Adult-onset Still`s Disease with Pulmonary and Cardiac Involvement and Response to Intravenous Immunoglobulin

Cardiopulmonary manifestations of adult-onset Still`s disease (AOSD) include pericarditis, pleural effusion, transient pulmonary infiltrates, pulmonary interstitial disease and myocarditis. Serositis are common but pneumonitis and myocarditis are not and bring elevated risk of mortality. They may manifest on disease onset or flares. Previously reported cases were treated with high-dose glucocorticoids and immunosupressants and, when refractory, intravenous immunoglobulin (IVIG). We report an AOSD patient whose flare presented with severe pleupneumonitis and myopericarditis and, following nonresponse to a methylprednisolone pulse, high dose of prednisone and cyclosporine A, recovered after a 2-day 1g/kg/day IVIG infusion.

Long-term follow-up of children with extrahepatic portal vein obstruction: impact of an endoscopic sclerotherapy program on bleeding episodes, hepatic function, hypersplenism, and mortality

Background: Endoscopic sclerotherapy (ES) has been the standard treatment for children with idiopathic extrahepatic portal vein obstruction (EHPVO). Portosystemic shunts are indicated when variceal bleeding cannot be controlled by ES. Recently, mesenteric left portal vein bypass was indicated as a surgical intervention and preventative measure for hepatic dysfunction in children with long-term EHPVO. Nevertheless, there is a lack Of published data confirming the extent of hepatic dysfunction, hypersplenism, and physical development in children with long-term follow-up. Method: We retrospectively verified the long-term outcomes in 82 children with EHPVO treated with ES protocol, focusing on mortality, control of bleeding, hypersplenism, and consequent hepatic dysfunction. Results: Of the children, 56% were free from bleeding after the initiation of ES. The most frequent cause of rebleeding was gastric varices (30%). Four patients had recurrent bleeding from esophageal varices (4.6%). Four patients underwent surgery as a consequence of uncontrolled gastric varices. There were no deaths. Most patients showed good physical development. We observed a mild but statistically significant drop in factor V motion, as well as leukocyte and platelet count. Conclusion: Endoscopic sclerotherapy is an efficient treatment for children with EHPVO. The incidence of rebleeding is low, and there was no mortality. Children develop mild liver dysfunction and hypersplenism with long-term follow-up. Only a few patients manifest symptoms of hypersplenism, portal biliopathy, or liver dysfunction before adolescence. (C) 2009 Elsevier Inc. All rights reserved.

Autoimmune hemolytic anemia in systemic lupus erythematosus: association with thrombocytopenia

Hematological disturbances are common in systemic lupus erythematous (SLE). Specifically, autoimmune hemolytic anemia (AHA) may manifest in SLE patients at the time of diagnosis or within the first year of the disease. AHA is often associated with thrombocytopenia, lupus nephritis, and central nervous system activity. In this study we investigated these associations in Brazilian patients with SLE. Forty-four consecutive SLE patients who had a history of AHA were age, gender, and disease duration matched with 318 SLE patients without AHA who formed the control group. All patients fulfilled the revised American College of Rheumatology criteria for SLE and were followed-up within our Service. Clinical and laboratorial manifestations were similar in both groups, except for the predominance of leukopenia, thrombocytopenia, and anti-dsDNA on univariate analysis in the AHA group. The multivariate logistic regression model revealed risk only for thrombocytopenia in the AHA group compared to the control group (odds ratio, 2.70; 95% confidence interval, 1.32-5.50). Our results corroborate previous data that AHA in SLE increases the risk of thrombocytopenia in individuals with SLE. This association suggests a common mechanism in AHA and SLE pathophysiologies.

