Autoinflammatory diseases: Mimics of Autoimmunity or part of its spectrum? Case presentation

Introduction: Autoinflammatory diseases are very rare diseases presenting within a wide clinical spectrum. Recognition of the main clinical features are challenging due to overlapping or mimicking with autoimmune diseases. Discussion: A case series is reviewed to illustrate typical and atypical features and the difficulties of these diagnoses in the low prevalence areas-a typical unrecognized case of familial Mediterranean fever (FMF) in a youngster, an atypical adult case with overlapping of IMF with Behcet disease, and an early presentation of FMF in infant presenting with inflammatory colitis, as well as the overlapping features within the cryopirin diseases spectrum in an 8-year-old boy who presented with systemic onset arthritis. Conclusion: These cases may represent examples of a very puzzling relationship among disorders of innate and adaptive immune systems and inflammation.

Analysis of patellar stabilizers muscles and patellar kinematics in anterior knee pain subjects

Patella stabilizer muscle response and patellar kinematics were evaluated in 19 women with anterior knee pain (AKP) and 20 healthy women during maximum voluntary isometric contraction (MVIC) with the knee positioned at 15 degrees, 30 degrees and 45 degrees flexion during open (OKC) and closed (CKC) kinetic chain exercises. Patellar kinematics was evaluated through patellar tilt and displacement, and the electrical activity of patellar stabilizers through the root mean square normalized during MVIC and OKC with the knee at 90 degrees flexion. Data revealed that the vastus medialis oblique muscle (VMO) was more active in the control group compared to the AKP group during OKC exercises with the knee at 45 degrees flexion. However, no difference in the patellar kinematics was observed between these groups; nevertheless, the correlation between these parameters also showed, with the knee at 45 degrees flexion, that lateral patellar tilt increase was associated with a reduction in the activity of lateral patellar stabilizers in the control group and with an increase in the VMO activity in the AKP group. In conclusion, electrical activity is an important factor in evaluating AKP and in AKP treatment evolution. (C) 2010 Elsevier Ltd. All rights reserved.

Assessment of the Expression of IR beta, IRS-1, IRS-2 and IGF-IR beta in a Rat Model of Intrauterine Growth Restriction

Objective: To investigate glomerular development and expression of insulin and insulin-like growth factor receptors in an experimental model of intrauterine growth restriction (IUGR). Material and Methods: We studied three groups of Sprague-Dawley fetuses: IUGR - restricted by ligation of the right uterine artery; C-IUGR - left horn controls, and EC - external controls (non-manipulated). Body and organs were weighed, and glomerular number and volume were analyzed. Expression of IR beta, IRS-1, IRS-2 and IGF-IR beta was analyzed in liver, intestine and kidneys by immunoblotting. Results: Organ/body weight ratios were similar. In IUGR, glomerular number and volume were increased compared to C-IUGR and EC (p < 0.001). In the IUGR liver, increases were found in IGF-IR beta compared to C-IUGR and EC; IR beta compared to EC, and IRS-2 compared to C-IUGR. However, decreases in IR beta were noted in IUGR compared to C-IUGR; IRS-1 compared to C-IUGR and EC, and IRS-2 compared to EC. In IUGR intestine, increases were detected in IR beta, IRS-1 and IGF-IR beta compared to C-IUGR and EC. In IUGR kidneys, increases were observed in IR beta and IGF-IR beta compared to C-IUGR and EC, and IRS-1 compared to EC. Decreased IRS-2 in the intestine and kidney were noticed in IUGR compared to C-IUGR and EC. Conclusion: IUGR fetuses had less glomeruli and alterations in insulin receptors, which may be associated with an increased risk of disease occurrence in adulthood. Copyright (C) 2010 S. Karger AG, Basel

The Essential Oil of Eucalyptus tereticornis and its Constituents, alpha- and beta-Pinene, Show Accelerative Properties on Rat Gastrointestinal Transit

The essential oil of Eucalyptus tereticornis (EOET) has pharmacological activities but their effects on the gastrointestinal tract are yet unknown. It possesses alpha- and beta-pinene as minor constituents, isomers largely used as food or drink additives. In this work, we studied their actions on gut motility. After feeding with a liquid test meal, conscious rats received perorally EOET, alpha-, or beta-pinene, and the fractional dye retention was determined. EOET and its constituents decreased the gastric retention. In anesthetized rats, pinenes increased gastric tonus, while enhancing the meal progression in the small intestine of conscious rats. Both alpha- and beta-pinene contracted gastric strips in vitro but relaxed the duodenum. Conversely, EOET relaxed both the gastric and duodenal strips. In conclusion, EOET accelerates the gastric emptying of liquid, and part of its action is attributed to the contrasting effects induced by alpha- and beta-pinene on the gut.

