Protein defects in neuromuscular diseases

Muscular dystrophies are a heterogeneous group of genetically determined progressive disorders of the muscle with a primary or predominant involvement of the pelvic or shoulder girdle musculature. The clinical course is highly variable, ranging from severe congenital forms with rapid progression to milder forms with later onset and a slower course. In recent years, several proteins from the sarcolemmal muscle membrane (dystrophin, sarcoglycans, dysferlin, caveolin-3), from the extracellular matrix (alpha2-laminin, collagen VI), from the sarcomere (telethonin, myotilin, titin, nebulin), from the muscle cytosol (calpain 3, TRIM32), from the nucleus (emerin, lamin A/C, survival motor neuron protein), and from the glycosylation pathway (fukutin, fukutin-related protein) have been identified. Mutations in their respective genes are responsible for different forms of neuromuscular diseases. Protein analysis using Western blotting or immunohistochemistry with specific antibodies is of the utmost importance for the differential diagnosis and elucidation of the physiopathology of each genetic disorder involved. Recent molecular studies have shown clinical inter- and intra-familial variability in several genetic disorders highlighting the importance of other factors in determining phenotypic expression and the role of possible modifying genes and protein interactions. Developmental studies can help elucidate the mechanism of normal muscle formation and thus muscle regeneration. In the last fifteen years, our research has focused on muscle protein expression, localization and possible interactions in patients affected by different forms of muscular dystrophies. The main objective of this review is to summarize the most recent findings in the field and our own contribution.

Terrorism as war by other means: national security and state support for terrorism

The conventional approach in the discipline of International Relations is to treat terrorist organizations as "non-state" actors of international relations. However, this approach is problematic due to the fact that most terrorist organizations are backed or exploited by some states. In this article, I take issue with the non-stateness of terrorist organizations and seek to answer the question of why so many states, at times, support terrorist organizations. I argue that in the face of rising threats to national security in an age of devastating wars, modern nation states tend to provide support to foreign terrorist organizations that work against their present and imminent enemies. I elaborate on my argument studying three cases of state support for terrorism: Iranian support for Hamas, Syrian support for the PKK, and American support for the MEK. The analyses suggest that, for many states, terror is nothing but war by other means.

Glucosamine and chondroitin sulfate in the repair of osteochondral defects in dogs - clinical-radiographic analysis

Among the proposed treatments to repair lesions of degenerative joint disease (DJD), chondroprotective nutraceuticals composed by glucosamine and chondroitin sulfate are a non-invasive theraphy with properties that favors the health of the cartilage. Although used in human, it is also available for veterinary use with administration in the form of nutritional supplement independent of prescription, since they have registry only in the Inspection Service, which does not require safety and efficacy testing. The lack of such tests to prove efficacy and safety of veterinary medicines required by the Ministry of Agriculture and the lack of scientific studies proving its benefits raises doubts about the efficiency of the concentrations of such active substances. In this context, the objective of this study was to evaluate the efficacy of a veterinary chondroprotective nutraceutical based on chondroitin sulfate and glucosamine in the repair of osteochondral defects in lateral femoral condyle of 48 dogs, through clinical and radiographic analysis. The animals were divided into treatment group (TG) and control group (CG), so that only the TG received the nutraceutical every 24 hours at the rate recommended by the manufacturer. The results of the four treatment times (15, 30, 60 and 90 days) showed that the chondroprotective nutraceutical, in the rate, formulation and administration at the times used, did not improve clinical signs and radiologically did not influence in the repair process of the defects, since the treated and control groups showed similar radiographic findings at the end of the treatments.

An investigation into support for restrictions on HIV carriers in the Chicago metropolitan area

An investigation into support for restrictions on people testing seropositive for HIV is reported on. Data were collected during telephone interviews with two-hundred adults aged eighteen to sixty-five in the Chicago metropolitan area. Using the analytic technique of LISREL, six models which attempt to explain support for restrictions were tested. It was found that the model best supported by the data indicates that two groups contribute to support for restrictions on HIV carriers - one due to intolerance of homosexuality and one to mistrust of public health officials regarding their control and management of the AIDS epidemic. The relevance of these findings for public health policy makers is discussed.

