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Background and O bjectives: Several factors can compromise patient safety, such as ineffective teamwork, failed organizational processes, and the physical and psychological overload of health professionals. Studies about associations between burn out and patient safety have shown different outcomes. Objective: To analyze the relationship between burnout and patient safety. Materials and Methods: A systematic review with a meta-analysis performed using PubMed and Web of Science databases during January 2018. Two searches were conducted with the following descriptors: (i) patient safety AND burnout professional safety AND organizational culture, and (ii) patient safety AND burnout professional safety AND safety management. Results: Twenty-one studies were analyzed, most of them demonstrating an association between the existence of burnout and the worsening of patient safety. High levels of burnout is more common among physicians and nurses, and it is associated with external factors such as: high workload, long journeys, and ineffective interpersonal relationships. Good patient safety practices are influenced by organized workflows that generate autonomy for health professionals. Through meta-analysis, we found a relationship between the development of burnout and patient safety actions with a probability of superiority of 66.4%. Conclusion: There is a relationship between high levels of burnout and worsening patient safety., Competing Interests: The authors declare no conflict of interest.

Benzene is one of the most important substances for assessment, due to its significant use, the environmental contamination resulting from its emission and the effects on human health. It is classified by the International Agency for Research on Cancer (IARC) as a known carcinogen to humans (group 1) and associated with the development of leukemia. In general, the population is exposed to this substance by inhaling contaminated air, which varies according to the location and intensity of its potential sources. The petrochemical industry is one of the most important sources of this compound. The municipality of Duque de Caxias, specifically the Campos Elíseos district, in Rio de Janeiro State, Brazil, houses the Industrial Complex of Campos Elíseos (PICE), a grouping of over 25 industries, which includes the second largest oil refinery in Brazil. Environmental contamination from the PICE has been recognized, but there is a lack of studies concerning its impact on the health of the surrounding population. S-phenylmercapturic acid (S-PMA) concentrations ranging from 0.80 to 8.01μg.g-1 creatinine were observed in the local population, apparently related to hematological changes also observed in exposed population. The quantifiable presence of urinary S-PMA from the benzene metabolism is associated with the fact that 60% of the participants present specific hematological changes, which may be due to the environmental benzene exposure. The allele and genotype frequencies of the CYP2E1 and NQO1 enzymes observed in the study population were similar to those reported in other studies. The presence of the variant allele in the NQO1 genotype may be a risk factor for the observed hematological changes.

The aim of this study was to evaluate the effects of turmeric and curcumin in the management of oral mucositis in cancer patients undergoing chemo and/or radiotherapy. The systematic review was reported according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses. The search was performed in the following database: Cochrane Library, LILACS, LIVIVO, PubMed, Scopus, and Web of Science. A gray literature search was undertaken using Google Scholar, Open Grey, and ProQuest. The methodology of included studies was evaluated by the Meta-Analysis of Statistics Assessment and Review Instrument. After a two-step selection process, four randomized and one nonrandomized clinical trials were included in the analysis. Two studies were categorized as low and three as moderate risk of bias. Turmeric/curcumin was applied topically as a gel or as a mouthwash. Patients treated with turmeric/curcumin experienced reduced grade of mucositis, pain, erythema intensity, and ulcerative area. Current evidence suggests that topical application of turmeric or curcumin is effective in controlling signs and symptoms of oral mucositis. Thus, further investigation is required to confirm the promising effect of turmeric and curcumin in oral inflammatory lesions., (© 2019 John Wiley & Sons, Ltd.)

