Glavobolja i afazija u mlade žene s nedostatkom proteina S i nefrotskim sindromom

We present sequential brain imaging findings in a young woman who while being evaluated for a newly discovered nephrotic syndrome developed headache followed by aphasia. The patient’s symptoms were due to cerebral venous thrombosis in the setting of protein S deficiency and oral contraception.Predstavljamo sekvencijski nalaz slikovnog prikaza mozga mlade žene kod koje je tijekom kliničke evaluacije novootkrivenog nefrotskog sindroma došlo do pojave glavobolje praćene afazijom. Simptomi su bili uzrokovani cerebralnom venskom trombozom koja se razvila uslijed nedostatka proteina S i uporabe oralnih kontraceptiva

HCV cirrhotic patients treated with direct acting antivirals: detection of tubular dysfunction and resolution after viral clearance

Background/aims: Hepatitis C virus (HCV) has been identified in tubular epithelial cells of infected patients, however the presence of tubular dysfunction, which is a risk factor for chronic kidney disease, has never been examined in vivo. The present prospective longitudinal study aimed to estimate the prevalence of tubular dysfunction alone or with glomerular damage and its evolution after HCV clearance in cirrhotic patients. Methods: One-hundred-thirty-five consecutive Child-Pugh-A cirrhotic patients were evaluated before antiviral treatment and six months after the end of therapy. Tubular dysfunction was evaluated by urinary-alpha1-microglobulin-to-creatinine-ratio (α1-MCR), glomerular damage was assessed by urinary-albumin-to-creatinine-ratio (ACR). Results: Almost all the patients (93.3%) showed a normal or mildly decreased e-GFR (KDIGO-G1/G2-categories). Tubular dysfunction was found in 23.7% (32/135) of patients, co-occurring with glomerular damage in 37.5% (12/32) of cases, while glomerular damage was found in 16.3% (22/135) of patients. In multiple logistic regression, glomerular damage and the concomitant presence of diabetes and hypertension were the only predictors significantly associated with tubular dysfunction. After HCV-clearance, patients experienced a significant reduction of α1-MCR levels (21.0 vs 10.5 μg/mg, p=0.009) and tubular dysfunction resolved in 57.1% of subjects. Conclusions: Tubular dysfunction is an unrecognized feature of HCV-related kidney disease in cirrhotic patients and its presence should be primarily investigated in subjects with glomerular damage, diabetes and hypertension, despite normal e-GFR. Tubular dysfunction resolves in the majority of cases after HCV clearance, however, it may persist after antiviral treatment and further studies should evaluate its long term impact on kidney function

Outcomes of Reduced Intensity Conditioning Allogeneic Hematopoietic Stem Cell Transplantation for Hodgkin Lymphomas: A Retrospective Multicenter Experience by the Rete Ematologica Pugliese (REP)

Patients with Hodgkin lymphomas progressing after autologous stem cell transplantation (SCT) have a very poor outcome. Our retrospective analysis confirms that reduced-intensity conditioning allogeneic SCT may be an effective salvage strategy for patients who relapse after an autologous SCT and that outcomes are similar for both sibling and matched-unrelated donor transplants. Patients with active disease at transplantation have poor outcomes. Background: Hodgkin lymphoma (HL) is a potentially curable disease, and modern therapy is expected to successfully cure more than 80% of the patients. However, patients progressing after intensive treatments, such as autologous stem cell transplantation (SCT), have a very poor outcome. Allogeneic SCT offers the only strategy with a curative potential for these patients. This study reports a retrospective multicenter experience of the Rete Ematologica Pugliese (REP) over the past 17 years, aiming to define the impact of each patient's disease and transplant-related characteristics on outcomes. Patients and Methods: We retrospectively studied 72 patients with HL who received allogeneic SCT from 2000 to 2017. At the time of allogeneic SCT, 33 (46%) patients had chemosensitive disease, and 39 (54%) were chemo-refractory. All patients received reduced-intensity conditioning, 50% received grafts from a matched sibling donor, and 50% from a matched-unrelated donor. Results: With a median follow-up of 48 months (range, 3-195 months), 30 patients are alive, and 42 have died. The Kaplan-Meier estimates of overall survival and progression-free survival at 5 years were 35% and 34%, respectively. Following transplantation, 12 (17%) patients died of non-relapse mortality at a median of 90 days (range, 1 day-20 months). The causes of death included infection (n = 7), graft-versus-host disease (n = 3), and multi-organ failure (n = 2). Conclusions: Allogeneic SCT results extend survival in selected patients with relapsed/refractory HL, showing low treatment-related mortality. Patients with active disease at the time of allogeneic transplantation have poor outcomes. Allogeneic SCT may be an effective salvage strategy for patients who relapse after an autologous SCT

