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Note The designations employed and the presentation of material in this publication do not imply the expression of any opinion whatsoever on the part of the European Commission or the European Environment Agency concerning the legal status of any country or territory, and the boundaries shown on maps do not imply official endorsement or acceptance. A number(More)
Niemann-Pick disease type C (NP-C) is a rare, autosomal-recessive, progressive neurological disease caused by mutations in either the NPC1 gene (in 95% of cases) or the NPC2 gene. This observational, multicentre genetic screening study evaluated the frequency and phenotypes of NP-C in consecutive adult patients with neurological and psychiatric symptoms.(More)
Niemann-Pick disease type C (NP-C) is a devastating, neurovisceral lysosomal storage disorder which is characterised by variable manifestation of visceral signs, progressive neuropsychiatric deterioration and premature death, caused by mutations in the NPC1 and NPC2 genes. Due to the complexity of diagnosis and the availability of an approved therapy in the(More)
There are a plethora of codes of practice emerging to help business manage and communicate social and environmental performance, particularly the relationship with suppliers in developing countries. These codes lie at the heart of ethical trading or ethical sourcing, and often tackle issues central to sustainable business. However, there are often separate(More)
Three hundred feedlot steers (320 +/- 2 kg) were assigned to freeze brand, hot-iron brand, and sham branding treatments according to a randomized branding arrangement. Behaviors believed to be indicative of pain (i.e., tail-flicking, kicking, falling, and vocalizing) were recorded during branding. Escape behavior, measured as the amount and duration of(More)
Niemann-Pick disease type C (NP-C) is a neurovisceral lysosomal cholesterol trafficking and lipid storage disorder caused by mutations in one of the two genes, NPC1 or NPC2. Diagnosis has often been a difficult task, due to the wide range in age of onset of NP-C and clinical presentation of the disease, combined with the complexity of the cell biology(More)
Executive summary This report, based on a survey of 1,027 employees in Hong Kong, reveals the general improvement in work-life balance of Hong Kong employees compared with our same study done in 2004. Results of a questionnaire-based survey distributed in cooperation with Hong Kong employers showed the following:-Compared with 2004, employees now work 5(More)