Primary immunodeficiencies unravel critical aspects of the pathophysiology of autoimmunity and of the genetics of autoimmune disease

Background Primary Immunodeficiencies (PIDs) represent unique opportunities to understand the operation of the human immune system. Accordingly, PIDs associated with autoimmune manifestations provide insights into the pathophysiology of autoimmunity as well as into the genetics of autoimmune diseases (AID). Epidemiological data show that there are PIDs systematically associated with AID, such as immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX), Omenn syndrome, autoinunune polyendocrinopathy-candidiasis-ectodertnal dystrophy (APECED), autoinumine lymphoproliferative syndrome (ALPS), and C1q deficiency, while strong associations are seen with a handful of other deficits. Conclusion We interpret such stringent disease associations, together with a wealth of observations in experimental systems, as indicating first of all that natural tolerance to body components is an active, dominant process involving many of the components that ensure responsiveness, rather than, as previously believed, the result of the mere purge of autoreactivities. More precisely, it seems that deficits of Treg cell development, functions, numbers, and T cell receptor repertoire are among the main factors for autoimmunity pathogenesis in many (if not all) PIDs most frequently presenting with autoimmune features. Clearly, other pathophysiological mechanisms are also involved in autoimmunity, but these seem less critical in the process of self-tolerance. Comparing the clinical picture of IPEX cases with those, much less severe, of ALPS or APECED, provides some assessment of the relative importance of each set of mechanisms.

Analyses of Individual and Organizational Competences Associated with Knowledge Management Practice

The research analyzed critical aspects of the knowledge management process based on the analyses of knowledge, abilities and attitudes required to individual knowledge workers and to organizations responsible for the management process. In the present work a characterization of the knowledge management process was developed and information and knowledge wokers defined. Competence concept was discussed and specialists gave opinions about critical competences to knowledge management process. The opinions were organized and analyzed by the Delphi method. The results aggregate to the management context by discussing an extremely important resource to organizations - knowledge - and because they support its management process. The research identified wide critical aspects that are compatible with current organizational challenges, directing the process management to important themes as: the worker able to create, the organization able to convert individual knowledge into organizational knowledge, knowledge sharing while still tacit, the maximization organizational knowledge use, information and knowledge generation and preservation, among others important topics to be observed by knowledge workers and by administrators responsible for the knowledge management process.

Nutritional aspects of the prevention and treatment of osteoporosis

Osteoporosis is a global health problem characterized by low bone mass and microarchitectural deterioration of bone tissue with a consequent increase in bone fragility and susceptibility to fracture. Nutrition plays a critical role in reducing the risk of osteoporosis through its effect on all of these fragility factors, especially on the development and maintenance of bone mass. An adequate calcium, vitamin D and protein intake resulted in reduced bone remodeling, better calcium retention, reduced age-related bone loss, and reduced fracture risk. Recent evidence indicates that a healthy dietary pattern including dairy products (mainly fat free), fruit and vegetables and adequate amounts of meat, fish and poultry is positively related to bone health. Furthermore, mineral and vitamin supplementation should be closely monitored by health professionals since it could have adverse effects and be insufficient to ensure optimal protection of bone health.

Nutrional aspects of the prevention and treatment of osteoporosis

A osteoporose é um problema de saúde global, caracterizada por baixa massa óssea e deterioração da microarquitetura do tecido ósseo, com consequente aumento da fragilidade óssea e suscetibilidade a fraturas. A nutrição desempenha um papel fundamental na redução do risco de osteoporose por seu efeito sobre todos os fatores relacionados à fragilidade óssea, principalmente no desenvolvimento e na manutenção da massa óssea. Uma adequada ingestão de cálcio, vitamina D e proteína leva à redução da remodelação óssea, à maior retenção de cálcio, à redução da perda óssea relacionada à idade e à redução do risco de fraturas. Evidências recentes indicam que uma alimentação saudável, incluindo a ingestão de produtos lácteos (principalmente os desnatados), frutas e verduras, e uma quantidade adequada de carnes, peixes e aves, está relacionada positivamente com a saúde óssea. Além disso, a suplementação de vitaminas e minerais deve ser monitorada de perto, por profissionais de saúde, uma vez que pode ter efeitos adversos e ser insuficiente para assegurar uma eficaz proteção à saúde óssea

