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.

Gradually truncated log-normal in USA publicly traded firm size distribution

We study the statistical distribution of firm size for USA and Brazilian publicly traded firms through the Zipf plot technique. Sale size is used to measure firm size. The Brazilian firm size distribution is given by a log-normal distribution without any adjustable parameter. However, we also need to consider different parameters of log-normal distribution for the largest firms in the distribution, which are mostly foreign firms. The log-normal distribution has to be gradually truncated after a certain critical value for USA firms. Therefore, the original hypothesis of proportional effect proposed by Gibrat is valid with some modification for very large firms. We also consider the possible mechanisms behind this distribution. (c) 2006 Published by Elsevier B.V.

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).

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.

Assessment and statistics of Brazilian hydroelectric power plants: Dam areas versus installed and firm power

The Brazilian relief, predominantly composed by small mountains and plateaus, contributed to formation of rivers with high amount of falls. With exception to North-eastern Brazil, the climate of this country are rainy, which contributes to maintain water flows high. These elements are essential to a high hydroelectric potential, contributing to the choice of hydroelectric power plants as the main technology of electricity generation in Brazil. Though this is a renewable source, whose utilized resource is free, dams must to be established which generates a high environmental and social impact. The objective of this study is to evaluate the impact caused by these dams through the use of environmental indexes. These indexes are ratio formed by installed power with dam area of a hydro power plant, and ratio formed by firm power with this dam area. In this study, the greatest media values were found in South, Southeast, and Northeast regions respectively, and the smallest media values were found in North and Mid-West regions, respectively. The greatest encountered media indexes were also found in dams established in the 1950s. In the last six decades, the smallest indexes were registered by darns established in the 1980s. These indexes could be utilized as important instruments for environmental impact assessments, and could enable a dam to be established that depletes an ecosystem as less as possible.

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.

Building sustainable values in organizations with the support of human resource management: Evidence from one firm considered as the 'best place to work' in Brazil

Researchers and other professionals unanimously agree that companies should become more sustainable, but this will not happen without the support of human resource management. Paradoxically, there is a lack of information on the support human resource management offers to organizational sustainability applied to real cases. Therefore, this research presents a case study on this topic that was carried out in a leading Brazilian company, which is considered as a model and has been selected as 'the best place to work in the country'. The results provide practical examples of how this family company has been working to guarantee an increasingly sustainable performance with the support of human resources, highlighting the achievements and challenges the company has faced. One of the main results indicates that companies seeking to achieve sustainability need the assistance of the human resource field in order to design a communication system which bridges the gap between practices and sustainable values. © 2012 Management Centre for Human Values.

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.