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2. New results on focusing of gamma-rays with Laue lenses

Author

Ferrari, F., Frontera, F., Loffredo, G., Virgilli, E., Guidorzi, C., Carassiti, V., Evangelisti, F., Landi, L., Chiozzi, S., Squerzanti, S., Caroli, E., Stephen, J. B., Schiavone, F., Basili, A., Andersen, K. H., and Courtois, P.

Subjects
Astrophysics - Instrumentation and Methods for Astrophysics
Abstract

We report on new results on the development activity of broad band Laue lenses for hard X-/gamma-ray astronomy (70/100-600 keV). After the development of a first prototype, whose performance was presented at the SPIE conference on Astronomical Telescopes held last year in Marseille (Frontera et al. 2008), we have improved the lens assembling technology. We present the development status of the new lens prototype that is on the way to be assembled., Comment: 8 pages, 11 figures, to be Published in SPIE Proceedings, vol.7437-19, 2009

Published
2009
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3. Focusing of gamma-rays with Laue lenses: first results

Author

Frontera, F., Loffredo, G., Pisa, A., Nobili, F., Carassiti, V., Evangelisti, F., Landi, L., Squerzanti, S., Caroli, E., Stephen, J. B., Andersen, K. H., Courtois, P., Auricchio, N., Milani, L., and Negri, B.

Subjects
Astrophysics, Physics - Instrumentation and Detectors
Abstract

We report on the first results obtained from our development project of focusing gamma-rays ($>$60 keV) by using Laue lenses. The first lens prototype model has been assembled and tested. We describe the technique adopted and the lens focusing capabilities at about 100 keV., Comment: 8 pages 9 figures, to be published in SPIE Procs. 7011, 2008

Published
2008
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4. Development status of a Laue lens project for gamma-ray astronomy

Author

Frontera, F., Loffredo, G., Pisa, A., Milani, L., Nobili, F., Auricchio, N., Carassiti, V., Evangelisti, F., Landi, L., Squerzanti, S., Andersen, K. H., Courtois, P., Amati, L., Caroli, E., Landini, G., Silvestri, S., Stephen, J. B., Poulsen, J. M., Negri, B., and Pareschi, G.

Subjects
Astrophysics
Abstract

We report the status of the HAXTEL project, devoted to perform a design study and the development of a Laue lens prototype. After a summary of the major results of the design study, the approach adopted to develop a Demonstration Model of a Laue lens is discussed, the set up described, and some results presented., Comment: 11 pages, 11 figures, 2007 SPIE Conference on Optics for EUV, X-Ray, and Gamma-Ray Astronomy III

Published
2007
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5. Gamma-ray lens development status for a European Gamma-Ray Imager

Author

Frontera, F., Pisa, A., Carassiti, V., Evangelisti, F., Loffredo, G., Pellicciotta, D., Andersen, K. H., Courtois, P., Amati, L., Caroli, E., Franceschini, T., Landini, G., Silvestri, S., and Stephen, J. B.

Subjects
Astrophysics
Abstract

A breakthrough in the sensitivity level of the hard X-/gamma-ray telescopes, which today are based on detectors that view the sky through (or not) coded masks, is expected when focusing optics will be available also in this energy range. Focusing techniques are now in an advanced stage of development. To date the most efficient technique to focus hard X-rays with energies above 100 keV appears to be the Bragg diffraction from crystals in transmission configuration (Laue lenses). Crystals with mosaic structure appear to be the most suitable to build a Laue lens with a broad passband, even though other alternative structures are being investigated. The goal of our project is the development of a broad band focusing telescope based on gamma-ray lenses for the study of the continuum emission of celestial sources from 60 keV up to >600 keV. We will report details of our project, its development status and results of our assessment study of a lens configuration for the European Gamma Ray Imager (GRI) mission now under study for the ESA plan "Cosmic Vision 2015-2025"., Comment: 12 pages, 12 figures

Published
2006
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6. Laue Lens Development for Hard X-rays (>60 keV)

Author

Pellicciotta, D., Frontera, F., Loffredo, G., Pisa, A., Andersen, K., Courtois, P., Hamelin, B., Carassiti, V., Melchiorri, M., and Squerzanti, S.

Subjects
Astrophysics
Abstract

Results of reflectivity measurements of mosaic crystal samples of Cu (111) are reported. These tests were performed in the context of a feasibility study of a hard X-ray focusing telescope for space astronomy with energy passband from 60 to 600 keV. The technique envisaged is that of using mosaic crystals in transmission configuration that diffract X-rays for Bragg diffraction (Laue lens). The Laue lens assumed has a spherical shape with focal length $f$. It is made of flat mosaic crystal tiles suitably positioned in the lens. The samples were grown and worked for this project at the Institute Laue-Langevin (ILL) in Grenoble (France), while the reflectivity tests were performed at the X-ray facility of the Physics Department of the University of Ferrara., Comment: 6 pages, 12 figures, accepted for publication in IEEE Transactions on Nuclear Science

Published
2005
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7. Exploring the Hard X-/soft gamma-ray Continuum Spectra with Laue Lenses

Author

Frontera, F., Pisa, A., De Chiara, P., Loffredo, G., Pellicciotta, D., Carassiti, V., Evangelisti, F., Andersen, K., Courtois, P., Hamelin, B., Amati, L., Auricchio, N., Bassani, L., Caroli, E., Landini, G., Orlandini, M., Stephen, J. B., Comastri, A., Knodlseder, J., and von Ballmoos, P.

Subjects
Astrophysics
Abstract

The history of X-ray astronomy has shown that any advancement in our knowledge of the X-ray sky is strictly related to an increase in instrument sensitivity. At energies above 60 keV, there are interesting prospects for greatly improving the limiting sensitivity of the current generation of direct viewing telescopes (with or without coded masks), offered by the use of Laue lenses. We will discuss below the development status of a Hard X-Ray focusing Telescope (HAXTEL) based on Laue lenses with a broad bandpass (from 60 to 600 keV) for the study of the X-ray continuum of celestial sources. We show two examplesof multi-lens configurations with expected sensitivity orders of magnitude better ($\sim 1 \times 10^{-8}$ photons cm$^{-2}$ s$^{-1}$ keV$^{-1}$ at 200 keV) than that achieved so far. With this unprecedented sensitivity, very exciting astrophysical prospects are opened., Comment: 4 pages, 10 figures, to be published in the Proc. of the 39th ESLAB Symosium, 19-21 April 2005

Published
2005

8. Feasibility study of a Laue lens for hard X-rays for space astronomy

Author

Pisa, A., Frontera, F., De Chiara, P., Loffredo, G., Pellicciotta, D., Landini, G., Franceschini, T., Silvestri, S., Andersen, K., Courtois, P., and Hamelin, B.

Subjects
Astrophysics, Astrophysics - Instrumentation and Methods for Astrophysics
Abstract

We report on the feasibility study of a Laue lens for hard X-rays (>60 keV)based on mosaic crystals, for astrophysical applications. In particular we discuss the scientific motivations, its functioning principle, the procedure followed to select the suitable crystal materials, the criteria adopted to establish crystal dimensions and their distribution on the lens in order to obtain the best lens focusing capabilities, and the criteria for optimizing the lens effective area in a given passband. We also discuss the effects of misalignments of the crystal tiles due to unavoidable mechanical errors in assembling the lens. A software was developed to face all these topics and to evaluate the expected lens performance., Comment: 10 pages, corrected Fig. 1b and Fig. 2, which are wrong in the published version, corrected typos

Published
2004
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9. Immunochip analysis identifies novel susceptibility loci in the human leukocyte antigen region for acquired thrombotic thrombocytopenic purpura

Author

Mancini, I., Ricaño‐Ponce, I., Pappalardo, E., Cairo, A., Gorski, M.M., Casoli, G., Ferrari, B., Alberti, M., Mikovic, D., Noris, M., Wijmenga, C., Peyvandi, F., Rinaldi, E., Melpignano, A., Campus, S., Podda, R.A., Caria, C., Caddori, A., Di Francesco, E., Giuffrida, G., Agostini, V., Roncarati, U., Mannarella, C., Fragasso, A., Podda, G.M., Bertinato, E., Cerbone, A.M., Tufano, A., Loffredo, G., Poggi, V., Pizzuti, M., Re, G., Ronchi, M., Codeluppi, K., Facchini, L., De Fanti, A., Amarri, S., Trisolini, S.M., Capria, S., Aprile, L., Defina, M., and Cerù, S.

