Mulibrey nanism : Clinical characteristics and pathophysiologic features of growth restriction, insulin resistance and tumour development

Background: Mulibrey nanism (MUL; Muscle-liver-brain-eye nanism; OMIM 253250) is an autosomal recessive growth disorder more prevalent in Finland than elsewhere in the world. Clinical characteristics include severe prenatal onset growth restriction, cardiopathy, multiple organ manifestations but no major neurological handicap. MUL is caused by mutations in the TRIM37 gene on chromosome 17q22-23, encoding a peroxisomal protein TRIM37 with ubiquitin E3-ligase activity. Nineteen different mutations have been detected, four of them present in the Finnish patients. Objective: This study aimed to characterize clinical and histopathological features of MUL in the national cohort of Finnish patients. Patients and methods: A total of 92 Finnish patients (age 0.7 to 77 years) participated in the clinical follow-up study. Patients hospital records and growth charts were reviewed. Physical, radiographic and laboratory examinations were performed according to a clinical protocol. Thirty patients (18 females) were treated with recombinant human GH for a median period of 5.7 years. Biopsies and autopsy samples were used for the histopathological and immunohistochemical analyses. Results: MUL patients were born small for gestational age (SGA) with immature craniofacial features after prenatal-onset growth restriction. They experienced a continuous deceleration in both height SDS and weight-for-height (WFH) postnatally. In infancy feeding difficulties and frequent pneumonias were common problems. At the time of diagnosis (median age 2.1 years) characteristic craniofacial, radiological and ocular features were the most constant findings. MUL patients showed a dramatic change in glucose metabolism with increasing age. While the children had low fasting glucose and insulin levels, 90% of the adults were insulin resistant, half had type 2 diabetes and an additional 42% showed impaired glucose tolerance (IGT). Seventy percent fulfilled the National Cholesterol Education Program (NCEP) Adult Treatment Panel III criteria for metabolic syndrome as adults. GH therapy improved pre-pubertal growth but had only minor impact on adult height (+5 cm). Interestingly, treated subjects were slimmer and had less frequent metabolic concerns as young adults. MUL patients displayed histologically a disturbed architecture with ectopic tissues and a high frequency of both benign and malignant tumours present in several internal organs. A total of 232 tumorous lesions were detected in our patient cohort. The majority of the tumours showed strong expression of endothelial cell marker CD34 as well as α-smooth muscle actin (α-SMA). Fifteen of the tumours were malignant and seven of them (five Wilms tumours) occurred in the kidney. Conclusions: MUL patients present a distinct postnatal growth pattern. Short-term response of GH treatment is substantial but the long-term impact remains modest. Although MUL patients form a distinct clinical and diagnostic entity, their clinical findings vary considerably from infancy to adulthood. While failure to thrive dominates early life, MUL adults develop metabolic syndrome and have a tendency for malignancies and vascular lesions in several organs. This speaks for a central role of TRIM37 in regulation of key cellular functions, such as proliferation, migration, angiogenesis and insulin signalling.

Snow characteristics in Dronning Maud Land, Antarctica

Snow cover is very sensitive to climate change and has a large feedback effect on the climate system due to the high albedo. Snow covers almost all surfaces in Antarctica and small changes in snow properties can mean large changes in absorbed radiation. In the ongoing discussion of climatic change, the mass balance of Antarctica has received increasing focus during recent decades, since its reaction to global warming strongly influences sea-level change. The aim of the present work was to examine the spatial and temporal variations in the physical and chemical characteristics of surface snow and annual accumulation rates in western Dronning Maud Land, Antarctica. The data were collected along a 350-km-long transect from the coast to the plateau during the years 1999-2004 as a part of the Finnish Antarctic Research Programme (FINNARP). The research focused on the most recent annual accumulation in the coastal area. The results show that the distance from the sea, and the moisture source, was the most predominant factor controlling the variations in both physical (conductivity, grain size, oxygen isotope ratio and accumulation) and chemical snow properties. The sea-salt and sulphur-containing components predominated in the coastal region. The local influences of nunataks and topographic highs were also visible on snow. The variations in all measured properties were wide within single sites mostly due to redistribution by winds and sastrugi topography, which reveals the importance of the spatially representative measurements. The mean accumulations occurred on the ice shelf, in the coastal region and on the plateau: 312 ± 28, 215 ± 43 and 92 ± 25 mm w.e., respectively. Depth hoar layers were usually found under the thin ice crust and were associated with a low dielectric constant and high concentrations of nitrate. Taking into account the vast size of the Antarctic ice sheet and its geographic characteristics, it is important to extend investigation of the distribution of surface snow properties and accumulation to provide well-documented data.

