RESUMO
BACKGROUND: 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. OBJECTIVES: To evaluate the effectiveness and safety of enzyme replacement therapy compared to other interventions, placebo or no interventions, for treating Anderson-Fabry disease. SEARCH METHODS: 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. SELECTION CRITERIA: Randomized controlled trials of agalsidase alfa or beta in participants diagnosed with Anderson-Fabry disease. DATA COLLECTION AND ANALYSIS: Two authors selected relevant trials, assessed methodological quality and extracted data. MAIN RESULTS: 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). AUTHORS' CONCLUSIONS: Six small, poor quality randomised controlled trials provide no robust evidence for use of either agalsidase alfa and beta to treat Anderson-Fabry disease.
Assuntos
Terapia de Reposição de Enzimas/métodos , Doença de Fabry/tratamento farmacológico , Isoenzimas/administração & dosagem , alfa-Galactosidase/administração & dosagem , Doença de Fabry/enzimologia , Feminino , Humanos , Masculino , Medição da Dor , Ensaios Clínicos Controlados Aleatórios como Assunto , Proteínas Recombinantes , Fatores de Tempo , Triexosilceramidas/análise , Triexosilceramidas/sangueRESUMO
BACKGROUND: This is an update of a Cochrane Review first published in The Cochrane Library in Issue 2, 2006 and previously updated in 2009.Noise-induced hearing loss can be prevented by eliminating or lowering noise exposure levels. Where the source of the noise cannot be eliminated, workers have to rely on hearing protection equipment. Several trials have been conducted to study the effectiveness of interventions to influence the wearing of hearing protection. OBJECTIVES: To evaluate the effectiveness of interventions to enhance the wearing of hearing protection among persons regularly exposed to high noise levels. SEARCH METHODS: We searched the Cochrane Ear, Nose and Throat Disorders Group Trials Register; the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2011, Issue 1); PubMed; EMBASE; CINAHL; Web of Science; BIOSIS Previews; Cambridge Scientific Abstracts; ICTRP and additional sources for published and unpublished trials. The date of the most recent search was 8 April 2011. SELECTION CRITERIA: We included studies if they had a randomised design, if they were among noise exposed (> 80 dB(A)) persons, if they included an intervention to promote the wearing of hearing protection (compared to another intervention or no intervention), and if the outcome measured was the amount of use of hearing protection or a proxy measure thereof. DATA COLLECTION AND ANALYSIS: Two authors selected relevant trials, assessed risk of bias and extracted data. MAIN RESULTS: Seven studies, involving 4670 participants, were included.A computer-based intervention lasting 30 minutes, tailored to the risk of an individual worker, was not found to be more effective than a video providing general information among workers, around 80% of whom already used hearing protection.A four-year school-based hearing loss prevention programme found that the intervention group was twice as likely to wear some kind of hearing protection as the control group that received a baseline hearing test and two additional tests at years two and three.We conducted two meta-analyses for the comparisons 'tailored strategy (the use of communication or other types of interventions that are specific to an individual or a group and aim to change behaviour) versus non-tailored strategy' and 'tailored strategy versus a commercial video on the use of hearing protection' to look at mean percentage use of hearing protective devices (HPDs), that showed improvement in the mean use of HPDs for the tailored group. A meta-analysis of the comparison 'mixed interventions' (classroom instruction, distribution of HPDs, mailings, noise level assessments and audiometric testing) versus control (audiometric testing alone) also showed improvement in self reported use of HPDs when shooting firearms.Tailored education showed an improvement in HPD use of 8.3% versus targeted education (6.1%). AUTHORS' CONCLUSIONS: The evidence found in this review shows that some interventions improve the mean use of hearing protection devices compared to non-intervention. Future trials should have standard outcomes and interventions to allow the combining of results in meta-analysis.
Assuntos
Dispositivos de Proteção das Orelhas , Perda Auditiva Provocada por Ruído/prevenção & controle , Ruído Ocupacional/efeitos adversos , Doenças Profissionais/prevenção & controle , Educação em Saúde/métodos , Humanos , Ensaios Clínicos Controlados Aleatórios como AssuntoRESUMO
BACKGROUND: Music is ever present in our daily lives, establishing a link between humans and the arts through the senses and pleasure. Sound technicians are the link between musicians and audiences or consumers. Recently, general concern has arisen regarding occurrences of hearing loss induced by noise from excessively amplified sound-producing activities within leisure and professional environments. Sound technicians' activities expose them to the risk of hearing loss, and consequently put at risk their quality of life, the quality of the musical product and consumers' hearing. The aim of this study was to measure the prevalence of high frequency hearing loss consistent with noise exposure among sound technicians in Brazil and compare this with a control group without occupational noise exposure. METHODS: This was a cross-sectional study comparing 177 participants in two groups: 82 sound technicians and 95 controls (non-sound technicians). A questionnaire on music listening habits and associated complaints was applied, and data were gathered regarding the professionals' numbers of working hours per day and both groups' hearing complaint and presence of tinnitus. The participants' ear canals were visually inspected using an otoscope. Hearing assessments were performed (tonal and speech audiometry) using a portable digital AD 229 E audiometer funded by FAPESP. RESULTS: There was no statistically significant difference between the sound technicians and controls regarding age and gender. Thus, the study sample was homogenous and would be unlikely to lead to bias in the results. A statistically significant difference in hearing loss was observed between the groups: 50% among the sound technicians and 10.5% among the controls. The difference could be addressed to high sound levels. CONCLUSION: The sound technicians presented a higher prevalence of high frequency hearing loss consistent with noise exposure than did the general population, although the possibility of residual confounding due to unmeasured factors such as socioeconomic status cannot be ruled out.
Assuntos
Perda Auditiva Provocada por Ruído/epidemiologia , Música , Ruído Ocupacional/efeitos adversos , Exposição Ocupacional/efeitos adversos , Adulto , Idoso , Audiometria , Brasil/epidemiologia , Estudos de Casos e Controles , Estudos Transversais , Feminino , Perda Auditiva Provocada por Ruído/induzido quimicamente , Perda Auditiva Provocada por Ruído/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Exposição Ocupacional/estatística & dados numéricos , Prevalência , Inquéritos e QuestionáriosRESUMO
RATIONALE AND AIM: Over the past 12 years, thousands of authors working with the Cochrane Collaboration around the world have produced systematic reviews to reduce uncertainty in health care decision making. We evaluated the conclusions from Cochrane systematic reviews of randomized controlled trials in terms of their recommendations for clinical practice and research. METHODS: In our cross-sectional study of systematic reviews published in the Cochrane Library, we randomly selected and analysed completed systematic reviews published across all 50 Cochrane Collaborative Review Groups. RESULTS: We analysed 1016 completed systematic reviews. Of these, 44% concluded that the interventions studied were likely to be beneficial, of which 1% recommended no further research and 43% recommended additional research. Also, 7% of the reviews concluded that the interventions were likely to be harmful, of which 2% did not recommend further studies and 5% recommended additional studies. In total, 49% of the reviews reported that the evidence did not support either benefit or harm, of which 1% did not recommend further studies and 48% recommended additional studies. Overall, 96% of the reviews recommended further research. CONCLUSIONS: Cochrane systematic reviews were about evenly split between those in which the authors concluded that at least one of the interventions was beneficial and those in which the evidence neither supported nor refuted the intervention tested. The Cochrane Collaboration needs to include clinical trial protocol summaries with a study design optimized to answer the relevant research questions.