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BACKGROUND Risdiplam is an orally administered therapy that modifies pre-mRNA splicing of the survival of motor neuron 2 (SMN2) gene and is approved for the treatment of spinal muscular atrophy. The FIREFISH study is investigating the safety and efficacy of risdiplam in treated infants with type 1 spinal muscular atrophy versus historical controls. The primary endpoint of part 2 of the FIREFISH study showed that infants with type 1 spinal muscular atrophy attained the ability to sit without support for at least 5 s after 12 months of treatment. Here, we report on the safety and efficacy of risdiplam in FIREFISH part 2 over 24 months of treatment. METHODS FIREFISH is an ongoing, multicentre, open-label, two-part study. In FIREFISH part 2, eligible infants (aged 1-7 months at enrolment, with a genetically confirmed diagnosis of spinal muscular atrophy, and two SMN2 gene copies) were enrolled in 14 hospitals in ten countries across Europe, North America, South America, and Asia. Risdiplam was orally administered once daily at 0·2 mg/kg for infants between 5 months and 2 years of age; once an infant reached 2 years of age, the dose was increased to 0·25 mg/kg. Infants younger than 5 months started at 0·04 mg/kg (infants between 1 month and 3 months old) or 0·08 mg/kg (infants between 3 months and 5 months old), and this starting dose was adjusted to 0·2 mg/kg once pharmacokinetic data were available for each infant. The primary and secondary endpoints included in the statistical hierarchy and assessed at month 12 have been reported previously. Here we present the remainder of the secondary efficacy endpoints that were included in the statistical hierarchy at month 24: the ability to sit without support for at least 30 s, to stand alone, and to walk alone, as assessed by the Bayley Scales of Infant and Toddler Development, third edition gross motor subscale. These three endpoints were compared with a performance criterion of 5% that was defined based on the natural history of type 1 spinal muscular atrophy; the results were considered statistically significant if the lower limit of the two-sided 90% CI was above the 5% threshold. FIREFISH is registered with ClinicalTrials.gov, NCT02913482. Recruitment is closed; the 36-month extension period of the study is ongoing. FINDINGS Between March 13 and Nov 19, 2018, 41 infants were enrolled in FIREFISH part 2. After 24 months of treatment, 38 infants were ongoing in the study and 18 infants (44% [90% CI 31-58]) were able to sit without support for at least 30 s (p

Tourist satisfaction is the most important component in the analysis of tourism behavior, since it affects the choice of destination and the purchase of products, as well as the potential decision of the tourist to revisit the destination. The purpose of this paper is to examine the satisfaction of tourists visiting the Ionian Islands in Greece, by use of quantitative methods that capture the factors influencing the satisfaction of tourists and their desire to repeat the visit, their relationship and consideration of the causes that shape tourism behavior. Research results reveal factors that affect tourist satisfaction, the relationships among these factors, the relationship between satisfaction and revisit to destination, the assessment of satisfaction by segmentation of tourists with regard to their motivation, the usability of information in satisfaction and the effect of tourism experience in travel behavior. The tourism policy recommendations arising from the research results will contribute to the diversification and enrichment of the tourism product, while also to a further enhancement in the satisfaction of tourists visiting the Ionian Islands., tourismos, Vol. 14 No. 2 (2019)

Objective: To estimate breast-feeding prevalence in Greece in 2007 and 2017, compare breast-feeding indicators and maternity hospital practices between these years, and investigate breast-feeding determinants. Design: Two national cross-sectional studies (2007 and 2017) using systematic cluster sampling of babies with the same sampling design, data collection and analysis methodology. Setting: Telephone interview with babies’ mothers or fathers. Participants: Representative sample of infants who participated in the national neonatal screening programme (n 549 in 2017, n 586 in 2007). Results: We found that breast-feeding indicators were higher in 2017 compared with 10 years before. In 2017, 94 % of mothers initiated breast-feeding. Breast-feeding rates were 80, 56 and 45 % by the end of the 1st, 4th and 6th completed month of age, respectively. At the same ages, 40, 25 and

