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2. Conceptual design of the AGATA 2[formula omitted] array at LNL

Author

Valiente-Dobón, J.J., Menegazzo, R., Goasduff, A., Agguiaro, D., Aguilera, P., Angelini, F., Balogh, M., Bazzacco, D., Benito, J., Benzoni, G., Bez, N., Bolognesi, M., Bottoni, S., Brugnara, D., Carollo, S., Cocconi, P., Cogo, A., Collado, J., Crespi, F.C.L., Ertoprak, A., Escudeiro, R., Galtarossa, F., Gamba, E.R., Gambalonga, A., Servín, B. Góngora, Gottardo, A., Gozzelino, A., Gulmini, M., Huang, Z., Marchi, T., Mengoni, D., Modanese, P., Napoli, D.R., Pellumaj, J., Pérez-Vidal, R.M., Pigliapoco, S., Pilotto, E., Ramina, L., Rampazzo, M., Raniero, W., Rebeschini, M., Rezynkina, K., Rosso, D., Scarcioffolo, M., Scarpa, D., Sedlák, M., Smith, R., Toniolo, N., Veronese, F., Volpe, V., Zago, L., Zanon, I., Zhang, G., Abels, R., Allegrini, M.L., Aufranc, C., Baulieu, G., Belkhiria, C., Benettoni, M., Benini, D., Bentley, M., Biasotto, M., Blaizot, M., Miquel, J. Blasco, Boiano, C., Boston, A., Boston, H., Boujrad, A., Bourgault, P., Bracco, A., Brambilla, S., Burrows, I., Camera, F., Capra, S., Capsoni, A., Cash, R., Civera, J.V., Clément, E., Coelli, S., Cordwell, M., Corradi, L., Coudert, S., De Angelis, G., De Ruvo, L., Debras, G., Del Fabbro, M., Diklić, J., Dosme, N., Duchene, G., Duclos, B., Dudouet, J., Eberth, J., Elloumi, S., Everett, C., Fantinel, S., Fillinger, M., Fioretto, E., Fransen, C., Gadea, A., Gibelin, L., González, V., Goupil, J., Görgen, C., Grant, A., Green, K., Ha, J., Hartnett, T., Henseler, K., Hess, H., Hirsch, R., Houarner, C., Jacob, J., Joannem, T., Judson, D.S., Karkour, N., Karolak, M., Kebbiri, M., Kieffer, J., Labiche, M., Lafay, X., Le Jeannic, P., Lefevre, A., Legay, E., Legruel, F., Lenzi, S., Leoni, S., Linget, D., Liptrot, M., López-Martens, A., Lotodé, A., Manara, L., Ménager, L., Mijatović, T., Million, B., Minarello, A., Montagnoli, G., Morrall, P., Mullacrane, I., Nyberg, J., Philippon, G., Polettini, M., Popieul, F., Pullia, A., Recchia, F., Reiter, P., Richardt, G., Rocchini, M., Roger, A., Saillant, F., Sanchis, E., Laskar, Md.S.R., Secci, G., Sigward, M.-H., Simpson, J., Solenne, N., Spee, F., Stefanini, A.M., Stézowski, O., Szilner, S., Templeton, N., Theisen, Ch., Thiel, S., Tomasi, F., Tzvetkov, S., Vigano, D., Viscione, E., Wieland, O., Wimmer, K., Wittwer, G., and Zielińska, M.

Published
2023
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3. GET: A generic electronics system for TPCs and nuclear physics instrumentation

Author

Pollacco, E.C., Grinyer, G.F., Abu-Nimeh, F., Ahn, T., Anvar, S., Arokiaraj, A., Ayyad, Y., Baba, H., Babo, M., Baron, P., Bazin, D., Beceiro-Novo, S., Belkhiria, C., Blaizot, M., Blank, B., Bradt, J., Cardella, G., Carpenter, L., Ceruti, S., De Filippo, E., Delagnes, E., De Luca, S., De Witte, H., Druillole, F., Duclos, B., Favela, F., Fritsch, A., Giovinazzo, J., Gueye, C., Isobe, T., Hellmuth, P., Huss, C., Lachacinski, B., Laffoley, A.T., Lebertre, G., Legeard, L., Lynch, W.G., Marchi, T., Martina, L., Maugeais, C., Mittig, W., Nalpas, L., Pagano, E.V., Pancin, J., Poleshchuk, O., Pedroza, J.L., Pibernat, J., Primault, S., Raabe, R., Raine, B., Rebii, A., Renaud, M., Roger, T., Roussel-Chomaz, P., Russotto, P., Saccà, G., Saillant, F., Sizun, P., Suzuki, D., Swartz, J.A., Tizon, A., Trifiró, A., Usher, N., Wittwer, G., and Yang, J.C.

Published
2018
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4. Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: results from the European Network of Rare Bleeding Disorders

Author

PEYVANDI, F., PALLA, R., MENEGATTI, M., SIBONI, S.M., HALIMEH, S., FAESER, B., PERGANTOU, H., PLATOKOUKI, H., GIANGRANDE, P., PEERLINCK, K., CELKAN, T., OZDEMIR, N., BIDLINGMAIER, C., INGERSLEV, J., GIANSILY‐BLAIZOT, M., SCHVED, J.F., GILMORE, R., GADISSEUR, A., BENEDIK‐DOLNIČAR, M., KITANOVSKI, L., MIKOVIC, D., MUSALLAM, K.M., and ROSENDAAL, F.R.

Published
2012
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21. Pharmacokinetic properties of recombinant FVIIa in inherited FVII deficiency account for a large volume of distribution at steady state and a prolonged pharmacodynamic effect

Author

Morfini, M, Batorova, A, Mariani, G, Auerswald, G, Bernardi, Francesco, Di Minno, G, Dolce, A, Fede, C, Giansily Blaizot, M, Ingerslev, J, Martinowitz, U, Napolitano, M, Pinotti, Mirko, Schved, Jf, International FVII [IF7], Seven Treatment Evaluation Registry [STER] Study Groups, Morfini, M, Batorova, A, Mariani, G, Auerswald, G, Bernardi, F, DI MINNO, Giovanni, Dolce, A, Fede, C, Giansily Blaizot, M, Ingerslev, J, Martinowitz, U, Napolitano, M, Pinotti, M, Schved, Jf, Morfini M, Batorova A, Mariani G, Auerswald G, Bernardi F, Di Minno G, Dolce A, Fede C, Giansily-Blaizot M, Ingerslev J, Martinowitz U, Napolitano M, Pinotti M, and Schved JF.

