Studies of vertical wind profiles at Cape Kennedy, Florida Final report

Vertical wind profiles spectral analysis and numerical wind forecasts at Cape Kenned

Key Issues In Inhibitor Management In Patients With Haemophilia

[No abstract available]12SUPPL.1s319s329Bray, G.L., Gomperts, E.D., Courter, S., A multicenter study of recombinant factor VIII (recombinate): Safety, efficacy, and inhibitor risk in previously untreated patients with hemophilia A. The recombinate study group (1994) Blood, 83, pp. 2428-2435Lusher, J.M., Arkin, S., Abildgaard, C.F., Schwartz, R.S., Recombinant factor VIII for the treatment of previously untreated patients with hemophilia A. Safety, efficacy, and development of inhibitors. Kogenate previously untreated patient study group (1993) N Engl J Med, 328, pp. 453-459Hay, C.R., Baglin, T.P., Collins, P.W., The diagnosis and management of factor VIII and IX inhibitors: A guideline from the UK Haemophilia Centre Doctors' Organization (UKHCDO) (2000) Br J Haematol, 111, pp. 78-90Lawrence, J.S., Johnson, J.B., The presence of a circulating anticoagulant in a male member of a hemophiliac family (1941) Trans Am Clin Climatol Assoc, 57, pp. 223-231Konkle, B.A., Ebbesen, L.S., Erhardtsen, E., Randomized, prospective clinical trial of recombinant factor VIIa for secondary prophylaxis in hemophilia patients with inhibitors (2007) J Thromb Haemost, 5, pp. 1904-1913Leissinger, C., Gringeri, A., Antmen, B., Anti-inhibitor coagulant complex prophylaxis in hemophilia with inhibitors (2011) N Engl J Med, 365, pp. 1684-1692Gouw, S.C., Van Den Berg, H.M., Oldenburg, J., F8 gene mutation type and inhibitor development in patients with severe hemophilia A: Systematic review and meta-analysis (2012) Blood, 119, pp. 2922-2934Gouw, S.C., Van Der Bom, J.G., Auerswald, G., Recombinant versus plasma-derived factor VIII products and the development of inhibitors in previously untreated patients with severe hemophilia A: The CANAL cohort study (2007) Blood, 109, pp. 4693-4697Iorio, A., Halimeh, S., Holzhauer, S., Rate of inhibitor development in previously untreated hemophilia A patients treated with plasma-derived or recombinant factor VIII concentrates: A systematic review (2010) J Thromb Haemost, 8, pp. 1256-1265Wight, J., Paisley, S., The epidemiology of inhibitors in haemophilia A: A systematic review (2003) Haemophilia, 9, pp. 418-435Hay, C.R., The epidemiology of factor VIII inhibitors (2006) Haemophilia, 12 (SUPPL. 6), pp. 23-28. , discussion 8-9Astermark, J., Basic aspects of inhibitors to factors VIII and IX and the influence of non-genetic risk factors (2006) Haemophilia, 12 (SUPPL. 6), pp. 8-13Hay, C.R., Palmer, B., Chalmers, E., Incidence of factor VIII inhibitors throughout life in severe hemophilia A in the United Kingdom (2011) Blood, 117, pp. 6367-6370Wacey, A.I., Kemball-Cook, G., Kazazian, H.H., The haemophilia A mutation search test and resource site, home page of the factor VIII mutation database: HAMSTeRS (1996) Nucleic Acids Res, 24, pp. 100-102Green, P.M., Montandon, A.J., Ljung, R., Haemophilia B mutations in a complete Swedish population sample: A test of new strategy for the genetic counselling of diseases with high mutational heterogeneity (1991) Br J Haematol, 78, pp. 390-397Viel, K.R., Ameri, A., Abshire, T.C., Inhibitors of factor VIII in black patients with hemophilia (2009) N Engl J Med, 360, pp. 1618-1627Viel, K.R., Machiah, D.K., Warren, D.M., A sequence variation scan of the coagulation factor VIII (FVIII) structural gene and associations with plasma FVIII activity levels (2007) Blood, 109, pp. 3713-3724Santos, A., Annichino-Bizzacchi, J.M., Ozelo, M.C., Inhibitors of factor VIII in hemophilia (2009) N Engl J Med, 361, pp. 309-310. , author reply 10Oldenburg, J., Pavlova, A., (2006) Genetic risk factors for inhibitors to factors VIII and IX.Haemophilia, 12 (SUPPL. 