Background: A better understanding of sickle cell anemia (SCA) and improvements in drug therapy and health policy have contributed to the emergence of a large population of adults living with this disease. The mechanisms by which SCA produces adverse effects on the respiratory system of these patients are largely unknown. Fractional-order (FrOr) models have a high potential to improve pulmonary clinical science and could be useful for diagnostic purposes, offering accurate models with an improved ability to mimic nature. Part 2 of this two-part study examines the changes in respiratory mechanics in patients with SCA using the new perspective of the FrOr models. These results are compared with those obtained in traditional forced oscillation (FOT) parameters, investigated in Part 1 of the present study, complementing this first analysis., Methodology/principal Findings: The data consisted of three categories of subjects: controls (n = 23), patients with a normal spirometric exam (n = 21) and those presenting restriction (n = 24). The diagnostic accuracy was evaluated by investigating the area under the receiver operating characteristic curve (AUC). Initially, it was observed that biomechanical changes in SCA included increased values of fractional inertance, as well as damping and hysteresivity (p<0.001). The correlation analysis showed that FrOr parameters are associated with functional exercise capacity (R = -0.57), pulmonary diffusion (R = -0.71), respiratory muscle performance (R = 0.50), pulmonary flows (R = -0.62) and airway obstruction (R = 0.60). Fractional-order modeling showed high diagnostic accuracy in the detection of early respiratory abnormalities (AUC = 0.93), outperforming spirometry (p<0.03) and standard FOT analysis (p<0.01) used in Part 1 of this study. A combination of machine learning methods with fractional-order modeling further improved diagnostic accuracy (AUC = 0.97)., Conclusions: FrOr modeling improved our knowledge about the biomechanical abnormalities in adults with SCA. Changes in FrOr parameters are associated with functional exercise capacity decline, abnormal pulmonary mechanics and diffusion. FrOr modeling outperformed spirometric and traditional forced oscillation analyses, showing a high diagnostic accuracy in the diagnosis of early respiratory abnormalities that was further improved by an automatic clinical decision support system. This finding suggested the potential utility of this combination to help identify early respiratory changes in patients with SCA., Competing Interests: The authors have declared that no competing interests exist.

Trypanosoma cruzi has a complex life cycle where two infective developmental stages, known as trypomastigote and amastigote, can be found in the vertebrate host. Both forms can invade a large variety of cellular types and induce the formation of a parasitophorous vacuole (PV), that, posteriorly, disassembles and releases the parasites into the host cell cytoplasm. The biogenesis of T. cruzi PVs has not been analyzed in professional phagocytic cells. We investigated the biogenesis of PVs containing trypomastigotes or amastigotes in peritoneal macrophages. We observed the presence of profiles of the endoplasmic reticulum and lysosomes from the host cell near PVs at early stages of interaction in both developmental stages, suggesting that both organelles may participate as possible membrane donors for the formation of the PVs. The Golgi complex, however, was observed only near already formed PVs. Electron microscopy tomography and FIB-SEM microscopy followed by 3D reconstruction of entire PVs containing amastigotes or trypomastigotes confirmed the presence of both endoplasmic reticulum and lysosomes in the initial stages of PV formation. In addition, Golgi complex and mitochondria localize around PVs during their biogenesis. Taken together these observations provide a whole view of the invasion process in a professional phagocytic cell., (Copyright © 2019. Published by Elsevier Inc.)

Background: Familial partial lipodystrophy (FPL) is a rare genetic disease characterized by body fat abnormalities that lead to insulin resistance (IR). Clinical conditions linked to milder IR, such as type 2 diabetes (T2D) and metabolic syndrome, are associated with abnormalities of the hypothalamic-pituitary-adrenal (HPA) axis, but little is known about its activity in FPL., Methods: Patients meeting the clinical criteria for FPL were subjected to anthropometric, biochemical and hormone analyses. A genetic study to identify mutations in the genes encoding peroxisome proliferator-activated receptor gamma (PPARγ) was performed. Polycystic ovary syndrome and hepatic steatosis were investigated, and the patient body compositions were analyzed via dual X-ray energy absorptiometry (DXA). The HPA axis was assessed via basal [cortisol, adrenocorticotrophic hormone (ACTH), cortisol binding globulin, nocturnal salivary cortisol and urinary free cortisol (UFC)] as well as dynamic suppression tests (cortisol post 0.5 mg and post 1 mg dexamethasone)., Results: Six patients (five female and one male) aged 17 to 42 years were included. In DXA analyses, the fat mass ratio between the trunk and lower limbs (FMR) was > 1.2 in all phenotypes. One patient had a confirmed mutation in the PPARγ gene: a novel heterozygous substitution of p. Arg 212 Trp (c.634C>T) at exon 5. HPA sensitivity to glucocorticoid feedback was preserved in all six patients, and a trend towards lower basal serum cortisol, serum ACTH and UFC values was observed., Conclusions: Our findings suggest that FPL is not associated with overt abnormalities in the HPA axis, despite a trend towards low-normal basal cortisol and ACTH values and lower UFC levels. These findings suggest that the extreme insulin resistance occurring in FPL may lead to a decrease in HPA axis activity without changing its sensitivity to glucocorticoid feedback, in contrast to the abnormalities in HPA axis function in T2D and common metabolic syndrome.

