Incidence of Shwachman-Diamond syndrome

No abstract availabl

Structural Change in the Automotive Industry and its Regional Impacts: The Case of Brazil

From the late 1980s onward, the Brazilian automotive industry has undergone significant change as it has embraced trade liberalization and growing inward FDI. Using an inter-regional input-output model, this paper analyses the evolution of the industry from a spatial perspective. It is shown that new investment has resulted in a more dispersed locational framework for the industry. However, not all the benefits of new investment are garnered by the recipient regions. The paper poses a number of policy questions which center on the issue of inter-regional dependence

Impacto Das Exportações Brasileiras Para O Mercosul, União Européia E Nafta Sobre Produção E Emprego: Uma Análise De Insumo-Produto Para 1997 -2001

The Brazilian exports and the degree of openness of the Brazilian economy have increased in the recent period. Brazil is searching for new trade partners in order to improve its share in the international trade. This paper has as principal aim analyze the impact of those strategies upon production and employment. In order to reach this aim we will use the input-output matrices for the Brazilian economy estimated by Guilhoto and Sesso (2005). We will open the exports component of final demand into four trade blocks

Absence of acquired copy number neutral loss of heterozygosity (CN-LOH) of chromosome 7 in a series of 10 patients with Shwachman-Diamond syndrome

We report that acquired copy number neutral loss of heterozygosity (CN-LOH) of chromosome 7 was not identified in a series of 10 patients with Shwachman\u2013Diamond syndrome (SDS)

Diet-induced Obesity In Rats Leads To A Decrease In Sperm Motility.

Obesity is rapidly becoming a worldwide epidemic that affects children and adults. Some studies have shown a relationship between obesity and infertility, but until now it remains controversial. Thus, the aim of the present study was to investigate the effect of high-fat diet-induced obesity on male reproductive parameters. In a first experiment, male Wistar rats were fed a high-fat diet (HFD) or standard chow (SD) for 15, 30 or 45 weeks, after which they were evaluated by adiposity index, serum leptin levels, reproductive organ weights and sperm counts. In a second experiment, rats received HFD or SD only for 15 weeks, long enough to cause obesity. Sexual hormones and sexual behavior were evaluated in these animals, as well as fertility after natural mating. Another group of rats was submitted to motility analysis and fertility evaluation after in utero insemination. After 15, 30 or 45 weeks, HFD-fed animals presented significant increases in obesity index and serum leptin levels. Reproductive organ weights and sperm counts in the testis and epididymis were similar between the two groups at all timepoints studied. Sexual behavior was not altered by the diet regimen, and HFD fertility after natural mating was also similar to SD-fed animals. Intergroup testosterone levels were also comparable, but estradiol levels were increased in HFD rats. Furthermore, sperm quality was reduced in HFD animals as evidenced by their decreased percentage of sperm with progressive movement. This altered motility parameter was followed by a trend toward reduction in fertility potential after artificial in utero insemination. The results reported herein showed that obesity can affect sperm quality, by reducing sperm motility, without affecting other sperm parameters. The low sperm quality caused a slight reduction in fertility potential, showing that obesity may lead to impairment in male fertility.93

Investigation of Rubble-Masonry Wall Construction Practice in Latium, Central Italy

The 2016-2017 Central Italy seismic sequence severely affected existing unreinforced-masonry constructions in four regions. Those in Latium region proved the most prone to fragmentation because of an unfortunate combination of undressed natural stone units and very low lime content in mortar. Within the framework of a research project funded by the regional government, shake table tests are planned to investigate masonry disintegration as well as possible intervention techniques, as described in a companion paper. All specimens will have natural stone units retrieved from the debris in Collespada, a settlement of the municipality of Accumoli, one of the most affected by the seismic sequence. To push further the representativeness of the specimens with respect to field conditions, wall geometry, masonry fabric and mortar recipe are carefully designed. The wall thickness will be approximately equal to 0.5 m, close to average thickness surveyed in the area. Following the survey of several vertical sections of actual masonry walls, the specimens will present unconnected external leaves with a limited nucleus. Based on tests on mortar sampled from collapsed buildings, mortars will be prepared by a part of natural lime every nine parts of sand. Shear tests on sampled mortar delivered apparent cohesion and friction coefficient that are used as preliminary values of a finite-discrete element model, which can account for masonry fragmentation in dynamic non-linear analyses. The numerical model was tested under the envisioned sequence of records, belonging to the Amatrice station and related to the East component, approximately fault normal, of the two main seismic events, 24 August and 30 October, 2016

