A clinical score to guide in decision making for monogenic type I IFNopathies.

Objective: To develop a set of clinical criteria that identifies patients with a potential autoinflammatory IFNopathy.

Methods: Based on a literature review, a set of clinical criteria identifying genetically confirmed monogenic IFNopathies was selected. For validation, the clinical score was assessed in healthy controls (HCs) and 18 disease controls, including 2 known autoimmune IFNopathies, juvenile systemic lupus erythematosus (JSLE, n = 4) and dermatomyositis (JDM, n = 4); adenosine deaminase 2 deficiency (DADA2, n = 4); and oligoarticular juvenile idiopathic arthritis (oJIA, n = 6).

Baricitinib experience on STING-associated vasculopathy with onset in infancy: A representative case from Turkey.

Stimulator of interferon genes associated vasculopathy with onset in infancy (SAVI), caused by heterozygote gain-of-function mutations in TMEM173, is characterized by fever attacks with ulcerating cutaneous manifestations on cold-sensitive areas and interstitial lung disease. A six-month-old boy was admitted to our hospital with fever, cough, and rash on the external surface of both upper and lower extremities. Respiratory symptoms consistent with ILD developed and skin lesions evolved to eschar formation particularly on acral regions.

Non-Leishmania Parasite in Fatal Visceral Leishmaniasis-Like Disease, Brazil.

Through whole-genome sequencing analysis, we identified non-Leishmania parasites isolated from a man with a fatal visceral leishmaniasis-like illness in Brazil. The parasites infected mice and reproduced the patient's clinical manifestations. Molecular epidemiologic studies are needed to ascertain whether a new infectious disease is emerging that can be confused with leishmaniasis.

[What place for an Ethics committee in a comprehensive cancer centre? For an ethics embodied in real life].

The Ethics committee of Gustave Roussy cancer center is devoted to both reflection and action. The group has 40 members, professionals, patients and outside experts. These meet in plenary meetings or in specific working sessions and intervene at the request of any professional faced with ethical questions in the care.

Aged biochar changed copper availability and distribution among soil fractions and influenced corn seed germination in a copper-contaminated soil.

Biochar has been recommended as a multi-beneficial amendment for the in situ remediation of heavy metals contaminated soils due to its high recalcitrance, stability, specific surface area and retention capacity, which leads to a long-lasting influence on the immobilization of soil contaminants. The influence of biochar on the availability of heavy metals such as copper is not fully understood and may be related to a change in copper association with soils fractions. Therefore, a long-time laboratory incubation study was set up as a completely randomized design to test the effect of biochar from different sources (coconut husks-CHB, orange bagasse-OBB and sewage sludge-SSB) at two rates of application (30 and 60 t ha) on the distribution of copper in a copper-contaminated soil after 24 months incubation.

Macrophages From Subjects With Isolated GH/IGF-I Deficiency Due to a GHRH Receptor Gene Mutation Are Less Prone to Infection by .

Isolated growth hormone (GH) deficiency (IGHD) affects approximately 1 in 4,000 to 1 in 10,000 individuals worldwide. We have previously described a large cohort of subjects with IGHD due to a homozygous mutation in the GH releasing hormone (GHRH) receptor gene. These subjects exhibit throughout the life very low levels of GH and its principal mediator, the Insulin Growth Factor-I (IGF-I).

Survival of outbreak, food, and environmental strains of Listeria monocytogenes on whole apples as affected by cultivar and wax coating.

The 2014-2015 U.S. nationwide outbreak of listeriosis linked to apples used in commercially produced, prepackaged caramel apples was the first implication of whole apples in outbreaks of foodborne illnesses.

Association Between Zika Virus Microcephaly in Newborns With the rs3775291 Variant in Toll-Like Receptor 3 and rs1799964 Variant at Tumor Necrosis Factor-α Gene.