Subjects
Heredity, Pharmacokinetic, inherited Factor VII deficiency, Factor VII Deficiency, Socio-culturale, Factor VIIa, Pharmacology, Severity of Illness Index, Pharmacokinetics, Predictive Value of Tests, hemic and lymphatic diseases, Humans, Medicine, Genetic Predisposition to Disease, FVII deficiency, Registries, cardiovascular diseases, Blood Coagulation, Volume of distribution, biology, Coagulants, business.industry, Vascular biology, rFVIIa, Hematology, Factor VII, Recombinant Proteins, Phenotype, Treatment Outcome, pharmacokinetics, Recombinant factor VIIa, Pharmacodynamics, biology.protein, Blood Coagulation Tests, Steady state (chemistry), Drug Monitoring, business
Abstract

Pharmacokinetic properties of recombinant FVIIa in inherited FVII deficiency account for a large volume of distribution at steady state and a prolonged pharmacodynamic effect

Published
2014

22. Invasive procedures and minor surgery in factor VII deficiency

Author

Mariani,G, Dolce, A, Ingerslev, J, Giansily Blaizot, M, DI Minno, MD, Auerswald, G, DE Saez, AR, Tagliaferri, A, Batorova, A., NAPOLITANO, Mariasanta, Mariani, Guglielmo, Dolce, A., Napolitano, M., Ingerslev, J., Giansily Blaizot, M., DI MINNO, Matteo, Auerswald, G., De Saez, A. R., Tagliaferri, A., Batorova, A., Mariani,G, Dolce, A, Napolitano, M, Ingerslev, J, Giansily-Blaizot, M, DI Minno, MD, Auerswald, G, DE Saez, AR, Tagliaferri, A, and Batorova, A

Subjects
Adult, Male, MINOR SURGERY, INVASIVE PROCEDURES, FACTOR VII DEFICIENCY, Adolescent, Factor VII Deficiency, Blood Loss, Surgical, Infant, Hemorrhage, Hematology, Factor VII, Hemostasi, Middle Aged, Settore MED/15 - Malattie Del Sangue, Minor Surgical Procedure, Young Adult, Coagulant, Child, Preschool, Surgical Procedures, Operative, Female, Child, Genetics (clinical), Aged, Human
Published
2012

23. Replacement Therapy for Minor Surgery and Invasive Procedures in Factor VII Deficiency: The STER Experience

Author

Napolitano, M., Dolce, A., Ingerslev, J., Giansily-blaizot, M., Minno, M., Auerswald, G., Saez, A., Batorova, A., Caimi, T., Chuansumrit, A., Fernandez, M., Bertrand, M., Verdy, E., Zanon, E., Abecassis, L., Aronis, S., Siragusa, S., Orecchioni, A., Mariani, G., Napolitano, M., Dolce, A., Ingerslev, J., Giansily-Blaizot, M., Minno, M., Auerswald, G., Saez, A., Batorova, A., Caimi, T., Chuansumrit, A., Fernandez, M., Bertrand, M., Verdy, E., Zanon, E., Abecassis, L., Aronis, S., Siragusa, S., Orecchioni, A., and Mariani, G.

Subjects
Adverse events, Inherited Factor VII deficiency
Published
2011

24. Comunicazione orale

Author

Di Minno, M., Batorova, A., Dolce, A., Minno, G., Bernardi, F., Schved, J., Ingerslev, J., Giansily Blaizot, M., Auerswald, G., Mariani, G., NAPOLITANO, Mariasanta, Di Minno, M., Batorova, A., Napolitano, M., Dolce, A., Minno, G., Bernardi, F., Schved, J., Ingerslev, J., Giansily-Blaizot, M., Auerswald, G., and Mariani, G.

Subjects
Bleeding Phenotype,Factor VII deficiency
Published
2011

25. Recombinant, activated factor VII for surgery in factor VII deficiency: a prospective evaluation - the surgical STER

Author

Mariani, G, Dolce, A, Batorova, A, Auerswald, G, Schved, JF, Siragusa, S, Napolitano, M, Knudsen, JB, Ingerslev, J, Aronis, S, Grandone, E, Tagariello, G, Tagliaferri, A, Caimi, TM, Lopez Fernandez, F, Lapecorella, M, Orecchioni, A, Jansily Blaizot, M, Wulff, K., DI MINNO, GIOVANNI, Mariani, G, Dolce, A, Batorova, A, Auerswald, G, Schved, Jf, Siragusa, S, Napolitano, M, Knudsen, Jb, Ingerslev, J, Aronis, S, DI MINNO, Giovanni, Grandone, E, Tagariello, G, Tagliaferri, A, Caimi, Tm, Lopez Fernandez, F, Lapecorella, M, Orecchioni, A, Jansily Blaizot, M, and Wulff, K.

Published
2011

26. Chapter 11: Petroleum systems of Syria, in L. Marlow, C. Kendall and L. Yose eds, Petroleum systems of the Tethyan region

Author

Barrier, Eric, Machlour, L., Blaizot, M., Institut des Sciences de la Terre de Paris (iSTeP), Université Pierre et Marie Curie - Paris 6 (UPMC)-Centre National de la Recherche Scientifique (CNRS), and Queyroy, Marie José

Subjects
[SDU.STU] Sciences of the Universe [physics]/Earth Sciences, [SDU.STU]Sciences of the Universe [physics]/Earth Sciences, ComputingMilieux_MISCELLANEOUS
Abstract

International audience

Published
2014

27. A New Front-End High-Resolution Sampling Board for the New-Generation Electronics of EXOGAM2 and NEDA Detectors

Author

Egea, F. J., Gonzalez, V., Tripon, M., Jastrzab, M., Triossi, A., Gadea, A., Blaizot, M., and 0-Belirlenecek