Published
2016
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11. Needs and Challenges of Daily Life for People with Down Syndrome Residing in the City of Rome, Italy

Author

Bertoli, M., Biasini, G., Calignano, M. T., Celani, G., De Grossi, G., Digilio, M. C., Fermariello, C. C., Loffredo, G., Luchino, F., Marchese, A., Mazotti, S., Menghi, B., Razzano, C., Tiano, C., Zambon Hobart, A., Zampino, G., and Zuccala, G.

Abstract

Background: Population-based surveys on the quality of life of people with Down syndrome (DS) are difficult to perform because of ethical and legal policies regarding privacy and confidential information, but they are essential for service planning. Little is known about the sample size and variability of quality of life of people with DS living in the city of Rome, which has a population of 2.7 million inhabitants. The aim of the present study is to explore the needs and challenges in health, social integration and daily life, of people with DS living in Rome. Methodology: A cross-sectional, census-based survey was conducted in 2006. All family doctors (3016 in total) of the National Health Service were involved by the Statistical Bureau of the Municipality of Rome. As per the census, every resident citizen is registered with a family doctor and every person with disabilities is coded. Associations for Down Syndrome encouraged their members to participate in the research. Questionnaires were completed by families of people with DS, in accordance with privacy laws. Findings: An initial survey, conducted via a letter and a telephone contact with family doctors, identified 884 people with DS residing in the city of Rome. Data on the medical and social conditions of 518 people with DS, ranging in age from 0 to 64 years, were collected. Some 88% of these were living with their original family; 82.1% had one or more siblings, and 19.5% had lost one or both parents. A full 100% of children with DS were enrolled in the public school system. This ensures that they are fully occupied and entirely integrated in society. After secondary school there is a lack of opportunities. Thus, only 10% of adults were working with a regular contract. A mere 42.2% of people with DS aged 25-30 were involved in some form of regular activity (although not always on a daily basis). After the age of 30, the percentage of people demonstrating decline in function increased sharply, while disability-related support decreased. In other words, as people with DS age, daily life evolves increasingly around the home, with only occasional outdoor activities. Conclusion: The health, employment and social needs of the majority of people with DS in the city of Rome are not being met. The findings of this study underscore the urgent need for more comprehensive inclusion in society of adults with DS and for the provision of support services to create an enabling environment for inclusion. Because of the variability of performance among individuals with DS, there is a need to create more case-specific options in terms of work, living arrangements, social networking and medical services. Schooling and social inclusion in childhood alone do not guarantee a satisfactory quality of life in adulthood. It is argued herewith that policy of inclusion and support should extend over the entire lifetime of people with DS.

Published
2011
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13. The features of acquired thrombotic thrombocytopenic purpura occurring at advanced age

Author

Agosti, P, Mancini, I, Artoni, A, Ferrari, B, Pontiggia, S, Trisolini, Sm, Facchini, L, Peyvandi, F, Capria, S, Codeluppi, K, Rinaldi, E, Pastore, D, Campus, S, Podda, Ra, Caria, C, Caddori, A, Nicolosi, D, Giuffrida, G, Agostini, V, Roncarati, U, Mannarella, C, Fragasso, A, Podda, Gm, Birocchi, S, Cerbone, Am, Tufano, A, Loffredo, G, Menna, G, Pizzuti, M, Ronchi, M, De Fanti, A, Amarri, S, Defina, M, Bocchia, M, Ceru, S, Gattillo, S, Agosti, P., Mancini I., Artoni A., Ferrari B., Pontiggia S., Trisolini S. M., Facchini L., Peyvandi F., and Tufano, A.

Subjects
medicine.medical_specialty, Thrombotic microangiopathy, ADAMTS13 Protein, Hemorrhage, Disease, 030204 cardiovascular system & hematology, Kidney, Adamts13 activity, 03 medical and health sciences, 0302 clinical medicine, Elderly, Older patients, Internal medicine, medicine, Humans, Registries, Acute thrombotic thrombocytopenic purpura management, ADAMTS13, Rare disease, Aged, Acquired Thrombotic Thrombocytopenic Purpura, Purpura, Thrombotic Thrombocytopenic, business.industry, Hematology, Microangiopathic hemolytic anemia, medicine.disease, Cross-Sectional Studies, 030220 oncology & carcinogenesis, Female, business
Abstract

Introduction: Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare life-threatening thrombotic microangiopathy (TMA) affecting more frequently women of 30–50 years of age. There is scarce information on the clinical features of aTTP occurring in the elderly. Our goal was to evaluate the impact of an elderly-onset disease on the expression, severity and management of aTTP. Materials and methods: We performed a cross-sectional study of patients enrolled in the Milan TTP Registry (www.ttpdatabase.org) after a first acute episode of aTTP from January 2002 to March 2018. The aTTP diagnosis was suspected on the basis of the presence of thrombocytopenia and microangiopathic hemolytic anemia with no alternative causes, and was confirmed centrally by a severe plasma deficiency of ADAMTS13 activity (

Published
2020

14. PediaVirus chatline: all together against COVID-19

Author

Iafusco, M, Ciampa, C, De Maddi, F, Palamone, G, Quarantiello, F, De Luca, G, Iannello, C, Pisano, S, Nocerino, A, Russo, R, Orlando, F, Iafusco, D, Aiello, A, Amorosi, W, Arena, S, Aschettino, M, Basile, P, Battista, A, Bozza, L, Cafiero, M, Caldore, M, Capuano, V, Carbone, Mg, Carbone, V, Casaburo, F, Cavaliere, P, Cerciello, L, Cervone De Martino, M, Cesiro, G, Ciampa, L, Ciao, C, Cimaduomo, L, Cioffi, L, Cirillo, C, Confetto, S, Coppola, G, Crisci, A, Cuzzolin, M, D’Avino, A, De Rosa, I, De Rosa, S, De Siena, M, Del Giudice, G, Del Zotti, F, Della Rotonda, G, D’Errico, U, Di Benedetto, L, Di Prisco, A, Di Stasio, F, Dinardo, M, Donadio, P, Emiliano, M, Esposito, A, Esposito, C, Esposito, L, Esposito, Sl, Fabiani, I, Federico, A, Ferrara, D, Ferraro, E, Filippi, A, Fiorinastro, F, Foria, G, Fusco, A, Gambardella, A, Gicchino, Mf, Giuliano, M, Grano, S, Greco, P, Iacono, A, Iafusco, F, Iannello, G, Iervolino, C, Illiano, G, Imperatore, C, Indolfi, C, Indolfi, P, Iovino, A, Iuliano, R, Kosova, P, Lieto, A, Liotta, L, Lisbino, M, Lodato, G, Loffredo, G, Maida, W, Maione, R, Maiorino, C, Marigliano, Ae, Marinelli, G, Marrone, G, Minervino, G, Napoli, M, Nappo, V, Narciso, V, Nardo, S, Noviello, D, Opallo, A, Orbinato, F, Ottaviano, C, Pace, M, Palma, R, Palmentieri, P, Palmieri, L, Palmieri, S, Parrella, G, Parrotta, G, Pellegrini, F, Peluso, C, Pesole, F, Petito, A, Pezzolla, L, Pignatelli, A, Pinto, L, Piroli, C, Piscopo, A, Pullano, F, Raineri, L, Rigante, Donato, Rivezzi, G, Rocco, C, Rocco, D, Rostan, E, Russo, N, Sannino, C, Sarno, C, Savanelli, L, Serra, S, Sibilio, M, Spera, M, Speranza, P, Stagni, A, Stanco, A, Torino, M, Uccello, A, Ummarino, M, Ummarino, D, Viano, V, Vigorita, S, Villani, S, Viola, R, Vitale, A, Vitiello, R, Vuillemieur, P, and Zanfardino, A

Subjects
Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Covid-19
Published
2020

16. Laue lens development for hard X-rays (>60 keV)

Author

Pellicciotta, D., Frontera, F., Loffredo, G., Pisa, A., Andersen, K., Courtois, P., Hamelin, B., Carassiti, V., Melchiorri, M., and Squerzanti, S.