Slogans of Change : Three Outlooks on Finnish Television

The study Slogans of Change. Three Outlooks on Finnish Television Contents is concerned with alleged changes of television contents during the 1990s and 2000s, such as dumbing down, tabloidisation, entertainisation , and the like. Specifically, the focus is on the ways these changes might manifest in Finnish television. The aim of the study has been threefold: 1. To operationalise public and academic discussions about changes via specific slogans emerging from the debates; 2. Consequently, to study the slogans empirically and reflect on the findings with earlier research, including studies on institutional and audience-related aspects; 3. Finally, to suggest what the findings might mean regarding discussions about television s role, and what kinds of slogans or concepts might best serve future discussions and research. The empirical outlooks presented in this study offer analyses with three different sets of opposing slogans of change. The outlooks also follow three different traditions of the study of television. The first outlook focuses on quantity, as it gives a longitudinal (1993-2004), macro-level view on programme structures. The methodological approach is derived from media economic and policy studies. The claims that frame the analysis are convergence versus diversification of programme structures. The second outlook provides quantitative and qualitative views on the characteristics and quality  the term signifying essence as well as worth  of Finnish television journalism during sample weeks from the years 2002 and 2003. This outlook follows the traditions of quantitative content analysis found in journalism studies coupled with descriptive qualitative content analyses. The slogans reflected in this section are the lightening or widening of journalism. The third outlook narrows down the material and focuses at a micro-level on form; that is, communicative conventions in a small array of selected programmes in 1993, 2000 and during 2002-2004. The analyses have been inspired by the method of conversation analysis of verbal interaction, and coupled with qualitative close readings, with the focus of different communicative situations in the programmes. The catchphrases employed in this part are emotainment versus democratainment, coupled with more specific claims of discursive hybridisation and conversationalisation. The findings depict that, empirically, changes in Finnish television contents are not clear linear trends and cannot easily be moulded into neat slogans. The quantitative outlook on programme output during 1993-2004 depicts a tendency towards differentiation of channels, paving the way for the multi-channel digital system. The change in programme structures, however, is not dramatic on the level of total output. The second outlook suggests that the dualistic concepts, such as the pair information-entertainment, are not sufficient in understanding the array and changes of programmes that could be called journalism. The outlook on communicative conventions highlights hybridisation in the manner of television talk and its relation to broader debates on contents. Despite the three dissimilar empirical approaches, unifying aspects emerge. The outlooks suggest, albeit in different ways, tendencies toward distinction and polarisation. This study proposes that in order to facilitate a more nuanced understanding of the changes in television contents, dualistic slogans should be replaced with a multi-dimensional understanding of the concept of diversity.

