Major CD4 T-Cell Depletion and Immune Senescence in a Patient with Chronic Granulomatous Disease

Chronic granulomatous disease (CGD) results from primary defects in phagocytic reactive oxygen species (ROS) production. T-cell evaluation is usually neglected during patients’ follow-up, although T-cell depletion has been reported in CGD through unknown mechanisms. We describe here a 36-year-old patient with X-linked CGD with severe CD4 T-cell depletion <200 CD4 T-cells/μl, providing insights into the mechanisms that underlie T-cell loss in the context of oxidative burst defects. In addition to the typical infections, the patient featured a progressive T-cell loss associated with persistent lymphocyte activation, expansion of interleukin (IL)-17-producing CD4 T-cells, and impaired thymic activity, leading to a reduced replenishment of the T-cell pool. A relative CD4 depletion was also found at the gut mucosal level, although no bias to IL-17-production was documented. This immunological pattern of exhaustion of immune resources favors prompt, potentially curative, therapeutic interventions in CGD patients, namely, stem-cell transplantation or gene therapy. Moreover, this clinical case raises new research questions on the interplay of ROS production and T-cell homeostasis and immune senescence

Satellites and large doping- and temperature-dependence of electronic properties in hole-doped BaFe2As2

Over the last years, superconductivity has been discovered in several families of iron-based compounds. Despite intense research, even basic electronic properties of these materials, such as Fermi surfaces, effective electron masses, or orbital characters are still subject to debate. Here, we address an issue that has not been considered before, namely the consequences of dynamical screening of the Coulomb interactions among Fe-d electrons. We demonstrate its importance not only for correlation satellites seen in photoemission spectroscopy, but also for the low-energy electronic structure. From our analysis of the normal phase of BaFe2As2 emerges the picture of a strongly correlated compound with strongly doping- and temperature-dependent properties. In the hole overdoped regime, an incoherent metal is found, while Fermi-liquid behavior is recovered in the undoped compound. At optimal doping, the self-energy exhibits an unusual square-root energy dependence which leads to strong band renormalizations near the Fermi level

Couple’s Relationship After the Death of a Child: a Systematic Review

When a child dies, the parents must address the changes in their relationship as well as the way that these changes affect their individual adjustment. These two perspectives are addressed in this systematic review. Five databases were systematically searched for papers published in English between January 2000 and February 2014. Of the 646 publications, 24 papers met the inclusion criteria. The results suggest that a child’s death can cause cohesive as well as detrimental effects on a couple’s relationship. Variables that may produce differential outcomes for the marital relationship include situational factors, such as the cause and type of death and the child’s age at the time of death; dyad-level factors, such as surviving children, the pre-death characteristics of the relationship, communication and incongruent grieving; and individuallevel factors, such as the family of origin’s processing of trauma, social support, religious affiliation and finding meaning. Aspects such as marital quality and the couple’s interdependence were found to influence each parent’s individual adjustment. Larger, prospective, ethically conducted studies should be implemented to consolidate these findings. Mental health professionals may benefit from a deeper understanding of the risk and protective factors regarding marital adjustment after a child’s death

Approaches in biotechnological applications of natural polymers

Natural polymers, such as gums and mucilage, are biocompatible, cheap, easily available and non-toxic materials of native origin. These polymers are increasingly preferred over synthetic materials for industrial applications due to their intrinsic properties, as well as they are considered alternative sources of raw materials since they present characteristics of sustainability, biodegradability and biosafety. As definition, gums and mucilages are polysaccharides or complex carbohydrates consisting of one or more monosaccharides or their derivatives linked in bewildering variety of linkages and structures. Natural gums are considered polysaccharides naturally occurring in varieties of plant seeds and exudates, tree or shrub exudates, seaweed extracts, fungi, bacteria, and animal sources. Water-soluble gums, also known as hydrocolloids, are considered exudates and are pathological products; therefore, they do not form a part of cell wall. On the other hand, mucilages are part of cell and physiological products. It is important to highlight that gums represent the largest amounts of polymer materials derived from plants. Gums have enormously large and broad applications in both food and non-food industries, being commonly used as thickening, binding, emulsifying, suspending, stabilizing agents and matrices for drug release in pharmaceutical and cosmetic industries. In the food industry, their gelling properties and the ability to mold edible films and coatings are extensively studied. The use of gums depends on the intrinsic properties that they provide, often at costs below those of synthetic polymers. For upgrading the value of gums, they are being processed into various forms, including the most recent nanomaterials, for various biotechnological applications. Thus, the main natural polymers including galactomannans, cellulose, chitin, agar, carrageenan, alginate, cashew gum, pectin and starch, in addition to the current researches about them are reviewed in this article.. }To the Conselho Nacional de Desenvolvimento Cientfíico e Tecnológico (CNPq) for fellowships (LCBBC and MGCC) and the Coordenação de Aperfeiçoamento de Pessoal de Nvíel Superior (CAPES) (PBSA). This study was supported by the Portuguese Foundation for Science and Technology (FCT) under the scope of the strategic funding of UID/BIO/04469/2013 unit, the Project RECI/BBB-EBI/0179/2012 (FCOMP-01-0124-FEDER-027462) and COMPETE 2020 (POCI-01-0145-FEDER-006684) (JAT)