This chapter describes methods related to the diagnosis of genetic dental diseases. Based on the present knowledge, clinical phenotyping and next-generation sequencing techniques are discussed. Methods necessary for Sanger sequencing, multiplex ligation-dependent probe amplification, and epigenetic modification methods are detailed. In addition, protocols for cell culture establishment and characterization from patients with inherited dental anomalies are described.

Trypanosoma cruzi epimastigotes internalize macromolecules avidly by endocytosis. Previously, we identified a tubule-vesicular network likely to correspond to the early-endosomes. However, a detailed ultrastructural characterization of these endosomes was missing. Here, we combined endocytosis assays with ultrastructural data from high-resolution electron microscopy to produce a 3D analysis of epimastigote endosomes and their interactions with endocytic organelles. We showed that endocytic cargo was found in carrier vesicles budding from the cytopharynx. These vesicles appeared to fuse with a tubule-vesicular network of early endosomes identified by ultrastructural features including the presence of intermembrane invaginations and coated membrane sections. Within the posterior region of the cell, endosomes localized preferentially on the side nearest to the cytopharynx microtubules. At 4°C, cargo accumulated at a shortened cytopharynx, and subsequent temperature shift to 12°C led to slow cargo delivery to endosomes and, later, to reservosomes. Bridges between reservosomes and endosomes resemble heterotypic fusion. Reservosomes are excluded from the posterior end of the cell, with no preferential cargo delivery to reservosomes closer to the nucleus. Our 3D analysis indicates that epimastigotes accomplish high-speed endocytic traffic by cargo transfer to a bona fide early-endosome and then directly from endosomes to reservosomes, via multiple and simultaneous heterotypic fusion events., (Copyright © 2018 Elsevier GmbH. All rights reserved.)

Alzheimer's disease (AD) is a disabling and highly prevalent neurodegenerative condition, for which there are no effective therapies. Soluble oligomers of the amyloid-β peptide (AβOs) are thought to be proximal neurotoxins involved in early neuronal oxidative stress and synapse damage, ultimately leading to neurodegeneration and memory impairment in AD. The aim of the current study was to evaluate the neuroprotective potential of mesenchymal stem cells (MSCs) against the deleterious impact of AβOs on hippocampal neurons. To this end, we established transwell cocultures of rat hippocampal neurons and MSCs. We show that MSCs and MSC-derived extracellular vesicles protect neurons against AβO-induced oxidative stress and synapse damage, revealed by loss of pre- and postsynaptic markers. Protection by MSCs entails three complementary mechanisms: 1) internalization and degradation of AβOs; 2) release of extracellular vesicles containing active catalase; and 3) selective secretion of interleukin-6, interleukin-10, and vascular endothelial growth factor to the medium. Results support the notion that MSCs may represent a promising alternative for cell-based therapies in AD., (© 2018 by The American Society for Biochemistry and Molecular Biology, Inc.)