6), pp. 15-22Goodeve, A.C., Peake, I.R., The molecular basis of hemophilia A: Genotype-phenotype relationships and inhibitor development (2003) Semin Thromb Hemost, 29, pp. 23-30Jacquemin, M., Vantomme, V., Buhot, C., CD4+ T-cell clones specific for wild-type factor VIII: A molecular mechanism responsible for a higher incidence of inhibitor formation in mild/moderate hemophilia A (2003) Blood, 101, pp. 1351-1358Kane, W.H., Davie, E.W., Cloning of a cDNA coding for human factor V, a blood coagulation factor homologous to factor VIII and ceruloplasmin (1986) Proc Natl Acad Sci USA, 83, pp. 6800-6804Lacroix-Desmazes, S., Repesse, Y., Kaveri, S.V., Dasgupta, S., The role of VWF in the immunogenicity of FVIII (2008) Thromb Res, 122 (SUPPL. 2), pp. S3-6Rivard, G.E., Lillicrap, D., Poon, M.C., Can activated recombinant factor VII be used to postpone the exposure of infants to factor VIII until after 2 years of age? (2005) Haemophilia, 11, pp. 335-339Hermans, C., De Moerloose, P., Fischer, K., Management of acute haemarthrosis in haemophilia A without inhibitors: Literature review, European survey and recommendations (2011) Haemophilia, 17, pp. 383-392Kurnik, K., Bidlingmaier, C., Engl, W., New early prophylaxis regimen that avoids immunological danger signals can reduce FVIII inhibitor development (2010) Haemophilia, 16, pp. 256-262Gouw, S.C., Van Den Berg, H.M., Fischer, K., Intensity of factor VIII treatment and inhibitor development in children with severe hemophilia A: The RODIN study (2013) BloodKreuz, W., Ettingshausen, C.E., Zyschka, A., Inhibitor development in previously untreated patients with hemophilia A: A prospective long-term follow-up comparing plasmaderived and recombinant products (2002) Semin Thromb Hemost, 28, pp. 285-290Santagostino, E., Mannucci, P.M., Bianchi Bonomi, A., Guidelines on replacement therapy for haemophilia and inherited coagulation disorders in Italy (2000) Haemophilia, 6, pp. 1-10Rezende, S.M., Pinheiro, K., Caram, C., Registry of inherited coagulopathies in Brazil: First report (2009) Haemophilia, 15, pp. 142-149Manco-Johnson, M.J., Abshire, T.C., Shapiro, A.D., Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia (2007) N Engl J Med, 357, pp. 535-544Kruse-Jarres, R., Inhibitors: Our greatest challenge.Can we minimize the incidence? (2013) Haemophilia, 19 (SUPPL. 1), pp. 2-7Lobet, S., Detrembleur, C., Francq, B., Hermans, C., Natural progression of blood-induced joint damage in patients with haemophilia: Clinical relevance and reproducibility of threedimensional gait analysis (2010) Haemophilia, 16, pp. 813-821Leissinger, C.A., Prevention of bleeds in hemophilia patients with inhibitors: Emerging data and clinical direction (2004) Am J Hematol, 77, pp. 187-193Gringeri, A., Mantovani, L.G., Scalone, L., Cost of care and quality of life for patients with hemophilia complicated by inhibitors: The COCIS Study Group (2003) Blood, 102, pp. 2358-2363Fischer, K., Van Der Bom, J.G., Molho, P., Prophylactic versus on-demand treatment strategies for severe haemophilia: A comparison of costs and long-term outcome (2002) Haemophilia, 8, pp. 745-752Morfini, M., Haya, S., Tagariello, G., European study on orthopaedic status