Background: STAT3 gain-of-function (GOF) germline mutations have been recently described. A comprehensive overview of this early-onset multiorgan autoimmune and lymphoproliferative disease has not yet been compiled. Objective:We have conducted a systematic review of published STAT3 GOF cases in order to describe clinical, diagnostic and therapeutic aspects of the disease.Methods: A systematic review including articles published before 10/10/2018 in Pubmed, WoS and CENTRAL databases was performed. We described cases of patients with STAT3 GOF germline mutations with genetic analysis and a concordant phenotype if functional analyses were not performed for the mutation. Results:The search identified 18 publications describing 42 unique patients. Twenty-eight different mutations were described. Onset of disease was very early with an average age of 3 (0.5-5) years old. The most frequent manifestations were: autoimmune cytopenias (28/42), lymphoproliferation (27/42), enteropathy (24/42), interstitial lung disease (15/42), thyroiditis (13/42), diabetes (10/42), and post-natal growth failure (15/21). Immunodeficiency was not always a predominant feature. Most patients required significant immunosuppressive therapy.Five patients received hematopoietic stem cell transplantation, 4 died from complications. Improvement of symptoms was observed for 8 out of 9 patients that received targeted biotherapies.

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This paper assesses the impact on household incomes of the COVID‐19 pandemic and governments’ policy responses in April 2020 in four large and severely hit EU countries: Belgium, Italy, Spain and the UK. We provide comparative evidence on the level of relative and absolute welfare resilience at the onset of the pandemic, by creating counterfactual scenarios using the European tax‐benefit model EUROMOD combined with COVID‐19‐related household surveys and timely labor market data. We find that income poverty increased in all countries due to the pandemic while inequality remained broadly the same. Differences in the impact of policies across countries arose from four main sources: the asymmetric dimension of the shock by country, the different protection offered by each tax‐benefit system, the diverse design of discretionary measures and differences in the household level circumstances and living arrangements of individuals at risk of income loss in each country.

When the 2008 crisis hit, social safety nets in Europe were not in the best of shape. This article examines what, if anything, governments did to adjust minimum income protection after two decades of relative neglect. In view of the hardship brought on by the crisis, this question is of importance in itself. In addition, there is a long-standing interest in the role crises play in re-shaping policies, possibly in a radical way. Building on purpose-collected data for twenty-four European countries, this article shows that many countries introduced supportive measures during the first years of the crisis, particularly in the form of additional benefit increases and more generous child benefits. Behavioural requirements imposed on minimum income recipients were not relaxed but in some countries activation efforts were intensified. Although the evidence shows that the crisis did trigger a response, there is little evidence for a structural change of course towards more adequate safety nets.

This paper looks at the level of the minimum income guarantees for able-bodied persons at working age and assesses benefi t trends since the 1990s. Our dataset comprises 25 EU countries (EU27 except Cyprus and Malta), 3 American States (Nebraska, New Jersey and Texas) and Norway. The degree of welfare erosion is measured by three indicators: real benefi t trends, benefi t trends relative to the development of average wages and benefi t trends relative to changes in median equivalent income. We therefore assess net disposable income of families relying on social assistance, taking account of the impact of i.a. child benefi ts and housing allowances. A central question in this paper is the extent to which trends in social assistance benefi t packages are linked to the statutory mechanism that is being used to adjust benefi t levels.

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C.D. were involved in clinical follow-up of the patient, participated in the conceptualization and design of the case report, contributed to the acquisition of data, critically revised the manuscript, and approved the final manuscript as submitted; M. Baque participated in the conceptualization and design of the case report, contributed to the acquisition of the computed tomography scan, critically revised the manuscript, and approved the final manuscript as submitted; D.A. participated in the conceptualization and design of the case report, contributed to the acquisition of accessory salivary gland biopsy data, critically revised the manuscript, and approved the final manuscript as submitted; and D.F. participated in the conceptualization and design of the case report, analyzed BAL cytospins, critically revised the manuscript, and approved the final manuscript as submitted.

This article assesses the current variation in activation strategies directed towards able-bodied persons of working age relying on a minimum income guarantee in 19 EU member states. First, we argue that the active inclusion notion developed by the European Commission in its Recommendation on the active inclusion of persons excluded from the labour market provides a useful tool to categorize current activation strategies towards minimum income protection (MIP) recipients. Consequently, we assess the empirical viability of active inclusion strategies in a fuzzy set ideal type analysis of purpose-collected institutional data. We find that there are only few countries where the activation discourse has remained a dead letter. Most countries implement policy measures that aim to discourage benefit dependency among MIP recipients. Nevertheless, behind the realities of activation strategies towards MIP recipients seldom lies the notion of active inclusion as defined by the European Commission. Particularly, many countries focus predominantly on incentives to increase labour market participation rates of MIP recipients, rather than enabling measures.