Background: The improvement in sickle cell anemia (SCA) care resulted in the emergence of a large population of adults living with this disease. The mechanisms of lung injury in this new population are largely unknown. The forced oscillation technique (FOT) represents the current state-of-the-art in the assessment of lung function. The present work uses the FOT to improve our knowledge about the respiratory abnormalities in SCA, evaluates the associations of FOT with the functional exercise capacity and investigates the early detection of respiratory abnormalities., Methodology/principal Findings: Spirometric classification of restrictive abnormalities resulted in three categories: controls (n = 23), patients with a normal exam (n = 21) and presenting pulmonary restriction (n = 24). FOT analysis showed that, besides restrictive changes (reduced compliance; p<0.001), there is also an increase in respiratory resistance (p<0.001) and ventilation heterogeneity (p<0.01). FOT parameters are associated with functional exercise capacity (R = -0.38), pulmonary diffusion (R = 0.66), respiratory muscle performance (R = 0.41), pulmonary volumes (R = 0.56) and airway obstruction (R = 0.54). The diagnostic accuracy was evaluated by investigating the area under the receiver operating characteristic curve (AUC). A combination of FOT and machine learning (ML) classifiers showed adequate diagnostic accuracy in the detection of early respiratory abnormalities (AUC = 0.82)., Conclusions: In this study, the use of FOT showed that adults with SCA develop a mixed pattern of respiratory disease. Changes in FOT parameters are associated with functional exercise capacity decline, abnormal pulmonary mechanics and diffusion. FOT associated with ML methods accurately diagnosed early respiratory abnormalities. This suggested the potential utility of the FOT and ML clinical decision support systems in the identification of respiratory abnormalities in patients with SCA.

Objectives: to verify the influence of dimethylsulfoxide and pentoxifylline on the vitality of cutaneous flaps in rats and the tissue repair process., Methods: were studied 30 Wistar rats, submitting them to a 2cm wide by 8cm long dorsal cutaneous flap, of caudal base. We distributed the animals in three groups: Control Group (n=10) with application gauze moistened with 0.9% Saline in the flap bed for 30 seconds; Dimethylsulfoxide group (n=10), with administration of 1ml of 5% dimethylsulfoxide divided into five injections of 0.2ml in the transition of the flap segments; Pentoxifylline group (n=10), with administration of pentoxifylline 20mg/kg, diluted to 1ml and divided into five injections of 0.2ml in the transition of the flap segments. Drugs were administered intraoperatively, in a single dose and subcutaneously. We observed the skin flaps for changes in color and texture. On the 10th postoperative day, we checked the dimensions of viable and necrotic tissues, followed by excision of the specimen for histological analysis., Results: the measurements of length of the viable and necrotic tissues between groups showed no differences. Histological analysis showed that the Dimethylsulfoxide group presented neovascularization, inflammatory infiltrate with leukocytes and more structured conjunctival stroma. The Pentoxifylline group showed neovascularization and inflammatory infiltrate, with moderate to intense granulation. The control group evolved with a higher rate of necrosis in the distal segment., Conclusion: dimethylsulfoxide and pentoxifylline influenced the vitality of the flap and the tissue repair process. However, they did not prevent necrosis macroscopically.