Allergic rhinitis in the child and associated comorbidities

Allergic rhinitis (AR) typically presents after the second year of life, but the exact prevalence in early life is unknown. AR affects 10-30% of the population, with the greatest frequency found in children and adolescents. It appears that the prevalence has increased in the pediatric population. As the childs` immune system develops between the 1st and 4th yr of life, those with an atopic predisposition begin to express allergic disease with a clear Th(2) response to allergen exposure, resulting in symptoms. In pediatric AR, two or more seasons of pollen exposure are generally needed for sensitization, so allergy testing to seasonal allergens (trees, grasses, and weeds) should be conducted after the age of 2 or 3 years. Sensitization to perennial allergens (animals, dust mites, and cockroaches) may manifest several months after exposure. Classification of AR includes measurement of frequency and duration of symptoms. Intermittent AR is defined as symptoms for < 4 days/wk or < 4 consecutive weeks. Persistent AR is defined as occurring for more than 4 days/wk and more than 4 consecutive weeks. AR is associated with impairments in quality of life, sleep disorders, emotional problems, and impairment in activities such as work and school productivity and social functioning. AR can also be graded in severity - either mild or moderate/severe. There are comorbidities associated with AR. The chronic effects of the inflammatory process affect lungs, ears, growth, and others. AR can induce medical complications, learning problems and sleep-related complaints, such as obstructive sleep apnea syndrome and chronic and acute sinusitis, acute otitis media, serous otitis media, and aggravation of adenoidal hypertrophy and asthma.

Abdominal compartment syndrome in trauma resuscitation

Purpose of review Swelling is inexorably linked to shock and resuscitation in trauma. In many forms, swelling complicates and interacts with traumatic injury to raise pressures in the abdomen, resulting in intraabdominal hypertension, which may overtly manifest as abdominal compartment syndrome (ACS) driving multiple organ failure. Despite renewed clinical interest in posttraumatic intraabdominal pressure, there remains a chiasm between knowledge of the risks and clinical interventions to mitigate them. This review provides a concise overview of definitions, risk factors, diagnosis and management using an illustrative trauma case. Recent findings Intraabdominal pressure commonly increases following trauma, wherein ACS may manifest earlier than generally appreciated and complicate other insults such as shock and hemorrhage. Contemporary resuscitation strategies may exacerbate intraabdominal hypertension, particularly massive crystalloid resuscitation. Although unproven, the recent transition to crystalloid restriction and high plasma resuscitation strategies may influence the prevalence of ACS. Nonetheless, aggressive intraabdominal pressure monitoring should be mandatory in the critically ill. Despite potential nonoperative options, decompressive laparotomy remains the only definitive but often morbid treatment. Summary ACS results from many dysfunctions acting in concert with each other in self-propagating vicious cycles. Starting with greater awareness, it is imperative that the growing knowledge should be translated into clinical practice.

Short Report: Co-Infection with Paracoccidioidomycosis and Human Immunodeficiency Virus: Report of a Case with Esophageal Involvement

Paracoccidioiodomycosis (PCM) is a systemic and deep mycosis endemic in Latin America, especially in Brazil. In patients infected with human immunodeficiency virus (HIV), PCM can manifest with prominent involvement of the reticuloendothelial system. There are no reports in the literature of esophageal involvement by PCM in that population. We report a case of PCM with pulmonary and esophageal involvement without radiologic evidence of an esophageal-bronchial fistula in an HIV-infected patient.

Short-term urea feeding decreases in vitro hatching of bovine blastocysts

Cows fed high-protein diets may have impaired reproductive performance. Although the pathogenesis has not been completely elucidated, it appears that not only the uterus, but also the follicle and oocyte, are affected by excessive plasma urea nitrogen (PUN) concentrations. Thus, the objective was to determine the effects of short-term urea feeding on the competence of bovine oocytes. Forty crossbred heifers (Bos indicus vs Bos taurus) were allocated to two groups, namely CONTROL (maintenance diet) and UREA (maintenance diet supplemented with 75 g of urea/day), following a cross-over design. Heifers received their respective diets for 6 d (without adaptation). On the sixth day, blood samples were harvested both before and 3 h after feeding, and cumulus oocyte complexes (COCs) were collected by ovum pick-up. Although PUN concentrations were higher in UREA than CONTROL heifers (31.31 mg/dL +/- 1.13 vs 22.12 mg/dL +/- 0.86; mean +/- SEM), neither the number of COCs recovered (8.8 +/- 1.0 vs 9.2 +/- 0.8, UREA vs CONTROL, respectively) nor their quality (based on morphology) differed significantly between groups. Next, oocytes were fertilized and cultured in vitro to assess developmental rates. There was an absence of significant differences between groups for rates of cleavage (Day 3) or blastocyst formation (Days 6, 7 and 9), but the hatched blastocyst rate on Day 11 after fertilization was lower (P < 0.05) in the UREA than the CONTROL groups (64.3 vs 83.5%). Therefore, we inferred that the effects of urea were only manifest later in development. In conclusion, high PUN concentrations decreased oocyte competence in heifers, reinforcing the hypothesis that poor reproductive performance in cows with high PUN was due, at least in part, to a deleterious effect on oocytes. (C) 2011 Elsevier Inc. All rights reserved.