Subjects
0-Belirlenecek
Abstract

19th IEEE-NPSS Real Time Conference (RT) -- MAY 26-30, 2014 -- Nara, JAPAN, WOS: 000380588000136, The present paper aims to present the final design and results of the FADC Mezzanine for EXOGAM and NEDA detectors, focusing on the resolution performance. Finally, the conclusion shows how a common digitizing device has been integrated in an experimental environment involving low-noise and fast sampling rates. Not only the integration fulfilled the expected specifications on both systems, but also, it shows how a study of synergy between detectors has led to a reduction of resources and time by applying a common strategy., Osaka Univ, Res Nucl Phys, IEEE Nucl & Plasma Sci Soc

Published
2014

30. Design and test of a high-speed flash ADC mezzanine card for high-resolution and timing performance in nuclear structure experiments [Conference Paper]

Author

Egea, X., Sanchis, E., Gonzalez, V., Gadea, A., Blasco, J.M., Barrientos, D., Wadsworth, R., Valiente Dobon, J.J., Tripon, M., Boujrad, A., Houarner, C., Jastrzab, M., Blaizot, M., Bourgault, P., De Angelis, G., Erduran, Mustafa Nizamettin, Erturk, S., De France, G., Huyuk, T., Jaworski, G., Di Nitto, A., Nyberg, J., Palacz, M., Pipidis, A., Tarnowski, R., and Triossi, A.

Subjects
Neutron detectors, Germanium, Ultraviolet spectroscopy, Nuclear physics, Historic preservation, Scintillation counters
Abstract

2012 18th IEEE-NPSS Real Time Conference, RT 2012 -- 9 June 2012 through 15 June 2012 -- Berkeley, CA -- 95754 This board will be part of the upgrade for the new electronics for the EXOGAM2 (HP-Ge detector array) and NEDA (BC501A-based neutron detector array), therefore it was necessary to deal with the problem of providing a sampling card with high resolution for new gamma spectroscopy experiments while sampling at very high rates, with a broad bandwidth in order to preserve the shape for further analysis. Pulse shape analysis is of paramount importance in neutron detectors, such as NEDA, based on scintillators that are sensitive to ?-rays as well. High resolution and high speed are often two parameters which conform a trade-off and it is hard to achieve both simultaneously. The aforementioned constraints and the urge of building new sampling electronics to improve the signal analysis in nuclear physics experiments, led to the development of this FADC mezzanine This involves sampling rates up to 250 Msps preserving a high resolution of 11.3 effective bits in order to satisfy the experiment demands. In this work is described the design and the test bench proposed for a proper high speed ADC characterization system and the results obtained up to now. © 2012 IEEE.

Published
2012

31. Coagulation Factor Activity Level and Clinical Bleeding Severity in Rare Bleeding Disorders: Results Foam the European Network of Rare Bleeding Disorders (EN-RBD)

Author

Peyvandi, F., Palla, R., Menegatti, M., Siboni, S.M., Halimeh, S., Faeser, B., Pergantou, H., Platokouki, H., Giangrande, P., Peerlink, K., Celkan, T., Ozdemir, N., Bidlingmaier, C., Ingerslev, J., Giansily-Blaizot, M., Schved, J.F., Gilmore, R., Gadisseur, A.P., Benedik-Dolnicar, M., Kitanovski, L., Mikovic, D., Musallam, K.M., and Rosendaal, F.R.

Published
2011

32. Factor VII deficiency: evidence that in minor surgery one-day replacement therapy is sufficient

Author

Mariani, G, Dolce, A, Ingerslev, J, Batorova, A, Schved, JF, Di Minno, G, Auerswald, G, Giansilly Blaizot,M, Aronis, S, Ruiz de Saez, A, Tagliaferri, A., NAPOLITANO, Mariasanta, SIRAGUSA, Sergio, Mariani, G, Dolce, A, Ingerslev, J, Batorova, A, Napolitano, M, Schved, JF, Di Minno, G, Auerswald, G, Giansilly-Blaizot,M, Aronis, S, Siragusa, S, Ruiz de Saez, A, and Tagliaferri, A

Subjects
Inherited FACTOR VII DEFICIENCY, MINOR SURGERY, INVASIVE PROCEDURES, Settore MED/15 - Malattie Del Sangue
Published
2011

35. Circulating FVIII-specific IgG, IgA and IgM memory B cells from haemophilia A patients.

Author

Diaz, I., Bolloré, K., Tuaillon, E., Lapalud, P., Giansily ‐ Blaizot, M., Vendrell, J. P., Schved, J. F., and Lavigne ‐ Lissalde, G.

Subjects
BLOOD coagulation factor VIII antibodies, IMMUNOGLOBULIN M, B cell differentiation, HEMOPHILIACS, HEMOPHILIA treatment, MEDICAL care, THERAPEUTICS
Abstract

Introduction Approximately, 25% of haemophilia A ( HA) patients treated by factor VIII ( FVIII), develop antibodies, known as inhibitors, neutralizing the activity of infused FVIII. This immune response involves B cells ( BC), including FVIII-specific memory B cells ( MBC). Production of anti- FVIII antibodies after stimulation of FVIII-specific MBC suggests a role of these cells in the immune response to FVIII. Animal models allowed the study of circulating FVIII-specific cells, however few data are available on HA patients. Aim and methods In the present study, we simultaneously detected, via ELISpot assay, different isotypes of MBC in the blood of HA patients, after polyclonal activation. Patients included: three with active inhibitors; three with a history of inhibitors; six without any past or active inhibitor. Results FVIII-specific MBC were detected in peripheral blood of HA patients: (i) patients with active inhibitors (IgG: 4-5.2/106 BC; IgA: 2.9-4/106 BC) (ii) patients with a past of inhibitors (no IgG BC; IgA: 5-7.5/106 BC) (iii) patients without inhibitors (no IgG BC or IgA BC except one patient had two FVIII-specific IgA BC/106 BC). Conclusion FVIII-specific IgA MBC were detected in HA patients with past and current immune responses against FVIII and FVIII-specific IgG MBC were found only in those with positive inhibitors. This study shows the possibility to detect and characterize easily and simultaneously the MBC from patient blood and that MBC seem different according to anti- FVIII immune history. It could be a useful tool to study anti- FVIII response and Immune Tolerance Induction cellular mechanisms. [ABSTRACT FROM AUTHOR]

Published
2016
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36. Women with congenital factor VII deficiency: clinical phenotype and treatment options from two international studies.