Subjects
X-ray telescope -- Equipment and supplies, Lenses -- Optical properties, Lenses -- Analysis, Business, Electronics, Electronics and electrical industries
Abstract

Results of reflectivity measurements of mosaic crystal samples of Cu (111) are reported. These tests were performed in the context of a feasibility study of a hard X-ray focusing telescope for space astronomy based on mosaic crystals in transmission configuration (Laue lens). The Laue lens assumed has the shape of a sphere segment with radius R equal to 2 times the focal length f of the lens and is made of flat mosaic crystal tiles properly positioned in the lens. The goal is to develop lenses for astrophysical applications with broad energy passband from 60 to 600 keV. The samples were grown and worked for this project at the Institute Laue-Langevin (ILL) in Grenoble, France, while the reflectivity tests were performed at the X-ray facility of the Physics Department of the University of Ferrara. Index Terms--Astronomy, Bragg, crystals, Laue, telescope, X-ray.

Published
2006

20. MYH9-Related Disease: A Novel Prognostic Model to Predict the Clinical Evolution of the Disease Based on Genotype-Phenotype Correlations

Author

Pecci, A, Klersy, C, Gresele, P, Lee, K, De Rocco, D, Bozzi, V, Russo, G, Heller, Pg, Loffredo, G, Ballmaier, M, Fabris, F, Beggiato, E, Kahr, Wha, Pujol-Moix, N, Platokouki, H, Matthijs, G, Noris, P, Yerram, P, Hermans, C, Batzios, S, De Groot, M, Zieger, B, De Candia, E, Fraticelli, V, Kersseboom, R, Piccoli, Gb, Zimmermann, S, Zaninetti, C, Nicchia, E, Baronci, C, Seri, M, Knight, Pj, Balduini, Cl, Savoia, A, Van Geet, C, Geber, B, Economou, M, Fierro, T, Glembotsky, Ac, Vianello, F, Guthner, C., Pecci, A, Klersy, C, Gresele, P, Lee, Kj, De Rocco, D, Bozzi, V, Russo, G, Heller, Pg, Loffredo, G, Ballmaier, M, Fabris, F, Beggiato, E, Kahr, Wh, Pujol-Moix, N, Platokouki, H, Van Geet, C, Noris, P, Yerram, P, Hermans, C, Gerber, B, Economou, M, De Groot, M, Zieger, B, De Candia, E, Fraticelli, V, Kersseboom, R, Piccoli, Gb, Zimmermann, S, Fierro, T, Glembotsky, Ac, Vianello, F, Zaninetti, C, Nicchia, E, Güthner, C, Baronci, C, Seri, M, Knight, Pj, Balduini, Cl, Savoia, A., DE ROCCO, Daniela, Pujol Moix, N, Nicchia, Elena, Savoia, Anna, and University of Zurich

Subjects
Male, Oncology, thrombocytopenia, Medicina Clínica, Disease, Malattia MYH9 associata, Myh9 related disease, MYH9 RELATED DISEASE, Myh9, MYH9, Risk Factors, purl.org/becyt/ford/3.2 [https], Genotype, Genetics(clinical), CRYSTAL-STRUCTURE, Age of Onset, IIA, Genetics (clinical), deafne, MYOSIN HEAVY-CHAIN, EPSTEIN-SYNDROME, Molecular Motor Proteins, FECHTNER SYNDROMES, Trombocitopenia, Penetrance, POWER STROKE STATE, Phenotype, Italy, nephropathy, Female, purl.org/becyt/ford/3 [https], Miosina No Muscular Iia, Adult, 2716 Genetics (clinical), medicine.medical_specialty, CIENCIAS MÉDICAS Y DE LA SALUD, MOTOR DOMAIN, Hearing Loss, Sensorineural, Genetic counseling, 610 Medicine & health, Biology, nonmuscle myosin, Article, Cataract, Nephropathy, 1311 Genetics, Internal medicine, deafness, Genetics, medicine, Humans, Hematología, Gene, Genetic Association Studies, Hereditaria, Myosin Heavy Chains, MUTATIONS, Settore MED/09 - MEDICINA INTERNA, medicine.disease, Amino Acid Substitution, Epstein Syndrome, Mutation, 10032 Clinic for Oncology and Hematology, Linear Models, SMOOTH-MUSCLE MYOSIN
Abstract

MYH9-related disease (MYH9-RD) is a rare autosomal-dominant disorder caused by mutations in the gene for nonmuscle myosin heavy chain IIA (NMMHC-IIA). MYH9-RD is characterized by a considerable variability in clinical evolution: patients present at birth with only thrombocytopenia, but some of them subsequently develop sensorineural deafness, cataract, and/or nephropathy often leading to end-stage renal disease (ESRD). We searched for genotype–phenotype correlations in the largest series of consecutive MYH9-RD patients collected so far (255 cases from 121 families). Association of genotypes with noncongenital features was assessed by a generalized linear regression model. The analysis defined disease evolution associated to seven different MYH9 genotypes that are responsible for 85% of MYH9-RD cases. Mutations hitting residue R702 demonstrated a complete penetrance for early-onset ESRD and deafness. The p.D1424H substitution associated with high risk of developing all the noncongenital manifestations of disease. Mutations hitting a distinct hydrophobic seam in the NMMHC-IIA head domain or substitutions at R1165 associated with high risk of deafness but low risk of nephropathy or cataract. Patients with p.E1841K, p.D1424N, and C-terminal deletions had low risk of noncongenital defects. These findings are essential to patients' clinical management and genetic counseling and are discussed in view of molecular pathogenesis of MYH9-RD. Fil: Pecci, Alessandro. University of Pavia; Italia Fil: Klersy, Catherine. IRCCS Policlinico San Matteo Foundation; Italia Fil: Gresele, Paolo. Università di Perugia; Italia Fil: Lee, Kieran J. D.. University of Leeds; Reino Unido Fil: De Rocco, Daniela. Università degli Studi di Trieste; Italia Fil: Bozzi, Valeria. University of Pavia; Italia Fil: Russo, Giovanna. University of Catania; Italia Fil: Heller, Paula Graciela. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Médicas. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Médicas; Argentina Fil: Loffredo, Giuseppe. Pausilipon Hospital. Department of Oncology; Italia Fil: Ballmaier, Matthias. Hannover Medical School; Alemania Fil: Fabris, Fabrizio. Università di Padova; Italia Fil: Beggiato, Eloise. Hospital “Città della Salute e Della Scienza”; Italia Fil: Kahr, Walter H. A.. University of Toronto; Canadá. Hospital for Sick Children. Division of Hematology/Oncology; Canadá Fil: Pujol Moix, Nuria. Universitat Autònoma de Barcelona; España Fil: Platokouki, Helen. “Aghia Sophia” Children's Hospital; Grecia Fil: Van Geet, Christel. University of Leuven. Center for Molecular and Vascular Biology; Bélgica Fil: Noris, Patrizia. University of Pavia; Italia Fil: Yerram, Preethi. University of Missouri; Estados Unidos Fil: Hermans, Cedric. St-Luc University Hospital; Bélgica Fil: Gerber, Bernhard. University Hospital Zurich, Division of Hematology; Suiza Fil: Economou, Marina. Aristotle University; Grecia Fil: De Groot, Marco. University of Groningen; Países Bajos Fil: Zieger, Barbara. University Medical Center Freiburg; Alemania Fil: De Candia, Erica. Catholic University of Rome; Italia Fil: Fraticelli, Vincenzo. Giovanni Paolo II Foundation; Italia Fil: Kersseboom, Rogier. Erasmus Medical Centre; Países Bajos Fil: Piccoli, Giorgina B.. Università di Torino; Italia Fil: Zimmermann, Stefanie. Goethe Universitat Frankfurt; Alemania Fil: Fierro, Tiziana. Università di Perugia; Italia Fil: Glembotsky, Ana Claudia. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Médicas. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Médicas; Argentina Fil: Vianello, Fabrizio. Università di Padova; Italia Fil: Zaninetti, Carlo. University of Pavia; Italia Fil: Nicchia, Elena. Università degli Studi di Trieste; Italia Fil: Güthner, Christiane. Stadtspital Triemli. Department of Medical Oncology and Hematology; Italia Fil: Baronci, Carlo. Pediatric Hospital "Bambino Gesù"; Italia Fil: Seri, Marco. Università di Bologna; Italia Fil: Knight, Peter J.. University of Leeds; Reino Unido Fil: Balduini, Carlo L.. University of Pavia; Italia Fil: Savoia, Anna. Università degli Studi di Trieste; Italia