Organotins are a group of chemical compounds that have a tin atom covalently bound to one or more organic groups. The best-studied organotin is tributyltin chloride, which is an environmental pollutant and an endocrine disruptor. Tributyltin chloride has been shown to bind to PPARγ/RXRα and induces adipogenesis in different mammalian cells. However, there are few studies with other organotin compounds, such as dibutyltins. The aim of this study was to investigate the effect of dibutyltins diacetate, dichloride, dilaurate, and maleate on the transcriptional activity of the nuclear PPARγ and RXRα receptors, and on adipogenesis and inflammation. Analogous to tributyltin chloride, in reporter gene assay using HeLa cells, we observed that dibutyltins diacetate, dichloride, dilaurate, and maleate are partial agonists of PPARγ. Unlike tributyltin chloride, which is a full agonist of RXRα, dibutyltins dichloride and dilaurate are partial RXRα agonists. Additionally, the introduction of the C285S mutation, which disrupts tributyltin chloride binding to PPARγ, abrogated the dibutyltin agonistic activity. In 3T3-L1 preadipocytes, all dibutyltin induced adipogenesis, although the effect was less pronounced than that of rosiglitazone and tributyltin chloride. This adipogenic effect was confirmed by the expression of adipogenic markers Fabp4, Adipoq, and Glut4. Exposure of 3T3-L1 cells with dibutyltin in the presence of T0070907, a specific PPARγ antagonist, reduced fat accumulation, suggesting that adipogenic effect occurs through PPARγ. Furthermore, dibutyltins dichloride, dilaurate, and maleate inhibited the expression of proinflammatory genes in 3T3-L1 cells, such as Vcam1, Dcn, Fn1, S100a8, and Lgals9. Additionally, in RAW 264.7 macrophages, tributyltin chloride and dibutyltin dilaurate reduced LPS-stimulated TNFα expression. Our findings indicate that dibutyltins diacetate, dichloride, dilaurate, and maleate are PPARγ partial agonists and that dibutyltins dichloride and dilaurate are also partial RXRα agonists. Furthermore, dibutyltins induce adipogenesis in a PPARγ-dependent manner and repress inflammatory genes in 3T3-L1 and RAW 264.7 cells. Although dibutyltins display some partial PPARγ/RXRα agonistic effects, the translation of cell-based results assays into in vivo effects on inflammation and insulin resistance is not entirely known. Nevertheless, further studies are necessary to address their effects in different periods of life and to elucidate the actions of organostanic compounds in whole-body context.

Nitric oxide synthase 2, inducible (Nos2) expression is necessary for the microbicidal activity of macrophages. However, NOS2 over-activation causes multiple inflammatory disorders, suggesting a tight gene regulation is necessary. Using cytosolic flagellin as a model for inflammasome-dependent NOS2 activation, we discovered a surprising new role for NLRC4/caspase-1 axis in regulating chromatin accessibility of the Nos2 promoter. We found that activation of two independent mechanisms is necessary for NOS2 expression by cytosolic flagellin: caspase-1 and NF-κB activation. NF-κB activation was necessary, but not sufficient, for NOS2 expression. Conversely, caspase-1 was necessary for NOS2 expression, but dispensable for NF-κB activation, indicating that this protease acts downstream NF-κB activation. We demonstrated that epigenetic regulation of Nos2 by caspase-1 involves cleavage of the chromatin regulator PARP1 (also known as ARTD1) and chromatin accessibility of the NF-κB binding sites located at the Nos2 promoter. Remarkably, caspase-1-mediated Nos2 transcription and NO production contribute to the resistance of macrophages to Salmonella typhimurium infection. Our results uncover the molecular mechanism behind the constricted regulation of Nos2 expression and open new therapeutic opportunities based on epigenetic activities of caspase-1 against infectious and inflammatory diseases., Competing Interests: The authors declare no competing financial interests.

