75 resultados para Autoinflammatory syndromes


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Abstract OBJECTIVE To assess the nursing workload (NW) in Semi-intensive Therapy Unit, specialized in the care of children with Craniofacial anomalies and associated syndromes; to compare the amount of workforce required according to the Nursing Activities Score (NAS) and the COFEN Resolution 293/04. METHOD Cross-sectional study, whose sample was composed of 72 patients. Nursing workload was assessed through retrospective application of the NAS. RESULTS the NAS mean was 49.5%. Nursing workload for the last day of hospitalization was lower in patients being discharged to home (p<0.001) and higher on the first compared to last day of hospitalization (p< 0.001). The number of professionals required according to NAS was superior to the COFEN Resolution 293/04, being 17 and 14, respectively. CONCLUSION the nursing workload corresponded to approximately 50% of the working time of nursing professional and was influenced by day and outcome of hospitalization. The amount of professionals was greater than that determined by the existing legislation.

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This study describes the reproductive system of Stachytarpheta maximiliani (Verbenaceae), including its floral biology, nectar and pollen availability and insect foraging patterns, identifying whose species act as pollinators. It was carried out in a Brazilian Atlantic rain forest site. Observations on the pollination biology of the Verbenaceae S. maximiliani indicate that their flowering period extends from September through May. Anthesis occurs from 5:30 a.m. to 5:00 p.m. and nectar and pollen are available during all the anthesis. Many species of beetles, hemipterans, flies, wasps, bees and butterflies visit their flowers, but bees and butterflies are the most frequent visitors. The flowers are generally small, gathered in dense showy inflorescences. A complex of floral characteristcs, such as violet-blue color of flowers, long floral tubes, without scents, nectar not exposed, high concentration of sugar in nectar (about 32%), allowed identification of floral syndromes (melittophily and psicophily) and function for each visitor. The bees, Bombus morio, B. atratus, Trigonopedia ferruginea, Xylocopa brasilianorum and Apis mellifera and the butterflies Corticea mendica mendica, Corticea sp., Vehilius clavicula, Urbanus simplicius, U. teleus and Heraclides thoas brasiliensis, are the most important pollinators.

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Iliotibial band lesions constitute a potential source of discomfort and are frequently confounded with other conditions which cause pain on the lateral aspect of the hip, thigh and knee. Ultrasonography is usually regarded as a first-line imaging modality in the assessment of such conditions because of its excellent diagnostic accuracy, low cost and wide availability. The correct identification of the structure involved in the production of symptoms leads to an appropriate management and to a higher probability of resolution of the clinical complaint. The present article is aimed at reviewing the different sonographic presentations of iliotibial band syndromes.

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Whole-body imaging in children was classically performed with radiography, positron-emission tomography, either combined or not with computed tomography, the latter with the disadvantage of exposure to ionizing radiation. Whole-body magnetic resonance imaging (MRI), in association with the recently developed metabolic and functional techniques such as diffusion-weighted imaging, has brought the advantage of a comprehensive evaluation of pediatric patients without the risks inherent to ionizing radiation usually present in other conventional imaging methods. It is a rapid and sensitive method, particularly in pediatrics, for detecting and monitoring multifocal lesions in the body as a whole. In pediatrics, it is utilized for both oncologic and non-oncologic indications such as screening and diagnosis of tumors in patients with genetic syndromes, evaluation of disease extent and staging, evaluation of therapeutic response and post-therapy follow-up, evaluation of non neoplastic diseases such as multifocal osteomyelitis, vascular malformations and syndromes affecting multiple regions of the body. The present review was aimed at describing the major indications of whole-body MRI in pediatrics added of technical considerations.

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AbstractPenetrating atherosclerotic aortic ulcer is a rare entity with poor prognosis in the setting of acute aortic syndrome. In the literature, cases like the present one, located in the aortic arch, starting with chest pain and evolving with dysphonia, are even rarer. The present report emphasizes the role played by computed tomography in the diagnosis of penetrating atherosclerotic ulcer as well as in the differentiation of this condition from other acute aortic syndromes. Additionally, the authors describe a new therapeutic approach represented by a hybrid endovascular surgical procedure for treatment of the disease.