Multi-messenger observations of a binary neutron star merger

On 2017 August 17 a binary neutron star coalescence candidate (later designated GW170817) with merger time 12:41:04 UTC was observed through gravitational waves by the Advanced LIGO and Advanced Virgo detectors. The Fermi Gamma-ray Burst Monitor independently detected a gamma-ray burst (GRB 170817A) with a time delay of ~1.7 s with respect to the merger time. From the gravitational-wave signal, the source was initially localized to a sky region of 31 deg2 at a luminosity distance of 40+8-8 Mpc and with component masses consistent with neutron stars. The component masses were later measured to be in the range 0.86 to 2.26 Mo. An extensive observing campaign was launched across the electromagnetic spectrum leading to the discovery of a bright optical transient (SSS17a, now with the IAU identification of AT 2017gfo) in NGC 4993 (at ~40 Mpc) less than 11 hours after the merger by the One- Meter, Two Hemisphere (1M2H) team using the 1 m Swope Telescope. The optical transient was independently detected by multiple teams within an hour. Subsequent observations targeted the object and its environment. Early ultraviolet observations revealed a blue transient that faded within 48 hours. Optical and infrared observations showed a redward evolution over ~10 days. Following early non-detections, X-ray and radio emission were discovered at the transient’s position ~9 and ~16 days, respectively, after the merger. Both the X-ray and radio emission likely arise from a physical process that is distinct from the one that generates the UV/optical/near-infrared emission. No ultra-high-energy gamma-rays and no neutrino candidates consistent with the source were found in follow-up searches. These observations support the hypothesis that GW170817 was produced by the merger of two neutron stars in NGC4993 followed by a short gamma-ray burst (GRB 170817A) and a kilonova/macronova powered by the radioactive decay of r-process nuclei synthesized in the ejecta

Atualização da diretriz brasileira de insuficiência cardíaca crônica - 2012

Esta atualização da Diretriz de Insuficiência Cardíaca Crônica (IC) - 2012 surge para reavaliar as recomendações através de uma avaliação criteriosa das pesquisas (considerando-se a qualidade dos estudos), fundamental para que se atinja esse propósito. Para tanto, foi dada ênfase ao efeito em desfechos de morte, à qualidade "CONSORT" (Consolidated Standards of Reporting Trials), à descrição qualitativa e quantitativa da otimização da medicação, à população realmente incluída, às metanálises somente de estudos qualidade "CONSORT", à custo-efetividade, à existência de efeito de classe, ao número de pacientes incluídos e à análise de subgrupos apenas para gerar hipóteses. Na área da epidemiologia, as recentes abordagens das características da IC com fração de ejeção preservada (ICFEP) e da importância da IC como causa de morte no Brasil foram revisadas. Além disso, este documento contempla a reavaliação do valor dos biomarcadores no diagnóstico e no seguimento da IC, o papel diagnóstico da angiotomografia coronariana nos casos de risco intermediário ou baixo risco de doença coronariana, a não recomendação de rotina do telemonitoramento; o surgimento da avaliação familiar como recomendação importante, e a reavaliação da restrição da adição de sal na dieta. As clínicas de IC e reabilitação física, apesar de alguns resultados negativos ou controversos quanto à mortalidade, continuam com recomendação importante. No campo do tratamento farmacológico, abrange-se a reavaliação da indicação do nebivolol, introduz-se a ivabradina como um novo paradigma no tratamento, os antagonistas da aldosterona não têm efeito de classe reconhecido, o ômega 3 passa a ser recomendado, o ferro administrado por via endovenosa e o sildenafil recebem indicação em casos selecionados. Todas as recomendações para outras etiologias são expandidas para a Doença de Chagas. Na área da anticoagulação, recomenda-se a utilização dos escores CHA2DS2VASC e o HAS-BLED na fibrilação atrial, com introdução de inibidores da trombina e do fator Xa como alternativas na anticoagulação. No tratamento cirúrgico da IC, considerou-se que resultados neutros do estudo STICH influenciaram as recomendações, o transplante cardíaco continua a ser o tratamento indicado nas fases evolutivas tardias de IC, os dispositivos de assistência circulatória mecânica para terapia de destino passam a ter recomendação, a duração do QRS foi fundamental na indicação de TRV-AV, e o CDI continua com recomendação I para miocardiopatia isquêmica. Entretanto, baseada em análise crítica dos estudos considerando-se o custo-efetividade, o CDI não atingiu recomendação I para classes menos graves devido as limitações dos estudos. Também a importância da cardiotoxicidade por drogas para tratamento de neoplasias foi ressaltada, o tratamento da IC na gravidez e da miocardite foi revisado. Novos potenciais métodos de tratamento em fase de pesquisa são apresentados e novos fluxogramas de diagnóstico e tratamento da IC, reformulados, foram incluído