Coping by relatives of critical care patients

OBJECTIVE: To describe the coping strategies used by the relatives of patients hospitalized in an intensive care unit. METHODS: This is a descriptive study that uses a convenience sample and both qualitative and quantitative methods. The study was conducted at a tertiary university hospital in Brazil. Participants included 41 relatives who were selected during the first 96 hours of patient hospitalization in the intensive care unit. RESULTS: The participants reported that they more frequently used Coping Strategies Based on the Stressor, followed by Religiosity/Fantasy Thinking and Seeking for Social Support. There was a statistically significant relationship (P <.01) between the use of the strategy Seeking for Social Support and elevated Acute Physiology, Age, and Chronic Health Evaluation 11 scores. Qualitative analysis allowed a clearer understanding of the relation between the patient`s condition and changes in the coping strategies used by the patient`s relatives. CONCLUSION: This study describes the coping strategies used by patients` relatives during the early hospitalization period. This investigation allowed for a better understanding of the relatives` psychologic aspects and their relation with the patient`s clinical condition. The results shall assist the design of specific interventions directed at facilitating positive coping responses on the part of relatives. (Heart Lung (R) 200 38:217-227.)

Spectrofluorimetric Determination of Second Critical Micellar Concentration of SDS and SDS/Brij 30 Systems

Potentially useful stead-state fluorimetric technique was used to determine the critical micellar concentrations (CMC(1) and CMC(2)) for two micellar media, one formed by SDS and the other by SDS/Brij 30. A comparative study based on conductimetric and surfacial tension measurements suggests that the CMC(1) estimated by the fluorimetric method is lower than the value estimated by these other techniques. Equivalent values were observed for SDS micelles without Brij 30 neutral co-surfactant. The use of acridine orange as fluorescent probe permitted to determine both CMC(1) and CMC(2). Based on it an explanation on aspects of micelle formation mechanism is presented, particularly based on a spherical and a rod like structures.

Repair of critical-size defects with autogenous periosteum-derived cells combined with bovine anorganic apatite/collagen: an experimental study in rat calvaria

The aim of this study was to evaluate the bone repair using autogenous periosteum-derived cells (PDC) and bovine anorganic apatite and collagen (HA-COL). PDC from Wistar rats (n=10) were seeded on HA-COL discs and subjected to osteoinduction during 6 days. Critical-size defects in rat calvarias were treated with blood clot (G1), autogenous bone (G2), HA-COL (G3) and HA-COL combined with PDC (G4) (n=40), and then analyzed 1 and 3 months after surgeries. Radiographic analysis exhibited no significant temporal change. G1 and G2 had discrete new marginal bone, but the radiopacity of graft materials in G2, G3 and G4 impaired the detection of osteogenesis. At 3 months, histopathological analysis showed the presence of ossification islets in G1, which was more evident in G2, homogeneous new bone around HA-COL in G3 and heterogeneous new bone around HA-COL in G4 in addition to moderate presence of foreign body cells in G3 and G4. Histomorphometric analysis showed no change in the volume density of xenograft (p>0.05) and bone volume density in G2 was twice greater than in G1 and G4 after 3 months (p<0.05), but similar to G3. The PDC did not increase bone formation in vivo, although the biomaterial alone showed biocompatibility and osteoconduction capacity.

Critical applications of SW 846 US EPA methods to evaluation of marine samples quality

Technical evaluation of analytical data is of extreme relevance considering it can be used for comparisons with environmental quality standards and decision-making as related to the management of disposal of dredged sediments and the evaluation of salt and brackish water quality in accordance with CONAMA 357/05 Resolution. It is, therefore, essential that the project manager discusses the environmental agency's technical requirements with the laboratory contracted for the follow-up of the analysis underway and even with a view to possible re-analysis when anomalous data are identified. The main technical requirements are: (1) method quantitation limits (QLs) should fall below environmental standards; (2) analyses should be carried out in laboratories whose analytical scope is accredited by the National Institute of Metrology (INMETRO) or qualified or accepted by a licensing agency; (3) chain of custody should be provided in order to ensure sample traceability; (4) control charts should be provided to prove method performance; (5) certified reference material analysis or, if that is not available, matrix spike analysis, should be undertaken and (6) chromatograms should be included in the analytical report. Within this context and with a view to helping environmental managers in analytical report evaluation, this work has as objectives the discussion of the limitations of the application of SW 846 US EPA methods to marine samples, the consequences of having data based on method detection limits (MDL) and not sample quantitation limits (SQL), and present possible modifications of the principal method applied by laboratories in order to comply with environmental quality standards.