Introduction: Standardization is important across the life cycle of medicinal products, supporting the diagnosis, treatment, and prevention of a wide range of diseases. For rare diseases, standardization is even more important, as patient groups are small, presenting significant challenges in the design, conduct, analysis, and interpretation of clinical studies. It is here that standardization institutions, including the UK’s National Institute for Biological Standards and Control (NIBSC), can have a key role. Areas covered: A considerable proportion of NIBSC’s work supports the better understanding, diagnosis, treatment, and prevention of rare diseases. NIBSC is also part of the UK’s Medicines and Healthcare products Regulatory Agency (MHRA), creating an agency that is uniquely placed to combine scientific and regulatory expertize for the benefit of public health. This review provides an overview of NIBSC’s work in rare diseases and highlights the positive impact of the work of standardization institutions in this field. Expert opinion: Standardization in product development is key for patients with rare diseases. The work of standardization institutions is increasingly being recognized as crucial for supporting scientific and clinical advancements, and early and collaborative interactions can provide drug developers with the necessary expertize, when standards matter most.

Tourism satisfaction is one of the most important components in the analysis of tourism behavior as, it not only affects the choice of tourism destination and the consumption of products, but also affects tourist's future decision to return to the same destination. The main objective of this paper is to investigate the satisfaction of tourists visiting the Ionian Islands of Greece through quantitative methods that capture the factors influencing tourists' satisfaction and their choice to revisit those destinations, the relationship between them and the consideration of the causes that shape tourism behavior. Field research was conducted at four Ionian islands, namely Corfu, Zakynthos, Lefkada and Kefalonia. Those islands have functional interdependencies and are sensitive to influences from mainland regions. The conclusions of the research reveal the factors that affect tourism satisfaction, the relationships among those factors, the relationship between satisfaction and the revisit to a destination, the assessment of satisfaction according to the segmentation of tourists regarding their motive, the usability of information in satisfaction and the impact of the tourism experience in travel behavior. The tourism policy recommendations arising from the results of this research can lead to the diversification and the enrichment of the tourism product, but also to a further increase of the satisfaction of tourists visiting the Ionian Islands in Greece.

As the economic crisis becomes more intense, affecting regions' economic activities to different degrees, new issues have aroused regarding the ability of each region to respond positively to these changes by maintaining its performance at high levels. As a result, the notion of resilience, and in particular economic resilience, has become a field of study for many researchers. Regional economic resilience is defined as the ability of a region to recover successfully from shocks that affect its economy. The term has two meanings: the first one is based on equilibrium analysis, in which resilience is the ability of a region to return to a pre-existing state in a single equilibrium system and the second one defines resilience in terms of complex adaptive systems and refers to the region's ability to adjust and change as response to sudden pressures, shocks and negative impacts. The aim of this paper is to investigate the impact of economic crisis on Greek regions and to assess the displayed degree of resilience. More specifically we focus on regional resilience regarding tourism industry. Tourism, an important economic activity and a fast growing industry worldwide, is one of the main 'sources of income for many countries, including Greece. Its importance and contribution to Greek economy is significant, especially given the poor performance of most other economic sectors. In 2014 tourism's contribution to GDP was more than 20%. According to the Greek Tourism Confederation (SETE), in 2014 the volume of paid employment provided by the tourism industry increased by 23 percent. During July 2014, Greece's tourism industry generated 137,139 jobs, compared to the same month in 2013. For the purposes of the paper we will use data for three periods: 2007 (i.e. the beginning of the crisis), 2010 (i.e. on the middle of the crisis), 2013 (i.e. on the peak of the crisis). Data refer to NUTS 2 regions and involve: 1) GVA for the tourism industry, 2) Employment in the tourism sector, 3) Number of arrivals (national and international tourists), 4) Number of nights spent (national and international tourists), 5) Number of establishments, 6) Number of available beds. A first analysis of the above data shows that tourism was affected slightly by the economic crisis. In fact, some regions showed an increase on specific tourism indicators. Decrease has been observed on data referring to national tourists, GVA and employment. In order to assess the regions' degree of resilience we introduce an index which is formed by the aforementioned indicators and reveals if a region is economically resilient, shock resistant or non- resilient. Results show that the country's tourism industry could be considered as resilient. Nevertheless, there are significant variations in the resilience of tourism among regions. It is noticeable that the degree of resilience depends on the region's geographical position i.e. whether it is a mainland or an island region. As a result new issues have aroused regarding the investments on tourism per region and the necessity for a regional tourism planning and policies.

