Comparative studies of Toll-like receptor signalling using zebrafish

Zebrafish model systems for infectious disease are increasingly used for the functional analysis of molecular pattern recognition processes. These studies benefit from the high conservation level of all innate immune factors in vertebrates. Zebrafish studies are strategically well positioned for this because of the ease of comparisons with studies in other fish species of which the immune system also has been intensively studied, but that are currently still less amendable to detailed genetic or microscopic studies. In this paper we focus on Toll-like receptor (TLR) signalling factors, which currently are the best characterized in mammalian systems. We review the knowledge on TLR signalling in the context of recent advances in zebrafish studies and discuss possibilities for future approaches that can complement studies in cell cultures and rodent models. A focus in these comparisons is the role of negative control mechanisms in immune responses that appear very important in a whole organism to keep adverse systemic responses in check. We also pay much attention to comparisons with studies in common carp that is highly related to zebrafish and that because of its large body mass can complement immune studies in zebrafish.     

Hyaline fibromatosis of Hoffa’s fat pad in a patient with a mild type of hyaline fibromatosis syndrome

Hyaline fibromatosis syndrome (HFS) is a rare, homozygous, autosomal recessive disease, characterized by deposition of hyaline material in skin and other organs, resulting in esthetic problems, disability, and potential life-threatening complications. Most patients become clinically apparent in the first few years of life, and the disorder typically progresses with the appearance of new lesions. We describe a rare case of a 20-year-old patient with juvenile-onset mild HFS who presented with a history of progressive anterior knee pain. Detailed magnetic resonance (MR) imaging findings with histopathological correlation are presented of hyaline fibromatosis of Hoffa’s fat pad, including differential diagnosis. The diagnosis of HFS is generally made on basis of clinical and histopathological findings. Imaging findings, however, may contribute to the correct diagnosis in patients who present with a less typical clinical course of HFS.     

From Relevant Capabilities to Relevant Indicators: Defining an Indicator System for Children’s Well-Being in Spain

In the last two decades, studies dealing with the measurement of children’s well-being have proliferated. These studies develop mainly from the need to address the topic from a multidimensional perspective, capable of integrating approaches into a more comprehensive view of reality. In this regard, key issues have been tackled and discussions are still open, such as those on the inclusion of boys and girls as active agents in the definition of their needs, or on the consideration of aspects that affect both present and future needs as part of well-being. The capability theory sets a very interesting theoretical framework within this context. This work will, first of all, try to approach the topic of children’s well-being from such perspective in conjunction with the Life Sustainability proposition. There is, however, no translation of these theoretical contributions to the development of indicators, even though the actual need for them is well acknowledged, given the political and social-action implications of bringing this progress to the realm of the tangible. There are two main reasons for this. First, the scarcity of data about children that could allow the empirical development of valid and reliable measurements in this field. Second, the methodological difficulty of appropriately defining this kind of factors, which are very often linked to subjective and/or intangible aspects, for quantification purposes. Keeping all this in mind, the second part of this work aims at making some progress in this direction and proposes a system of indicators to support what is first analyzed from a conceptual point of view.