Local and systemic immunomodulatory mechanisms triggered by Human Papillomavirus transformed cells: a potential role for G-CSF and neutrophils

Cervical cancer is the last stage of a series of molecular and cellular alterations initiated with Human Papillomavirus (HPV) infection. The process involves immune responses and evasion mechanisms, which culminates with tolerance toward tumor antigens. Our objective was to understand local and systemic changes in the interactions between HPV associated cervical lesions and the immune system as lesions progress to cancer. Locally, we observed higher cervical leukocyte infiltrate, reflected by the increase in the frequency of T lymphocytes, neutrophils and M2 macrophages, in cancer patients. We observed a strong negative correlation between the frequency of neutrophils and T cells in precursor and cancer samples, but not cervicitis. In 3D tumor cell cultures, neutrophils inhibited T cell activity, displayed longer viability and longer CD16 expression half-life than neat neutrophil cultures. Systemically, we observed higher plasma G-CSF concentration, higher frequency of immature low density neutrophils, and tolerogenic monocyte derived dendritic cells, MoDCs, also in cancer patients. Interestingly, there was a negative correlation between T cell activation by MoDCs and G-CSF concentration in the plasma. Our results indicate that neutrophils and G-CSF may be part of the immune escape mechanisms triggered by cervical cancer cells, locally and systemically, respectively.Tis study was supported by Sao Paulo Research foundation: grants 2008/57889-1, 2010/20010-4, 2014/19326-6, by the Brazilian National Counsel of Technological and Scientifc Development: grant 573799/2008-3. KLFA and RAMR had PhD fellowships by Sao Paulo Research Foundation, CRSF has a Coordination for the Improvement of Higher Education Personnel PhD fellowship. We thank the Pathology Department of the School of Medicine, coordinated by Prof. Venâncio Avancini Ferreira Alves, Universidade de São Paulo for the slides containing histological samples from the biopsies used in this study. We thank Sandra Alexandre Alves for her technical support.info:eu-repo/semantics/publishedVersio

Uncovering the multifaceted roles played by neutrophils in allogeneic hematopoietic stem cell transplantation

Allogeneic hematopoietic stem cell transplantation (alloHSCT) is a life-saving procedure used for the treatment of selected hematological malignancies, inborn errors of metabolism, and bone marrow failures. The role of neutrophils in alloHSCT has been traditionally evaluated only in the context of their ability to act as a first line of defense against infection. However, recent evidence has highlighted neutrophils as key effectors of innate and adaptive immune responses through a wide array of newly discovered functions. Accordingly, neutrophils are emerging as highly versatile cells that are able to acquire different, often opposite, functional capacities depending on the microenvironment and their differentiation status. Herein, we review the current knowledge on the multiple functions that neutrophils exhibit through the different stages of alloHSCT, from the hematopoietic stem cell (HSC) mobilization in the donor to the immunological reconstitution that occurs in the recipient following HSC infusion. We also discuss the influence exerted on neutrophils by the immunosuppressive drugs delivered in the course of alloHSCT as part of graft-versus-host disease (GVHD) prophylaxis. Finally, the potential involvement of neutrophils in alloHSCT-related complications, such as transplant-associated thrombotic microangiopathy (TA-TMA), acute and chronic GVHD, and cytomegalovirus (CMV) reactivation, is also discussed. Based on the data reviewed herein, the role played by neutrophils in alloHSCT is far greater than a simple antimicrobial role. However, much remains to be investigated in terms of the potential functions that neutrophils might exert during a highly complex procedure such as alloHSCT