During the last decade a new concept has emerged in the regional science debate, the notion of regional resilience, defined as the ability of a region to prevent, prepare, respond and recover after a disturbance, in order that this disturbance does not stand as an obstacle to the region¢s development. Regional resilience is characterized by the capacity of a regional economy to i) withstand external pressures, ii) to respond positively to external changes and iii) to adjust and to learn. According to the international literature, a region should exhibit certain characteristics in order to be considered as resilient, including resourcefulness, performance, redundancy, diversity, innovative learning, connectedness, robustness and rapidity. The aim of this paper is to explore the notion of resilience in the tourism industry. Tourism, an important economic activity and fast growing industry worldwide, is one of the main income ‎sources for many countries, including Greece. In Greece, tourism represents over 17% of the country's GNP and 18,3% of total employment. During the years of recent economic crisis, the Greek tourism sector has been affected less than other economic activities, indicating its importance for the Greek economy. Nevertheless, significant variations in the resilience of tourism on economic crisis impact seem to have occurred among regions and therefore research regarding regional tourism resilience has grown in importance. This paper aims to investigate the extent to which Greek regions are resilient as far as tourism industry is concerned. More specifically, we valuate the adaptability of regions, tourism destinations and actors to the socio-economic changes and the degree of resilience of each region during the economic crisis i.e. whether they can cope with it, overcome it and recover from it. Furthermore, we intend to investigate how the evaluation of present resilience can contribute to the improvement of resilience planning and management on a regional basis. The central thesis of this research is that regional variations in the tourism industry resilience call for regionally adjusted tourism planning and management policies. In order to achieve the aim of the research, we will examine the impact of economic crisis on regional hospitality industry employment, measured by the change in the number of jobs in hospitality business (hotels) within each region, and attempt to model employment resilience after the industry experiences an economic shock. Tourism industry is a critical source of regional economic activity, and therefore it is important to understand what happens to industry employment due to economic crisis and the mechanisms by which regional tourism industry resilience is achieved. In this way, we will investigate the impact of the socio-economic changes in regions and through the noted declinations we will estimate the extent to which these regions are tourism resilient in economic shocks.

In recent times, the attention given to the importance of tourism as a particularly efficient way to promote regional development has significantly increased, owing to the capacity of tourism to create income and employment and to generate synergies within other economic sectors. Tourism is worldwide considered to have a key role in the economic development of regions, and therefore, is widely used to reduce regional disparities and to improve quality of life standards in local communities. Greece has a long tradition in tourism, mainly due to a gifted natural environment and rich cultural heritage. However, tourism development across regions is highly unbalanced. This is attributed particularly to the highly insular and mountainous geomorphology of the country, the uneven distribution of natural, socio-cultural and environmental resources, and the lack of appropriate regional and tourism development policies during the post war decades. The dominance of the mass tourism model adopted since the early seventies in the country and the extensive structural inefficiencies, are effectively hindering the existing potential for a balanced and viable tourism development. The main objective of this paper is to investigate tourism development disparities among Greek regions and to identify factors and occurring patterns that affect the tourism development of regions in order to formulate a strategy and policy framework for balanced regional tourism development. In the first section of this paper an overview of the theoretical approaches related to tourism and balanced regional development is provided. Next, the role of tourism in the Greek national economy and the country's position in the international tourism market are examined. Following an analysis of the evolution of regional tourism disparities in Greece during the last decades in the third section, quantitative techniques are used to assess intra- and cross-regional tourism inequalities in the fourth section. The results reveal the existence of strong regional disparities in tourism development among Greek regions, although the development pattern differs among different types of regions. Finally, in the last section some concluding remarks and policy recommendations are presented, leading to the identification of issues for further research.