Job control, job demands, social support at work and health among adolescent workers

OBJECTIVE: To evaluate physical and psychological dimensions of adolescent labor (such as job demands, job control, and social support in the work environment), and their relation to reported body pain, work injuries, sleep duration and daily working hours. METHODS: A total of 354 adolescents attending evening classes at a public school in São Paulo, Brazil, answered questionnaires regarding their living and working conditions (Karasek's Job Content Questionnaire, 1998), and their health status. Data collection took place in April and May 2001. Multiple logistic regression analysis was used to determine relations among variables. RESULTS: Psychological job demands were related to body pain (OR=3.3), higher risk of work injuries (OR=3.0) and reduced sleep duration in weekdays (Monday to Thursday) (p<0.01). Lower decision authority in the workplace (p=0.03) and higher job security (p=0.02) were related to longer daily working hours. CONCLUSIONS: It was concluded that besides physical stressors, psychological factors are to be taken into account when studying adolescent working conditions, as they may be associated with negative job conditions and health effects.

Demand for and availability of online support to stop smoking

OBJECTIVES: Estimate the frequency of online searches on the topic of smoking and analyze the quality of online resources available to smokers interested in giving up smoking. METHODS: Search engines were used to revise searches and online resources related to stopping smoking in Brazil in 2010. The number of searches was determined using analytical tools available on Google Ads; the number and type of sites were determined by replicating the search patterns of internet users. The sites were classified according to content (advertising, library of articles and other). The quality of the sites was analyzed using the Smoking Treatment Scale- Content (STS-C) and the Smoking Treatment Scale - Rating (STS-R). RESULTS: A total of 642,446 searches was carried out. Around a third of the 113 sites encountered were of the 'library' type, i.e. they only contained articles, followed by sites containing clinical advertising (18.6) and professional education (10.6). Thirteen of the sites offered advice on quitting directed at smokers. The majority of the sites did not contain evidence-based information, were not interactive and did not have the possibility of communicating with users after the first contact. Other limitations we came across were a lack of financial disclosure as well as no guarantee of privacy concerning information obtained and no distinction made between editorial content and advertisements. CONCLUSIONS: There is a disparity between the high demand for online support in giving up smoking and the scarcity of quality online resources for smokers. It is necessary to develop interactive, customized online resources based on evidence and random clinical testing in order to improve the support available to Brazilian smokers.

Gender differences in social support and leisure-time physical activity

OBJECTIVE To identify gender differences in social support dimensions’ effect on adults’ leisure-time physical activity maintenance, type, and time.METHODS Longitudinal study of 1,278 non-faculty public employees at a university in Rio de Janeiro, RJ, Southeastern Brazil. Physical activity was evaluated using a dichotomous question with a two-week reference period, and further questions concerning leisure-time physical activity type (individual or group) and time spent on the activity. Social support was measured with the Medical Outcomes Study Social Support Scale. For the analysis, logistic regression models were adjusted separately by gender.RESULTS A multinomial logistic regression showed an association between material support and individual activities among women (OR = 2.76; 95%CI 1.2;6.5). Affective support was associated with time spent on leisure-time physical activity only among men (OR = 1.80; 95%CI 1.1;3.2).CONCLUSIONS All dimensions of social support that were examined influenced either the type of, or the time spent on, leisure-time physical activity. In some social support dimensions, the associations detected varied by gender. Future studies should attempt to elucidate the mechanisms involved in these gender differences.

Risk of dependence associated with health, social support, and lifestyle

OBJECTIVE To analyze the prevalence of individuals at risk of dependence and its associated factors.METHODS The study was based on data from the Catalan Health Survey, Spain conducted in 2010 and 2011. Logistic regression models from a random sample of 3,842 individuals aged ≥ 15 years were used to classify individuals according to the state of their personal autonomy. Predictive models were proposed to identify indicators that helped distinguish dependent individuals from those at risk of dependence. Variables on health status, social support, and lifestyles were considered.RESULTS We found that 18.6% of the population presented a risk of dependence, especially after age 65. Compared with this group, individuals who reported dependence (11.0%) had difficulties performing activities of daily living and had to receive support to perform them. Habits such as smoking, excessive alcohol consumption, and being sedentary were associated with a higher probability of dependence, particularly for women.CONCLUSIONS Difficulties in carrying out activities of daily living precede the onset of dependence. Preserving personal autonomy and function without receiving support appear to be a preventive factor. Adopting an active and healthy lifestyle helps reduce the risk of dependence.

