Enzyme replacement therapy for Anderson-Fabry disease.

Anderson-Fabry disease is an X-linked defect of glycosphingolipid metabolism. Progressive renal insufficiency is a major source of morbidity, additional complications result from cardio- and cerebro-vascular involvement. Survival is reduced among affected males and symptomatic female carriers. To evaluate the effectiveness and safety of enzyme replacement therapy compared to other interventions, placebo or no interventions, for treating Anderson-Fabry disease. We searched 'Clinical Trials' on The Cochrane Library, MEDLINE, EMBASE, LILACS and the Cystic Fibrosis and Genetic Disorders Group's Inborn Errors of Metabolism Trials Register (date of the most recent search: 11 September 2012). The original search was performed in September 2008.Date of the most recent search of the Cystic Fibrosis and Genetic Disorders Group's Inborn Errors of Metabolism Trials Register: 11 September 2012. Randomized controlled trials of agalsidase alfa or beta in participants diagnosed with Anderson-Fabry disease. Two authors selected relevant trials, assessed methodological quality and extracted data. Six trials comparing either agalsidase alfa or beta in 223 participants fulfilled the selection criteria.Both trials comparing agalsidase alfa to placebo reported on globotriaosylceramide concentration in plasma and tissue; aggregate results were non-significant. One trial reported pain scores, there was a statistically significant improvement for participants receiving treatment at up to three months, mean difference -2.10 (95% confidence interval (CI) -3.79 to -0.41); at up to five months, mean difference -1.90 (95% CI -3.65 to -0.15); and at up to six months, mean difference -2.00 (95% CI -3.66 to -0.34). There was a significant difference in pain-related quality of life at over five months and up to six months, mean difference -2.10 (95% CI -3.92 to -0.28) but not at other time-points. Neither trial reported deaths.One of the three trials comparing agalsidase beta to placebo reported on globotriaosylceramide concentration in plasma and tissue and showed significant improvement: kidney, mean difference -1.70 (95% CI -2.09 to -1.31); heart, mean difference -0.90 (95% CI -1.18 to -0.62); and composite results (renal, cardiac, and cerebrovascular complications and death), mean difference -4.80 (95% CI -5.45 to -4.15). There was no significant difference between groups for death; no trials reported on pain.Only one trial compared agalsidase alfa to agalsidase beta. There was no significant difference between the groups for any adverse events, risk ratio 0.36 (95% CI 0.08 to 1.59), or any serious adverse events; risk ratio 0.30; 95% CI 0.03 to 2.57). Six small, poor quality randomised controlled trials provide no robust evidence for use of either agalsidase alfa and beta to treat Anderson-Fabry disease.

Staphylococcus sp na produção primária de leite: aspectos de saúde pública

Staphylococcus is one of the more important causes of the called Foodborne Disease(FD), being that from the 40 species described from genus, the more important is Staphylococcus aureus. During years believed that the S. aureus was the only specie from genus able to produce enterotoxins, responsable for the clinical frame in humans, but latest studies report the isolation of other species both positive coagulase (PC) as negative with enterotoxigenic potential. The symptoms of this intoxication appear after a short period of incubation (2-6 hours) and usually characterized by nausea, vomits, abdominal ache, diarrhea, and rarely is fatal. For the toxin to be formed in food is necessary that bacteria population to be at least 105 UFC/g, being that such toxins characterized by presenting great resistance front of gastrointestinal proteases and of homemade termical treatment. Among the main foods that might carry the microorganism, the milk and its derivatives have highlights. The contamination of the product might happen as from the milk from cows with clinical and/or subclinical mastitis, as the Staphylococcus genus is one of the main agents etiologic from this disease, equipments utensils badly sanitized equipments and utensils and from the manipulators. The control of these factors configures as fundamental condition for the achievement of a safe, quality product, which doesn’t offer risk to the consumers

Obtenção de hibridos lapdesf fur-fd e atividade anti-inflamatória com potencial atividade neuroprotetora

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)