Background: Sickle cell anemia (SCA) is characterized by a broad spectrum of abnormalities that affect most body organs and systems. To date, there is few data on the influence of these patients' clinical characteristics on the functional exercise capacity., Aim: To investigate the effect of the clinical complications on the functional exercise capacity of adult SCA patients., Method: Cross-sectional study, where 45 SCA patients underwent clinical evaluations, echocardiography, pulmonary function testing, and determination of six-minute walking distance (6MWD)., Results: A significant correlation (P < 0.001) was found between 6MWD and hemoglobin (Hb) level, tricuspid regurgitation velocity, forced vital capacity, acute chest syndrome, and diffusing capacity for carbon monoxide. The prediction model for 6MWD explained 67% of the 6MWD variability (P < 0.001)., Conclusions: Hemodynamics, cardiovascular function, pulmonary function, and episodes of acute lung injury seem to impact the 6MWD in adults with SCA., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Aim: To systematically evaluate the accuracy of host-derived salivary biomarkers in the diagnosis of periodontal disease based on the given sensitivity and specificity information., Materials and Methods: Studies were eligible for inclusion if they had compared the diagnostic application of salivary biomarkers with clinical examination of periodontal disease. A detailed search was performed in five databases without restrictions on subject age, chronology, or language. Additionally, a partial grey-literature search was conducted. The revised Quality Assessment of Diagnostic Accuracy Studies tool and Meta-analysis were used to evaluate the selected studies., Results: From the 905 screened studies, four were included in the qualitative and quantitative analysis. One biomarker, macrophage inflammatory protein-1α (MIP-1α), had excellent diagnostic accuracy and two, interleukin-1β (IL-1β) and interleukin-6 (IL-6), showed acceptable diagnostic values. However, the only biomarker considered excellent was evaluated in a single study, which may reduce the robustness of the results., Conclusion: There is currently limited evidence to confirm the diagnostic capability of salivary biomarkers in the clinical assessment of periodontal disease. Notwithstanding, the summary findings showed the growing importance of salivary biomarker, and can guide larger, well-controlled, diagnostic accuracy studies. Likewise, although not conclusive, MIP-1α, IL-1β, and IL-6 may be promising biomarkers for future studies., (© 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: Beige adipocytes comprise a unique thermogenic cell type in the white adipose tissue (WAT) of rodents and humans, and play a critical role in energy homeostasis. In this scenario, recruitment of beige cells has been an important focus of interest for the development of novel therapeutic strategies to treat obesity. PPARγ activation by full agonists (thiazolidinediones, TZDs) drives the appearance of beige cells, a process so-called browning of WAT. However, this does not translate into increased energy expenditure, and TZDs are associated with weight gain. Partial PPARγ agonists, on the other hand, do not induce weight gain, but have not been shown to drive WAT browning. The present study was designed to investigate the effects of GQ-16 on BAT and on browning of WAT in obese mice., Methods: Male Swiss mice with obesity and hyperglycemia induced by high fat diet were treated with vehicle, rosiglitazone (4 mg/kg/d) or the TZD-derived partial PPARγ agonist GQ-16 (40 mg/kg/d) for 14 days. Fasting blood glucose, aspartate aminotransferase, alanine aminotransferase and lipid profile were measured. WAT and brown adipose tissue (BAT) depots were excised for determination of adiposity, relative expression of Ucp-1, Cidea, Prdm16, Cd40 and Tmem26 by RT-qPCR, histological analysis, and UCP-1 protein expression analysis by immunohistochemistry. Liver samples were also removed for histological analysis and determination of hepatic triglyceride content., Results: GQ-16 treatment reduced high fat diet-induced weight gain in mice despite increasing energy intake. This was accompanied by reduced epididymal fat mass, reduced liver triglyceride content, morphological signs of increased BAT activity, increased expression of thermogenesis-related genes in interscapular BAT and epididymal WAT, and increased UCP-1 protein expression in interscapular BAT and in epididymal and inguinal WAT., Conclusion: This study suggests for the first time that a partial PPARγ agonist may increase BAT activity and induce the expression of thermogenesis-related genes in visceral WAT., General Significance: These findings suggest that PPARγ activity might be modulated by partial agonists to induce WAT browning and treat obesity.