Published
2014

21. Alteration of liver enzymes is a feature of the MYH9-related disease syndrome

Author

Pecci A, Biino G, Fierro T, Bozzi V, Mezzasoma A, Patrizia Noris, Ramenghi U, Loffredo G, Fabris F, Momi S, Magrini U, Pirastu M, Savoia A, Balduini C, Gresele P, Myh, Italian Registry For -Releated Diseases, Pecci, A., Biino, G., Fierro, T., Bozzi, V., Mezzasoma, A., Noris, P., Ramenghi, U., Loffredo, G., Fabris, F., Momi, S., Magrini, U., Pirastu, M., Savoia, Anna, Balduini, C., Gresele, P., and Italian Registry for MYH9 releated, Diseases

Subjects
MYH9, Thrombocytopenia, Male, Pathology, MYH9 Mutations, Anatomy and Physiology, Biopsy, lcsh:Medicine, Disease, Biochemistry, Liver Function Tests, may-heggly, Odds Ratio, lcsh:Science, IIA, Child, inherited thrombocytopenias, Aged, 80 and over, Multidisciplinary, medicine.diagnostic_test, Molecular Motor Proteins, Liver Diseases, Fatty liver, fechtner-syndrome, myosin heavy chain, diagnostic algorithm, epstein-syndrome, cell motility, Macrothrombocytopenia, Syndrome, Hematology, Middle Aged, Immunohistochemistry, Malattia MYH9 correlata, Enzymes, Liver, Autosomal Dominant, Child, Preschool, Medicine, Female, Research Article, Adult, Platelets, medicine.medical_specialty, Adolescent, Gastroenterology and Hepatology, Neurological System, Nephropathy, Enzyme Regulation, Young Adult, medicine, Humans, Clinical significance, Abnormalities, Multiple, Biology, Aged, Demography, Clinical Genetics, Myosin Heavy Chains, business.industry, lcsh:R, Case-control study, Infant, Odds ratio, medicine.disease, Case-Control Studies, Mutation, lcsh:Q, business, Liver function tests, Follow-Up Studies, Neuroscience
Abstract

BACKGROUND: MYH9-related disease (MYH9-RD) is a rare autosomal dominant genetic syndrome characterized by congenital thrombocytopenia associated with the risk of developing progressive nephropathy, sensorineural deafness, and presenile cataract. During the collection of a large case-series of patients with MYH9-RD we noticed several cases with unexplained elevation of liver enzymes. Our aim was to evaluate if the alteration of liver tests is a feature of the MYH9-RD and to define its clinical significance. METHODS AND FINDINGS: Data concerning liver tests, prospectively recorded in the Italian Registry for MYH9-RD, were collected and compared with those of three control populations: patients with autoimmune thrombocytopenia, patients with inherited thrombocytopenias other than MYH9-RD, and the participants to a large epidemiologic survey in an Italian geographic isolate. Thirty-eight of 75 evaluable MYH9-RD patients (50.7%) showed an elevation of ALT and/or AST, and 17 of 63 (27.0%) an increase of GGT. The increases ranged from 1.9 ± 0.7 to 2.7 ± 1.6 fold the upper normal limit. The prevalence of liver test alterations was significantly higher in MYH9-RD patients than in each of the control populations, with odds ratios ranging from 8.2 (95% CIs 2.2-44.8) to 24.7 (14.8-40.8). Clinical follow-up and more detailed liver studies of a subset of patients, including ultrasound liver scan, liver elastography and liver biopsy in one case, did not show any significant structural damage or evolution towards liver insufficiency. CONCLUSIONS: Elevation of liver enzymes is a frequent and previously unrecognized feature of the MYH9-RD syndrome; however, this defect does not appear to have poor prognostic value.

Published
2012

23. Effects of R216Q mutation of GATA-1 on erythropoisis and platelet production

Author

Balduini, Cl, Pecci, A, Loffredo, G, Izzo, P, Noris, P, Grosso, M, Bergamaschi, G, Rosti, V, Magrini, U, Ceresa, If, Conti, V, Poggi, V, Savoia, Anna, Balduini, Cl, Pecci, A, Loffredo, G, Izzo, P, Noris, P, Grosso, M, Bergamaschi, G, Rosti, V, Magrini, U, Ceresa, If, Conti, V, Poggi, V, and Savoia, Anna

Published
2004

24. Platelet size for distinguishing between inherited thrombocytopenias and immune thrombocytopenia: A multicentric, real life study

Author

Patrizia Noris, Klersy C, Gresele P, Giona F, Giordano P, Minuz P, Loffredo G, Pecci A, Melazzini F, Civaschi E, Mezzasoma A, Piedimonte M, Semeraro F, Veneri D, Menna F, Ciardelli L, Cl, Balduini, and Italian Gruppo di Studio delle Piastrine

Subjects
Adult, Blood Platelets, Male, medicine.medical_specialty, Immune thrombocytopenia, Inherited thrombocytopenias, Platelet diameter, Platelet size, Platelet volume, Adolescent, Diagnostic accuracy, Gastroenterology, Sensitivity and Specificity, Diagnosis, Differential, Young Adult, Reference Values, hemic and lymphatic diseases, Internal medicine, Diagnosis, medicine, Humans, Platelet, Mean platelet volume, Aged, Female, Middle Aged, Platelet Count, Purpura, Thrombocytopenic, Idiopathic, Thrombocytopenia, Cell Size, Purpura, business.industry, Platelets, Haemostasis and Thrombosis, Hematology, Idiopathic, Peripheral blood, Thrombocytopenic, Immunology, Differential, Differential diagnosis, business, Life study
Abstract

The most frequent forms of inherited thrombocytopenia (IT) are characterized by platelet size abnormalities and it has been suggested that this parameter is useful for their differentiation from immune thrombocytopenia (ITP). Recently, a monocentric study identified cut-off values for mean platelet volume (MPV) and mean platelet diameter (MPD) with good diagnostic accuracy in this respect. To validate these cut-off values in a different and larger case series of patients, we enrolled 130 subjects with ITP and 113 with IT in six different centres. The platelet count and MPV was each measured by the instrument routinely used in each institution. In some centres, platelet count was also measured by optical microscopy. MPD was evaluated centrally by image analysis of peripheral blood films. The previously identified cut-off value for MPV had 91% specificity in distinguishing ITP from inherited macrothrombocytopenias (mono and biallelic Bernard-Soulier, MYH9-related disease), while its sensitivity was greatly variable depending on the instrument used. With an appropriate instrument, specificity was 83%. The diagnostic accuracy of MPD was lower than that obtained with MPV. We concluded that MPV is a useful parameter for differentiating ITP from IT provided that it is measured by appropriate cell counters.