Nonword repetition and young children's receptive vocabulary: A longitudinal study

A longitudinal study investigated the claim that phonological memory contributes to vocabulary acquisition in young children. In the first phase, children were given tests of receptive vocabulary, receptive grammar, nonword repetition, phonological sensitivity (or awareness), and performance IQ. In the second phase, children were given the nonword repetition and receptive vocabulary tests. In Session 1, both nonword repetition and phonological sensitivity accounted for variation in receptive vocabulary and grammar after performance IQ effects were controlled. When phonological sensitivity was also controlled, nonword repetition did not account for significant additional variation in receptive vocabulary and grammar, When performance IQ and autoregression effects were controlled, all Session I verbal ability measures predicted Session 2 vocabulary, but only Session 1 vocabulary predicted Session 2 nonword repetition. When phonological sensitivity was also controlled. Session 1 nonword repetition (leniently scored) predicted Session 2 vocabulary. Overall, these findings show qualified support for the claim that the capacity component of nonword repetition contributes directly to vocabulary in young children. They suggest that the association between nonword repetition and vocabulary in young children may, to a substantial extent, reflect a latent phonological processing ability that is also manifest in phonological sensitivity.

Hormone physiology of pea mutants prevented from flowering by mutations gi or veg1

The veg1 (vegetative) mutant in pea (Pisum sativum L.) does not flower under any circumstances and gi (gigas) mutants remain vegetative under certain conditions. gi plants are deficient in production of floral stimulus, whereas veg1 plants lack a response to floral stimulus. During long days in particular, these non-flowering mutant plants eventually enter a stable compact phase characterised by a large reduction in internode length, small leaves and growth of lateral shoots from the upper-stem (aerial) nodes. The first-order laterals in turn produce second-order laterals and so on in a reiterative pattern. The apical bud is reduced in size but continues active growth. Endogenous hormone measurements and gibberellin application studies with gi-1, gi-2 and veg1 plants indicate that a reduction in gibberellin and perhaps indole-3-acetic acid level may account, at least partially, for the compact aerial shoot phenotype. In the gi-1 mutant, the compact phenotype is rescued by transfer from a 24- to an 8-h photoperiod. We propose that in plants where flowering is prevented by a lack of floral stimulus or an inability to respond, the large reduction in photoperiod gene activity during long days may lead to a reduction in apical sink strength that is manifest in an altered hormone profile and weak apical dominance.

Atom lasers, coherent states, and coherence II. Maximally robust ensembles of pure states

As discussed in the preceding paper [Wiseman and Vaccaro, preceding paper, Phys. Rev. A 65, 043605 (2002)], the stationary state of an optical or atom laser far above threshold is a mixture of coherent field states with random phase, or, equivalently, a Poissonian mixture of number states. We are interested in which, if either, of these descriptions of rho(ss) as a stationary ensemble of pure states, is more natural. In the preceding paper we concentrated upon the question of whether descriptions such as these are physically realizable (PR). In this paper we investigate another relevant aspect of these ensembles, their robustness. A robust ensemble is one for which the pure states that comprise it survive relatively unchanged for a long time under the system evolution. We determine numerically the most robust ensembles as a function of the parameters in the laser model: the self-energy chi of the bosons in the laser mode, and the excess phase noise nu. We find that these most robust ensembles are PR ensembles, or similar to PR ensembles, for all values of these parameters. In the ideal laser limit (nu=chi=0), the most robust states are coherent states. As the phase noise or phase dispersion is increased through nu or the self-interaction of the bosons chi, respectively, the most robust states become more and more amplitude squeezed. We find scaling laws for these states, and give analytical derivations for them. As the phase diffusion or dispersion becomes so large that the laser output is no longer quantum coherent, the most robust states become so squeezed that they cease to have a well-defined coherent amplitude. That is, the quantum coherence of the laser output is manifest in the most robust PR ensemble being an ensemble of states with a well-defined coherent amplitude. This lends support to our approach of regarding robust PR ensembles as the most natural description of the state of the laser mode. It also has interesting implications for atom lasers in particular, for which phase dispersion due to self-interactions is expected to be large.