Yrittäjyyden arvottaminen akateemisten nuorten argumentoinnissa

Evaluation of entrepreneurship in the speech of academic students and newly qualified young academics a summary of a qualitative attitude study. In Finland very few university students plan to become entrepreneurs. The aim of this research was to examine entrepreneurial attitudes expressed in speech. The material was gathered from interviews with university students and newly qualified young academic adults. The interviewees commented on twelve different sentences with claims formulated using research literature and views that have appeared in public discussions. The interviewees were divided into three different groups based on their self-expressed entrepreneurial intentions. The method of qualitative attitude research (Vesala & Rantanen 1999, 2007) was used in the interviews. The research material was studied using two interpretative theories: (1) The planned behaviour theory (Ajzen 1985, 1991a, b), which makes it possible to focus on the separate elements (attitude towards an act, subjective norms and perceived feasibility) necessary for intentions to develop; and (2) The theory of the two images of entrepreneurship (Vesala 1996), where individualism and relationism can be seen as resources for evaluating entrepreneurship. The subject of the research was how university students and newly qualified young adults viewed entrepreneurship as a general phenomen and in relation to the academic world. A second focus was on the attitudes expressed toward entrepreneurial university education and the possibility of combining entrepreneurship and academic knowledge. Of interest were also questions such as whether academic studies, knowledge and the university itself are resources or barriers to entrepreneurial intentions and entrepreneurship whether university students received any support for their entrepreneurial ambitions from the university and their fellow academic students. The problems tackled by this research were thus the following: How was entrepreneurship seen, both as a general phenomen and in an academic context, when it was evaluated positively, negatively or neutrally by the interviewees? In what way was entrepreneurship constructed in the interviewees attitudes? How were entrepreneurship and the academic world related in the interviewees attitudes? What kind of role did the university as an academic context play in the interviewees attitudes for example were university education and academic knowledge seen as resources or barriers to their entrepreneurial intentions. Traditional attitude studies claim that attitudes are a stable property of an individual. In contrast, rhetorical social psychological and qualitative attitude studies emphasize the contextual and linguistic aspects of attitude, and they offered an alternative viewpoint for this research. The study was based on two general assumptions: attitudes have objects and are evaluative. Here attitude was defined as an evaluative interpresentation made towards an object; adopting an attitude is a contextual process in the sense that attitudes are always concerned with the action context of the persons presenting them. Entrepreneurship, both as a general phenomen and in an academic context, was specified as the object to which an attitude was taken. From a theoretical point of view, qualitative methods suited the general structure of this research well. In a particular, qualitative approach which emphasized contextual elements proved to be both empirically valid and useful for avoiding the problematic assumptions associated with traditional attitude study. The subject of the analysis was the argumentative speech produced by the interviewees. The results of the study show the subjects responses to three main ways of viewing entrepreneurships. The first was an individualistic, ideal image of entrepreneurship. This was mostly evaluated positively and gained wide approval especially among interviewees who included entrepreneurship among their employment choices. Entrepreneurship was seen as the decision to earn one s living independently. In this individualistic image of entrepreneurship, the social context was hardly ever mentioned. Elements which were seen to threaten this ideal image were evaluated negatively. When entrepreneurship was evaluated negatively using the individualistic image of entrepreneurship, it was mentioned that it forced one into a never ending cycle of work and uninterested duties. The relationistic image of entrepreneurship was used as a speech resource when the social context was constructed as an economic resource or a threat to the ideal image of entrepreneurship. In the second view, entrepreneurship was characteristically seen as being based on economics, which was seen as a threat to the ideal individualistic image of entrepreneurship. The risk of economic failure was seen as a limiting factor to entrepreneurial ambitions as it forced entrepreneurs to work around the clock. The third view concerned the relationship between entrepreneurship and the academic world. Entrepreneurship as an employment choice for university educated persons was evaluated as relevant, and thus positively, when university education was constructed as a resource for entrepreneurship - and irrelevant and thus negatively when it was construed as an obstacle, too wide, or when successful entrepreneurship was seen as being mostly based on an individual s personal characteristics. The interviewees with no entrepreneurial intentions expressed the view that academic education didn t provide the proper skills and knowledge for entrepreneurship. The interviewees also expressed interest in university entrepreneurship education, although none had experience on this. The interviewees emphasized the fact that the University didn t encourage them to consider entrepreneurship as a relevant employment choice. The assumption made by this study was that becoming an entrepreneur is a conscious decision, the environment may influence an individual s decisions on how to make a living as it tends to socialise people to act in accordance with cultural traditions. Keywords: Entrepreneurship, Attitudes towards entrepreneurship, Intentional behaviour, Entrepreneurial intention, University entrepreneurship education, Qualitative attitude research (Vesala & Rantanen 1999, 2007), Rhetorical social psychology (Billig 1986), The theory of entrepreneuship s two images: individualism and relationism (Vesala 1996 ), The planned behaviour theory (Ajzen 1985, 1991a, b)

