Trials comparing enzyme replacement therapy to placebo show significant improvement with enzyme replacement therapy in regard to microvascular endothelial deposits of globotriaosylceramide and in pain-related quality of life. There is, however, no evidence identifying if the alfa or beta form is superior or the optimal dose or frequency of enzyme replacement therapy. With regards to safety, adverse events (i.e., rigors, fever) were more significant in the agalsidase beta as compared to placebo. The long-term influence of enzyme replacement therapy on risk of morbidity and mortality related to Anderson-Fabry disease remains to be established. This review highlights the need for continued research into the use of enzyme replacement therapy for Anderson-Fabry disease.

Historically, Escherichia coli is among the most studied organisms and serves as the basis for understanding many fundamental biochemical and genetic concepts. In addition, it displays 9 pathogenesis groups, with the Shiga toxin-producing (STEC) group being the main representative regarding foodborne pathogenesis. Its typical characteristic is the presence of 2 distinct toxins and variants: stx1 (stx1a, stx1c, and stx1d), and stx2 (stx2a, stx2b, stx2c, stx2d, stx2e, stx2f, and stx2g). The main challenge regarding the study of E. coli is the standardization of a high sensitivity method including all pathotypes, that allows for enrichment of STEC cells and a decrease of background microbiota. The ability of some E. coli cells belonging to other pathogenic groups, such as O104:H4, to acquire genes unique to the STEC group, increases the pathogenic power and the risk of new outbreaks related to these bacteria. In addition, animals with a high concentration of pathogenic E. coli cells present in feces (above 10 4 CFU/g), designated as supershedding animals, may be the primary transmission factor among ruminants. Therefore, the purpose of this review is to address pathogenicity factors and the importance of supershedding animals in the transmission of this pathogen, discussing the main methods currently applied, to focus on the occurrence of STEC in beef.

Mycobacterium bovis is the causative agent of bovine tuberculosis (BTB), the pathogen responsible for serious economic impact on the livestock sector. In order to obtain data on isolated M. bovis strains and assist in the control and eradication program for BTB, a cross sectional descriptive molecular epidemiology study in the Brazilian Midwest was conducted. Through spoligotyping and 24-loci MIRU-VNTR methods, 37 clinical isolates of M. bovis circulating in the region were analyzed, 10 isolated from the state of Mato Grosso, 12 from the state of Mato Grosso do Sul and 15 from the state of Goiás. The spoligotyping analysis identified 10 distinct M. bovis profiles (SB0121 n = 14, SB0295 n = 6, SB0140 n = 6, SB0881 n = 3, SB1144 n = 2, SB1145 n = 2, SB0134 n = 1, SB1050 n = 1, SB1055 n = 1, SB1136 n = 1) grouped in six clusters and four orphan patterns. The MIRU-VNTR 24-loci grouped the same isolates in six clusters and 22 unique orphan patterns, showing higher discriminatory power than spoligotyping. When associating the results of both techniques, the isolates were grouped in five clusters and 24 unique M. bovis profiles. Among the 24-loci MIRU-VNTR evaluated, two, ETR-A and QUB 11b loci, showed high discriminatory ability (h = ≥ 0.50), while MIRU 16, MIRU 27, ETR-B, ETR-C, Mtub21 and QUB 26 loci showed moderate ability (h = 0.33 or h = 0.49) and were the most effective in evaluating the genotypic similarities among the clinical M. bovis isolate samples. Herein, the 29 patterns found amongst the 37 isolates of M. bovis circulating in the Brazilian Midwest can be due to the animal movement between regions, municipalities and farms, thus causing the spread of various M. bovis strains in herds from Midwest Brazil.

Salmonella is one of the major causative agents of foodborne infections. Salmonellosis becomes more dangerous when strains resistant to several antibiotics are found in food, especially in chicken, one of the primary transmission vehicles of this pathogen for humans. The present study aimed to estimate the occurrence of Salmonella in chicken carcasses from the state of Mato Grosso, Brazil, as well as determine the antibiotic resistance profile and genotypic characteristic of multi-drug resistant (MDR) isolates. During a 15-month period, from 01/2014 to 05/2015, 850 samples of chilled fresh chicken carcasses were sampled from a slaughterhouse and submitted to Salmonella determinations according to the ISO-6579/2002 method, serotyping and multiplex PCR. The disc diffusion test was applied for 17 antibiotics, according to CLSI (2014). Five isolates were genotyped by repetitive sequence-based PCR using the semi-automated DiversiLab (bioMérieux®) system. The occurrence of Salmonella in chicken carcasses was of 3.7% (31/850), with only 4 strains (12.9%) presenting as MDR, and 6 strains (19.35%) displaying ESBL. The predominant serovars were Salmonella Infantis (35.4%, 11/31), and S. Abony (25.8%, 8/31), followed by serovars S. Agona (12.9%, 4/31), S. Schwarzengrund (9.7%, 3/31), S. Anatum and Salmonella enterica O:4,5 (6.5%, 2/31), and only one Salmonella enterica O:6,7 strain (3.2%, 1/31). All isolates were resistant to one to 5 classes of antibiotics in decreasing order: folate pathway inhibitors, β-lactams (cephalosporins, penicillin, monobactams), tetracyclines, chloramphenicol, and gentamicin. However, strains sensitive to florfenicol, streptomycin, nalidixic acid, ciprofloxacin, enrofloxacin, and nitrofurantoin were also found in this study. Genotyping revealed 98 to 99% homology between 3 Salmonella strains, which displayed high phenotypic resistance similarity to β-lactams and folate pathway inhibitors. Detection of MDR non-typhoid Salmonella in chicken slaughterhouses with quality assurance systems such as Hazard Analysis and Critical Points and Implemented Good Manufacturing Practices is a concern, reinforcing the need for constant monitoring of these pathogens, with the purpose of safeguarding the safety of their products.