A framework for extreme-event control in wastewater treatment

In this paper an approach to extreme event control in wastewater treatment plant operation by use of automatic supervisory control is discussed. The framework presented is based on the fact that different operational conditions manifest themselves as clusters in a multivariate measurement space. These clusters are identified and linked to specific and corresponding events by use of principal component analysis and fuzzy c-means clustering. A reduced system model is assigned to each type of extreme event and used to calculate appropriate local controller set points. In earlier work we have shown that this approach is applicable to wastewater treatment control using look-up tables to determine current set points. In this work we focus on the automatic determination of appropriate set points by use of steady state and dynamic predictions. The performance of a relatively simple steady-state supervisory controller is compared with that of a model predictive supervisory controller. Also, a look-up table approach is included in the comparison, as it provides a simple and robust alternative to the steady-state and model predictive controllers, The methodology is illustrated in a simulation study.

MR image-based measurement of rates of change in volumes of brain structures. Part 1: Method and validation

A detailed analysis procedure is described for evaluating rates of volumetric change in brain structures based on structural magnetic resonance (MR) images. In this procedure, a series of image processing tools have been employed to address the problems encountered in measuring rates of change based on structural MR images. These tools include an algorithm for intensity non-uniforniity correction, a robust algorithm for three-dimensional image registration with sub-voxel precision and an algorithm for brain tissue segmentation. However, a unique feature in the procedure is the use of a fractional volume model that has been developed to provide a quantitative measure for the partial volume effect. With this model, the fractional constituent tissue volumes are evaluated for voxels at the tissue boundary that manifest partial volume effect, thus allowing tissue boundaries be defined at a sub-voxel level and in an automated fashion. Validation studies are presented on key algorithms including segmentation and registration. An overall assessment of the method is provided through the evaluation of the rates of brain atrophy in a group of normal elderly subjects for which the rate of brain atrophy due to normal aging is predictably small. An application of the method is given in Part 11 where the rates of brain atrophy in various brain regions are studied in relation to normal aging and Alzheimer's disease. (C) 2002 Elsevier Science Inc. All rights reserved.

A mudan??a de pol??tica na crise do Estado de Bem Estar Social: uma an??lise comparada

O artigo faz uma identifica????o das caracter??sticas da policy change que vem sendo assumidas nos ??ltimos anos, nos diversos setores da interven????o p??blica, com base no exame das mudan??as das pol??ticas nas principais democracias contempor??neas integrantes da OCDE. Focaliza a tend??ncia, que parece ser mais real e de longo prazo, expressa na passagem do Estado social para o Estado gerencial.

O desenho da criança de cinco anos : investigando/refletindo as formas produzidas a partir de imagem da arte

O desenho infantil comprova significados e sentidos estabelecidos historicamente e revela experiências comuns da criança pensar e idealizar o mundo. Diante dessa constatação, o cerne desta pesquisa é investigar e refletir sobre as nuances no desenho da criança de cinco anos quando apresentadas às imagens de arte, analisando e compreendendo como ela se expressa verbal e graficamente diante de informações mediadas pelo professor de arte, quando manifestam suas experiências individuais e/ou em grupo, representando suas vivências e apropriando-se da linguagem artística, e assim constituindo-se como autoras. Para sustentar a pesquisa, adotamos os princípios da pesquisa qualitativa utilizando como recurso a observação participante em sala de aula, que vem ao encontro às nossas propostas por oportunizar o registro de dados in locus, potencializando assim, a estratégia da produção de dados. Os sujeitos dessa pesquisa são vinte e uma crianças de cinco anos que frequentaram o Grupo Cinco, do Centro de Educação Infantil Criarte/Ufes. A pesquisa buscou entender o desenho infantil como expressão intrínseca da criança, que, oportunizado por ações que colaboram para esse fazer, dará significados ao objeto, que, associado a sua história, sua vivência, possivelmente contribuirá para a formação artística e imagética da criança, significando sua expressão e criação. Trazemos para esse estudo conceitos do desenho infantil, bem como questões que abarcam todo o processo de elaboração e maturação do desenho, fundamentados nas pesquisas de Rosa Iavelberg, Analice Pilar, César Cola, Arno Stern e Viktor Lowenfeld, dentre outros, os quais possibilitaram nossa análise a partir do desenho e da fala das crianças. As reflexões apresentadas procuram contribuir para compreendermos o processo de diálogo, envolvimento e representação gráfica das crianças com as imagens de arte.