Finnish and Austrian Wood Markets in a Changing Environment

At present the operating environment of sawmills in Europe is changing and there are uncertainties related in raw material supply in many countries. The changes in the operating environment of roundwood markets and the effects followed by these changes have brought up several interesting issues from the viewpoint of research. Lately new factors have been influencing the roundwood markets, such as increasing interest towards wood-based energy and implementation of new energy policies as well as changes in wood trade flows that affect the domestic markets in many countries. This Master’s thesis studies the adaptation ability of Finnish roundwood markets in a changing operating environment, aiming to produce an up-to-date analysis considering new development trends. The study concentrates on the roundwood markets from the viewpoint of sawmill industry since the industry is dependent on the functioning of the markets and sawmills are highly affected by the changes on the roundwood markets. To facilitate international comparison, the study is implemented by comparing Finnish and Austrian roundwood markets and analysing changes happening in the two countries. Finland and Austria share rather similar characteristics in the roundwood market structures, forest resources and forest ownership as well as production of roundwood and sawnwood. In addition they both are big exporters of forest industry products. In this study changes in the operating environment of sawmill industry both in Finland as well as in Austria are compared to each other aiming to recognise the main similarities and differences between the countries. In addition both development possibilities as well as challenges followed by the changes are discussed. The aim of the study is to define the main challenges and possibilities confronted by the actors on the markets and also to find new perspectives to approach these. The study is implemented as a qualitative study. The theoretical framework of the study describes the operating environment of wood markets from the viewpoint of the sawmill industry and represents the effects of supply and demand on the wood markets. The primary research material of the study was gathered by interviewing high level experts of forestry and sawmill industry in both Finland and Austria. The aim was to receive as extensive country specific viewpoint from the markets as possible, hence interviewees represented different parties of the markets. After creating country-specific profiles based on the theoretical framework a cross-country comparison was implemented. As a consequence the main similarities and differences in the operating environment and on the roundwood markets of Finland and Austria were recognized. In addition the main challenges and possibilites were identified. The results of the study offer a wide analysis regarding the main similarities and differences of the wood markets of Finland and Austria and their operating environments as well as concerning challenges and possibilities faced on the markets.

An Experimental Study of Home Gateway Characteristics

Many residential and small business users connect to the Internet via home gateways, such as DSL and cable modems. The characteristics of these devices heavily influence the quality and performance of the Internet service that these users receive. Anecdotal evidence suggests that an extremely diverse set of behaviors exists in the deployed base, forcing application developers to design for the lowest common denominator. This paper experimentally analyzes some characteristics of a substantial number of different home gateways: binding timeouts, queuing delays, throughput, protocol support and others.

Nature-based entrepreneurship in private forests – The preconditions for the sustainable co-operation between private forest owners and entrepreneurs