Institutional and matrix support and its relationship with primary healthcare

OBJECTIVE To analyze whether the level of institutional and matrix support is associated with better certification of primary healthcare teams.METHODS In this cross-sectional study, we evaluated two kinds of primary healthcare support – 14,489 teams received institutional support and 14,306 teams received matrix support. Logistic regression models were applied. In the institutional support model, the independent variable was “level of support” (as calculated by the sum of supporting activities for both modalities). In the matrix support model, in turn, the independent variables were the supporting activities. The multivariate analysis has considered variables with p < 0.20. The model was adjusted by the Hosmer-Lemeshow test.RESULTS The teams had institutional and matrix supporting activities (84.0% and 85.0%), respectively, with 55.0% of them performing between six and eight activities. For the institutional support, we have observed 1.96 and 3.77 chances for teams who had medium and high levels of support to have very good or good certification, respectively. For the matrix support, the chances of their having very good or good certification were 1.79 and 3.29, respectively. Regarding to the association between institutional support activities and the certification, the very good or good certification was positively associated with self-assessment (OR = 1.95), permanent education (OR = 1.43), shared evaluation (OR = 1.40), and supervision and evaluation of indicators (OR = 1.37). In regards to the matrix support, the very good or good certification was positively associated with permanent education (OR = 1.50), interventions in the territory (OR = 1.30), and discussion in the work processes (OR = 1.23).CONCLUSIONS In Brazil, supporting activities are being incorporated in primary healthcare, and there is an association between the level of support, both matrix and institutional, and the certification result.

Influence of the support offered to breastfeeding by maternity hospitals

ABSTRACT OBJECTIVE To evaluate whether the support offered by maternity hospitals is associated with higher prevalences of exclusive and predominant breastfeeding. METHODS This is a cross-sectional study including a representative sample of 916 infants less than six months who were born in maternity hospitals, in Ribeirao Preto, Sao Paulo, Southeastern Brazil, 2011. The maternity hospitals were evaluated in relation to their fulfillment of the Ten Steps to Successful Breastfeeding. Data were collected regarding breastfeeding patterns, the birth hospital and other characteristics. The individualized effect of the study factor on exclusive and predominant breastfeeding was analyzed using Poisson multiple regression with robust variance. RESULTS Predominant breastfeeding tended to be more prevalent when the number of fulfilled steps was higher (p of linear trend = 0.057). The step related to not offering artificial teats or pacifiers to breastfed infants and that related to encouraging the establishment of breastfeeding support groups were associated, respectively, to a higher prevalence of exclusive (PR = 1.26; 95%CI 1.04;1.54) and predominant breastfeeding (PR = 1.55; 95%CI 1.01;2.39), after an adjustment was performed for confounding variables. CONCLUSIONS We observed a positive association between support offered by maternity hospitals and prevalences of exclusive and predominant breastfeeding. These results can be useful to other locations with similar characteristics (cities with hospitals that fulfill the Ten Steps to Successful Breastfeeding) to provide incentive to breastfeeding, by means of promoting, protecting and supporting breastfeeding in maternity hospitals.

Dot-ELISA-IgM in saliva for the diagnosis of human leptospirosis using polyester fabric-resin as support (Preliminary Report)

In order to improve the diagnosis of human leptospirosis, we standardized the dot-ELISA for the search of specific IgM antibodies in saliva. Saliva and serum samples were collected simultaneously from 20 patients with the icterohemorrhagic form of the disease, from 10 patients with other pathologies and from 5 negative controls. Leptospires of serovars icterohaemorrhagiae, canicola, hebdomadis, brasiliensis and cynopteri grown in EMJH medium and mixed together in equal volumes, were used as antigen at individual protein concentration of 0.2 µg/µl. In the solid phase of the test we used polyester fabric impregnated with N-methylolacrylamide resin. The antigen volume for each test was 1µl, the saliva volume was 8 µl, and the volume of peroxidase-labelled anti-human IgM conjugate was 30 µl. A visual reading was taken after development in freshly prepared chromogen solution. In contrast to the classic nitrocellulose membrane support, the fabric support is easy to obtain and to handle. Saliva can be collected directly onto the support, a fact that facilitates the method and reduces the expenses and risks related to blood processing.