Published
2013

25. Clinical and Laboratory Features of of 103 Patients From 42 Italian Families with Inherited Thrimbocytopenia Derived from the Monoallelic Ala156Val Mutation of GPIb Alpha (Bolzano Mutation)

Author

Balduini, C., Perrotta, S., Bottega, R., Pecci, A., Melazzini, F., Civaschi, E., Russo, S., Magrin, S., Loffredo, G., Di Salvo, V., Russo, G., Casale, M., De Rocco, D., Grignani, C., Cattaneo, M., Baronci, C., Dragani, A., Albano, V., Jankovic, M., Scianguetta, S., Savoia, A., Noris, P., Brian Cannon, C., Balduini, S., Perrotta, Bottega, Roberta, A., Pecci, F., Melazzini, E., Civaschi, S., Russo, S., Magrin, G., Loffredo, V., Di Salvo, G., Russo, M., Casale, DE ROCCO, Daniela, C., Grignani, M., Cattaneo, C., Baronci, A., Dragani, V., Albano, M., Jankovic, S., Scianguetta, Savoia, Anna, and P., Noris

Subjects
GPIb-alpha Bolzano mutations Bernard-Soulier syndrome Mutational screening
Published
2011

27. Platelet diameters in inherited thrombocytopenias: analysis of 376 patients with all known disorders

Author

Noris, P, Biino, G, Pecci, A, Civaschi, E, Savoia, A, Seri, M, Melazzini, F, Loffredo, G, Russo, G, Bozzi, V, Notarangelo, Ld, Gresele, P, Heller, Pg, Pujol Moix, N, Kunishima, S, Cattaneo, M, Bussel, J, De Candia, Erica, Cagioni, C, Ramenghi, U, Barozzi, S, Fabris, F, Balduini, Cl, De Candia, Erica (ORCID:0000-0003-0942-2819), Noris, P, Biino, G, Pecci, A, Civaschi, E, Savoia, A, Seri, M, Melazzini, F, Loffredo, G, Russo, G, Bozzi, V, Notarangelo, Ld, Gresele, P, Heller, Pg, Pujol Moix, N, Kunishima, S, Cattaneo, M, Bussel, J, De Candia, Erica, Cagioni, C, Ramenghi, U, Barozzi, S, Fabris, F, Balduini, Cl, and De Candia, Erica (ORCID:0000-0003-0942-2819)

Abstract

Abnormalities of platelet size are one of the distinguishing features of inherited thrombocytopenias (ITs), and evaluation of blood films is recommended as an essential step for differential diagnosis of these disorders. Nevertheless, what we presently know about this subject is derived mainly from anecdotal evidence. To improve knowledge in this field, we evaluated platelet size on blood films obtained from 376 patients with all 19 forms of IT identified so far and found that these conditions differ not only in mean platelet diameter, but also in platelet diameter distribution width and the percentage of platelets with increased or reduced diameters. On the basis of these findings, we propose a new classification of ITs according to platelet size. It distinguishes forms with giant platelets, with large platelets, with normal or slightly increased platelet size, and with normal or slightly decreased platelet size. We also measured platelet diameters in 87 patients with immune thrombocytopenia and identified cutoff values for mean platelet diameter and the percentage of platelets with increased or reduced size that have good diagnostic accuracy in differentiating ITs with giant platelets and with normal or slightly decreased platelet size from immune thrombocytopenia and all other forms of IT

Published
2014

28. Comparison of different biochemical and molecular methods for the identification of Vibrio parahaemolyticus

Author

Croci, L., Suffredini, E., Cozzi, L., Toti, L., Ottaviani, D., Pruzzo, Carla, Serratore, P., Fischetti, R., Goffredo, E., Loffredo, G., Mioni, R., Vibrio parahaemolyticus Working Group, L. Croci, E. Suffredini, L. Cozzi, L. Toti, D. Ottaviani, C. Pruzzo, P. Serratore, R. Fischetti, E. Goffredo, G. Loffredo, and R. Mioni and the Vibrio parahaemolyticus Working Group

Subjects
Identification methods, DNA, Bacterial, Concordance, BIOCHEMICAL METHODS, Applied Microbiology and Biotechnology, Polymerase Chain Reaction, MOLECULAR METHODS, Microbiology, law.invention, Bacterial Proteins, Vibrionaceae, law, False Positive Reactions, Polymerase chain reaction, Bacteriological Techniques, Chromatography, biology, Vibrio parahaemolyticus, General Medicine, equipment and supplies, biology.organism_classification, DNA-Binding Proteins, Genes, Bacterial, Biotechnology, Transcription Factors
Abstract

Aims: Multicentre evaluation of biochemical and molecular methods for the identification of Vibrio parahaemolyticus. Methods and Results: For the biochemical identification methods, API 20E and API 20NE and Alsina's scheme were evaluated in intra- and interlaboratory tests in order to determine the accuracy and concordance of each method. Both in intra- and interlaboratory tests, the Alsina's scheme showed the highest sensitivity (86% of correct identifications in the interlaboratory test). False-positive results were obtained by all methods (specificity was 95% for API 20E, 73% for API 20NE and 84% for Alsina's scheme) and concordance varied from 65% of API 20NE to 84% of API 20E. For the molecular identifications, polymerase chain reaction (PCR) for the detection of toxR gene, tl gene and pR72H fragment were tested on 30 strains by two laboratories. The PCR for toxR showed the highest inclusivity (96%), exclusivity (100%) and concordance (97%). Conclusions: Among the biochemical identification methods tested, the Alsina's scheme gave more reliable results; however, in order to avoid false-positive results, all the biochemical identifications should be confirmed by means of molecular methods. Significance and Impact of the Study: Availability of an efficient identification method of Vibrio parahaemolyticus to use in official control of fisheries products.

Published
2006

30. Mutations in the 5′ UTR of ANKRD26, the ankirin repeat domain 26 gene, cause an autosomal-dominant form of inherited thrombocytopenia, THC2

Author

Pippucci, T. (Tommaso), Savoia, A. (Anna), Perrotta, S. (Silverio), Pujol-Moix, N. (Nuria), Noris, P. (Patrizia), Castegnaro, G. (Giovanni), Pecci, A. (Alessandro), Gnan, C. (Chiara), Punzo, F. (Francesca), Marconi, C. (Caterina), Gherardi, S. (Samuele), Loffredo, G. (Giuseppe), Rocco, D. (Daniela) de, Scianguetta, S., Barozzi, S. (Serena), Magini, P. (Pamela), Bozzi, V. (Valeria), Dezzani, L. (Luca), Stazio, M. (Mariateresa) di, Ferraro, M. (Marcella), Perini, G. (Giovanni), Seri, M. (Marco), Balduini, C.L. (Carlo), Pippucci, T. (Tommaso), Savoia, A. (Anna), Perrotta, S. (Silverio), Pujol-Moix, N. (Nuria), Noris, P. (Patrizia), Castegnaro, G. (Giovanni), Pecci, A. (Alessandro), Gnan, C. (Chiara), Punzo, F. (Francesca), Marconi, C. (Caterina), Gherardi, S. (Samuele), Loffredo, G. (Giuseppe), Rocco, D. (Daniela) de, Scianguetta, S., Barozzi, S. (Serena), Magini, P. (Pamela), Bozzi, V. (Valeria), Dezzani, L. (Luca), Stazio, M. (Mariateresa) di, Ferraro, M. (Marcella), Perini, G. (Giovanni), Seri, M. (Marco), and Balduini, C.L. (Carlo)

Abstract

THC2, an autosomal-dominant thrombocytopenia described so far in only two families, has been ascribed to mutations in MASTL or ACBD5. Here, we show that ANKRD26, another gene within the THC2 locus, and neither MASTL nor ACBD5, is mutated in eight unrelated families. ANKRD26 was also found to be mutated in the family previously reported to have an ACBD5 mutation. We identified six different ANKRD26 mutations, which were clustered in a highly conserved 19 bp sequence located in the 5′ untranslated region. Mutations were not detected in 500 controls and are absent from the 1000 Genomes database. Available data from an animal model and Dr. Watson's genome give evidence against haploinsufficiency as the pathogenetic mechanism for ANKRD26-mediated thrombocytopenia. The luciferase reporter assay suggests that these 5′ UTR mutations might enhance ANKRD26 expression. ANKRD26 is the ancestor of a family of primate-specific genes termed POTE, which have been recently identified as a family of proapoptotic proteins. Dysregulation of apoptosis might therefore be the pathogenetic mechanism, as demonstrated for another thrombocytopenia, THC4. Further investigation is needed to provide evidence supporting this hypothesis.

