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.

Intervenções para redução de diarréia em pacientes recebendo quimioterapia para câncer colorretal: revisão sistematica

Pós-graduação em Bases Gerais da Cirurgia - FMB

Efetividade da finasterida no tratamento da síndrome da dor pélvica crônica: revisão sistemática e metanálise

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Eficácia do tratamento reparador quando comparado ao tratamento conservador na perfuração esofágica: revisão sistemática e estudo oerdenado de séries de casos

Pós-graduação em Bases Gerais da Cirurgia - FMB

A contagem de plaquetas pré-operatória é fator de risco em pacientes cirróticos com hepatocarcinoma submetidos a hepatectomia?: revisão sistemática e metanálise de estudos coorte

Pós-graduação em Bases Gerais da Cirurgia - FMB

Revisão sistemática dos marcadores de otiminização da perfusão tecidual em ensaios clínicos aleatórios de cirurgias de alto risco

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

Eficácia da sublobectomia comparada à lobectomia no tratamento do cãncer de pulmão de não pequenas células em estádios iniciais: metanálise proporcional de série de casos

Pós-graduação em Bases Gerais da Cirurgia - FMB

Colocação de endoprótese vascular versus cirurgia para coarctação de aorta: revisão sistemática

Pós-graduação em Bases Gerais da Cirurgia - FMB

Eficácia do tratamento cirúrgico no tórax instável: revisão sistemática e metanálise

Pós-graduação em Bases Gerais da Cirurgia - FMB

Recuperação acelerada após reparo cirúrgico aberto eletivo do aneurisma da aorta abdominal: revisão sistemática da literatura

Pós-graduação em Anestesiologia - FMB

Phytotherapy: an introduction to its history, use and application

There has been a great advance in the pharmacognosy field, which has increased the use of medicinal plants by health professionals and practitioners of folk medicine. This systematic review explored the history and the present day application of phytotherapeutic medicines. We searched the databases Cochrane Library, Embase, Lilacs, PubMed, Scielo and a specialized bibliography. We concluded that there are many therapeutic potentialities in the use of phytotherapeutic medicines and medicinal plants. We also concluded that despite the increasing number of publications in the field each year, there is still a lack of reviews and meta-analyses that could promote a better integration of the knowledge produced. In addition, research about pharmacological interactions and multidisciplinary studies may promote a quicker and safer process from the workbench up to the clinical trials.

Supplementation and therapeutic use of vitamin D in patients with multiple sclerosis: Consensus of the Scientific Department of Neuroimmunology of the Brazilian Academy of Neurology

A esclerose múltipla (EM) é uma doença inflamatória, autoimune, desmielinizante e degenerativa do sistema nervoso central. Estudos epidemiológicos têm identificado associações de hipovitaminose D com doenças autoimunes. O principal objetivo desta revisão é responder se há evidências que indiquem o uso terapêutico de vitamina D em monoterapia para pacientes com EM. Por meio dos sites PUBMED, EMBASE, LILACS e Scielo foram realizadas buscas usando os descritores “vitamin D”, e “multiple sclerosis” até 12/09/2013. Estudos clínicos randomizados, controlados e duplo-cegos foram selecionados para avaliar a resposta terapêutica da vitamina D na EM. Não foram encontradas evidências científicas que justifiquem o uso da vitamina D em monoterapia no tratamento da EM, na prática clínica.

Revisão sistemática do efeito do envelhecimento no andar livre e adaptativo

Aging seems to impair the walking. However, it is not clear the effects of aging on walking. The aim of this study was to determine changes in kinematic, kinetic and electromyographic parameters of the free and adaptive gait, in preferred velocity, caused by aging. The initial search strategy was performed to identify all articles that examined the free and adaptive gait. The electronic databases analyzed were: MEDLINE, PubMed, EMBASE, CINAHL, Sports Discus, DARE, PsychInfo, ERIC, AusportMed, AMI, Cochrane and PEDro. Twenty-three articles were reviewed in full. Elderly are slower, with shorter step length and longer double support duration than young adults during free and adaptive gait. Even, they showed higher muscular demands, with redistribution of joint power and torque and decreased force in the propulsion and absorption phases. It was concluded that elderly present altered kinematic, kinetic and electromyographic parameters of free and adaptive gait compared to young adults.

Terapia nutricional perioperatória em pacientes submetidos à hepatectomia devido carcinoma hepatocelular (CHC): revisão sistem[atica de ensaios clínicos randonizados

Pós-graduação em Bases Gerais da Cirurgia - FMB

Enhanced Recovery after Elective Open Surgical Repair of Abdominal Aortic Aneurysm: A Complementary Overview through a Pooled Analysis of Proportions from Case Series Studies

Objectives: To evaluate the efficacy and safety of enhanced recovery after surgery (ERAS) programs in elective open surgical repair (OSR) of abdominal aortic aneurysm (AAA).Background: Open surgical repair of AAA is associated with high morbidity and mortality, prolonged hospital stay and high costs. ERAS programs contribute to the optimization of treatment by reducing hospital stay and improving clinical outcomes.Methods: A review of PubMed, EMBASE and LILACS databases was conducted. As only one randomized controlled trial was found, a pooled analysis of proportions from case series was conducted, considering it a complementary overview of the topic. Inclusion criteria were case series with more than five cases reported, adult patients who underwent an elective OSR of AAA and use of an ERAS program. ERAS was compared to conventional perioperative care. The pooled proportion and the confidence interval (CI) are shown for each outcome. The overlap of the CI suggests similar effect of the interventions studied.Results: Thirteen case series studies with ERAS involving 1,250 patients were compared to six case series with conventional care with a total of 1,429 patients. The pooled, respective proportions for ERAS and conventional care were: mortality, 1.51% [95% CI: 0.0091, 0.0226] and 3.0% [95% CI 0.0183, 0.0445]; and incidence of complications, 3.82% [95% CI 0.0259, 0.0528] and 4.0% [95% CI 0.03, 0.05].Conclusion: This review shows that ERAS and conventional care therapies have similar mortality and complication rates in OSR of AAA.