Europe as we know it, the European Union of 27 countries, has evolved from the European Coal and Steel Union of a few developed countries in a comprehensive economic and political union, which now embraces and unites most of the European continent (EU 2011). Each successive transformation and enlargement of the Union has brought in new people and countries with large differences and particularities. Countries of southern Europe and later the former eastern socialist republics joined the initial core of the developed Western economies. Today, between countries of the European Union, different zones are distinguished in relation to levels of economic and social development. One group consists of the prosperous western and northern economies and includes countries such as Germany, Finland, the Netherlands, or Denmark. The second group includes regional and Mediterranean countries with less developed economies such as Spain, Portugal, Italy, Greece or Ireland. Finally, the third group includes countries of former Eastern economies, relatively weak, completing the transition from socialism to capitalism. In this group are countries such as Bulgaria, Poland, Romania, Latvia. The existence of many different countries in a broad geographic area designates the regional problem at three levels. The first level concerns the groups of countries mentioned above. At a second level variation exists within countries. Finally, at a third level, in the unified Europe, differences are between regions across national borders. Many studies argue that while disparities between member countries are decreasing gradually, the disparities within countries are increasing. As a result, the overall gap between the rich and developed regions on one hand and the less developed regions on the other hand is expanding. The current economic crisis has affected almost all European countries but the countries of the European south and the former eastern socialist republics have suffered the most. Our objective in this paper is to quantify regional disparities as expressed by several growth indicators, such as GDP per capita, employment/unemployment rates, household savings and use them to compare the regional disparities at the three levels described above before and after the crisis. Keywords: Economic Crisis, Regional Disparities, GDP, Unemployment. JEL Classification: R10, R11, R15 Other possible choices for the theme: E or special session ZJ

The distribution of income has always been a main concern of economic theory and policy. Classical economists were concerned with the distribution of income between the main factors of production, land, labour and capital. Modern economists, on the other hand, are concerned with the distribution of income across individuals and households. Furthermore, the unequal distribution of personal income and wealth is one of the most prominent features of our society and one which has a profound effect on economic and social relationships. The theoretical aspects of income distribution and a number of income inequality measures have been presented in some previous work (Dimaki et al. 2001). In this paper we focus on income inequality at a regional level. Almost all countries face regional disparities, due to a variety of reasons, historic, socioeconomic, structural and geographic, leading to a number of adverse consequences for the less favored regions. Hence, Governments take certain alternative measures to alleviate those disparities and assist the less developed regions. Our objective in this paper is to: - Define a measure expressing a region's current state of development and future prospects. - Assess the changes in that measure over time, resulting from both endogenous development and the implementation of alternative state measures for its improvement. - Relate the changes in that measure to the respective changes in the regional income inequality measures over the same period of time. The theoretical findings will be applied to the case of Greek regions over a period of time and the results will be presented and critically discussed.

The development of regions and especially those facing spatial discontinuity has been the focus in some previous work (Angelis 1980, 1999) and the region's Image has been defined as a variable expressing its relative attractiveness and its prospects of future development. The findings have been applied to selected Greek regions and the results have been discussed. Among the factors affecting a region's Image, accessibility to markets, resources and influence centres, seems to be a very crucial one and its improvement will facilitate its development. Although several measures for improving a region's accessibility have been suggested, they seem to have little or no effect for regions with spatial discontinuity. In this case, alternative measures have been introduced and a modification of the initial region's Image concept has been proposed so as to take into account the variables related to those measures (Gaki, Angelis, Dimaki 2009). The use of the modified Image functions for regions with spatial discontinuity seems to be a step in the right direction since, on one hand, they give a better view of their current state of development and future prospects and, on the other hand, they may be used as the basis for the design, but also for the evaluation, of alternative specific measures aiming to assist them in overcoming their problem. One of the measures suggested for this purpose is the development of local business activities, i.e. activities basically using local resources and selling their products to local markets. For such activities discontinuity is not a handicap since their development doesn't require frequent transportation of physical entities to-and-from other regions. Our objective in this paper is to focus on this solution. Toward this end we: - Determine the characteristics that a region should have in order to implement such a solution - Redefine the Image function so as to include those characteristics - Apply the theoretical findings to selected Greek regions - Compare the findings with those obtained when the initial Image function was used and summarize the conclusions.