Published
2011
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31. Needs and challenges of daily life for people with Down syndrome residing in the city of Rome, Italy

Author

Bertoli, M, Biasini, G, Calignano, Mt, Celani, G, De Grossi, G, Digilio, Mc, Fermariello, Cc, Loffredo, G, Luchino, F, Marchese, Alessandra Maria, Mazotti, S, Menghi, B, Razzano, C, Tiano, C, Zambon Hobart, A, Zampino, Giuseppe, Zuccalà, G., Zampino, Giuseppe (ORCID:0000-0003-3865-3253), Bertoli, M, Biasini, G, Calignano, Mt, Celani, G, De Grossi, G, Digilio, Mc, Fermariello, Cc, Loffredo, G, Luchino, F, Marchese, Alessandra Maria, Mazotti, S, Menghi, B, Razzano, C, Tiano, C, Zambon Hobart, A, Zampino, Giuseppe, Zuccalà, G., and Zampino, Giuseppe (ORCID:0000-0003-3865-3253)

Abstract

Population-based surveys on the quality of life of people with Down syndrome (DS) are difficult to perform because of ethical and legal policies regarding privacy and confidential information, but they are essential for service planning. Little is known about the sample size and variability of quality of life of people with DS living in the city of Rome, which has a population of 2.7 million inhabitants. The aim of the present study is to explore the needs and challenges in health, social integration and daily life, of people with DS living in Rome.

Published
2011

32. JEM-X: The X-ray monitor on INTEGRAL

Author

Flanagan, Kathryn A, Siegmund, Oswald H. W, Budtz-Jørgensen, Carl, Lund, Niels, Westergaard, Niels Jørgen Stenfeldt, Brandt, Søren Kristian, Hornstrup, Allan, Rasmussen, Ib Lundgaard, Laursen, Steen, Pedersen, S.M., Kristensen, R.E., Mogensen, P.B., Andersen, K.H., Polny, Jozef, Jensen, Poul Anker, Oxborrow, Carol Anne, Chenevez, Jérôme, Omoe, K., Kämäräinen, V.J., Andersson, Toke Bech, Vilhu, O.R., Huovelin, J., Costa, E., Feroci, M., Rubini, A., Morelli, E., Morbidini, A., Frontera, F., Pelliciari, C., Loffredo, G., Zavattini, G., Carassiti, V., Morawski, M., Juchnikowski, G., Reglero, V., Peris, J., Collado, V., Rodirgo, J.M., Perez, F., Requena, J.L., Larsson, S., Svensson, R., Zdziarski, A., Castro-Tirado, A.J., Schnopper, H.W., Flanagan, Kathryn A, Siegmund, Oswald H. W, Budtz-Jørgensen, Carl, Lund, Niels, Westergaard, Niels Jørgen Stenfeldt, Brandt, Søren Kristian, Hornstrup, Allan, Rasmussen, Ib Lundgaard, Laursen, Steen, Pedersen, S.M., Kristensen, R.E., Mogensen, P.B., Andersen, K.H., Polny, Jozef, Jensen, Poul Anker, Oxborrow, Carol Anne, Chenevez, Jérôme, Omoe, K., Kämäräinen, V.J., Andersson, Toke Bech, Vilhu, O.R., Huovelin, J., Costa, E., Feroci, M., Rubini, A., Morelli, E., Morbidini, A., Frontera, F., Pelliciari, C., Loffredo, G., Zavattini, G., Carassiti, V., Morawski, M., Juchnikowski, G., Reglero, V., Peris, J., Collado, V., Rodirgo, J.M., Perez, F., Requena, J.L., Larsson, S., Svensson, R., Zdziarski, A., Castro-Tirado, A.J., and Schnopper, H.W.

Abstract

The INTEGRAL X-ray monitor, JEM-X, (together with the two gamma ray instruments, SPI and IBIS) provides simultaneous imaging with arcminute angular resolution in the 3-35 keV band. The good angular resolution and low energy response of JEM-X plays an important role in the detection and identification of gamma ray sources as well as in the analysis and scientific interpretation of the combined X-ray and gamma ray data. JEM-X is a coded aperture X-ray telescope consisting of two identical detectors. Each detector has a sensitive area of 500 cm(2), and views the sky through its own coded aperture mask. The coded masks are located 3.4 m above the detector windows. The detector field of view is constrained by X-ray collimators (6.6degrees FOV, FWHM).

Published
2004

33. Clinical and laboratory features of 103 patients from 42 Italian families with inherited thrombocytopenia derived from the monoallelic Ala156Val mutation of GPIb (Bolzano mutation)

Author

Noris, P., primary, Perrotta, S., additional, Bottega, R., additional, Pecci, A., additional, Melazzini, F., additional, Civaschi, E., additional, Russo, S., additional, Magrin, S., additional, Loffredo, G., additional, Di Salvo, V., additional, Russo, G., additional, Casale, M., additional, De Rocco, D., additional, Grignani, C., additional, Cattaneo, M., additional, Baronci, C., additional, Dragani, A., additional, Albano, V., additional, Jankovic, M., additional, Scianguetta, S., additional, Savoia, A., additional, and Balduini, C. L., additional

Published
2011
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34. JEM-X: The X-ray monitor aboard INTEGRAL

Author

Lund, Niels, Budtz-Jørgensen, Carl, Westergaard, Niels Jørgen Stenfeldt, Brandt, Søren Kristian, Rasmussen, Ib Lundgaard, Hornstrup, Allan, Oxborrow, Carol Anne, Chenevez, Jérôme, Jensen, Poul Anker, Laursen, Steen, Andersen, KH., Mogensen, P.B., Rasmussen, Ib, Omo, K., Pedersen, Søren Møller, Polny, Jozef, Andersson, H., Andersson, T., Kamarainen, V., Vilhu, O., Huovelin, J., Maisala, S., Morawski, M., Juchnikowski, G., Costa, E., Feroci, M., Rubini, A., Rapisarda, M., Morelli, E., Carassiti, V., Frontera, F., Pelliciari, C., Loffredo, G., Nunez, S.M., Reglero, V., Velasco, T., Larsson, S., Svensson, R., Zdziarski, A.A., Castro-Tirado, A., Attina, P., Goria, M., Giulianelli, G., Cordero, F., Rezazad, M., Schmidt, M.N., Carli, R., Gomez, C., Jensen, Peter Løkke, Sarri, G., Tiemon, A., Orr, A., Much, R., Kretschmar, P., Schnopper, H.W., Lund, Niels, Budtz-Jørgensen, Carl, Westergaard, Niels Jørgen Stenfeldt, Brandt, Søren Kristian, Rasmussen, Ib Lundgaard, Hornstrup, Allan, Oxborrow, Carol Anne, Chenevez, Jérôme, Jensen, Poul Anker, Laursen, Steen, Andersen, KH., Mogensen, P.B., Rasmussen, Ib, Omo, K., Pedersen, Søren Møller, Polny, Jozef, Andersson, H., Andersson, T., Kamarainen, V., Vilhu, O., Huovelin, J., Maisala, S., Morawski, M., Juchnikowski, G., Costa, E., Feroci, M., Rubini, A., Rapisarda, M., Morelli, E., Carassiti, V., Frontera, F., Pelliciari, C., Loffredo, G., Nunez, S.M., Reglero, V., Velasco, T., Larsson, S., Svensson, R., Zdziarski, A.A., Castro-Tirado, A., Attina, P., Goria, M., Giulianelli, G., Cordero, F., Rezazad, M., Schmidt, M.N., Carli, R., Gomez, C., Jensen, Peter Løkke, Sarri, G., Tiemon, A., Orr, A., Much, R., Kretschmar, P., and Schnopper, H.W.