of haemophilia patients with inhibitors (2007) Haemophilia, 13, pp. 606-612Soucie, J.M., Cianfrini, C., Janco, R.L., Joint range-of-motion limitations among young males with hemophilia: Prevalence and risk factors (2004) Blood, 103, pp. 2467-2473Hay, C.R., Dimichele, D.M., The principal results of the International Immune Tolerance Study: A randomized dose comparison (2012) Blood, 119, pp. 1335-1344Astermark, J., Donfield, S.M., Dimichele, D.M., A randomized comparison of bypassing agents in hemophilia complicated by an inhibitor: The FEIBA NovoSeven Comparative (FENOC) Study (2007) Blood, 109, pp. 546-551Sorensen, B., Dargaud, Y., Kenet, G., On-demand treatment of bleeds in haemophilia patients with inhibitors: Strategies for securing and maintaining predictable efficacy with recombinant activated factor VII (2012) Haemophilia, 18, pp. 255-262Kenet, G., Martinowitz, U., Single-dose recombinant activated factor VII therapy in hemophilia patients with inhibitors (2008) Semin Hematol, 45, pp. S38-41Treur, M.J., McCracken, F., Heeg, B., Efficacy of recombinant activated factor VII vs. Activated prothrombin complex concentrate for patients suffering from haemophilia complicated with inhibitors: A Bayesian meta-regression (2009) Haemophilia, 15, pp. 420-436Knight, C., Dano, A.M., Kennedy-Martin, T., Systematic review of efficacy of rFVIIa and aPCC treatment for hemophilia patients with inhibitors (2009) Adv Ther, 26, pp. 68-88Valentino, L.A., The benefits of prophylactic treatment with APCC in patients with haemophilia and high-titre inhibitors: A retrospective case series (2009) Haemophilia, 15, pp. 733-742Teitel, J., Berntorp, E., Dolan, G., A consensus statement on clinical trials of bypassing agent prophylaxis in inhibitor patients (2011) Haemophilia, 17, pp. 516-521Young, G., Auerswald, G., Jimenez-Yuste, V., When should prophylaxis therapy in inhibitor patients be considered? (2011) Haemophilia, 17, pp. e849-e857Dimichele, D., Negrier, C., A retrospective postlicensure survey of FEIBA efficacy and safety (2006) Haemophilia, 12, pp. 352-362Valentino, L.A., Assessing the benefits of FEIBA prophylaxis in haemophilia patients with inhibitors (2010) Haemophilia, 16, pp. 263-271Brackmann, H.H., Schwaab, R., Effenberger, W., Hemophilia treatment. Side effects during immune tolerance induction (2000) Haematologica, 85, pp. 75-77Young, G., McDaniel, M., Nugent, D.J., Prophylactic recombinant factor VIIa in haemophilia patients with inhibitors (2005) Haemophilia, 11, pp. 203-207Morfini, M., Auerswald, G., Kobelt, R.A., Prophylactic treatment of haemophilia patients with inhibitors: Clinical experience with recombinant factor VIIa in European Haemophilia Centres (2007) Haemophilia, 13, pp. 502-507Jimenez-Yuste, V., Alvarez, M.T., Martin-Salces, M., Prophylaxis in 10 patients with severe haemophilia A and inhibitor: Different approaches for different clinical situations (2009) Haemophilia, 15, pp. 203-209Young, G., Auerswald, G., Jimenez-Yuste, V., PRO-PACT: Retrospective observational study on the prophylactic use of recombinant factor VIIa in hemophilia patients with inhibitors (2012) Thromb ResGupta, S., Siddiqi, A.E., Soucie, J.M., The effect of secondary prophylaxis versus episodic treatment on the range of motion of target joints in patients with haemophilia (2013) Br J Haematol, 161, pp. 424-433Carcao, M., Lambert, T., Prophylaxis in haemophilia with inhibitors: Update from international experience (2010) Haemophilia, 16 (SUPPL. 