Author

Napolitano, M., Di Minno, M. N. D., Batorova, A., Dolce, A., Giansily ‐ Blaizot, M., Ingerslev, J., Schved, J. ‐ F., Auerswald, G., Kenet, G., Karimi, M., Shamsi, T., Ruiz de Sáez, A., Dolatkhah, R., Chuansumrit, A., Bertrand, M. A., and Mariani, G.

Subjects
HEMOPHILIA, BLOOD coagulation factor VIII antibodies, PATHOLOGICAL physiology, HEMOPHILIA treatment, MENORRHAGIA, GENITALIA, FOREIGN study, DISEASE risk factors
Abstract

Introduction A paucity of data exists on the incidence, diagnosis and treatment of bleeding in women with inherited factor VII ( FVII) deficiency. Aim Here we report results of a comprehensive analysis from two international registries of patients with inherited FVII deficiency, depicting the clinical picture of this disorder in women and describing any gender-related differences. Methods A comprehensive analysis of two fully compatible, international registries of patients with inherited FVII deficiency (International Registry of Factor VII deficiency, IRF7; Seven Treatment Evaluation Registry, STER) was performed. Results In our cohort ( N = 449; 215 male, 234 female), the higher prevalence of mucocutaneous bleeds in females strongly predicted ensuing gynaecological bleeding (hazard ratio = 12.8, 95% CI 1.68-97.6, P = 0.014). Menorrhagia was the most prevalent type of bleeding (46.4% of patients), and was the presentation symptom in 12% of cases. Replacement therapies administered were also analysed. For surgical procedures ( n = 50), a receiver operator characteristic analysis showed that the minimal first dose of rFVIIa to avoid postsurgical bleeding during the first 24 hours was 22 μg kg−1, and no less than two administrations. Prophylaxis was reported in 25 women with excellent or effective outcomes when performed with a total weekly rFVIIa dose of 90 μg kg−1 (divided as three doses). Conclusion Women with FVII deficiency have a bleeding disorder mainly characterized by mucocutaneous bleeds, which predicts an increased risk of ensuing gynaecological bleeding. Systematic replacement therapy or long-term prophylaxis with rFVIIa may reduce the impact of menorrhagia on the reproductive system, iron loss and may avoid unnecessary hysterectomies. [ABSTRACT FROM AUTHOR]

Published
2016
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38. A New Front-End High-Resolution Sampling Board for the New-Generation Electronics of EXOGAM2 and NEDA Detectors.

Author

Egea Canet, F. J., Gonzalez, V., Tripon, M., Jastrzab, M., Triossi, A., Gadea, A., de France, G., Valiente-Dobon, J. J., Barrientos, D., Sanchis, E., Boujrad, A., Houarner, C., Blaizot, M., Bourgault, P., de Angelis, G., Erduran, M. N., Erturk, S., Huyuk, T., Jaworski, G., and Luo, X. L.

Subjects
GAMMA ray spectrometer, NEUTRON counters, EXOTIC nuclei, ELECTRONICS, NUCLEAR science
Abstract

This paper presents the final design and results of the FADC Mezzanine for the EXOGAM (EXOtic GAMma array spectrometer) and NEDA (Neutron Detector Array) detectors. The measurements performed include those of studying the effective number of bits, the energy resolution using HP-Ge detectors, as well as timing histograms and n/\gamma discrimination performance. Finally, the conclusion shows how a common digitizing device has been integrated in the experimental environment of two very different detectors which combine both low-noise acquisition and fast sampling rates. Not only the integration fulfilled the expected specifications on both systems, but it also showed how a study of synergy between detectors could lead to the reduction of resources and time by applying a common strategy. [ABSTRACT FROM PUBLISHER]

Published
2015
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39. Digital Front-End Electronics for the Neutron Detector NEDA.

Author

Egea Canet, F. J., Houarner, C., Boujrad, A., Gonzalez, V., Tripon, M., Jastrzab, M., Triossi, A., de France, G., Gadea, A., Valiente-Dobon, J. J., Barrientos, D., Sanchis, E., Blaizot, M., Bourgault, P., de Angelis, G., Erduran, M. N., Erturk, S., Huyuk, T., Jaworski, G., and Luo, X. L.

Subjects
NEUTRON counters, PHOTOMULTIPLIERS, DIGITAL electronics -- Research, NUCLEAR structure, NUCLEAR science
Abstract

This paper presents the design of the NEDA (Neutron Detector Array) electronics, a first attempt to involve the use of digital electronics in large neutron detector arrays. Starting from the front-end modules attached to the PMTs (PhotoMultiplier Tubes) and ending up with the data processing workstations, a comprehensive electronic system capable of dealing with the acquisition and pre-processing of the neutron array is detailed. Among the electronic modules required, we emphasize the front-end analog processing, the digitalization, digital pre-processing and communications firmware, as well as the integration of the GTS (Global Trigger and Synchronization) system, already used successfully in AGATA (Advanced Gamma Tracking Array). The NEDA array will be available for measurements in 2016. [ABSTRACT FROM PUBLISHER]

Published
2015
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40. Design and test of a high-speed flash ADC mezzanine card for high-resolution and timing performance in nuclear structure experiments.

Author

Egea, X., Sanchis, E., Gonzalez, V., Gadea, A., Blasco, J.M., Barrientos, D., Valiente Dobon, J.J., Tripon, M., Boujrad, A., Houarner, C., Jastrzab, M., Blaizot, M., Bourgault, P., de Angelis, G., Erduran, M.N., Erturk, S., de France, G., Huyuk, T., Jaworski, G., and di Nitto, A.