Culex quinquefasciatus vitellogenesis: morphological and biochemical aspects

The vitellogenic process in Culex quinquefasciatus, which is triggered by a blood meal, involves the synthesis, distribution and storage of the nutrients necessary for embryo development. The fat body of an adult female Cx. quinquefasciatus revealed two cell types: large trophocytes and small, eosinophilic, "oenocyte-like" cells, which show no morphological changes throughout the gonotrophic cycle. Trophocytes, which only begin to synthesise vitellogenin (Vg) 12 h post-blood meal (PBM), undergo a series of morphological changes following engorgement. These changes include the expansion of the rough endoplasmic reticulum (RER) and Golgi complex, which are later destroyed by autophagosomes. At 84 h PBM, trophocytes return to their pre-engorgement morphology. The ovarian follicles of non-blood-fed Cx. quinquefasciatus contain a cluster of eight undifferentiated cells surrounded by follicular epithelium. After engorgement, the oocyte membrane facing the perioocytic space increases its absorptive surface by microvilli development; large amounts of Vg and lipids are stored between 24 and 48 h PBM. Along with yolk storage in the oocyte, follicular cells exhibit the development of RER cisternae and electron-dense granules begin to fill the perioocytic space, possibly giving rise to endochorion. Later in the gonotrophic cycle, electron-dense vesicles, which are possible exochorion precursors, fuse at the apical membrane of follicular cells. This fusion is followed by follicular cell degeneration.

An analytical solution for the critical number of particles for stable bose-einstein condensation under the influence of an anisotropic potential

We have considered a Bose gas in an anisotropic potential. Applying the the Gross-Pitaevskii Equation (GPE) for a confined dilute atomic gas, we have used the methods of optimized perturbation theory and self-similar root approximants, to obtain an analytical formula for the critical number of particles as a function of the anisotropy parameter for the potential. The spectrum of the GPE is also discussed.

Some aspects of acetylation of untreated and mercerized sisal cellulose

We report here on some aspects of the acetylation in LiCl/N,N-dimethylacetamide, DMAc, of untreated and mercerized sisal cellulose, hereafter designated as sisal and M-sisal, respectively. Fiber mercerization by NaOH solution has resulted in the following changes: 29.9% decrease in the index of crystallinity; 16.2% decrease in the degree of polymerization and 9.3% increase in α-cellulose content. A light scattering study of solutions of sisal, M-sisal, microcrystalline and cotton celluloses in LiCl/DMAc has shown that they are present as aggregates, with (an apparent) average aggregation numbers of 5.2, 3.2, 9.8, and 35.3, respectively. The presence of these aggregates affects the accessibility of cellulose during its functionalization. A study of the evolution of the degree of substitution, DS, of cellulose acetate as a function of reaction time showed an increase up to 5 h, followed by a decrease at 7 h. Possible reasons for this decrease are discussed. As expected, M-sisal gave a higher DS that its untreated counterpart.

Ecological aspects of the surf-zone ichthyofauna of Itamambuca Beach, Ubatuba, SP

Sandy beaches are among the most extensive environments in Brazil and are generally threatened by disorderly urban growth due to their location along the coast, where human populations tend to concentrate. This is especially true in southeastern Brazil, where the largest urban areas are located. Thus, better knowledge regarding these natural resources is of considerable importance. The surf zone of sandy beaches is inhabited by a number of fish species, including juveniles seeking both food and shelter from predators. An ecological survey was conducted in the surf zone of Itamambuca beach in the city of Ubatuba, São Paulo State, Brazil, in order to evaluate the composition, structure and dynamics of the fish fauna using community descriptors. Diurnal and nocturnal samples were taken with a beach seine at five oceanographic stations over a 12-month period. Water temperature and salinity were also recorded. A total of 2,147 individuals representing 34 species and 18 families were sampled. The main variation in fish fauna was detected on the diel level, followed by a spatial repartition possibly related to salinity. Seasonal variation was also observed. The five most important species in the overall context were Atherinella brasiliensis, Eucinostomus melanopterus, Mugil sp., Trachinotus goodei and Atherinella blackburni.

