A survey of 21 haemophilia doctors, throughout Europe, who care for a total of approximately 5000 patients with bleeding disorders addressing practice and opinions regarding prophylaxis in patients aged 16-24 years and adults aged over 50 years, is presented. The outcome of adolescent patients who reduced or stopped prophylaxis was recorded. Eighteen of 19 respondents would consider modification of established prophylaxis in the adolescent age group, principal considerations being avoidance of risks of further concentrate exposure, predicted poor compliance and treatment costs. The preferred age for modification was 16-20 years, but there was very little consensus on the particular prophylactic regime recommended. Approximately, half of a cohort of 218 patients with severe haemophilia successfully reduced or stopped prophylaxis when they reached adolescence. Only 26 of 92 (28%) of the patient cohort who stopped prophylaxis, required reintroduction of a prophylactic regime and 12 of 59 (20%) of those who reduced the intensity of prophylaxis had to reintroduce a more intensive regime. A majority of respondents would consider starting prophylaxis in those over 50 years. There was no consensus as to indications for this practice or the nature of the prophylaxis protocol. We conclude that there is an absence of consensus on the management of patients with severe haemophilia, as they pass through adolescence and young adulthood, and reach the age of 50. Aggregate outcome data suggest a significant proportion of patients in the 18-22 years age range may be able to reduce or stop prophylaxis. A substantial number of older patients are on prophylaxis.

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We report on the optical readout of the gas electron multiplier (GEM) operated with a gaseous mixture suitable for the detection of thermal neutrons: 3 He-CF 4 . A CCD system operating in the 400-1000 nm band was used to collect the light. Spectroscopic data on the visible and NIR scintillation of He-CF 4 are presented. Images of the tracks of the proton and triton recorded with a triple GEM detector are also shown. r

We report on the operation of a GEM-based small TPC using an optical readout. The detector was operated with a mixture of Ar+CF 4 using 5.48 MeV alpha particles obtained from a 241 Am source and the GEM scintillation was concurrently read by a CCD camera and a photomultiplier. Precision collimators were used to define the track orientation. Qualitative results on the accuracy of the track angle, length and charge deposition measurements are presented. r