Abstract

This board will be part of the upgrade for the new electronics for the EXOGAM2 (HP-Ge detector array) and NEDA (BC501A-based neutron detector array), therefore it was necessary to deal with the problem of providing a sampling card with high resolution for new gamma spectroscopy experiments while sampling at very high rates, with a broad bandwidth in order to preserve the shape for further analysis. Pulse shape analysis is of paramount importance in neutron detectors, such as NEDA, based on scintillators that are sensitive to ?-rays as well. High resolution and high speed are often two parameters which conform a trade-off and it is hard to achieve both simultaneously. The aforementioned constraints and the urge of building new sampling electronics to improve the signal analysis in nuclear physics experiments, led to the development of this FADC mezzanine This involves sampling rates up to 250 Msps preserving a high resolution of 11.3 effective bits in order to satisfy the experiment demands. In this work is described the design and the test bench proposed for a proper high speed ADC characterization system and the results obtained up to now. [ABSTRACT FROM PUBLISHER]

Published
2012
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41. Two new double mutant alleles of the F7 gene and a literature review on alleles with two mutations in FVII deficiency.

Author

Giansily‐Blaizot, M., Kolaitis, N., Borhany, M., Moulis, G., Guillot, O., and Shamsi, T.

Subjects
*LITERATURE reviews, *ALLELES, *HEMOPHILIA, *MOLECULAR biology, *GENETIC polymorphisms
Abstract

The article discusses a literature review on alleles with two mutations in inherited factor VII (FVII) deficiency, and the two new double mutant alleles of the F7 gene. Topics include the molecular analysis of the gene, the common F7 polymorphisms, and the double mutant alleles of disease genes, particularly in small genes, like the F7 gene.

Published
2016
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43. A Haut-Doubs FVII variant depending on species-derivedthromboplastin reagent (F7:p.Arg337His).

Author

Mourey, G., Tachon, G., Pellequer, J.‐L., Zawadzki, C., Trossaërt, M., Bertrand, M.‐A., Schved, J.‐F., and Giansily‐Blaizot, M.

Subjects
THROMBOPLASTIN, HEMOPHILIA, HEMOPHILIACS, CHEMICAL reagents, MEDICAL genetics
Abstract

The article discusses the use of a species-derived thromboplastin reagent when developing a Haut-Doubs congenital factor variant. Topics covered include the inheritance of the reagent through an autosomal recessive manner. Also mentioned is the participation of several patients with haemophilia A in the study.

Published
2014
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44. A digital front-end electronics for the neutron detector NEDA.

Author

Egea, F.J., Houarner, C., Boujrad, A., Gonzalez, V., Tripon, M., Jastrzab, M., Triossi, A., de France, G., Gadea, A., Valiente-Dobon, J.J., Sanchis, E., Barrientos, D., Blaizot, M., Bourgault, P., de Angelis, G., Erduran, M.N., Erturk, S., Huyuk, T., Jaworski, G., and Luo, X.L.

Published
2014
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45. Women with congenital factor VII deficiency: clinical phenotype and treatment options from two international studies

Author

Alberto Dolce, G. Mariani, Gili Kenet, Muriel Giansily-Blaizot, A. Ruiz De Sáez, Guenter Auerswald, Angelika Batorova, J.-F. Schved, Marie-Anne Bertrand, Ampaiwan Chuansumrit, Mehran Karimi, M. N. D. Di Minno, Roya Dolatkhah, Mariasanta Napolitano, Tahir Shamsi, Jørgen Ingerslev, Département d'hématologie biologique[Montpellier], Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-CHU Saint-Eloi, Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Napolitano, M., Di Minno, M., Batorova, A., Dolce, A., Giansily-Blaizot, M., Ingerslev, J., Schved, J., Auerswald, G., Kenet, G., Karimi, M., Shamsi, T., Ruiz de Sáez, A., Dolatkhah, R., Chuansumrit, A., Bertrand, M., Mariani, G., Napolitano, M, DI MINNO, Matteo, Giansily Blaizot, M., Schved, J. F., and Bertrand, M. A.

Subjects
Male, Pediatrics, Factor VII Deficiency, 030204 cardiovascular system & hematology, Cohort Studies, chemistry.chemical_compound, 0302 clinical medicine, Antifibrinolytic agent, gynaecological bleeding, Registries, Child, Genetics (clinical), Aged, 80 and over, Factor VII, Incidence (epidemiology), Hazard ratio, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, Hematology, General Medicine, Middle Aged, Antifibrinolytic Agents, Recombinant Proteins, 3. Good health, Phenotype, Treatment Outcome, Child, Preschool, Cohort, Female, women, inherited factor VII deficiency, recombinant activated factor VII, Cohort study, Adult, medicine.medical_specialty, Adolescent, Mucocutaneous zone, Hemorrhage, Factor VIIa, Young Adult, 03 medical and health sciences, medicine, Humans, Menorrhagia, Aged, Proportional Hazards Models, Coagulants, business.industry, Proportional hazards model, Infant, Surgery, ROC Curve, chemistry, business, 030215 immunology
Abstract

Introduction A paucity of data exists on the incidence, diagnosis and treatment of bleeding in women with inherited factor VII (FVII) deficiency. Aim Here we report results of a comprehensive analysis from two international registries of patients with inherited FVII deficiency, depicting the clinical picture of this disorder in women and describing any gender-related differences. Methods A comprehensive analysis of two fully compatible, international registries of patients with inherited FVII deficiency (International Registry of Factor VII deficiency, IRF7; Seven Treatment Evaluation Registry, STER) was performed. Results In our cohort (N = 449; 215 male, 234 female), the higher prevalence of mucocutaneous bleeds in females strongly predicted ensuing gynaecological bleeding (hazard ratio = 12.8, 95% CI 1.68–97.6, P = 0.014). Menorrhagia was the most prevalent type of bleeding (46.4% of patients), and was the presentation symptom in 12% of cases. Replacement therapies administered were also analysed. For surgical procedures (n = 50), a receiver operator characteristic analysis showed that the minimal first dose of rFVIIa to avoid postsurgical bleeding during the first 24 hours was 22 μg kg−1, and no less than two administrations. Prophylaxis was reported in 25 women with excellent or effective outcomes when performed with a total weekly rFVIIa dose of 90 μg kg−1 (divided as three doses). Conclusion Women with FVII deficiency have a bleeding disorder mainly characterized by mucocutaneous bleeds, which predicts an increased risk of ensuing gynaecological bleeding. Systematic replacement therapy or long-term prophylaxis with rFVIIa may reduce the impact of menorrhagia on the reproductive system, iron loss and may avoid unnecessary hysterectomies.