American cutaneous leishmaniasis in the Pontal of Paranapanema - SP, Brazil: ecological and entomological aspects

American cutaneous leishmaniasis (ACL) occurs in epidemic outbreaks and in sporadic cases with small annual variation in the Pontal of Paranapanema, SP. There is little research on the sandfly fauna of this region. The last outbreaks were related to the Movement of the Landless Workers (MST) and with the ecological tourism in preserved forest of the Parque Estadual do Morro do Diabo (PEMD). AIM: identification of the sandfly fauna within the PEMD, mainly anthropophilic species already incriminated as vectors of ACL, as well as their seasonality, hourly frequency and data of the behavior. M&M: The captures were undertaken with CDC light and Shannon traps from 6:00 pm to 10:00 pm, monthly from May 2000 to December 2001. The temperature and relative humidity data were registered at hourly intervals. RESULTS: The captured species were: Brumptomyia brumpti, Nyssomyia neivai, Nyssomyia whitmani, Pintomyia fischeri and Pintomyia pessoai. The P. pessoai predominated (34.39%) and N. neivai was less found (0.74%), only being captured in CDC traps. Shannon trap captured more sandflies (63.01%) than the CDC traps (36.99%). Despite the environmental degradation anthropophilic species, indicates favorable bioecological conditions for persistence of vectors and potential transmission of leishmaniasis.

Epidemiological and ecological aspects related to malaria in the area of influence of the lake at Porto Primavera dam, in western São Paulo State, Brazil

A study was carried out in the area of influence of the Porto Primavera Hydroelectric Power Station, in western São Paulo State, to investigate ecological and epidemiological aspects of malaria in the area and monitor the profile of the anopheline populations following the environmental changes brought about by the construction of the lake. Mosquitoes captured were analyzed by standardized indicator species analysis (ISA) before and during different flooding phases (253 m and 257 m elevations). The local human population was studied by means of parasitological (thin/thick blood smears), molecular (PCR) and serological tests. Serological tests consisted of Enzyme Linked Immunosorbent Assay (ELISA) with synthetic peptides of the circumsporozoite protein (CSP) from classic Plasmodium vivax, P. vivax variants (VK247 and "vivax-like"), P. malariae and P. falciparum and Indirect Immunofluorescence Assay (IFA) with asexual forms of P. vivax, P. malariae and P. falciparum. The results of the entomological survey indicated that, although the Anopheles darlingi population increased after the flooding, the population density remained very low. No malaria, parasite infection or DNA was detected in the inhabitants of the study area. However, there was a low frequency of antibodies against asexual forms and a significant prevalence of antibodies against P. vivax, P. vivax variants, P. falciparum and P. malariae; the presence of these antibodies may result from recent or less recent contact with human or simian Plasmodium (a parallel study in the same area revealed the existence of a sylvatic cycle). Nevertheless, these results suggest that, as in other places where malaria is present and potential vectors circulate, the local epidemiological conditions observed could potentially support the transmission of malaria in Porto Primavera Lake if infected individuals are introduced in sufficient numbers. Further studies are required to elucidate the phenomena described in this paper.

Avaliação da qualidade da atenção básica utilizando a doença respiratória da infância como traçador, em um distrito sanitário do município de São Paulo

OBJECTIVES: to assess quality of the primary care using respiratory diseases as a tracer. METHODS: the evaluative approach is based on Donabedian's referencial connected to Kessner's tracers methodology. The sample is formed by 768 children from 0 to 5 years old, presenting respiratory symptoms, attended at seven basic health units from the Sanitary District of Brasilândia, in the city of São Paulo, State of São Paulo, Brazil. Medical registrations and interviews with mothers or responsibles were the information sources. RESULTS: more accurate child medical examination evidences significant association with favorable evolution, mainly on moderate clinical forms. Although aspects related to the accessibility demonstrated by the health team seem to influence decisively on care quality evaluated by users, mainly the medical doctor. Mothers education level appears to have no influence in the favorable evolution. CONCLUSIONS: the tracer methodology has the potentiality to facilitate the critical analysis of the health care. It is important to focus on the outcome of the health care. The children respiratory diseases are important tracer for the quality assessment on health services.