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Glucosidases are involved in key steps in the processing of oligosaccharides by cleaving O-glucose residues. Since they catalyze breaking and transfer reactions of glucosidic groups for the normal growth and development of all the cells, defects or genetic deficiencies in these enzymes are associated with serious disorders of the carbohydrate metabolism. Thus, glucosidases represent important targets to develop inhibitors, owing to their potential activities against viruses, tumoral growth and metastasis, diabetes, Gaucher's disease and other syndromes associated with the lisosomal storage of glucoesphingolipids, and osteoarthritis. This paper presents a description of the biochemical pathways and mechanisms of alpha and beta-glucosidases, and the currently available drugs capable to inhibit these enzymes.

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The autors report a case of jejunal mucosa prolapse after gastroenteroanastomosis, a rare postoperative complication. In the late postcholecistectomy period the patient had persistent vomit. Upper digestive endoscopy (UDE) showed obstruction of the second portion of duodenum, and a gastrojejunal anastomosis was performed. Soon after that, the patient had persistent vomit and upper digestive endoscopy (UDE) showed invagination of the jejunal mucosa. She was reoperated, a Roux Y gastrectomy was performed and the patient had a good evolution. The treatment for this complication is basically surgical, which intends to realieve the obstructive symptomatology.

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Most patients with partiaI gastrectomy have no postoperative problems. Some of them, however, develop severe nutritional disturbance, particularly those with Bilroth II reconstruction, which can be treated clinically in the majority of the cases. Those with important mal absorptive problems require surgery. In this article we present a technical alternative for those patients who need reintervention over a BII operation. Our technique has the advantage of not touching the duodenum previously sutured.

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Porcine circovirus types 1 and 2 (PCV1, PCV2) and porcine parvovirus (PPV) are widespread in pig populations around the world. Nevertheless, only PCV2 has been associated with different clinical syndromes, thus representing a major problem to the pig industry. The association of cases of swine abortions and stillborns with PCV1 and PCV2 and PPV was studied retrospectively (2005-2007). Additional pathogens were also investigated in lesioned fetuses. The studied litters included stillborn piglets and several mummified fetuses of varied sizes. Ventricular dilatation, myocardial pale areas, and mesocolic edema were the gross lesions. Escherichia coli was detected as co-infecting with PCV2 the cases in which mesocolic edema was seen. Microscopic lesions included non-suppurative myocarditis, myocardial necrosis and fibrosis, mineralization foci and intranuclear inclusion bodies in cardiomyocytes, and interstitial mononuclear pneumonia. Samples from 7 (5.78 per cent) of 121 aborted fetuses and stillborn piglets had lesions consistent with a viral cause and showed both positive anti-PCV2 immunostaining as well as PCV2-PCR. In samples from 3 (2.47 per cent) of these 7 fetuses, co-infection with PPV was confirmed by Nested-PCR. Both viruses were detected in fetuses at different stages of gestation. Viral antigens of PCV2 were detected by immunohistochemistry mainly in macrophages and myocytes. PCV1 individually was not detected in any of these affected fetuses, but it was associated with PCV2 and/or PPV in some of them. These findings indicate that PCV2 alone or in association with PPV should be kept in mind when investigating causes of infectious abortion in pigs in Brazil.

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Phalaris angusta is a South American natural grass that produces poisoning in sheep and cattle in Argentina and Brazil. Phalaris spp. can produce unrelated forms of poisoning in ruminants, acute and chronic syndromes. The objective of this paper was to describe an outbreak of acute and chronic Phalaris nervous syndrome in 53 of 980 fattening steers and heifers in a farm of Buenos Aires province. On September of 2006 the animals developed nervous signs and died after 3-5 days. The herd was removed to a phalarisfree pasture. Three months later (on December) 15 new clinical cases developed in the herd. Necropsy performed in one affected calf showed neither grossly nor microscopic changes. Microscopically, there were no major alterations in tissues. Nervous signs had been described in some field cases where neither pigment deposition nor axonal degeneration could be detected. Clinical findings displayed by affected cattle after consumption of Phalaris angusta pastures resemble those observed by other authors in Phalaris staggers. This is the first report in Argentina where both syndromes were seen in the same herd.

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This paper describes the epidemiological, clinical and immunohistochemical characteristics of canine lymphomas diagnosed in the region of Porto Alegre, Brazil. Thirty dogs were enrolled in the study; most of them were male (60%), mixed-breed (23%) and middle-aged or older. The majority (87%) of affected dogs showed the multicentric form. The B-cell phenotype was most frequently detected (62%); 37% of the animals were in clinical stage IV, and 83% were classified as sub-stage "b". Lymphadenopathy was observed in 67% of the cases, and dyspnea, prostration, decreased appetite and vomiting were the most common clinical signs encountered. Anemia was a frequently encountered laboratory alteration (57%), as were leukocytosis (40%), thrombocytopenia (33%), lymphopenia (30%), hyperglobulinemia (20%) and hypercalcemia (13%). The results of this study indicate that the clinical features of dogs with lymphoma in the region of Porto Alegre are similar to those observed worldwide.