Congenital Zika syndrome (CZS) is a cluster of malformation, and the mechanisms that lead it are still unclear. Using hypothesis-driven candidate genes and their function in viral infections, single-nucleotide polymorphisms (SNPs) were genotyped by quantitative polymerase chain reaction in a sample population from Sergipe State, Brazil. This study shows that rs3775291 SNP at Toll-like receptor 3, which triggers type I interferon antiviral responses in mothers infected by Zika virus during pregnancy, is associated with CZS occurrence (odds ratio [OR], 2.

Clinical and epidemiological indicators and spatial analysis of leprosy cases in patients under 15 years old in an endemic area of Northeast Brazil: an ecological and time series study.

Objective: This study aimed to analyse the clinical and epidemiological indicators, temporal trends and the spatial distribution of leprosy in patients under 15 years old in an endemic area of Northeast Brazil.

Design: Regional surveillance study of all reported cases.

Setting: State of Sergipe, endemic area of Northeast Brazil.

PSO-BP Neural Network-Based Strain Prediction of Wind Turbine Blades.

The full-scale static testing of wind turbine blades is an effective means to verify the accuracy and rationality of the blade design, and it is an indispensable part in the blade certification process. In the full-scale static experiments, the strain of the wind turbine blade is related to the applied loads, loading positions, stiffness, deflection, and other factors. At present, researches focus on the analysis of blade failure causes, blade load-bearing capacity, and parameter measurement methods in addition to the correlation analysis between the strain and the applied loads primarily.

[Isolation and protection, a contradiction and a necessity].

The association of the two terms 'isolating' and 'protecting', seems counterintuitive. The practice of isolation conjures up images of an asylum or even prison, rather than a notion of safeguarding. Isolation is defined within caregiving institutions by a vast range of practices, neither rare nor recent.

Ultrafine particles and PM in the air of cities around the world: Are they representative of each other?

Can mitigating only particle mass, as the existing air quality measures do, ultimately lead to reduction in ultrafine particles (UFP)? The aim of this study was to provide a broader urban perspective on the relationship between UFP, measured in terms of particle number concentration (PNC) and PM (mass concentration of particles with aerodynamic diameter < 2.5 μm) and factors that influence their concentrations. Hourly average PNC and PM were acquired from 10 cities located in North America, Europe, Asia, and Australia over a 12-month period.

Reproductive success of assisted reproductive technology in couples with chromosomal abnormalities.

Purpose: Infertility is estimated to affect 15% of couples, having chromosome abnormalities an important role in its etiology. The main objective of this work was to access the reproductive success of ART in infertile couples with chromosomal abnormalities comparing to a control group with normal karyotype.

Methods: A 7-year retrospective karyotype analysis of infertile couples was done.

Cell Cycle Kinase Polo Is Controlled by a Widespread 3' Untranslated Region Regulatory Sequence in Drosophila melanogaster.

Alternative polyadenylation generates transcriptomic diversity, although the physiological impact and regulatory mechanisms involved are still poorly understood. The cell cycle kinase Polo is controlled by alternative polyadenylation in the 3' untranslated region (3'UTR), with critical physiological consequences. Here, we characterized the molecular mechanisms required for alternative polyadenylation.

Supernumerary nasal tooth removed with a modified maxillary vestibular approach: case report and literature review.

Purpose: The aim of this present study is to describe a case of supernumerary nasal tooth removed with a modified maxillary vestibular approach with subperiosteal dissection.

Methods: Also, a review of English-language literature of supernumerary nasal teeth from 1959 to 2018 was performed.

Results: This study demonstrated that the modified maxillary approach with subperiosteal intranasal dissection is a useful approach for the exposure and removal of teeth impacted in the floor of the nasal cavity.

An evaluation of efficacy of the auto-dissemination technique as a tool for Aedes aegypti control in Madeira, Portugal.

Background: The frequency and intensity of arboviral epidemics is steadily increasing and posing an intractable public health burden. Current vector control methods are proving ineffectual and despite progress in the development of high technology approaches, there is an urgent need for the development of tools for immediate implementation. Several studies suggest that the auto-dissemination of pyriproxyfen (PPF) is a promising new approach to larviciding although there is little detail on the conditions under which it is optimally effective.