Nature-based tourism is one of the fastest growing tourism sectors at the moment. It is also the form of tourism that often benefits the economy of rural areas. In addition to state owned forests, nature-based tourism is in many countries situated in private forests, which are not owned by entrepreneurs themselves. Therefore, the ownership issues and property rights form central challenges for the business activities. The maintenance of good relationships between private forest owners and entrepreneurs, as well as combining their interests, becomes vital. These relationships are typically exceptionally asymmetrical, granting the forest owner unilateral rights regulating the business activities in their forests. Despite this, the co-operation is typically very informal and the existing economic compensation models do not necessarily cover all the forest owners’ costs. The ownership issues bring their own characteristics to the relationship. Therefore, we argue that different aspects of ownership, especially psychological ones, have to be more critically examined and taken into consideration in order to build truly successful relations between these parties. This is crucial for sustaining the business activities. The core of psychological ownership is the sense of possession. Psychological ownership can be defined as a state, in which individuals perceive the target of ownership, the object or idea, as “theirs”. The concept of psychological ownership has so far been mainly used in the context of professional organizations. In this research, it has been used to explain the relationships between private forest owners and nature-based entrepreneurs. The aim of this study is to provide new information concerning the effect of psychological ownership on the collaboration and to highlight the good practices. To address the complexity of the phenomenon, qualitative case study methods were adopted to understand the role of ownership at the level of subjective experience. The empirical data was based on 27 in-depth interviews with private forest owners and nature-based tourism entrepreneurs. The data was analysed by using the methods of qualitative analysis to construct different typologies to describe the essence of successful collaboration. As a result of the study, the special characteristics and the practical level expressions of the psychological ownership in the privately owned forest context were analysed. Four different strategies to perceive these ownership characteristics in co-operation relationships were found. By taking the psychological ownership into consideration via these strategies, the nature-based entrepreneurs aim to balance the co-operation relationship and minimise the risks in long term activities based on privately owned forests.

Growing up in Affiliation with a Religious Community : A Case Study of Seventh-day Adventist Youth in Finland

This study investigates how the religious community as a socialization context affects the development of young people's religious identity and values, using Finnish Seventh-day Adventism as a context for the case study. The research problem is investigated through the following questions: (1) What aspects support the intergenerational transmission of values and tradition in religious home education? (2) What is the role of social capital and the social networks of the religious community in the religious socialization process? (3) How does the religious composition of the peer group at school (e.g., a denominational school in comparison to a mainstream school) affect these young people s social relations and choices and their religious identity (as challenged versus as reinforced by values at school)? And (4) How do the young people studied negotiate their religious values and religious membership in the diverse social contexts of the society at large? The mixed method study includes both quantitative and qualitative data sets (3 surveys: n=106 young adults, n=100 teenagers, n=55 parents; 2 sets of interviews: n=10 young adults and n=10 teenagers; and fieldwork data from youth summer camps). The results indicate that, in religious home education, the relationship between parents and children, the parental example of a personally meaningful way of life, and encouraging critical thinking in order for young people to make personalized value choices were important factors in socialization. Overall, positive experiences of the religion and the religious community were crucial in providing direction for later choices of values and affiliations. Education that was experienced as either too severe or too permissive was not regarded as a positive influence for accepting similar values and lifestyle choices to those of the parents. Furthermore, the religious community had an important influence on these young people s religious socialization in terms of the commitment to denominational values and lifestyle and in providing them with religious identity and rooting them in the social network of the denomination. The network of the religious community generated important social resources, or social capital, for both the youth and their families, involving both tangible and intangible benefits, and bridging and bonding effects. However, the study also illustrates the sometimes difficult negotiations the youth face in navigating between differentiation and belonging when there is a tension between the values of a minority group and the larger society, and one wants to and does belong to both. It also demonstrates the variety within both the majority and the minority communities in society, as well as the many different ways one can find a personally meaningful way of being an Adventist. In the light of the previous literature about socialization-in-context in an increasingly pluralistic society, the findings were examined at four levels: individual, family, community and societal. These were seen as both a nested structure and as constructing a funnel in which each broader level directs the influences that reach the narrower ones. The societal setting directs the position and operation of religious communities, families and individuals, and the influences that reach the developing children and young people are in many ways directed by societal, communal and family characteristics. These levels are by nature constantly changing, as well as being constructed of different parts, like the pieces of a jigsaw puzzle, each of which alters in significance: for some negotiations on values and memberships the parental influence may be greater, whereas for others the peer group influences are. Although agency does remain somewhat connected to others, the growing youth are gradually able to take more responsibility for their own choices and their agency plays a crucial role in the process of choosing values and group memberships. Keywords: youth, community, Adventism, socialization, values, identity negotiations