Union Commitment and Participation Amongst Hong Kong Firefighters: A Development of an Integrative Model.

This paper examines the factors influencing members' intent to participate in their union, drawing on a study of Hong Kong firefighters. As in Western studies, the main direct predictor of intent to participate is affective union commitment, but instrumentality is a relatively more important antecedent of affective union commitment and participation than in the Western studies. We found some evidence of frustration-aggression effects on intent to participate in 'militant' and 'general' union activities, although the latter was significant only among individuals who perceive management-employee relations as co-operative and trusting.Department of Management and Marketin

Characterization and treatment of congenital thrombotic thrombocytopenic purpura.

Congenital Thrombotic Thrombocytopenic Purpura (cTTP) is an ultra rare thrombomicroangiopathy caused by an inherited deficiency of ADAMTS13. There is limited data on the genotype-phenotype correlation and no consensus on treatment. We reviewed the largest cohort of cTTP cases, diagnosed in the UK, over the past 15 years. 73 cases of cTTP were diagnosed, confirmed by genetic analysis. 93% were alive at the time of review. 36% had homozygous mutations and 64% compound heterozygous mutations. Two presentation peaks were seen, childhood (median diagnosis age 3.5 years) and adulthood, typically related to pregnancy (median diagnosis age 31 years). Genetic mutations differed by age of onset with pre-spacer mutations more likely to be associated with childhood-onset (p=0.0011). 69% of adult presentations were associated with pregnancy. Fresh Frozen Plasma (FFP) and intermediate purity factor VIII concentrate were used as treatment. 88% of patients with normal blood counts but headaches, lethargy or abdominal pain reported symptom resolution with prophylactic therapy although the most common currently used regimen of three weekly FFP proved insufficient for 70% of patients and weekly or fortnightly infusions were required. Stroke incidence was significantly reduced in patients receiving prophylactic therapy (2% versus 17%, p=0.04). Long term, there is a risk of end organ damage, seen in 75% of patients with a late diagnosis of cTTP. In conclusion, pre-spacer mutations are associated with earlier development of cTTP symptoms. Prophylactic ADAMTS13 replacement decreases the risk of end organ damage such as ischemic stroke and resolved previously unrecognized symptoms in patients with non-overt disease