Haemophilia A: mutation type determines risk of inhibitor formation

The formation of factor VIII antibodies is a major problem for replacement therapy of haemophilia A patients. Antibodies occur in 5-30% of patients with severe haemophilia A. The reason for antibody formation is still unknown. In this study we correlate for the first time different factor VIII gene mutations, stop- and missense mutations, large and small deletions and intrachromosomal intron 22 recombinations to antibody formation. A total of 364 patients with known inhibitor status of our institute, of the database, and of 3 studies representing intron-22-inversion data are included. The results show that the risk for developing factor VIII antibodies is strongly related to stop mutations. large deletions and intrachromosomal recombinations. A probable explanation could be the complete lack of endogenous circulating factor VIII protein in these cases. Other factors that might be important for the pathogenesis of inhibitor formation, e. g. the antenatal period, as well as possible therapeutic effects, are discussed.     

Prevention of bleeding after cardiopulmonary bypass with high-dose tranexamic acid. Double-blind, randomized clinical trial

This prospective, double-blind, randomized trial assessed the effectiveness of high-dose tranexamic acid given in the preoperative period on blood loss in patients undergoing cardiopulmonary bypass. One hundred fifty patients scheduled to undergo cardiac operations with cardiopulmonary bypass were randomized into three groups of equal size. The first group received 10 gm of tranexamic acid intravenously over 20 minutes before sternotomy and a placebo infusion over 5 hours. The second group received 10 gm of tranexamic acid over 20 minutes and then another 10 gm infused intravenously over 5 hours. The control group received a placebo bolus and a placebo infusion over 5 hours (0.9% normal saline solution). The blood loss after the operation was measured at 6 hours and 24 hours. The homologous blood and blood products given during and up to 48 hours after operation were recorded. Eighteen percent of the control group patients shed more than 750 ml blood in 6 hours compared with only 2% in both tranexamic acid groups. Patients who shed more than 750 ml blood required 93% more red blood cell transfusions than patients without excessive bleeding. Tranexamic acid (10 gm) given intravenously in the period before cardiopulmonary bypass reduced blood loss over 6 hours by 50% and over 24 hours by 35%. Continued tranexamic acid infusion (10 gm over 5 hours) did not reduce bleeding further. There was no difference in the coagulation profile before operation between patients with and without excessive bleeding. However, coagulation tests done in the postoperative period indicated ongoing fibrinolysis and platelet dysfunction in patients with excessive bleeding.     

A pathologist''s view of oral cancer in the north west

The significance of oral mucosal squamous cancer is considered in terms of the annual numbers of new cases and deaths, and the physical and psychological effects of the disease and its management. The sex, age and geographical distribution are outlined, and common presenting features and its natural clinical course are discussed. In addition, the presentation reviews the achievements and disappointments of clinicians and scientists involved in cancer management and research in recent years. Microvascular surgery and the adoption of a team approach are selected as achievements which have provided immediate benefits for the patient. The rising incidence of cancer among younger age groups, the occurrence of multiple oral cancers and systemic metastatic deposits are used to illustrate disquieting trends. A health education and screening programme to raise public awareness of oral cancer and the critical importance of early detection, and the application of molecular biology, are suggested as two areas offering hope for future progress.     

Photonic access concentrator for ATM gigabit switching fabrics

In future broadband communication networks the interest for purely photonic switches is due to the bandwidth mismatch between optical transmission networks and electronic switching nodes. Photonic ATM switching fabrics mainly based on wavelength-switching stages are therefore being studied, to implement high capacity switches with also concentration, multiplexing and demultiplexing functions, using state-of-the-art photonic technology. The architecture of an ATM photonic access concentrator is described in this paper, illustrating the design and implementation of its basic subsystems, the traffic concentrator and the cell multiplexer. The design guidelines are outlined in detail referring to an example, where 128 user lines at 622 Mb/s are given access to 4 outlets at 2.488 Gb/s. The corresponding implementation, based on the systematic use of cell wavelength encoding, makes use either of well-known photonic components, such as Fabry-Perot filters, fiber delay lines, splitters and combiners, either of recently developed devices, like high-speed optical gates and tunable filters and lasers. Finally, the system feasibility is demonstrated presenting the results obtained on a reduced size and speed experimental setup of the cell multiplexer     

Design and evaluation of an optically triggered monolithic sampleand hold circuit using GaAs MESFET technology

The design and evaluation of an optically triggered, fully integrated sample and hold circuit (OS/H) is described. Measured results are presented that demonstrate operation of this circuit at 250 Ms/s and with effective resolution approaching 8 bits. The integrated circuit, which measures 2.1 mm×1.4 mm, is realized in -1.0-V threshold, 20-GHz ft GaAs MESFET technology, consumes approximately 200 mW of power, and requires one optical address. The OS/H will find applications in high precision, hybrid, and integrated signal processing systems where high speed, high levels of parallelism, and low timing jitter are important. Measured results of a series photoconducting (Auston switch) OS/H realized in the same technology are presented for comparison purposes     

Impact of finite laser linewidth on the performance of OFDMnetworks employing single-cavity Fabry-Perot demultiplexers

In this paper we investigate the impact of finite laser linewidths (i.e. source partial coherence) on the crosstalk performance of optical frequency division multiplexing (OFDM) networks employing single-cavity Fabry-Perot demultiplexers. Results are presented that show the important limitation imposed by the finite laser linewidths on the attainable bit error rate (BER), maximum number of users, and the required power penalties to overcome this impairment     

Mine operating accurate stability control with optical fibersensing and Bragg grating technology: the European BRITE/EURAM STABILOSproject

Recent developments of stability control in mines, essentially based on Ge-doped fiber Bragg gratings (FBG) are reported including results about the different aspects of the system: accurate characterizations of FBG, sensor network topology and multiplexing method, user interface design and sensor packaging