O trabalho e a competência industriosa no beneficiamento de granitos : uma cartografia ergológica

O intuito inicial desta pesquisa foi acompanhar processos de trabalho à luz do referencial teórico da Ergologia e, portanto, concebendo o trabalho como relação dialética entre técnica e ação humana. O objetivo era cartografar o trabalho no processo de beneficiamento de granitos em uma organização de grande porte localizada no Espírito Santo e, após algum tempo em campo, o problema delineou- se do seguinte modo: como se constitui a competência industriosa no beneficiamento de granitos em uma organização de grande porte? A pesquisa justifica-se uma vez que, a despeito da relevância econômica, o cenário capixaba de rochas ornamentais apresenta problemas precários no que diz respeito à gestão. Para os Estudos Organizacionais, a relevância é reforçada pelo fato de aproximar desta área a abordagem ergológica e demarcar no debate sobre competência a noção de competência industriosa, ainda não explorada nesse campo de estudo. Para realização da pesquisa, foi praticada uma cartografia ergológica, a partir da articulação das pistas cartográficas com o referencial teórico-conceitual da Ergologia, sendo utilizadas como técnicas: observação participante durante 6 meses, com uma média de 3 visitas a campo por semana; 8 entrevistas semiestruturadas e em profundidade de cerca de 50 minutos cada com trabalhadores operacionais; uma entrevista com gerente de produção e outra com representante da área de Gestão de Pessoas; conversas com os demais trabalhadores, a fim de enriquecer o diário de campo; novas conversas e observações ao final da análise, para confrontação-validação com os trabalhadores. A sistematização dos procedimentos de análise pode ser assim descrita: a) leituras flutuantes com objetivo de fazer emergirem aspectos centrais relacionados às duas dimensões do trabalho, técnica e ação humana; b) leituras em profundidade com objetivo de fazer emergirem singularidades e especificidades relativas à dialética entre ambas; c) leituras em profundidade com objetivo de fazer emergirem aspectos relativos aos ingredientes da competência industriosa. A despeito da não delimitação de categorias analíticas e subcategorias, a partir da análise emergiram cinco eixos analíticos: 1) os procedimentos a serem empregados no processo de beneficiamento de granitos, englobando: as etapas do beneficiamento; as funções a serem desempenhadas e as tarefas a serem desenvolvidas; as normas regulamentadoras; os conhecimentos técnicos necessários para programação e operação de máquinas; a ordem de produção prescrita pelo setor comercial; 2) o trabalho real, diferenciado do trabalho como emprego de procedimentos pelo foco dado à ação humana no enfrentamento de situações reais, repletas de eventos e variabilidades, em todo o processo, englobando: preparo de carga; laminação; serrada; levigamento; resinagem; polimento-classificação; retoque; fechamento de pacote; ovada de contêiner; 3) diferentes modos de usos de si que, em tendência, são responsáveis pela constituição do agir em competência em cada etapa do processo, na dialética entre técnica e ação humana; 4) o modo como cada ingrediente da competência industriosa atua e se constitui, bem como sua concentração, em tendência, em cada etapa do processo, a partir dos tipos de usos de si que, também em tendência, são mais responsáveis pelo agir em competência, apresentando assim o perfil da competência industriosa no beneficiamento de granitos na empresa em análise; 5) dois possíveis fatores potencializadores dos ingredientes da competência industriosa, a saber, a transdução e os não-humanos. A partir de todo o exposto, as últimas considerações problematizam aspectos relativos ao debate sobre competências e práticas de gestão de pessoas a partir da competência compreendida da seguinte forma: mestria no ato de tirar partido do meio e de si para gerir situações de trabalho, em que a ação consiste na mobilização de recursos dificilmente perceptíveis e descritíveis, inerentes ao trabalhador, porém constituídos e manifestos por usos de si por si e pelos outros no e para o ato real de trabalho, marcadamente num nível infinitesimal, diante de situações que demandam aplicação de protocolos concomitante à gestão de variabilidades e eventos em parte inantecipáveis e inelimináveis.