Introduction: Rosiglitazone (RSG) is a synthetic full agonist of transcription factor peroxisome proliferator activated receptor gamma. Previous studies have suggested an anti-inflammatory effect of RSG on lipopolysaccharide-induced pulp inflammation. However, its role in other cellular events related to pulp repair has not been investigated. Therefore, the aim of the present study was to evaluate the effect of RSG on human dental pulp cell viability, proliferation, migration, and osteoblastic/odontoblastic differentiation., Methods: Cell proliferation was evaluated by [3H]-thymidine assay. Cell viability was assessed by a 3-[4,5-dimethylthiazol-2-yl]-2,5-diphenyltetrazolium bromide assay and by measuring the percentage of apoptotic cells by flow cytometry. Cell migration was estimated by scratch wound healing assay. Mineralization and cell differentiation were evaluated by alizarin red S staining and real-time polymerase chain reaction gene expression assay, respectively., Results: RSG significantly decreased cell proliferation and did not have effect on cell viability, apoptosis/necrosis, or migration. Alizarin red S showed that RSG accelerated calcified nodule formation. Results of real-time polymerase chain reaction demonstrated that RSG upregulated osteopontin expression, whereas expression of dentin sialophosphoprotein, dentin matrix protein-1, and osteocalcin was not affected., Conclusions: These findings suggest that RSG decreases human dental pulp cell proliferation, while positively regulating osteopontin expression., (Copyright © 2015 American Association of Endodontists. Published by Elsevier Inc. All rights reserved.)

Background: Raine syndrome (RS) is a rare autosomal recessive bone dysplasia typified by osteosclerosis and dysmorphic facies due to FAM20C mutations. Initially reported as lethal in infancy, survival is possible into adulthood. We describe the molecular analysis and clinical phenotypes of five individuals from two consanguineous Brazilian families with attenuated Raine Syndrome with previously unreported features., Methods: The medical and dental clinical records were reviewed. Extracted deciduous and permanent teeth as well as oral soft tissues were analysed. Whole exome sequencing was undertaken and FAM20C cDNA sequenced in family 1., Results: Family 1 included 3 siblings with hypoplastic Amelogenesis Imperfecta (AI) (inherited abnormal dental enamel formation). Mild facial dysmorphism was noted in the absence of other obvious skeletal or growth abnormalities. A mild hypophosphataemia and soft tissue ectopic mineralization were present. A homozygous FAM20C donor splice site mutation (c.784 + 5 g > c) was identified which led to abnormal cDNA sequence. Family 2 included 2 siblings with hypoplastic AI and tooth dentine abnormalities as part of a more obvious syndrome with facial dysmorphism. There was hypophosphataemia, soft tissue ectopic mineralization, but no osteosclerosis. A homozygous missense mutation in FAM20C (c.1487C > T; p.P496L) was identified., Conclusions: The clinical phenotype of non-lethal Raine Syndrome is more variable, including between affected siblings, than previously described and an adverse impact on bone growth and health may not be a prominent feature. By contrast, a profound failure of dental enamel formation leading to a distinctive hypoplastic AI in all teeth should alert clinicians to the possibility of FAM20C mutations.

Dengue fever induces a robust immune response, including massive T cell activation. The level of T cell activation may, however, be associated with more severe disease. In this study, we explored the level of CD8+ T lymphocyte activation in the first six days after onset of symptoms during a DENV2 outbreak in early 2010 on the coast of São Paulo State, Brazil. Using flow cytometry we detected a progressive increase in the percentage of CD8+ T cells in 74 dengue fever cases. Peripheral blood mononuclear cells from 30 cases were thawed and evaluated using expanded phenotyping. The expansion of the CD8+ T cells was coupled with increased Ki67 expression. Cell activation was observed later in the course of disease, as determined by the expression of the activation markers CD38 and HLA-DR. This increased CD8+ T lymphocyte activation was observed in all memory subsets, but was more pronounced in the effector memory subset, as defined by higher CD38 expression. Our results show that most CD8+ T cell subsets are expanded during DENV2 infection and that the effector memory subset is the predominantly affected sub population.

