Histopathology and fibrillin-1 distribution in severe early onset Marfan syndrome

Marfan syndrome (MFS) is an autosomal dominant condition which may involve the cardiovascular, ocular, skeletal, and other systems. Mutations causing MFS are found in the FBN1 gene, encoding fibrillin-1, an extracellular matrix protein involved in microfibril formation. In the most severe cases, mutations are generally found in exons 24-32, and children with these mutations usually die in the first years of life, of cardiopulmonary failure. We present clinical, molecular and histopathological studies on a patient with severe early onset MFS. He has a mutation in exon 25 of FBN1, a G > A transition at nucleotide position 3131 that converts the codon TGC, coding for cysteine at position 1044, to TAC, coding for tyrosine (C1044Y). This has resulted in abnormalities of the extracellular matrix and a severe clinical phenotype, although he has survived to the age of 14 years. (c) 2005 Wiley-Liss, Inc.

A qualitative cross-level investigation of safety climate perceptions

ver the last few decades occupational health and safety research has shifted its focus away from engineering and ergonomics as a means of improving workplace safety, and has given greater attention to examining the role played by organisational factors, such as safety climate. One factor constraining the advancement of our understanding of the safety climate construct is the tendency of researchers to remain steadfastly bound to the notion that safety climate is measured via a quantative measurement tool. Researchers in the area (e.g., Frone & Barling, 2004; Zohar, 2003) are now arguing for better triangulation of methodologies, in particular better qualitative research, to advance our knowledge and understanding. The present study extends the present body of safety climate literature in two ways; firstly, it addresses this methodological issue via the utilisation of a semi-structured interview methodology and secondly it examines the qualitative structure of safety climate perceptions across different levels (organisation, supervisor and co-worker) and different groups (managers vs. employees). Examination of the interview transcripts revealed qualitative differences and similarities between the different safety climate levels (organisational, supervisor and co-worker) and between manager and employee safety climate perceptions. Implications of these findings for safety climate theory and measurement are discussed.

Affective priming - is it only affect that primes?

A target word is classified faster as pleasant or unpleasant if preceded by a prime that matches the target word’s valence. This affective priming phenomenon is currently popular as an implicit measure of stimulus valence. The present set of experiments investigated whether rated stimulus arousal will affect target classification as well. In three experiments, word targets were preceded by prime stimuli that differed in rated arousal and valence. The basic priming effect was replicated in all experiments, however, priming was largest after high arousal unpleasant and low arousal pleasant primes, and reduced after low arousal unpleasant and high arousal pleasant primes. This finding emerged for picture and word primes and does not reflect the effect of differences in stimulus complexity. The difference in the effectiveness of the primes was not affected by SOA and seemed to hold across a wide range (50-200 ms for words and 200-500 ms for pictures). The present results suggest that some failures to find affective priming may not reflect on prime valence, but on prime arousal. Moreover, it suggests that increases in stimulus arousal have differential effects for the processing of pleasant and unpleasant stimuli.