Comparison of clinical features and drug therapies among European and Latin American patients with juvenile dermatomyositis

Objective To compare the demographic features, presenting manifestations, diagnostic investigations, disease course, and drug therapies of children with juvenile dermatomyositis (JDM) followed in Europe and Latin America. Methods Patients were inception cohorts seen between 1980 and 2004 in 27 paediatric rheumatology centres. The following information was collected through the review of patient charts: sex; age at disease onset; date of disease onset and diagnosis; onset type; presenting clinical features; diagnostic investigations; course type; and medications received during disease course. Results Four hundred and ninety patients (65.5% females, mean onset age 7.0 years, mean disease duration 7.7 years) were included. Disease presentation was acute or insidious in 57.1% and 42.9% of the patients, respectively. The course type was monophasic in 41.3% of patients and chronic polycyclic or continuous in 58.6% of patients. The more common presenting manifestations were muscle weakness (84.9%), Gottron`s papules (72.9%), heliotrope rash (62%), and malar rash (56.7%). Overall, the demographic and clinical features of the 2 continental cohorts were comparable. European patients received more frequently high-dose intravenous methylprednisolone, cyclosporine, cyclophosphamide, and azathioprine, while methotrexate and antimalarials medications were used more commonly by Latin American physicians. Conclusion The demographic and clinical characteristics of JDM are similar in European and Latin American patients. We found, however, several differences in the use of medications between European and Latin American paediatric rheumatologists.

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.

The Genome Sequence of Taurine Cattle: A Window to Ruminant Biology and Evolution

To understand the biology and evolution of ruminants, the cattle genome was sequenced to about sevenfold coverage. The cattle genome contains a minimum of 22,000 genes, with a core set of 14,345 orthologs shared among seven mammalian species of which 1217 are absent or undetected in noneutherian (marsupial or monotreme) genomes. Cattle-specific evolutionary breakpoint regions in chromosomes have a higher density of segmental duplications, enrichment of repetitive elements, and species-specific variations in genes associated with lactation and immune responsiveness. Genes involved in metabolism are generally highly conserved, although five metabolic genes are deleted or extensively diverged from their human orthologs. The cattle genome sequence thus provides a resource for understanding mammalian evolution and accelerating livestock genetic improvement for milk and meat production.

Star decompositions of cubes

Let Sk denote the complete bipartite graph K-1k and let e,, denote the ii-cube. We prove that the obvious necessary conditions for the existence of an S-k-decomposition of Q(n) are sufficient.

Trade relationships between the Gulf Cooperation Council and the European Union

This paper examines the trade relationship between the Gulf Cooperation Council (GCC) and the European Union (EU). A simultaneous equation regression model is developed and estimated to assist with the analysis. The regression results, using both the two stage least squares (2SLS) and ordinary least squares (OLS) estimation methods, reveal the existence of feedback effects between the two economic integrations. The results also show that during times of slack in oil prices, the GCC income from its investments overseas helped to finance its imports from the EU.

Urbanization, population transition, and growth

This paper analyzes a dual economy consisting of urban market areas and less developed rural areas with or without local markets. Urban areas have better opportunities for earnings and education than rural areas. Rural families choose whether to move to urban areas at costs that differ from location to location. As per capita output grows relative to the moving cost, urbanization proceeds, leading to lower fertility, more investments in human and physical capital per child relative to output per worker, and faster economic growth. These impacts are stronger if rural areas have no access to markets.

Trends in physical activity participation and the impact of integrated campaigns among Australian adults, 1997-99

Objectives: To determine whether awareness of the moderate physical activity message and prevalence of participation changed among Australian adults between 1997 and 1999, and if changes differed across Australia. Methods: Data were compared on awareness of the moderate physical activity message and on physical activity participation from identical national physical activity surveys in 1997 and 1999. Results: In 1999, following integrated public health efforts, recognition of the Active Australia campaign was substantially higher in NSW/ACT (61.7%) than elsewhere (29.3%). Knowledge about benefits of moderate activity increased between 1997 and 1999, more so in States with public health campaigns. National participation in 'sufficient physical activity' declined between 1997 and 1999, from 63% to 57%, but the decline was smaller in NSW/ACT (4.4%) than in the other States (6.0%). Conclusions and implications: Declining trends in physical activity in Australia require increased public health investments, including strategic planning and public education, such as occurred in NSW (1997/98).