Published
2016

46. Replacement therapy in inherited factor VII deficiency: occurrence of adverse events and relation with surgery

Author

Angelika Batorova, M. N. D. Di Minno, Kaan Kavakli, G. Mariani, Alberto Dolce, Pimlak Charoenkwan, Muriel Giansily-Blaizot, N B Mirbehbahani, M.-F. López Fernández, Jørgen Ingerslev, Mehran Karimi, Mariasanta Napolitano, Département d'hématologie biologique[Montpellier], Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-CHU Saint-Eloi, Université de Montpellier (UM), Napolitano, M, Dolce, A., Batorova, A., Giansily Blaizot, M., Ingerslev, J., Mirbehbahani, N., DI MINNO, Matteo, Lopez Fernandez, M. F., Karimi, M., Charoenkwan, P., Kavakli, K., Mariani, G., Napolitano, M., Giansily-Blaizot, M., Di Minno, M., and Lopez Fernandez, M.

Subjects
Male, medicine.medical_specialty, Pediatrics, Web of science, Factor VII Deficiency, 030204 cardiovascular system & hematology, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Internal medicine, medicine, Humans, Young adult, Adverse effect, Factor VII deficiency, Child, Adverse events, inherited factor VII deficiency replacement therapy, Genetics (clinical), ComputingMilieux_MISCELLANEOUS, Hematology, business.industry, Infant, Newborn, Infant, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, General Medicine, Middle Aged, Infant newborn, 3. Good health, Surgery, 030220 oncology & carcinogenesis, Child, Preschool, Female, business
Abstract

International audience

Published
2015

47. Digital Front-End Electronics for the Neutron Detector NEDA

Author

F. J. Egea Canet, C. Houarner, A. Boujrad, V. Gonzalez, M. Tripon, M. Jastrzab, A. Triossi, G. de France, A. Gadea, J. J. Valiente-Dobon, D. Barrientos, E. Sanchis, M. Blaizot, P. Bourgault, G. de Angelis, M. N. Erduran, S. Erturk, T. Huyuk, G. Jaworski, X. L. Luo, V. Modamio, M. Moszynski, A. Di Nitto, J. Nyberg, P-A. Soderstrom, M. Palacz, R. Wadsworth, Huyuk, Tayfun -- 0000-0003-0597-9767, Di Nitto, Antonio -- 0000-0002-9319-366X, Jaworski, Grzegorz -- 0000-0003-2241-0329, Triossi, Andrea -- 0000-0001-5140-9154, Gadea, Andres -- 0000-0002-4233-1970, Millan, Vicente Gonzalez -- 0000-0001-6014-2586, Jastrzab, Marcin -- 0000-0003-1457-7540, Soderstrom, Par-Anders -- 0000-0002-9504-2814, Sanchis Peris, Enrique -- 0000-0002-9689-9131, de France, Gilles -- 0000-0002-7439-1759, Barrientos, Diego -- 0000-0001-9693-2942, [Egea Canet, F. J. -- Gonzalez, V. -- Sanchis, E.] Univ Valencia, Dept Elect Engn, Escola Tecn Super Engn, Valencia, Spain -- [Houarner, C. -- Boujrad, A. -- Tripon, M. -- de France, G. -- Blaizot, M. -- Bourgault, P.] GANIL, F-14076 Caen, France -- [Jastrzab, M.] Polish Acad Sci, Niewodniczanski Inst Nucl Phys, Krakow, Poland -- [Triossi, A. -- Valiente-Dobon, J. J. -- Barrientos, D. -- de Angelis, G. -- Modamio, V.] Ist Nazl Fis Nucl, Lab Nazl Legnaro, I-35020 Padua, Italy -- [Gadea, A. -- Hueyuek, T.] Inst Fis Corpuscular CSIC UV, Valencia, Spain -- [Erduran, M. N.] Istanbul Sabahattin Zaim Univ, Fac Engn & Nat Sci, Istanbul, Turkey -- [Erturk, S.] Nigde Univ, Fen Edebiyat Falkultesi, Fiz Bolumu, Nigde, Turkey -- [Jaworski, G.] Warsaw Univ Technol, Fac Phys, Warsaw, Poland -- [Jaworski, G. -- Palacz, M.] Univ Warsaw, Heavy Ion Lab, Warsaw, Poland -- [Luo, X. L.] Natl Univ Def Technol, Dept Instrument Sci & Technol, Changsha, Hunan, Peoples R China -- [Luo, X. L. -- Nyberg, J. -- Soderstrom, P-A.] Uppsala Univ, Dept Phys & Astron, Uppsala, Sweden -- [Moszynski, M.] Natl Ctr Nucl Res, PL-05400 Otwock, Poland -- [Di Nitto, A.] Johannes Gutenberg Univ Mainz, D-55099 Mainz, Germany -- [Soderstrom, P-A.] RIKEN, Nishina Ctr, Wako, Saitama, Japan -- [Wadsworth, R.] Univ York, Dept Phys, York YO10 5DD, N Yorkshire, England, Canet, F. J. E., Houarner, C., Boujrad, A., Gonzalez, V., Tripon, M., Jastrzab, M., Triossi, A., De France, G., Gadea, A., Valiente-Dobon, J. J., Barrientos, D., Sanchis, E., Blaizot, M., Bourgault, P., De Angelis, G., Erduran, M. N., Erturk, S., Huyuk, T., Jaworski, G., Luo, X. L., Modamio, V., Moszynski, M., Di Nitto, A., Nyberg, J., Soderstrom, P. -A., Palacz, M., Wadsworth, R., Grand Accélérateur National d'Ions Lourds (GANIL), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de Physique Nucléaire et de Physique des Particules du CNRS (IN2P3)-Centre National de la Recherche Scientifique (CNRS), and 0-Belirlenecek