NEOTROPICAL XENARTHRANS: a data set of occurrence of xenarthran species in the Neotropics.

Xenarthrans-anteaters, sloths, and armadillos-have essential functions for ecosystem maintenance, such as insect control and nutrient cycling, playing key roles as ecosystem engineers. Because of habitat loss and fragmentation, hunting pressure, and conflicts with domestic dogs, these species have been threatened locally, regionally, or even across their full distribution ranges. The Neotropics harbor 21 species of armadillos, 10 anteaters, and 6 sloths.

Novel taxa in Cladochytriales (Chytridiomycota): (gen. nov.) and (sp. nov.).

Six species from Brazil were identified and purified on corn meal agar (CMA) plus glucose and Peptonized Milk-Tryptone-Glucose (PmTG) media and placed into a phylogenetic framework for the genus. New sequence data are presented for four species: , and and the new species . Our maximum likelihood and Bayesian analyses of combined 18S, 5.

[Perpetrators and victims of sexual violence].

Shaped by culture and education, human sexuality is organised around the principle of mutual consent, and other limits fixed by the law, between partners in emotional exchanges and sexual relationships. When this is not the case, complex issues of sexual violence, inflicted and suffered, arise. Certain health professionals then become involved, working either with the perpetrator and/or the victims of this violence.

Diagnosing acquired syphilis through oral lesions: the 12 year experience of an Oral Medicine Center.

Introduction: A resurgence of syphilis in Brazil has been reported in recent years.

Objective: With this in mind, the present study sought to investigate the frequency, demographics, and clinical characteristics of patients with acquired syphilis with oral involvement who received medical care at an Oral Medicine Reference Center in a Brazilian Public Hospital.

Methods: A retrospective study, spanning a period of 12 years, was performed to identify changing trends in syphilis over time.

Oxidized Low-Density Lipoprotein (Ox-LDL) and Triggering Receptor-Expressed Myeloid Cell (TREM-1) Levels Are Associated with Cardiometabolic Risk in Nonobese, Clinically Healthy, and Young Adults.

Oxidative and inflammatory substances play an important role in the genesis of processes related to cardiometabolic risk. High levels of oxidized low-density lipoprotein (Ox-LDL) and of triggering receptor-expressed myeloid cells (TREM-1) are associated with cardiovascular and inflammatory diseases. In this study, we evaluate the association of the plasma concentrations of Ox-LDL and serum levels of circulating TREM-1 (sTREM-1) with the components of cardiometabolic risk (CMR) and other associated risk parameters.

STIM1 holds a STING in its (N-terminal) tail.

The Ca sensor STIM1 is essential for adaptive immune responses, yet patients with hypomorphic STIM1 mutations develop both immunodeficiency and autoimmunity, implying that STIM1 also restrains immune responses. This study by Srikanth et al demonstrates that STIM1 tethers STING, a major inducer of the interferon (IFN) response, to the endoplasmic reticulum (ER) to prevent constitutive STING activation.

First dengue virus seroprevalence study on Madeira Island after the 2012 outbreak indicates unreported dengue circulation.

Background: In 2012, the first dengue virus outbreak was reported on the Portuguese island of Madeira with 1080 confirmed cases. Dengue virus of serotype 1 (DENV-1), probably imported from Venezuela, caused this outbreak with autochthonous transmission by invasive Aedes aegypti mosquitoes.

Results: We investigated the seroprevalence among the population on Madeira Island four years after the outbreak.

Enhancement of water solubility of poorly water-soluble drugs by new biocompatible N-acetyl amino acid N-alkyl cholinium-based ionic liquids.

The major challenge of the pharmaceutical industry is to find potential solvents for poorly water-soluble drug molecules. Ionic liquids (ILs) have attracted this industry as (co-) solvents due to their unique physicochemical and biological properties. Herein, a straightforward approach for the enhancement of the water solubility of paracetamol and sodium diclofenac is presented, using new biocompatible N-acetyl amino acid N-alkyl cholinium-based ionic liquids as co-solvents (0.