some high-tech companies located in Brazil. The research was conducted using the case study approach, focusing on eight companies that are representative of this sector. The main results are: (a) the most adopted GSCM practices in the studied high-tech companies located in Brazil are internal environmental management, investment recovery and reverse logistics and (b) Brazilian environmental legislation and international policies are very important in driving the adoption of GSCM practices. The internationalisation of companies was also found to be a variable that interferes with the adoption of GSCM practices. This is one of the first studies that examine the relationship between GSCM and the internationalisation of companies located in Brazil. © 2013 Springer-Verlag London.

Multinationals and Exports in a Large and Protected Developing Country

This paper examines an industry-level model developed to analyze the impact of affiliates of multinational firms (MNFs) on the host country's revealed comparative advantages (RCAs), which predicts that the referred impact is given by both technology service and industry orientation. Based on Brazilian manufacturing industries during the import-substitution industrialization, panel data estimates show that MNFs negatively affected RCA, which is explained by location advantages in industries presenting comparative disadvantages, as reinforced by a location model. Two other important results are: (i) import protection had a stronger anti-export effect on multinationals than on national firms; (ii) MNFs were concentrated in industries with lower world-export growth.

Thermoeconomic analysis method for optimization of combined heat and power systems

This article presents an thermoeconomic analysis of cogeneration plants, applied as a rational technique to produce electric power and saturated steam. The aim of this new methodology is the minimum exergetic manufacturing cost (EMC), based on the Second Law of Thermodynamics. The decision variables selected for the optimization are the pressure and the temperature of the steam leaving the boiler in the case of using steam turbine, and the pressure ratio, turbine exhaust temperature and mass flow in the case of using gas turbines. The equations for calculating the capital costs of the components and products are formulated as a function of these decision variables. An application of the method using real data of a multinational chemical industry located in São Paulo state is presented. The conditions which establish the minimum cost are presented as finals conclusions.

Logística reversa numa empresa de laminação de vidros: um estudo de caso

Este trabalho apresenta uma aplicação da Logística Reversa numa empresa multinacional que atua na área de laminação de vidros no Brasil. Na empresa estudada, a sucata gerada pelos seus clientes pode ser usada para realimentar o seu processo de produção. Inicialmente, são discutidos os principais aspectos relacionados ao tema, tais como, definição de Logística Reversa, ciclo de vida do produto, motivos e destinos do retorno, além dos fatores que influenciam a eficiência de um processo de Logística Reversa. A seguir, ilustradas pelo estudo de caso, são apresentadas e comentadas as etapas para uma implementação de um sistema de Logística Reversa ser bem sucedida. Finalmente, comentam-se as dificuldades, ganhos e vantagens competitivas obtidas por empresas que adotaram soluções da Logística Reversa, em particular na empresa em que o estudo de caso foi desenvolvido.

Inovação tecnológica na indústria automobilística: características e evolução recente

O objetivo principal deste artigo é analisar as características básicas da evolução recente do processo de inovação tecnológica na indústria automobilística. A hipótese de trabalho é que estariam ocorrendo mudanças na intensidade do processo de inovação técnica nessa indústria. O ponto de partida é a observação de que, nos últimos anos, tem havido na indústria automotiva - provavelmente em decorrência da intensificação da concorrência setorial e de uma maior oportunidade tecnológica - tanto uma ampliação dos gastos em P&D quanto uma crescente incorporação da microeletrônica (nos produtos e nos processos produtivos). Finalmente, são feitas também algumas considerações procurando relacionar os resultados obtidos neste artigo com a discussão do processo de de-maturity (rejuvenescimento) industrial, proposto por Abernathy e outros autores.

Empresas multinacionais e desempenho comercial do Brasil: uma revisão da literatura

Fazemos uma releitura dos estudos empíricos sobre empresas multinacionais (EMNs) e comércio exterior no Brasil a partir dos modelos teóricos e empíricos de comércio internacional com firmas multinacionais. Percebe-se que aqueles estudos não examinam como características econômicas do país (Brasil) condicionaram a emergência e tipo das EMNs e, assim, seus respectivos impactos sobre comércio exterior. Conseguem isolar, nas análises de impacto comercial, evidências dos serviços tecnológicos das estrangeiras, relativamente às domésticas do mesmo setor, mas não examinam se essas firmas se concentraram ou não em atividades com vantagens comparativas no país, o que seria crucial para determinar seu impacto comercial comparativo.