Background: Endothelial cell dysfunction is believed to play an important role in the pathogenesis of plasma leakage in patients with acute dengue virus (DENV) infection. Several factors, produced by activated endothelial cells, have been associated with plasma leakage or severe disease in patients with infectious diseases., Objectives: The aim of this study was to investigate which of these markers could serve as a surrogate marker for the occurrence of plasma leakage in patients with acute DENV infection., Study Design: A case-control study was performed in patients with acute DENV infection in Santos, Brazil. Plasma leakage was detected with X-ray and/or ultrasound examination at admission. Serum levels of soluble endoglin, endothelin-1, angiopoietin-2, VEGF, soluble VEGFR-2, MMP-2, MMP-9, TIMP-1 and TIMP-2 were determined using commercially available ELISAs., Results: Increased levels of angiopoietin-2, endothelin-1 and MMP-2 and decreased levels of soluble VEGFR-2 were significantly associated with the occurrence of plasma leakage. An unsupervised cluster analysis confirmed that angiopoietin-2 and soluble VEGFR-2 were strongly associated with clinical apparent vascular leakage., Conclusion: Angiopoietin-2 and soluble VEGFR-2 can serve as surrogate markers for the occurrence of plasma leakage in patients with acute DENV infection., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Introduction: Laryngectomy is a surgical procedure that aims to remove the hyoid bone and the larynx and its muscles; it is inferred that a destabilization of the hyoid-mandibular axis will occur, consequently changing chewing., Objective: To characterize the amplitude and speed of chewing in laryngectomies and to compare them with two groups of non-laryngectomized individuals differentiated by age., Method: 72 volunteers were divided into three groups: (A) 32 volunteers, mean age 22.3 years, 17 females; (B) 20 volunteers, mean age 53.2 years, 10 females, and (C) 20 volunteers who underwent total laryngectomy, mean age 61.5 years, one female. Electrognathography, a method that tracks and measures millimeter jaw movements, was used for evaluation., Results: Significant differences were observed between groups A and C in masticatory amplitude and speed., Conclusion: Age, and adaptive and compensatory changes appear to explain chewing better than factors related to total laryngectomy.

This study aimed to describe the contributions of nursing in the family relationships of scholars who have changes in school behavior and performance. It's an action research, based on the questioning method, using the Arc Method, implemented in five stages: reality observation, key-points, theory, possible solutions, and application to reality. The research happened in a municipal school, located in the west of Santa Maria-RS. 25 scholars' families participated in the sample, during March to May of 2012. It was noticed that the dream of having an ideal family exists, but the lived reality is still permeated by a lot of difficulties and require modifications. Thus, take care of the family relationships shows up as a possibility of society to develop worthily, considering the powerful influence it has on the people character development and formation.

The aim of this qualitative exploratory research is to understand how health workers relate to the main object of their work--the user--both subject and author of his/her life history. Eleven nursing practitioners from a Basic Health Unit participated in a semi-structured instrument, in March and April, 2008. The speeches revealed two converging themes: (1) Consideration of human peculiarities in attention to health care; and (2) dialogue and assistance as interactive possibilities. We found that the attention to health care is broadening the debates over valuing human peculiarities through dialogue and assistance as interactive possibilities.

In the Brazilian HIV-1 epidemic, subtypes B, C, and F1 are cocirculating in the population. Sequences of the partial pol gene from 463 HIV-1-infected patients were obtained from plasma samples and viral subtype was characterized. BF recombinants were found in 8% of the samples. Fifteen different patterns were observed. A CRF28&#95;BF and CRF29&#95;BF structure was found in 29.7% of the samples, CRF12&#95;BF in 13.5%, and CRF39&#95;BF in 2.7%. Two other patterns were identified in each of three samples. These findings could indicate a new CRF description, but to determine this a full length study is required.

Abuse, understood as the non-provisioning of fundamental rights, can affect workers' life quality and undermine their professional environments. This assay discusses institutional abuse and its effects on the work environment, and makes some proposals as to how alleviate them by stimulating the members of the health interdisciplinary team to improve and acknowledge their potential to transform and improve a reality perceived as unhealthy. It is essential that the health team strengthens its relationships and knowledge, reduces uneasiness, makes its routine flexible, and assesses specificities. This will allow making proposals in partnership for the improvement of inter- and intrapersonal relationships in health institutions, eventually building solidarity.