Subjects
Nuclear and High Energy Physics, Photomultiplier, front-end electronic, Physics::Instrumentation and Detectors, computer.software_genre, Analog signal processing, Neutron detection, Electronics, [PHYS.PHYS.PHYS-INS-DET]Physics [physics]/Physics [physics]/Instrumentation and Detectors [physics.ins-det], Electrical and Electronic Engineering, Digital electronics, Physics, neutron-gamma discrimination, Firmware, business.industry, neutron detectors, Detector, Electrical engineering, front-end electronics, Nuclear Energy and Engineering, Digital system, AGATA, neutron detector, business, Digital systems, computer
Abstract

19th Real Time Conference (RT) -- MAY 26-30, 2014 -- Nara, JAPAN, WOS: 000356458000029, This paper presents the design of the NEDA (Neutron Detector Array) electronics, a first attempt to involve the use of digital electronics in large neutron detector arrays. Starting from the front-end modules attached to the PMTs (PhotoMultiplier Tubes) and ending up with the data processing workstations, a comprehensive electronic system capable of dealing with the acquisition and pre-processing of the neutron array is detailed. Among the electronic modules required, we emphasize the front-end analog processing, the digitalization, digital pre-processing and communications firmware, as well as the integration of the GTS (Global Trigger and Synchronization) system, already used successfully in AGATA (Advanced Gamma Tracking Array). The NEDA array will be available for measurements in 2016., Osaka Univ, Res Nucl Phys, Generalitat Valenciana, Spain [PROMETEO/2010/101]; INFN, Italy; Spanish MINECO [AIC-D-2011-0746, FPA2011-29854, FPA2012-33650]; Swedish Research Council; Scientific and Technological Research Council of Turkey (TUBITAK); UK STFC; Science and Technology Facilities Council [ST/J000124/1, ST/L005735/1, ST/L005727/1], This work was supported by the Generalitat Valenciana, Spain, under grant PROMETEO/2010/101. Some authors were supported in part by INFN, Italy, and by the Spanish MINECO under grants AIC-D-2011-0746, FPA2011-29854, and FPA2012-33650. The Swedish Research Council, the Scientific and Technological Research Council of Turkey (TUBITAK), and the UK STFC also provided support.

Published
2015

48. A New Front-End High-Resolution Sampling Board for the New-Generation Electronics of EXOGAM2 and NEDA Detectors

Author

M. N. Erduran, Johan Nyberg, X.L. Luo, M. Jastrzab, Diego Barrientos, M. Palacz, V. Modamio, G. de Angelis, P-A Soderstrom, Vicente González, M. Moszynski, A. Boujrad, P. Bourgault, C. Houarner, M. Blaizot, A. Di Nitto, G. Jaworski, F.J. Egea, José-María Blasco, A. Triossi, M. Tripon, J. J. Valiente-Dobón, A. Gadea, T. Hüyük, S. Erturk, R. Wadsworth, Enrique Sanchis, Grand Accélérateur National d'Ions Lourds (GANIL), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de Physique Nucléaire et de Physique des Particules du CNRS (IN2P3)-Centre National de la Recherche Scientifique (CNRS), Egea Canet, F. J., Gonzalez, V., Tripon, M., Jastrzab, M., Triossi, A., Gadea, A., De France, G., Valiente-Dobon, J. J., Barrientos, D., Sanchis, E., Boujrad, A., Houarner, C., Blaizot, M., Bourgault, P., De Angelis, G., Erduran, M. N., Erturk, S., Huyuk, T., Jaworski, G., Luo, X. L., Modamio, V., Moszynski, M., Di Nitto, A., Nyberg, J., Soderstrom, P. -A., Palacz, M., and Wadsworth, R.

Subjects
Physics, Nuclear and High Energy Physics, Spectrometer, Physics::Instrumentation and Detectors, Detector, Bandwidth (signal processing), Acquisition in HP-Ge detectors, high-speed ADCs, Front and back ends, high-speed ADC, Effective number of bits, Acquisition in HP-Ge detector, Nuclear Energy and Engineering, low-noise electronics design, Electronic engineering, Neutron detection, Neutron, Electronics, [PHYS.PHYS.PHYS-INS-DET]Physics [physics]/Physics [physics]/Instrumentation and Detectors [physics.ins-det], Electrical and Electronic Engineering
Abstract

19th Real Time Conference (RT) -- MAY 26-30, 2014 -- Nara, JAPAN WOS: 000356458000028 This paper presents the final design and results of the FADC Mezzanine for the EXOGAM (EXOtic GAMma array spectrometer) and NEDA (Neutron Detector Array) detectors. The measurements performed include those of studying the effective number of bits, the energy resolution using HP-Ge detectors, as well as timing histograms and discrimination performance. Finally, the conclusion shows how a common digitizing device has been integrated in the experimental environment of two very different detectors which combine both low-noise acquisition and fast sampling rates. Not only the integration fulfilled the expected specifications on both systems, but it also showed how a study of synergy between detectors could lead to the reduction of resources and time by applying a common strategy. Osaka Univ, Res Nucl Phys Generalitat Valenciana, Spain [PROMETEO/2010/101]; INFN, Italy; Spanish MINECO [AIC-D-2011-0746, FPA2011-29854, FPA2012-33650]; Swedish Research Council; Scientific and Technological Research Council of Turkey (TUBITAK); UK STFC; Science and Technology Facilities Council [ST/L005735/1, ST/J000124/1, ST/L005727/1] This work was supported by the Generalitat Valenciana, Spain, under grant PROMETEO/2010/101. Some authors were supported in part by INFN, Italy, and by the Spanish MINECO under grants AIC-D-2011-0746, FPA2011-29854 and FPA2012-33650. This work was also supported by the Swedish Research Council, the Scientific and Technological Research Council of Turkey (TUBITAK) and the UK STFC.