A comprehensive characterization of chronic norovirus infection in immunodeficient hosts.

Letter to the edito

MTHFR Polymorphic Variant C677T Is Associated to Vascular Complications in Sickle-Cell Disease

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Vaso-occlusion is a determinant for most signs and symptoms of sickle-cell anemia (SCA). The mechanisms involved in the pathogenesis of vascular complications in SCA remain unclear. It is known that genetic polymorphisms associated with thrombophilia may be potential modifiers of clinical features of SCA. The genetic polymorphisms C677T and A1298C relating to the enzyme methylenetetrahydrofolate reductase (MTHFR), a clotting Factor V Leiden mutation (1691G -> A substitution of Factor V Leiden), and the mutant prothrombin 20210A allele were analyzed in this study. The aim was to find possible correlations with vascular complications and thrombophilia markers in a group of SCA patients in Pernambuco, Brazil. The study included 277 SCA patients, divided into two groups: one consisting of 177 nonconsanguineous SCA patients who presented vascular manifestations of stroke, avascular necrosis, leg ulcers, priapism, and acute chest syndrome (group 1); and the other consisting of 100 SCA patients without any reported vascular complication (group 2). Molecular tests were done using either polymerase chain reaction (PCR) restriction fragment length polymorphism or allele-specific PCR techniques. Comparisons between the groups were made using the chi(2) test. The 677 CT and TT genotypes showed a significant risk of vascular complications (p = 0.015). No significant associations between the groups were found when samples were analyzed for the MTHFR A1298C allele (p = 0.913), Factor V G1691 (p = 0.555), or prothrombin G20210A mutation (p = 1.000). The polymorphism MTHFR C677T seemed to be possibly predictive for the development of some vascular complications in SCA patients among this population.16910381043Science and Technology Support Foundation of the State of Pernambuco (Fundacao de Amparo a Ciencia e Tecnologia do Estado de PernambucoFACEPE)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq

Chilling requirement for seed germination and phenological observations on peach cultivars

In subtropical climate areas, the models and methods proposed to evaluate the chilling requirement of temperate fruit crops often do not provide satisfactory results, thus calling for the development of alternative techniques. The aim of this study was to evaluate the correlations between some phonological traits and chilling requirement for seed germination of 18 peach cultivars and one nectarine cultivar. Two experiments were installed separately for the correlation studies. In experiment 1, the phenological traits were observed in the field, while in experiment 2, the chilling requirement for 50 and 100% seed germination of each cultivar was assessed. The number of days for beginning of bloom (r = 0.70**, 0.61**) and full bloom (r = 0.72**, 0.76**) were both significantly correlated with the number of chilling units for 50% and 100% germination of seeds. The number of days for beginning of budding and dormancy break were both significantly correlated with the number of chilling units for 50% and 100% germination (r = 0.48*, 0.50*, respectively). However, the same significant effect for these phenological traits was not found between chilling units and 50% germination of seeds, as well as between chilling units and harvest dates