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otomy for aceruloplasminemia patients without symptomatic anemia.BACKGROUND Cultural and religious practices of African origin have decisively influenced traditional health practices in the Americas since the African diaspora. Plants are core elements in the religions of African origin. Compared with other parts of Brazil where the Afro-Brazilian presence is widely recognized, in Southern Brazil, these cultural practices are often socially invisible. Yet, there are several terreiros of three Afro-Brazilian religions Candomblé, Umbanda, and Ritual deAlmas e Angola. We hypothesize that the importance of plants in Afro-Brazilian religions is linked not only to spiritual and magical issues but also to the medicinal properties of these plants. We seek to answer the following questions (a) Which plants are used in the terreiros and what are their indications for use?; (b) Are there plants that stand out culturally in these religious groups?; and (c) What is the importance of the adaptive maintenance and replacement process in the use of plants in these religions, considering theensive knowledge about a highly consensual set of therapeutic plants that should be further investigated pharmacologically to understand the effect of their external use. Also, we emphasize the importance of recognizing and valuing this ancestral Afro-Brazilian knowledge and learning also from these people about their broader vision of health which also adds more spirituality in health care.BACKGROUND Niemann-Pick disease Type C (NP-C) is a rare, progressive neurodegenerative disorder characterized by progressive neurodegeneration and premature death. We report data at closure of the NPC Registry that describes the natural history, disease course and treatment experience of NP-C patients in a real-world setting. METHODS The NPC Registry was a prospective observational cohort study that ran between September 2009 and October 2017. Patients with a confirmed diagnosis of NP-C were enrolled regardless of treatment status. All patients underwent clinical assessments and medical care as determined by their physicians; data were collected through a secure internet-based portal. RESULTS At closure on October 19, 2017, 472 patients from 22 countries were enrolled in the NPC Registry. Mean (standard deviation) age at enrollment was 21.2 (15.0) years, and 51.9% of patients were male. First neurological symptom onset occurred during the early-infantile ( less then 2 years), late-infantile (2 to less then ase (at least 3 of the 4 domains having a decreased or unchanged score between enrollment and last follow-up). The NPC Registry did not identify any new safety signals associated with miglustat therapy. CONCLUSIONS The profiles of clinical manifestations in the final NPC Registry dataset agreed with previous clinical descriptions. Miglustat therapy was associated with a stabilization of neurological manifestations in most patients. The safety and tolerability of miglustat therapy was consistent with previous reports.OBJECTIVES EQ-5D is the most commonly used generic preference-based health-related quality of life (HRQoL) measure. The current study aimed at estimating the HRQoL index scores using EQ-5D-5 L measure in the capital of Iran; moreover, identifying some determinants of the HRQoL. METHODS A sample of 3060 subjects was selected by a stratified random sampling method from the general adult population of Tehran. Face-to-face interview was conducted to fill out the questionnaire, in this cross-sectional survey. EQ-5D-5 L utility score were estimated using an interim value set, based on a crosswalk methodology. Additionally, the relationships between HRQoL and sociodemographic characteristics were tested by generalized linear model, using STATA version 13. RESULTS The mean ± standard deviation utility and EQ-VAS scores were 0.79 ± 0.17 and 71.72 ± 19.37. The utility scores ranged 0.61 ± 0.19 in > 69 year-old females to 0.88 ± 0.12 in less then 30 year-old males. In mobility, self-care, and usual activity dimensions, most of the respondents reported "no problems" (70.47, 90.62, and 76.34%, respectively). However, in anxiety/depression and pain/discomfort dimensions, most of the respondents had problems (53.23 and 54.03%, respectively). Females had lower utility score than males; the utility score reduced with age increase; the educational level lead to higher utility scores; and the utility scores of individuals without spouse (divorced or widowed) were lower than those of the married individuals and never married ones. CONCLUSIONS The current study reported HRQoL norm data for the general adult population in the capital of Iran; these data could be very useful for policy making and economic evaluations. A significant percentage of people in Tehran reported anxiety/ depression, which highlights the risk of psychological problems. Effective interventions are needed to increase their HRQoL, especially for the vulnerable groups of the community.The genus Flavivirus encompasses several worldwide-distributed arthropod-borne viruses including, dengue virus, Japanese encephalitis virus, West Nile virus, yellow fever virus, Zika virus, and tick-borne encephalitis virus. Infection with these viruses manifest with symptoms ranging from febrile illness to life- threatening hypotensive shock and encephalitis. NCT-503 concentration Therefore, flaviviruses pose a great risk to public health. Currently, preventive measures are falling short to control epidemics and there are no antivirals against any Flavivirus.Flaviviruses carry a single stranded positive-sense RNA genome that plays multiple roles in infected cells it is translated into viral proteins, used as template for genome replication, it is the precursor of the subgenomic flaviviral RNA and it is assembled into new virions. Furthermore, viral RNA genomes are also packaged into extracellular vesicles, e.g. exosomes, which represent an alternate mode of virus dissemination.Because RNA molecules are at the center of Flavivirus replication cycle, viral and host RNA-binding proteins (RBPs) are critical determinants of infection. Numerous studies have revealed the function of RBPs during Flavivirus infection, particularly at the level of RNA translation and replication. These proteins, however, are also critical participants at the late stages of the replication cycle. Here we revise the function of host RBPs and the viral proteins capsid, NS2A and NS3, during the packaging of viral RNA and the assembly of new virus particles. Furthermore, we go through the evidence pointing towards the importance of host RBPs in mediating cellular RNA export with the idea that the biogenesis of exosomes harboring Flavivirus RNA would follow an analogous pathway.
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