A multi-service data management platform for scientific oceanographic products

Abstract. An efficient, secure and interoperable data platform solution has been developed in the TESSA project to provide fast navigation and access to the data stored in the data archive, as well as a standard-based metadata management support. The platform mainly targets scientific users and the situational sea awareness high-level services such as the decision support systems (DSS). These datasets are accessible through the following three main components: the Data Access Service (DAS), the Metadata Service and the Complex Data Analysis Module (CDAM). The DAS allows access to data stored in the archive by providing interfaces for different protocols and services for downloading, variables selection, data subsetting or map generation. Metadata Service is the heart of the information system of the TESSA products and completes the overall infrastructure for data and metadata management. This component enables data search and discovery and addresses interoperability by exploiting widely adopted standards for geospatial data. Finally, the CDAM represents the back-end of the TESSA DSS by performing on-demand complex data analysis tasks

GLUCOCORTICOID USE IN STEVENS-JOHNSON SYNDROME AND TOXIC EPIDERMAL NECROLYSIS CASES IN HOSPITAL NOSSA SENHORA DA CONCEIÇÃO - MARCH 2007 TO AUGUST 2014

Introdução: A síndrome de Stevens-Johnson e a necrólise epidérmica tóxica (NET) são reações cutâneas severas, associadas a lesões mucosas e comprometimento sistêmico. Os glicocorticoides foram empregues por muitos anos no tratamento da síndrome de Stevens-Johnson/necrólise epidérmica tóxica, mas não há evidências que sustentem esta conduta.Objetivo: Avaliação da mortalidade em casos de síndrome de Stevens-Johnson/necrólise epidérmica tóxica em um grupo de pacientes que fez uso de glicocorticoides sistêmico e em outro grupo não fez uso.Objetivo secundário: Descrição da população estudada; avaliação de infecção secundária e complicações extra-cutâneas nos dois grupos de pacientes.Material e Métodos: Coorte retrospectiva de pacientes avaliados por meio de consultorias solicitadas ao Serviço de Dermatologia do Hospital Nossa Senhora da Conceição, bem como pelo diagnóstico na internação ou óbito, durante o período de Março de 2007 a Agosto de 2014.Resultados: Mortalidade foi de 63.2% no grupo que usou corticoide e 9,1% no grupo que não usou corticoide, com p valor de 0,005 (IC 95% 1.79-163.8). Análise multivariada ajustada pelo SCORTEN demonstrou apenas uma tendência, com odds ratio para mortalidade no grupo de pacientes que usou glicocorticoides sendo de 7.38 (IC 95% 0.87-161.43) e p valor de 0.06.Conclusão: Este estudo sugere uma tendência de malefício relacionado ao uso de glicocorticoides para o tratamento dessas doenças, principalmente no que diz respeito à mortalidade.Introduction: Stevens-Johnson syndrome and toxic epidermal necrolysis are severe skin reactions associated with mucosal lesions and systemic involvement. Glucocorticoids were used for many years in the treatment of Stevens-Johnson syndrome / Toxic Epidermal Necrolysis, but there is no evidence to support this approach.Objective: Assessment of mortality in Stevens-Johnson syndrome/Toxic Epidermal Necrolysis cases in a group of patients that used systemic glucocorticoids and another group who did not.Secondary objective: Description of the study population; review of secondary infection and extra-cutaneous complications in both groups of patients.Material and Methods: Retrospective cohort of patients selected by consulting requested to the Dermatology Service of the Hospital Nossa Senhora da Conceição, as well as the diagnosis at admission or death, during the period March 2007 to August 2014.Results: Mortality rate was 63.2% in the group that used corticosteroids and 9.1% in the group that did not use steroids, with p value 0.005 (95% CI 1.79-163.8). Multivariate analysis adjusted for SCORTEN showed only a trend for harm, with an odds ratio for mortality in patients who used glucocorticoids being of 7:38 (95% CI 0.87-161.43) and p value 0.06.Conclusion: This study suggests a trend for harm associated with glucocorticoids use for treatment of these diseases, especially in regard to mortality