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O objetivo deste trabalho foi identificar as proporções das síndromes de dispersão de sementes em áreas de cerrado sensu stricto do Brasil Central, comparando-as com as de áreas isoladas de savanas amazônicas. Foram utilizadas listas de espécies de nove áreas de cerrado e cinco áreas de savanas amazônicas. Determinou-se a similaridade florística entre as áreas. As síndromes de dispersão de espécies ocorrentes nestas áreas foram determinadas a partir de dados da literatura. Utilizou-se o teste Qui-quadrado (chi²) a fim de detectar possíveis diferenças nas proporções de zoocoria e anemocoria entre as áreas de cerrado e entre as áreas de savanas amazônicas. As médias entre as duas regiões foram comparadas pelo teste t. Não foram encontradas diferenças significativas para as duas análises (p > 0,05). A média das proporções de zoocoria para o cerrado sensu stricto foi de 56,7% ± 5,4 DP e para as savanas amazônicas 55,6% ± 11,7 DP, e a média das proporções de anemocoria no cerrado sensu stricto foi de 39,7% ± 6,2 DP e para as savanas amazônicas 44,4% ± 11,7 DP. No cerrado sensu stricto, a autocoria variou de 1,4 a 5%, porém não se verificou a presença de espécies autocóricas nas savanas amazônicas. A autocoria é uma estratégia de dispersão raramente encontrada no cerrado e sua ausência nas áreas de savanas amazônicas pode estar relacionada à sua baixa eficiência em colonizar áreas isoladas.

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One dune habitat in the semi-arid Caatinga Biome, rich in endemisms, is described based on plant species composition, woody plant density, mean height and phenology and a multivariate analysis of the micro-habitats generated by variables associated to plants and topography. The local flora is composed mainly by typically sand-dweller species of Caatinga, suggesting the existence of a phytogeographic unity related to the sandy areas in the Caatinga biome, which seems to be corroborated by faunal distribution. Moreover, some species are probably endemic from the dunes, a pattern also found in vertebrates. The plant distribution is patchy, there is no conspicuous herbaceous layer and almost 50% of the ground represents exposed sand. Phenology is not synchronized among species, occurring leaves budding and shedding, flowers development and anthesis, fruits production and dispersion both in rainy and dry seasons. Leaf shedding is low compared to the level usually observed in Caatinga areas and about 50% of the woody individuals were producing leaves in both seasons. Spectrum of dispersal syndromes shows an unexpected higher proportion of zoochorous species among the phanerophytes, accounting for 31.3% of the species, 78.7% of the total frequency and 78.6% of the total density. The habitat of the dunes is very simple and homogeneous in structure and most of environmental variance in the area is explained by one gradient of woody plants density and another of increase of Bromelia antiacantha Bertol. (Bromeliaceae) and Tacinga inamoena (K. Schum.) N.P. Taylor & Stuppy (Cactaceae) toward valleys, which seem to determine two kinds of protected micro-habitats for the small cursorial fauna.

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Several human genetic syndromes have long been recognized to be defective in DNA repair mechanisms. This was first discovered by Cleaver (1968), who showed that cells from patients with xeroderma pigmentosum (XP) were defective for the ability to remove ultraviolet (UV)-induced lesions from their genome. Since then, new discoveries have promoted DNA repair studies to one of the most exciting areas of molecular biology. The present work intends to give a brief summary of the main known human genetic diseases related to DNA repair and how they may be linked to acquired diseases such as cancer

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We describe the identification of point mutations in the androgen receptor gene in five Brazilian patients with female assignment and behavior. The eight exons of the gene were amplified by the polymerase chain reaction (PCR) and analyzed for single-strand conformation polymorphism (SSCP) to detect the mutations. Direct sequencing of the mutant PCR products demonstrated single transitions in three of these cases: G®A in case 1, within exon C, changing codon 615 from Arg to His; G®A in case 2, within exon E, changing codon 752 from Arg to Gln, and C®T in case 3, within exon B, but without amino acid change.