Abstract

The JEM-X monitor provides X-ray spectra and imaging with arcminute angular resolution in the 3 to 35 keV band. The good angular resolution and the low energy response of JEM-X plays an important role in the identification of gamma ray sources and in the analysis and scientific interpretation of the combined X-ray and gamma ray data. JEM-X is a coded aperture instrument consisting of two identical, coaligned telescopes. Each of the detectors has a sensitive area of 500 cm(2), and views the sky through its own coded aperture mask. The two coded masks are inverted with respect to each other and provides an angular resolution of 3' across an effective field of view of about 10degrees diameter.

Published
2003

35. X-ray facility for the ground calibration of the X-ray monitor JEM-X on board INTEGRAL

Author

Loffredo, G., Pelliciari, C., Frontera, F., Carassiti, V., Chiozzi, S., Evangelisti, F., Landi, L., Melchiorri, M., Squerzanti, S., Brandt, Søren Kristian, Budtz-Jørgensen, Carl, Laursen, Steen, Lund, Niels, Polny, Jozef, Westergaard, Niels Jørgen Stenfeldt, Loffredo, G., Pelliciari, C., Frontera, F., Carassiti, V., Chiozzi, S., Evangelisti, F., Landi, L., Melchiorri, M., Squerzanti, S., Brandt, Søren Kristian, Budtz-Jørgensen, Carl, Laursen, Steen, Lund, Niels, Polny, Jozef, and Westergaard, Niels Jørgen Stenfeldt

Abstract

We describe the X-ray facility developed for the calibration of the X-ray monitor JEM-X on board the INTEGRAL satellite. The apparatus allowed the scanning of the detector geometric area with a pencil beam of desired energy over the major part of the passband of the instrument. The monochromatic radiation is obtained with the use of a double crystal monochromator at fixed exit. We discuss the facility performance.

Published
2003

36. JEM-X inflight performance

Author

Brandt, Søren Kristian, Budtz-Jørgensen, Carl, Lund, Niels, Westergaard, Niels Jørgen Stenfeldt, Rasmussen, Ib Lundgaard, Andersen, K.H., Chenevez, Jérôme, Hornstrup, Allan, Jensen, Poul Anker, Laursen, Steen, Omo, K., Oxborrow, Carol Anne, Pedersen, Søren Møller, Polny, Jozef, Andersson, H., Andersson, T., Vilhu, O., Huovelin, J., Maisala, S., Morawski, M., Juchnikowski, G., Costa, E., Feroci, M., Rubini, A., Rapisarda, M., Morelli, E., Frontera, F., Pelliciari, C., Loffredo, G., Carassiti, V., Reglero, V., Nunez, S.M., Larsson, S., Svensson, R., Zdziarski, A.A., Castro-Tirado, A., Goria, M., Giulianelli, G., Rezazad, M., Carli, R., Jensen, Peter Løkke, Cordero, F., Schmidt, M.N., Sarri, G., Gomez, C., Orr, A., Much, R., Schnopper, H.W., Kretschmar, P., Brandt, Søren Kristian, Budtz-Jørgensen, Carl, Lund, Niels, Westergaard, Niels Jørgen Stenfeldt, Rasmussen, Ib Lundgaard, Andersen, K.H., Chenevez, Jérôme, Hornstrup, Allan, Jensen, Poul Anker, Laursen, Steen, Omo, K., Oxborrow, Carol Anne, Pedersen, Søren Møller, Polny, Jozef, Andersson, H., Andersson, T., Vilhu, O., Huovelin, J., Maisala, S., Morawski, M., Juchnikowski, G., Costa, E., Feroci, M., Rubini, A., Rapisarda, M., Morelli, E., Frontera, F., Pelliciari, C., Loffredo, G., Carassiti, V., Reglero, V., Nunez, S.M., Larsson, S., Svensson, R., Zdziarski, A.A., Castro-Tirado, A., Goria, M., Giulianelli, G., Rezazad, M., Carli, R., Jensen, Peter Løkke, Cordero, F., Schmidt, M.N., Sarri, G., Gomez, C., Orr, A., Much, R., Schnopper, H.W., and Kretschmar, P.

Abstract

We summarize the inflight performance of JEM-X, the X-ray monitor on the INTEGRAL mission during the initial ten months of operations. The JEM-X instruments have now been tuned to stable operational conditions. The performance is found to be close to the pre-launch expectations. The ground calibrations and the inflight calibration data permit to determine the instruments characteristics to fully support the scientific data analysis.

Published
2003

37. New results on focusing of gamma-rays with Laue lenses

Author

Ferrari, F., primary, Frontera, F., additional, Loffredo, G., additional, Virgilli, E., additional, Guidorzi, C., additional, Carassiti, V., additional, Evangelisti, F., additional, Landi, L., additional, Chiozzi, S., additional, Squerzanti, S., additional, Caroli, E., additional, Stephen, J. B., additional, Schiavone, F., additional, Basili, A., additional, Andersen, K. H., additional, and Courtois, P., additional

Published
2009
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38. Focusing of gamma-rays with Laue lenses: first results

Author

Frontera, F., primary, Loffredo, G., additional, Pisa, A., additional, Nobili, F., additional, Carassiti, V., additional, Evangelisti, F., additional, Landi, L., additional, Squerzanti, S., additional, Caroli, E., additional, Stephen, J. B., additional, Andersen, K. H., additional, Courtois, P., additional, Auricchio, N., additional, Milani, L., additional, and Negri, B., additional

Published
2008
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39. Development status of a Laue lens project for gamma-ray astronomy

Author

Frontera, F., primary, Loffredo, G., additional, Pisa, A., additional, Milani, L., additional, Nobili, F., additional, Auricchio, N., additional, Carassiti, V., additional, Evangelisti, F., additional, Landi, L., additional, Squerzanti, S., additional, Andersen, K. H., additional, Courtois, P., additional, Amati, L., additional, Caroli, E., additional, Landini, G., additional, Silvestri, S., additional, Stephen, J. B., additional, Poulsen, J. M., additional, Negri, B., additional, and Pareschi, G., additional

Published
2007
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40. HAXTEL: A Laue lens telescope development project for a deep exploration of the hard X-ray sky (> 60 keV)

Author

Frontera, F., primary, Pisa, A., additional, Loffredo, G., additional, Pellicciotta, D., additional, Carassiti, V., additional, Evangelisti, F., additional, Andersen, K., additional, Courtois, P., additional, Amati, L., additional, Caroli, E., additional, Franceschini, T., additional, Landini, G., additional, Silvestri, S., additional, and Stephen, J., additional

Published
2006
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42. Verocytotoxin-producing Escherichia coli O157 in minced beef and dairy products in Italy

Author

Conedera, G., primary, Dalvit, P., additional, Martini, M., additional, Galiero, G., additional, Gramaglia, M., additional, Goffredo, E., additional, Loffredo, G., additional, Morabito, S., additional, Ottaviani, D., additional, Paterlini, F., additional, Pezzotti, G., additional, Pisanu, M., additional, Semprini, P., additional, and Caprioli, A., additional

Published
2004
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43. JEM-X: the x-ray monitor on INTEGRAL