Published
2015

49. A new front-end high-resolution sampling board for the new-generation electronics of EXOGAM2 and NEDA detectors

Author

F.J. Egea, V. Gonzalez, M. Tripon, M. Jastrzab, A. Triossi, A. Gadea, G. de France, J.J. Valiente-Dobon, J.M. Blasco, D. Barrientos, E. Sanchis, A. Boujrad, C. Houarner, M. Blaizot, P. Bourgault, G. de Angelis, M.N. Erduran, S. Erturk, T. Huyuk, G. Jaworski, X.L. Luo, V. Modamio, M. Moszynski, A. Di Nitto, J. Nyberg, P-A. Soderstrom, M. Palacz, R. Wadsworth, Triossi, Andrea -- 0000-0001-5140-9154, Di Nitto, Antonio -- 0000-0002-9319-366X, Huyuk, Tayfun -- 0000-0003-0597-9767, and [Egea, F. J. -- Gonzalez, V. -- Sanchis, E.] Univ Valencia, Dept Elect Engn, Escola Tecn Super Engn, Burjassot, Valencia, Spain -- [Gadea, A. -- Barrientos, D. -- Huyuk, T.] Inst Fis Corpuscular CSIC UV, Valencia, Spain -- [Tripon, M. -- de France, G. -- Boujrad, A. -- Houarner, C. -- Blaizot, M. -- Bourgault, P.] GANIL, F-14706 Caen, France -- [Jastrzab, M.] Polish Acad Sci, Niewodniczanski Inst Nucl Phys, Krakow, Poland -- [Triossi, A. -- Valiente-Dobon, J. J. -- Barrientos, D. -- de Angelis, G. -- Modamio, V.] Ist Nazl Fis Nucl, Lab Nazl Legnaro, IT-35020 Padua, Italy -- [Jaworski, G.] Warsaw Univ Technol, Fac Phys, Warsaw, Poland -- [Jaworski, G. -- Moszynski, M. -- Palacz, M.] Univ Warsaw, Heavy Ion Lab, Warsaw, Poland -- [Luo, X. L. -- Nyberg, J. -- Soderstrom, P-A.] Uppsala Univ, Dept Phys & Astron, Uppsala, Sweden -- [Moszynski, M.] Natl Ctr Nucl Res, PL-05400 Otwock, Poland -- [Luo, X. L.] Natl Univ Def Technol, Dept Instrument Sci & Technol, Changsha, Hunan, Peoples R China -- [Soderstrom, P-A.] RIKEN Nishina Ctr, Wako, Saitama, Japan -- [Di Nitto, A.] Johannes Gutenberg Univ Mainz, D-55099 Mainz, Germany -- [Erduran, M. N.] Istanbul Sabahattin Zaim Univ, Fac Engn & Nat Sci, Istanbul, Turkey -- [Erturk, S.] Nigde Univ, Fen Edebiyat Falkultesi, Fiz Bolumu, Nigde, Turkey -- [Wadsworth, R.] Univ York, Dept Phys, York, N Yorkshire, England

Abstract

19th IEEE-NPSS Real Time Conference (RT) -- MAY 26-30, 2014 -- Nara, JAPAN, WOS: 000380588000136, The present paper aims to present the final design and results of the FADC Mezzanine for EXOGAM and NEDA detectors, focusing on the resolution performance. Finally, the conclusion shows how a common digitizing device has been integrated in an experimental environment involving low-noise and fast sampling rates. Not only the integration fulfilled the expected specifications on both systems, but also, it shows how a study of synergy between detectors has led to a reduction of resources and time by applying a common strategy., Osaka Univ, Res Nucl Phys, IEEE Nucl & Plasma Sci Soc

Published
2014

50. Design and Test of a High-speed Flash ADC Mezzanine Card for High-resolution and Timing Performance in Nuclear Structure Experiments

Author

X. Egea, E. Sanchis, V. Gonzalez, A. Gadea, J.M. Blasco, D. Barrientos, J.J. Valiente Dobon, M. Tripon, A. Boujrad, C. Houarner, M. Jastrzab, M. Blaizot, P. Bourgault, G. de Angelis, M.N. Erduran, S. Erturk, G. de France, T. Huyuk, G. Jaworski, A. di Nitto, J. Nyberg, M. Palacz, A. Pipidis, R. Tarnowski, A. Triossi, R. Wadsworth, Grand Accélérateur National d'Ions Lourds (GANIL), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de Physique Nucléaire et de Physique des Particules du CNRS (IN2P3)-Centre National de la Recherche Scientifique (CNRS), EXOGAM, Egea, X., Sanchis, E., Gonzalez, V., Gadea, A., Blasco, J. M., Barrientos, D., Valiente Dobon, J. J., Tripon, M., Boujrad, A., Houarner, C., Jastrzab, M., Blaizot, M., Bourgault, P., De Angelis, G., Erduran, M. N., Erturk, S., De France, G., Huyuk, T., Jaworski, G., DI NITTO, Antonio, Nyberg, J., Palacz, M., Pipidis, A., Tarnowski, R., Triossi, A., and Wadsworth, R.

Subjects
Computer science, business.industry, Physics::Instrumentation and Detectors, High resolution, [PHYS.PHYS.PHYS-INS-DET]Physics [physics]/Physics [physics]/Instrumentation and Detectors [physics.ins-det], Flash ADC, business, Computer hardware, Test (assessment)
Abstract

International audience; This board will be part of the upgrade for the new electronics for the EXOGAM2 (HP-Ge detector array) and NEDA (BC501A-based neutron detector array), therefore it was necessary to deal with the problem of providing a sampling card with high resolution for new gamma spectroscopy experiments while sampling at very high rates, with a broad bandwidth in order to preserve the shape for further analysis. Pulse shape analysis is of paramount importance in neutron detectors, such as NEDA, based on scintillators that are sensitive to γ-rays as well. High resolution and high speed are often two parameters which conform a trade-off and it is hard to achieve both simultaneously. The aforementioned constraints and the urge of building new sampling electronics to improve the signal analysis in nuclear physics experiments, led to the development of this FADC mezzanine This involves sampling rates up to 250 Msps preserving a high resolution of 11.3 effective bits in order to satisfy the experiment demands. In this work is described the design and the test bench proposed for a proper high speed ADC characterization system and the results obtained up to now.

Published
2012

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