Perceptions of the usefulness of external support to immunization coverage in Guinea-Bissau: a Delphi analysis of the GAVI-Alliance cash-based support

INTRODUCTION: Although many countries have improved vaccination coverage in recent years, some, including Guinea-Bissau, failed to meet expected targets. This paper tries to understand the main barriers to better vaccination coverage in the context of the GAVI-Alliance (The Global Alliance for Vaccines and Immunisation) cash-based support provided to Guinea-Bissau. METHODS: The analysis is based on a document analysis and a three round Delphi study with a final consensus meeting. RESULTS: Consensus attributed about 25% of the failure to perform better to implementation problems; and about 10% to governance and also 10% to scarce resources. The qualitative analysis validates the importance of implementation issues and upgraded the relevance of the human resources crisis as an important drawback. The recommendations were balanced in their upstream-downstream focus but were blind to health information issues and logistical difficulties. CONCLUSIONS: It is commendable that such a fragile state, with all sorts of barriers, manages to sustain a slow steady growth of its vaccination coverage. Not reaching the targets set reflects the inappropriateness of those targets rather than a lack of commitment of the health workforce. In the unstable context of countries such as Guinea-Bissau, the predictability of the funds from global health initiatives like the GAVI-Alliance seem to make all the difference in achieving small consistent health gains even in the presence of other major bottlenecks.

Pelvic lymphoscintigraphy: contribution to the preoperative staging of rectal cancer

PURPOSE: Preservation of the anal sphincter in surgery for cancer of the distal rectum in an attempt to avoid colostomy has been a main concern of colorectal surgeons. Various proposed procedures contradict oncological principles, especially with respect to pelvic lymphadenectomy. Therefore, prior knowledge of pelvic lymph node involvement is an important factor in choosing the operative technique, i.e., radical or conservative resection. Introduction of ultrasound, computerized tomography, and magnetic resonance have made preoperative study of the area possible. Nevertheless, these resources offer information of an anatomical nature only. Lymphoscintigraphy enables the morphological and functional evaluation of the pelvic area and contributes toward complementing the data obtained with the other imaging techniques. The objective of this prospective study is twofold: to standardize the lymphoscintigraphy technique and to use it to differentiate patients with rectal cancer from those with other coloproctologic diseases. CASUISTIC AND METHODS: Sixty patients with various coloproctologic diseases were studied prospectively. Ages ranged from 21 to 96 years (average, 51 and median, 55 years). Twenty-six patients were male and 34 were female. Thirty patients had carcinoma of the distal rectum as diagnosed by proctologic and anatomic-pathologic examinations, 20 patients had hemorrhoids, 5 had chagasic megacolon, 2 had diverticular disease, 2 had neoplasm of the right colon, and 1 had ulcerative colitis as diagnosed by proctologic exam and/or enema. The lymphoscintigraphy method consisted of injecting 0.25 mL of a dextran solution marked with radioactive technetium-99m into the right and left sides of the perianal region and obtaining images with a gamma camera. The results were analyzed statistically with a confidence level of 95% (P < .05) using the following statistical techniques: arithmetic and medium average, Fisher exact test, chi-square test corrected for continuity according to Yates, and distribution tables for the number of patients. RESULTS: In rectal cancer, the tracer progresses unilaterally or is absent; in other patients, the progress of the tracer is bilateral and symmetrical, although its progress may be slow. Statistical tests showed with high significance that the agreement index between the clinical diagnosis and the result of the lymphoscintigraphic exam was 93%. CONCLUSIONS: Lymphoscintigraphy is a standardized, painless, and harmless test that can be performed in all cases; it differentiates patients with rectal cancer from those with other coloproctological diseases.

Percutaneous closure of atrial septal defects. The role of transesophageal echocardiography

PURPOSE: Evaluation of the role of transesophageal echocardiography in percutaneous closure of atrial septal defects (ASD) with the Amplatzer septal occluder. METHODS: Patients were selected for percutaneous closure of ASD by transesophageal echocardiography (TEE), which was also used to monitor the procedure, helping to select the appropriate size of the Amplatzer device, to verify its position, and to access the immediate results of the procedure. During the follow-up, TEE was used to evaluate the presence and magnitude of residual shunt (RS), device position, and right cardiac chamber diameters. RESULTS: Twenty-two (40%) of a total of 55 studied patients were selected. Thirteen underwent Amplatzer device implantation, eight are still waiting for it, and one preferred the conventional surgical treatment. All procedures were successful, which was mainly due to proper patient selection. Six (23%) patients acutely developed RS, which spontaneously disapeared at the three-month follow-up examination in three patients. There was a significant reduction in the right ventricle diastolic diameter, from 27mm (average) to 24mm and 20mm, one and three months after the procedure, respectively (p<0.0076). CONCLUSION: With the aid of TEE, percutaneous closure of ASD can be successfully, safely, and effectively performed.