Thermoeconomic analysis method for optimization of combined heat and power systems. Part I

This article presents a thermoeconomic analysis of cogeneration plants, applied as a rational technique to produce electric power and saturated steam. The aim of this new methodology is the minimum Exergetic Production Cost (EPC), based on the Second Law of Thermodynamics. The variables selected for the optimization are the pressure and the temperature of the steam leaving the boiler in the case of using steam turbine, and the pressure ratio, turbine exhaust temperature and mass flow in the case of using gas turbines. The equations for calculating the capital costs of the components and products are formulated as a function of these decision variables. An application of the method using real data of a multinational chemical industry located in São Paulo state is presented. The conditions which establish the minimum cost are presented as final output. (C) 2003 Elsevier Ltd. All rights reserved.

Anthropotechnological analysis of industrial accidents in Brazil

The Brazilian Ministry of Labour has been attempting to modify the norms used to analyse industrial accidents in the country. For this purpose, in 1994 it tried to make compulsory use of the causal tree approach to accident analysis, an approach developed in France during the 1970s,without having previously determined whether it is suitable for use under the industrial safety conditions that prevail in most Brazilian firms. In addition, apposition from Brazilian employers has blocked the proposed changes to the norms. The present study employed anthropotechnology to analyse experimental application of the causal tree method to work-related accidents in industrial firms in the region of Botucatu, São Paulo. Three work-related accidents were examined in three industrial firms representative of local, national and multinational companies. on the basis of the accidents analysed in this study, the rationale for the use of the causal tree method in Brazil can be summarized for each type of firm as follows:the method is redundant if there is a predominance of the type of risk whose elimination or neutralization requires adoption of conventional industrial safety measures (firm representative of local enterprises); the method is worth while if the company's specific technical risks have already largely been eliminated (firm representative of national enterprises); and the method is particularly appropriate if the firm has a good safety record and the causes of accidents are primarily related to industrial organization and management (multinational enterprise).

Juvenile localized scleroderma: Clinical and epidemiological features in 750 children. An international study

Objective. Juvenile localized scleroderma (JLS) includes a number of conditions often grouped together. With the long-term goal of developing uniform classification criteria, we studied the epidemiological, clinical and immunological features of children with JLS followed by paediatric rheumatology and dermatology centres. Methods. A large, multicentre, multinational study was conducted by collecting information on the demographics, family history, triggering environmental factors, clinical and laboratory features, and treatment of patients with JLS. Results. Seven hundred and fifty patients with JLS from 70 centres were enrolled into the study. The disease duration at diagnosis was 18 months. Linear scleroderma (LS) was the most frequent subtype (65%), followed by plaque morphea (PM) (26%), generalized morphea (GM) (7%) and deep morphea (DM) (2%). As many as 15% of patients had a mixed subtype. Ninety-one patients (12%) had a positive family history for rheumatic or autoimmune diseases; 100 (13.3%) reported environmental events as possible trigger. ANA was positive in 42.3% of the patients, with a higher prevalence in the LS-DM subtype than in the PM-GM subtype. Scl70 was detected in the sera of 3% of the patients, anticentromere antibody in 2%, anti-double-stranded DNA in 4%, anti-cardiolipin antibody in 13% and rheumatoid factor in 16%. Methotrexate was the drug most frequently used, especially during the last 5 yr. Conclusion. This study represents the largest collection of patients with JLS ever reported. The insidious onset of the disease, the delay in diagnosis, the recognition of mixed subtype and the better definition of the other subtypes should influence our efforts in educating trainees and practitioners and help in developing a comprehensive classification system for this syndrome. © 2006 Oxford University Press.

Integration practices for the technological innovation of products: Case studies at two large technological companies

Studies on innovation and technology management have emphasized the importance of integration between the research and development (R&D) department and others involved with the product development process (PDP) as a relevant practice for the good performance of technological innovation of product activities. This study addresses the topic of transfers of technologies to new product projects and also integration practices between the R&D department and others involved with the PDP. A qualitative study was conducted that was operationalized through two case studies at large high-tech companies: One is Brazilian and the other is a multinational subsidiary in Brazil. Among its main result, this paper represents and analyzes management practices that are favorable to integration in product development projects that demand development and transfer of technologies, such as: participation of R&D personnel in market activities, the adoption of virtual interaction mechanisms, and the application of methods such as technology roadmaps. © Universidad Alberto Hurtado, Facultad de Economía y Negocios.