2), pp. 16-23Dimichele, D.M., Hoots, W.K., Pipe, S.W., International workshop on immune tolerance induction: Consensus recommendations (2007) Haemophilia, 13 (SUPPL. 1), pp. 1-22Lenk, H., The German Registry of immune tolerance treatment in hemophilia-1999 update (2000) Haematologica, 85, pp. 45-47Brackmann, H.H., Oldenburg, J., Schwaab, R., Immune tolerance for the treatment of factor VIII inhibitors-twenty years' 'bonn protocol' (1996) Vox Sang, 70 (SUPPL. 1), pp. 30-35Oldenburg, J., Schwaab, R., Brackmann, H.H., Induction of immune tolerance in haemophilia A inhibitor patients by the 'Bonn Protocol': Predictive parameter for therapy duration and outcome (1999) Vox Sang, 77 (SUPPL. 1), pp. 49-54Mauser-Bunschoten, E.P., Nieuwenhuis, H.K., Roosendaal, G., Van Den Berg, H.M., Low-dose immune tolerance induction in hemophilia A patients with inhibitors (1995) Blood, 86, pp. 983-988Indications and recommended doses for treating patients with factor VIII inhibitors in hemophilia A (2008) Cross-Sectional Guidelines for Therapy with Blood Components and Plasma Derivatives. Executive Committee of the German Medical Association on the Recommendation of the Scientific Advisory Board, p. 91. , German Medical AssociationCoppola, A., Margaglione, M., Santagostino, E., Factor VIII gene (F8) mutations as predictors of outcome in immune tolerance induction of hemophilia A patients with highresponding inhibitors (2009) J Thromb Haemost, 7, pp. 1809-1815Freiburghaus, C., Berntorp, E., Ekman, M., Immunoadsorption for removal of inhibitors: Update on treatments in Malmo-Lund between 1980 and 1995 (1998) Haemophilia, 4, pp. 16-20Auerswald, G., Spranger, T., Brackmann, H.H., The role of plasmaderived factor VIII/von Willebrand factor concentrates in the treatment of hemophilia A patients (2003) Haematologica, 88, pp. EREP05Dimichele, D., The North American Immune Tolerance Registry: Contributions to the thirty-year experience with immune tolerance therapy (2009) Haemophilia, 15, pp. 320-328Mariani, G., Ghirardini, A., Bellocco, R., Immune tolerance in hemophilia-principal results from the International Registry. Report of the factor VIII and IX Subcommittee (1994) Thromb Haemost, 72, pp. 155-158Mariani, G., Kroner, B., Immune tolerance in hemophilia with factor VIII inhibitors: Predictors of success (2001) Haematologica, 86, pp. 1186-1193Franchini, M., Mannucci, P.M., Inhibitors of propagation of coagulation (factors VIII, IX and XI): A review of current therapeutic practice (2011) Br J Clin Pharmacol, 72, pp. 553-562Greninger, D.A., Saint-Remy, J.M., Jacquemin, M., The use of factor VIII/von Willebrand factor concentrate for immune tolerance induction in haemophilia A patients with high-titre inhibitors: Association of clinical outcome with inhibitor epitope profile (2008) Haemophilia, 14, pp. 295-302Gringeri, A., Musso, R., Mazzucconi, M.G., Immune tolerance induction with a high purity von Willebrand factor/VIII complex concentrate in haemophilia A patients with inhibitors at high risk of a poor response (2007) Haemophilia, 13, pp. 373-379Kurth, M.A., Dimichele, D., Sexauer, C., Immune tolerance therapy utilizing factor VIII/von Willebrand factor concentrate in haemophilia A patients with high titre factor VIII inhibitors (2008) Haemophilia, 14, pp. 50-55Astermark, J., Morado, M., Rocino, A., Current European practice in immune tolerance induction therapy in patients with haemophilia and inhibitors (2006) Haemophilia, 12, pp. 363-371Garvey, B., Rituximab in the treatment of autoimmune haematological disorders (2008) Br J Haematol, 