Cell Volume Regulation Mechanisms in Differentiated Astrocytes

The ability of astrocytes to control extracellular volume homeostasis is critical for brain function and pathology. Uncovering the mechanisms of cell volume regulation by astrocytes will be important for identifying novel therapeutic targets for neurological conditions, such as those characterized by imbalances to hydro saline challenges (as in edema) or by altered cell volume regulation (as in glioma). One major challenge in studying the astroglial membrane channels involved in volume homeostasis in cell culture model systems is that the expression patterns of these membrane channels do not resemble those observed in vivo. In our previous study, we demonstrated that rat primary astrocytes grown on nanostructured interfaces based on hydrotalcite-like compounds (HTlc) in vitro are differentiated and display molecular and functional properties of in vivo astrocytes, such as the functional expression of inwardly rectifying K+ channel (Kir 4.1) and Aquaporin-4 (AQP4) at the astrocytic microdomain. Here, we take advantage of the properties of differentiated primary astrocytes in vitro to provide an insight into the mechanism underpinning astrocytic cell volume regulation and its correlation with the expression and function of AQP4, Transient Receptor Potential Vanilloid 4 (TRPV4), and Volume Regulated Anion Channel (VRAC)

Allergens in allergy diagnosis: a glimpse at emerging new concepts and methodologies

Allergic diseases are important concern of public health. A reliable diagnosis is of utmost importance for the management of allergic patients both when immunotherapy is planned and when the treatment is essentially based on the avoidance of the allergy source. However, the available diagnostic systems sometimes fail to detect specific IgE antibodies thus impairing the correct diagnosis. The traditional test systems are generally based on the use of protein extracts derived from the allergenic sources whose composition is very variable and cannot be standardized. The development of a new methodology combining the so-called allergenic molecule-based diagnosis with the multiplex microarray technology and allowing the analysis of multiple purified allergens in a single test represents an important improvement in allergy diagnosis. In addition, the biochemical and immunological characterisation of individual allergens has provided new insights into the understanding of allergen-IgE recognition that could be exploited for further improvements of allergy diagnostic tests

SeaConditions: a web and mobile service for safer professional and recreational activities in the Mediterranean Sea

Abstract. Reliable and timely information on the environmental conditions at sea is key to the safety of professional and recreational users as well as to the optimal execution of their activities. The possibility of users obtaining environmental information in due time and with adequate accuracy in the marine and coastal environment is defined as sea situational awareness (SSA). Without adequate information on the environmental meteorological and oceanographic conditions, users have a limited capacity to respond, which has led to loss of lives and to large environmental disasters with enormous consequent damage to the economy, society and ecosystems. Within the framework of the TESSA project, new SSA services for the Mediterranean Sea have been developed. In this paper we present SeaConditions, which is a web and mobile application for the provision of meteorological and oceanographic observation and forecasting products. Model forecasts and satellite products from operational services, such as ECMWF and CMEMS, can be visualized in SeaConditions. In addition, layers of information related to bathymetry, sea level and ocean-colour data (chl a and water transparency) are displayed. Ocean forecasts at high spatial resolutions are included in the version of SeaConditions presented here. SeaConditions provides a user-friendly experience with a fluid zoom capability, facilitating the appropriate display of data with different levels of detail. SeaConditions is a single point of access to interactive maps from different geophysical fields, providing high-quality information based on advanced oceanographic models. The SeaConditions services are available through both web and mobile applications. The web application is available at www.sea-conditions.com and is accessible and compatible with present-day browsers. Interoperability with GIS software is implemented. User feedback has been collected and taken into account in order to improve the service. The SeaConditions iOS and Android apps have been downloaded by more than 105 000 users to date (May 2016), and more than 100 000 users have visited the web version