Immune tolerance induction using a factor VIII/von Willebrand factor concentrate (BIOSTATE), with or without immunosuppression, in Australian paediatric severe haemophilia A patients with high titre inhibitors: a multicentre, retrospective study.

INTRODUCTION: It has been postulated that factor VIII (FVIII) products containing von Willebrand factor (VWF) may improve immune tolerance induction (ITI) success rate in patients with haemophilia A and poor prognostic factors. MATERIALS AND METHODS: We conducted a retrospective cohort analysis of a FVIII/VWF concentrate (BIOSTATE) for ITI in paediatric patients with severe haemophilia A (SHA) and inhibitors, from January 2003 to December 2011 at 3 paediatric-only Haemophilia Treatment Centres in Australia. Response to ITI was assessed at or before 33 months and at completion of ITI. Fifteen male patients with SHA were included in the analysis. RESULTS: BIOSTATE was used for primary ITI in 8 patients (2 years, range 1.1-11.5 years) and for salvage ITI in 7 patients (9.9 years, range 1.1-15.4). At the end of the observation period there were 11 patients who achieved a complete response with BIOSTATE after a median duration of 21 months (range 5-85 months); a partial response was achieved in 2 patients in whom ITI is ongoing. Therefore, the overall response rate was 86.6%. Two patients were deemed treatment failures: one due to non-compliance after 18 months of ITI and another in whom a partial response had not been achieved after 22 months of ITI. CONCLUSION: BIOSTATE was well-tolerated and effective when used for primary or salvage ITI in this cohort of paediatric patients with SHA and a high-level inhibitor.

Behavioural stimulation and sensation-seeking among prisoners: applications to substance dependency.

BACKGROUND: Sensation-seeking among prisoners with substance dependence difficulties (drug and/or alcohol) was examined. This topic is under-researched in a prisoner sample. AIMS: The aims are to examine the association between sensation-seeking, other personality variables, and substance dependency among prisoners, and to examine if sensation-seeking can be refined. METHODS: Adult male prisoners (n=200) completed self-report measures examining the constructs of interest. RESULTS: Sensation-seeking comprised extraversion and openness to experience. It was more appropriately described as Behavioural-Stimulation-and-Sensation-Seeking (BStim-SS). BStim-SS is related to drug and poly-substance dependency but not alcohol-only dependency. Increased impulsivity was related to all substance use dependencies. CONCLUSIONS AND IMPLICATIONS FOR PRACTISE: BStim-SS presents as a valuable concept to apply to forensic populations. It captures the need for behavioural and emotional stimulation and lends support to Reward Discounting theory as valuable concept to apply across substance dependency. Implications for practise include: • A need to identify a broader concept of sensation-seeking for prisoner samples; • The recognition of differences within substance dependent samples, with impulsivity presenting differently across drug and/or alcohol dependent groups; • Recognition that concepts regularly applied to community samples need to be examined more specifically among forensic samples to ascertain validity.