141, pp. 149-169Franchini, M., Mengoli, C., Lippi, G., Immune tolerance with rituximab in congenital haemophilia with inhibitors: A systematic literature review based on individual patients' analysis (2008) Haemophilia, 14, pp. 903-912Sorensen, B., Johansen, P., Christiansen, K., Whole blood coagulation thrombelastographic profiles employing minimal tissue factor activation (2003) J Thromb Haemost, 1, pp. 551-558De Paula, E.V., Kavakli, K., Mahlangu, J., Recombinant factor VIIa analog (vatreptacog alfa [activated]) for treatment of joint bleeds in hemophilia patients with inhibitors: A randomized controlled trial (2012) J Thromb Haemost, 10, pp. 81-89Holmberg, H.L., Lauritzen, B., Tranholm, M., Ezban, M., Faster onset of effect and greater efficacy of NN1731 compared with rFVIIa, aPCC and FVIII in tail bleeding in hemophilic mice (2009) J Thromb Haemost, 7, pp. 1517-1522Moss, J., Scharling, B., Ezban, M., Moller Sorensen, T., Evaluation of the safety and pharmacokinetics of a fast-acting recombinant FVIIa analogue, NN1731, in healthy male subjects (2009) J Thromb Haemost, 7, pp. 299-305Sorensen, B., Persson, E., Ingerslev, J., Factor VIIa analogue (V158D/E296V/M298Q-FVIIa) normalises clot formation in whole blood from patients with severe haemophilia A (2007) Br J Haematol, 137, pp. 158-165Allen, G.A., Persson, E., Campbell, R.A., A variant of recombinant factor VIIa with enhanced procoagulant and antifibrinolytic activities in an in vitro model of hemophilia (2007) Arterioscler Thromb Vasc Biol, 27, pp. 683-689Mahlangu, J.N., Coetzee, M.J., Laffan, M., Phase I, randomized, double-blind, placebo-controlled, single-dose escalation study of the rFVIIa variant BAY 86-6150 in hemophilia (2012) J Thromb Haemost, 10, pp. 773-780Karpf, D.M., Sorensen, B.B., Hermit, M.B., Prolonged halflife of glycoPEGylated rFVIIa variants compared to native rFVIIa (2011) Thromb Res, 128, pp. 191-195Sen, P., Ghosh, S., Ezban, M., Effect of glycoPEGylation on factor VIIa binding and internalization (2010) Haemophilia, 16, pp. 339-348Toschi, V., OBI-1, porcine recombinant factor VIII for the potential treatment of patients with congenital hemophilia A and alloantibodies against human Factor VIII (2010) Curr Opin Mol Ther, 12, pp. 617-625Parker, E.T., Craddock, H.N., Barrow, R.T., Lollar, P., Comparative immunogenicity of recombinant B domain-deleted porcine factor VIII and Hyate: C in hemophilia A mice presensitized to human factor VIII (2004) J Thromb Haemost, 2, pp. 605-611Kempton, C.L., Abshire, T.C., Deveras, R.A., Pharmacokinetics and safety of OBI-1, a recombinant B domain-deleted porcine factor VIII, in subjects with haemophilia A (2012) Haemophilia, 18, pp. 798-804Abshire, T.C., Brackmann, H.H., Scharrer, I., Sucrose formulated recombinant human antihemophilic factor VIII is safe and efficacious for treatment of hemophilia A in home therapy-International Kogenate-FS Study Group (2000) Thromb Haemost, 83, pp. 811-816Tarantino, M.D., Collins, P.W., Hay, C.R., Clinical evaluation of an advanced category antihaemophilic factor prepared using a plasma/albumin-free method: Pharmacokinetics, efficacy, and safety in previously treated patients with haemophilia A (2004) Haemophilia, 10, pp. 428-437Young, G., Cooper, D.L., Gut, R.Z., Dosing and effectiveness of recombinant activated factor VII (rFVIIA) in congenital haemophilia with inhibitors by bleed type and location: The experience of the Haemophilia and Thrombosis Research Society (HTRS) Registry (2004-2008) (2012) Haemophilia, 18, pp. 990-99