Author

Budtz-Jorgensen, Carl, primary, Lund, N., additional, Westergaard, Niels J., additional, Brandt, S., additional, Hornstrup, Allan, additional, Rasmussen, Ib L., additional, Laursen, S., additional, Pedersen, S. M., additional, Kristansen, Rene E., additional, Mogensen, P. B., additional, Andersen, K. Harpo, additional, Rasmussen, I., additional, Polny, Josef, additional, Jensen, P. A., additional, Oxborrow, C. A., additional, Chenevez, J., additional, Omoe, K., additional, Kamarainen, Veikko J., additional, Andersson, Tor, additional, Vilhu, Osmi R., additional, Huovelin, J., additional, Costa, Enrico, additional, Feroci, Marco, additional, Rubini, Alda, additional, Morelli, E., additional, Morbidini, A., additional, Frontera, Filippo, additional, Pelliciari, Carlo, additional, Loffredo, G., additional, Zavattini, Guido, additional, Carassiti, Vittore, additional, Morawski, M., additional, Juchnikowski, G., additional, Reglero, Victor, additional, Peris, J., additional, Collado, V., additional, Rodrigo, Juana M., additional, Perez, F., additional, Requena, Jose-Luis, additional, Larsson, S., additional, Svensson, R., additional, Zdziarski, A., additional, Castro-Tirado, A. J., additional, and Schnopper, Herbert W., additional

Published
2004
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44. JEM–X inflight performance

Author

Brandt, S., primary, Budtz-Jørgensen, C., additional, Lund, N., additional, Westergaard, N. J., additional, Rasmussen, I. L., additional, Andersen, K. H., additional, Chenevez, J., additional, Hornstrup, A., additional, Jensen, P. A., additional, Laursen, S., additional, Omø, K., additional, Oxborrow, C. A., additional, Pedersen, S. M., additional, Polny, J., additional, Andersson, H., additional, Andersson, T., additional, Vilhu, O., additional, Huovelin, J., additional, Maisala, S., additional, Morawski, M., additional, Juchnikowski, G., additional, Costa, E., additional, Feroci, M., additional, Rubini, A., additional, Rapisarda, M., additional, Morelli, E., additional, Frontera, F., additional, Pelliciari, C., additional, Loffredo, G., additional, Carassiti, V., additional, Reglero, V., additional, Martínez Núñez, S., additional, Larsson, S., additional, Svensson, R., additional, Zdziarski, A. A., additional, Castro-Tirado, A., additional, Goria, M., additional, Giulianelli, G., additional, Rezazad, M., additional, Cordero, F., additional, Schmidt, M., additional, Carli, R., additional, Jensen, P. L., additional, Sarri, G., additional, Gomez, C., additional, Orr, A., additional, Much, R., additional, Schnopper, H. W., additional, and Kretschmar, P., additional

Published
2003
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45. JEM–X: The X-ray monitor aboard INTEGRAL

Author

Lund, N., primary, Budtz-Jørgensen, C., additional, Westergaard, N. J., additional, Brandt, S., additional, Rasmussen, I. L., additional, Hornstrup, A., additional, Oxborrow, C. A., additional, Chenevez, J., additional, Jensen, P. A., additional, Laursen, S., additional, Andersen, K. H., additional, Mogensen, P. B., additional, Rasmussen, I., additional, Omø, K., additional, Pedersen, S. M., additional, Polny, J., additional, Andersson, H., additional, Andersson, T., additional, Kämäräinen, V., additional, Vilhu, O., additional, Huovelin, J., additional, Maisala, S., additional, Morawski, M., additional, Juchnikowski, G., additional, Costa, E., additional, Feroci, M., additional, Rubini, A., additional, Rapisarda, M., additional, Morelli, E., additional, Carassiti, V., additional, Frontera, F., additional, Pelliciari, C., additional, Loffredo, G., additional, Martínez Núñez, S., additional, Reglero, V., additional, Velasco, T., additional, Larsson, S., additional, Svensson, R., additional, Zdziarski, A. A., additional, Castro-Tirado, A., additional, Attina, P., additional, Goria, M., additional, Giulianelli, G., additional, Cordero, F., additional, Rezazad, M., additional, Schmidt, M., additional, Carli, R., additional, Gomez, C., additional, Jensen, P. L., additional, Sarri, G., additional, Tiemon, A., additional, Orr, A., additional, Much, R., additional, Kretschmar, P., additional, and Schnopper, H. W., additional

Published
2003
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46. X–ray facility for the ground calibration of the X–ray monitor JEM-X on board INTEGRAL

Author

Loffredo, G., primary, Pelliciari, C., additional, Frontera, F., additional, Carassiti, V., additional, Chiozzi, S., additional, Evangelisti, F., additional, Landi, L., additional, Melchiorri, M., additional, Squerzanti, S., additional, Brandt, S., additional, Budtz-Joergensen, C., additional, Laursen, S., additional, Lund, N., additional, Polny, J., additional, and Westergaard, N. J., additional

Published
2003
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47. HAXTEL: A Laue lens telescope development project for a deep exploration of the hard X-ray sky (>60 keV).

Author

von Ballmoos, Peter, Frontera, F., Pisa, A., Loffredo, G., Pellicciotta, D., Carassiti, V., Evangelisti, F., Andersen, K., Courtois, P., Amati, L., Caroli, E., Franceschini, T., Landini, G., Silvestri, S., and Stephen, J.

Abstract

A review of the HAXTEL project devoted to the development of a Laue lens telescope for hard X-/gamma-ray observation of the continuum spectra of celestial sources is presented. Main design properties, open issues, the status of the project and an example of multi-lens configuration with sensitivity expectations are discussed. [ABSTRACT FROM AUTHOR]

Published
2006
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50. Inefficacy of immunosuppressive treatment in HBSAg-positive, delta-negative, moderate chronic active hepatitis in children

Author

L. Greco, G Orso, Buffolano W, Loffredo G, A Fontanella, Pietro Vajro, A D'Antonio, and Oggero

Subjects
Male, HBsAg, medicine.medical_specialty, Time Factors, Azathioprine, Placebo, Asymptomatic, Gastroenterology, Prednisone, Internal medicine, medicine, Humans, Hepatitis B e Antigens, Child, Transaminases, Hepatitis, Chronic, Hepatitis, Prothrombin time, Immunosuppression Therapy, Clinical Trials as Topic, Hepatitis B Surface Antigens, medicine.diagnostic_test, business.industry, medicine.disease, Hepatitis B, HBeAg, Child, Preschool, Pediatrics, Perinatology and Child Health, Immunology, Drug Therapy, Combination, Female, medicine.symptom, business, medicine.drug
Abstract

The efficacy of immunosuppressive treatment was evaluated in a group of 15 children (aged 2-10 years) affected by clinically asymptomatic, biochemically mild, HBSAg-positive, delta-antibody-negative, histologically moderate, chronic active hepatitis (CAH). Clinical, biochemical, serological, and histological features of chronic hepatitis B virus (HBV) infection were studied during a follow-up of 2 years. Eight of 15 patients received immunosuppressive treatment (prednisone plus azathioprine); seven received a placebo. No significant differences were found among treated and untreated patients, an overall improvement of nearly all parameters of disease (aminotransferases, albumin, prothrombin time, immunoglobulins, bromsulphalein at 45 min, and bile acid serum levels) being found in both groups. Treated patients showed an improvement in aminotransferase levels only at the first 3-month checkup. This finding, however, was not statistically significant in our study and disappeared at subsequent follow-up examinations. A high incidence of HBeAg was noted at entry into the study (10 of 15 patients checked). HBeAg seroconversion was observed in two of the six HBeAg-positive treated patients and in two of the four HBeAg-positive untreated patients. Finally, the histological picture showed similar patterns in both groups of patients studied. Our data suggest that no benefit from immunosuppressive therapy can be proven at this time in asymptomatic, moderately active, HBsAg-positive, delta-negative CAH in children.

Published
1985

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