Exploring Cortical Attentional System by Using fMRI during a Continuous Perfomance Test

Functional magnetic resonance imaging (fMRI) was performed in eight healthy subjects to identify the localization, magnitude, and volume extent of activation in brain regions that are involved in blood oxygen level-dependent (BOLD) response during the performance of Conners' Continuous Performance Test (CPT). An extensive brain network was activated during the task including frontal, temporal, and occipital cortical areas and left cerebellum. The more activated cluster in terms of volume extent and magnitude was located in the right anterior cingulate cortex (ACC). Analyzing the dynamic trend of the activation in the identified areas during the entire duration of the sustained attention test, we found a progressive decreasing of BOLD response probably due to a habituation effect without any deterioration of the performances. The observed brain network is consistent with existing models of visual object processing and attentional control and may serve as a basis for fMRI studies in clinical populations with neuropsychological deficits in Conners' CPT performance

Rejection-mediated Regression of Melanocytic Naevi in an Immunosuppressed Organ Transplant Recipient

© 2014 The Authors. doi: 10.2340/00015555-1789 Journal Compilation © 2014 Acta Dermato-Venereologica. ISSN 0001-5555 Eruptive melanocytic naevi and/or excess of melanocytic naevi have been reported in several groups of immunosuppressed patients. The eruption of melanocytic naevi after immunosuppression is a peculiar phenomenon indicating that the immune system may play a major role in limitating proliferation of melanocytes (1). In this article we describe a patient with excess of post-transplant melanocytic naevi that spontaneously disappeared after graft rejection

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O Brasil que o Brasil diz ser: como a mídia televisiva repercutiu nossa narrativa histórico-cultural na abertura dos Jogos Olímpicos Rio/2016

Oobjetivo deste estudo écompreender as narrativas simbólicas sobre o Brasil (história, cultura, personagens), expressa por jornalistas da televisão aberta na solenidade de abertura dos JO Rio/2016. As transmissõesde três redes do Brasil (Globo, Band, Record) e uma espanhola (TVE) foram gravadas e analisadas desde a perspectiva da teoria da convergência digital. Percebemos que mesmo havendo um guia de mídia, comum as emissoras, cada uma possui suas características de transmissão

A novel primary human immunodeficiency due to deficiency in the WASP-interacting protein WIP

A female offspring of consanguineous parents, showed features of Wiskott-Aldrich syndrome (WAS), including recurrent infections, eczema, thrombocytopenia, defective T cell proliferation and chemotaxis, and impaired natural killer cell function. Cells from this patient had undetectable WAS protein (WASP), but normal WAS sequence and messenger RNA levels. WASP interacting protein (WIP), which stabilizes WASP, was also undetectable. A homozygous c.1301C>G stop codon mutation was found in the WIPF1 gene, which encodes WIP. Introduction of WIP into the patient’s T cells restored WASP expression. These findings indicate that WIP deficiency should be suspected in patients with features of WAS in whom WAS sequence and mRNA levels are normal

The fusion approach – applications for understanding local government and European integration

The article explores the theoretical capabilities of the fusion approach as a conceptual ‘kit’ to explain the ‘bigger picture’ of European integration from a local government perspective. Fusion addresses the rationales and methods facilitating the transfer of policy-making competences to the European level. It understands European integration as a merging of public resources and policy instruments from multiple levels of government, whereby accountability and responsibilities for policy outcomes become blurred. The article argues that the fusion approach is useful to explain the systemic linkages between macro-trajectories and the corresponding change at the local level; the fusion dynamics of the local and European levels in a common policy-cycle; and the attitudes of local actors towards the EU. Although the article concludes that local government is rather modestly ‘fused’ into the EU, fusion approaches allow examining the extent to which the local level has become integrated into the European governance system

Pediatric Systemic Multi-Inflammatory Diseases in Italy During Sars-Cov-2 Epidemic: From Kawasaki Disease To Kawacovid

Introduction: Italy was affected by the SARS-CoV-2 epidemic after its outbreak in China. With a 4-weeks delay after the peak in adults, we observed an abnormal number of patients with characteristics of a multi-inflammatory disease and similarities with Kawasaki Disease (KD). Others reported similar cases, defined PIMS-TS or MIS-C.1,2 Objectives: To better characterize clinical features and treatment response of PIMS-TS and to explore its relationship with KD. Methods: We conducted an observational, retrospective, multicenter study. On April 24th-2020 the Rheumatology Study Group of the Italian Pediatric Society launched a national online survey, to enroll patients diagnosed with KD or with a multisystem inflammatory disease between February 1st 2020 and May 31st. The population was then divided into two different groups: 1) Classical and incomplete KD, named Kawasaki Disease Group (KDG); 2) KD-like multi-inflammatory syndrome, named KawaCOVID (KCG). An expert panel of pediatric rheumatologists re-analyzed every single patient to ensure appropriate classification. Data were collected with an online database. Results: 149 cases were studied, 96 with KDG and 53 with KCG. The two population significantly differed for clinical characteristics (see table 1). Lymphopenia, higher CRP levels, elevated Ferritin and Troponin-T characterized KCG such as lower WBC and platelets (all p values<0,05). KDG received more frequently immunoglobulins (IVIG) and acetylsalicylic acid (ASA) (81,3% vs 66%; p=0.04 and 71,9% vs 43,4%; p=0.001 respectively) as KCG more often received glucocorticoids (56,6% vs 14,6%; p<0.0001). SARS-CoV-2 assay more often resulted positive in KCG than in KDG (75,5% vs 20%; p<0.0001). Short-term follow data on KCG showed minor complications while on KDG a majority of patients had persistence of CAA. Comparing KDG with a KD-Historical Italian cohort (598 patients), no statistical difference was found in terms of clinical manifestations and laboratory data between the two groups Conclusion: Our study would suggest that SARS-CoV-2 infection might determine two distinct inflammatory diseases in children: KD, possibly triggered by SARS-CoV-2, and PIMS-TS. Older age at onset and clinical peculiarities, like the occurrence of myocarditis, characterize this multiinflammatory syndrome. Our patients had an optimal response to treatments and a good outcome, with few complications and no deaths

Clinical, immunological, and molecular features of typical and atypical severe combined immunodeficiency: Report of the italian primary immunodeficiency network

Severe combined immunodeficiencies (SCIDs) are a group of inborn errors of the immune system, usually associated with severe or life-threatening infections. Due to the variability of clinical phenotypes, the diagnostic complexity and the heterogeneity of the genetic basis, they are often difficult to recognize, leading to a significant diagnostic delay (DD). Aim of this study is to define presenting signs and natural history of SCID in a large cohort of patients, prior to hematopoietic stem cell or gene therapies. To this purpose, we conducted a 30-year retro-prospective multicenter study within the Italian Primary Immunodeficiency Network. One hundred eleven patients, diagnosed as typical or atypical SCID according to the European Society for Immune Deficiencies criteria, were included. Patients were subsequently classified based on the genetic alteration, pathogenic mechanism and immunological classification. A positive relationship between the age at onset and the DD was found. SCID patients with later onset were identified only in the last decade of observation. Syndromic SCIDs represented 28% of the cohort. Eight percent of the subjects were diagnosed in Intensive Care Units. Fifty-three percent had an atypical phenotype and most of them exhibited a discordant genotype-immunophenotype. Pre-treatment mortality was higher in atypical and syndromic patients. Our study broadens the knowledge of clinical and laboratory manifestations and genotype/phenotype correlation in patients with SCID and may facilitate the diagnosis of both typical and atypical forms of the disease in countries where newborn screening programs have not yet been implemented