Um estudo sobre os resultados da utiliza????o da Bolsa Eletr??nica de Compras no governo do estado de S??o Paulo

Esta ?? uma pesquisa com o objetivo de estudar e analisar os resultados da utiliza????o da Bolsa Eletr??nica de Compras (BEC) no Governo do Estado de S??o Paulo. O estudo permitiu responder a quest??es b??sicas, tais como: relacionamento com fornecedores, investimentos em treinamento, assimila????o, condi????es de trabalho e benef??cios operacionais. O estudo ?? uma pesquisa descritiva, que tenta obter uma descri????o completa e precisa de uma situa????o. A coleta de dados baseou-se em um question??rio, enviado por e-mail, respondido pelos usu??rios e operadores da BEC e pelos fornecedores. Os principais resultados obtidos demonstraram que utiliza????o da BEC n??o melhorou o relacionamento da Administra????o P??blica com seus fornecedores, por??m trouxe mais economia, credibilidade e transpar??ncia ao Governo do Estado de S??o Paulo.

As novas formas de explora????o do trabalho no capitalismo contempor??neo e as pol??ticas p??blicas de combate ?? desigualdade

O mercado de trabalho sofreu profundas transforma????es desde o come??o do capitalismo at?? o momento atual, sendo poss??vel compar??-lo a uma loteria, na qual os trabalhadores passaram a competir entre si por ascens??o profissional. Os bilhetes dessa loteria s??o os investimentos em capital humano. Nela haver??, necessariamente, vencedores e perdedores, com os primeiros apropriando-se do trabalho dos ??ltimos. Dessa realidade, surge uma nova forma de explora????o do trabalho, cujo instrumento de legitima????o ?? o capital humano, que refor??a a tend??ncia inerente do capitalismo ?? gera????o de desigualdade e coloca limita????es ?? vis??o tradicional de combate ?? desigualdade baseada na democratiza????o do capital humano. O crescimento tamb??m tem limita????es na redu????o da desigualdade, tendo em vista que a motiva????o microecon??mica que gera o crescimento macroecon??mico ?? justamente a busca pela desigualdade.

As estatais e as PPPs: o project finance como estrat??gia de garantia de investimentos em infra-estrutura

Este trabalho discute o papel das estatais nas PPPs, de modo a garantir investimentos em infraestrutura pelo financiamento via project finance. Partindo de um contexto de privatiza????es desse setor, que, no Brasil, efetivou-se na d??cada de 1990, buscou-se mostrar como surgiu espa??o para o estabelecimento de PPPs, advindas de novas institui????es resultantes do processo de reforma do Estado. O project finance ?? colocado como uma forma de garantir o financiamento desses investimentos. Sua estrutura financeira, baseada na cria????o de uma pessoa jur??dica para a gest??o do empreendimento, busca dirimir os riscos pela eleva????o do n??mero de participantes e pela securitiza????o de receitas futuras, tornando-as l??quidas em curto prazo. A discuss??o sobre o papel do project finance revela que essa ?? uma estrat??gia vi??vel para a garantia de investimentos por parte das estatais, em parceria com o governo e com outras empresas, que levem ao desenvolvimento socioecon??mico sustent??vel do Pa??s.

Mudan??as organizacionais no processo de moderniza????o da Secretaria da Fazenda do Estado de Pernambuco

A partir de 1998, teve in??cio, na Secretaria da Fazenda do Estado de Pernambuco, o Programa de Moderniza????o Fazend??ria (Promofaz), que promoveu diversas mudan??as organizacionais, concentrando esfor??os em projetos estruturadores, incluindo investimentos no planejamento de tecnologia da informa????o (TI). Dentre os projetos de TI, destacou-se a elabora????o das arquiteturas de sistemas e tecnol??gicas. Neste trabalho, foi realizada uma revis??o dos processos organizacionais da moderniza????o fazend??ria, abrangendo aspectos de cultura, mudan??a e aprendizagem organizacionais, confrontados com referenciais hist??ricos e te??ricos, e maior enfoque na ??rea de tecnologia da informa????o. Apesar das dificuldades, o processo de informatiza????o tem provocado fortes impactos nos componentes culturais da institui????o. Sem a utiliza????o da tecnologia da informa????o como ferramenta, provavelmente n??o se implantariam os novos modelos de gest??o adotados pela Sefaz, mas se observou que a tecnologia, sozinha, n??o faz o milagre da mudan??a, sendo necess??rio todo um conjunto de esfor??os e um trabalho paralelo com outros fatores de mudan??a.