Exploring strategies for classification of external stimuli using statistical features of the plant electrical response

This is the author accepted manuscript. The final version is available from the Royal Society via the DOI in this record.Plants sense their environment by producing electrical signals which in essence represent changes in underlying physiological processes. These electrical signals, when monitored, show both stochastic and deterministic dynamics. In this paper, we compute 11 statistical features from the raw non-stationary plant electrical signal time series to classify the stimulus applied (causing the electrical signal). By using different discriminant analysis-based classification techniques, we successfully establish that there is enough information in the raw electrical signal to classify the stimuli. In the process, we also propose two standard features which consistently give good classification results for three types of stimuli--sodium chloride (NaCl), sulfuric acid (H₂SO₄) and ozone (O₃). This may facilitate reduction in the complexity involved in computing all the features for online classification of similar external stimuli in future.The work reported in this paper was supported by project PLants Employed As SEnsor Devices (PLEASED), EC grant agreement number 296582

Background suppression in massive TeO2_2 bolometers with Neganov-Luke amplified light detectors

Bolometric detectors are excellent devices for the investigation of neutrinoless double-beta decay (0$\nu\beta\beta$). The observation of such decay would demonstrate the violation of lepton number, and at the same time it would necessarily imply that neutrinos have a Majorana character. The sensitivity of cryogenic detectors based on TeO$_2$ is strongly limited by the alpha background in the region of interest for the 0$\nu\beta\beta$ of $^{130}$Te. It has been demonstrated that particle discrimination in TeO$_2$ bolometers is possible measuring the Cherenkov light produced by particle interactions. However an event-by-event discrimination with NTD-based light detectors has to be demonstrated. We will discuss the performance of a highly-sensitive light detector exploiting the Neganov-Luke effect for signal amplification. The detector, being operated with NTD-thermistor and coupled to a 750 g TeO$_2$ crystal, shows the ability for an event-by-event identification of electron/gamma and alpha particles. The extremely low detector baseline noise, RMS 19 eV, demonstrates the possibility to enhance the sensitivity of TeO$_2$-based 0$\nu\beta\beta$ experiment to an unprecedented level

Reliability and concurrent validity of the Dutch hip and knee replacement expectations surveys

Background: Preoperative expectations of outcome of total hip and knee arthroplasty are important determinants of patients' satisfaction and functional outcome. Aims of the study were (1) to translate the Hospital for Special Surgery Hip Replacement Expectations Survey and Knee Replacement Expectations Survey into Dutch and (2) to study test-retest reliability and concurrent validity. Methods: Patients scheduled for total hip (N = 112) or knee replacement (N = 101) were sent the Dutch Expectations Surveys twice with a 2 week interval to determine test-retest reliability. To determine concurrent validity, the Expectation WOMAC was sent. Results: The results for the Dutch Hip Replacement Expectations Survey revealed good test-retest reliability (ICC 0.87), no bias and good internal consistency (alpha 0.86) (N = 72). The correlation between the Hip Expectations Score and the Expectation WOMAC score was 0.59 (N = 86). The results for the Dutch Knee Replacement Expectations Survey revealed good test-retest reliability (ICC 0.79), no bias and good internal consistency (alpha 0.91) (N = 46). The correlation with the Expectation WOMAC score was 0.52 (N = 57). Conclusions: Both Dutch Expectations Surveys are reliable instruments to determine patients' expectations before total hip or knee arthroplasty. As for concurrent validity, the correlation between both surveys and the Expectation WOMAC was moderate confirming that the same construct was determined. However, patients scored systematically lower on the Expectation WOMAC compared to the Dutch Expectation Surveys. Research on patients' expectations before total hip and knee replacement has only been performed in a limited amount of countries. With the Dutch Expectations Surveys it is now possible to determine patients' expectations in another culture and healthcare setting

Dual trigger protocol is an effective IVF strategy in both normal and high responders without compromising pregnancy outcomes

To compare pregnancy outcomes between normal versus high responders after dual trigger of final oocyte maturation with gonadotropin-releasing hormone (GnRH) agonist and human chorionic gonadotropin (hCG) in fresh in-vitro fertilization (IVF) cycles, where ovarian stimulation was achieved by a flexible GnRH antagonist protocol