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8 results on '"Roma, E."'

1. Respiratory Needs in Patients with Type 1 Spinal Muscular Atrophy Treated with Nusinersen

Author

Sansone, V. A., Pirola, A., Albamonte, E., Pane, M., Lizio, A., D'Amico, A., Catteruccia, M., Cutrera, R., Bruno, C., Pedemonte, M., Messina, S., Rao, F., Roma, E., Salmin, F., Coratti, Giorgia, Di Bari, A., De Sanctis, R., Pera, C. M., Sframeli, M., Piastra, Marco, Macagno, F., Vita, G., Bertini, E., Mercuri, Eugenio Maria, Coratti G. (ORCID:0000-0001-6666-5628), Piastra M. (ORCID:0000-0002-3144-8970), Mercuri E. (ORCID:0000-0002-9851-5365), Sansone, V. A., Pirola, A., Albamonte, E., Pane, M., Lizio, A., D'Amico, A., Catteruccia, M., Cutrera, R., Bruno, C., Pedemonte, M., Messina, S., Rao, F., Roma, E., Salmin, F., Coratti, Giorgia, Di Bari, A., De Sanctis, R., Pera, C. M., Sframeli, M., Piastra, Marco, Macagno, F., Vita, G., Bertini, E., Mercuri, Eugenio Maria, Coratti G. (ORCID:0000-0001-6666-5628), Piastra M. (ORCID:0000-0002-3144-8970), and Mercuri E. (ORCID:0000-0002-9851-5365)

Abstract

Objective: To evaluate the effects of nusinersen on respiratory function of patients with type 1 spinal muscular atrophy. Study design: Observational, longitudinal cohort study. We collected respiratory data from 118 children with type 1 spinal muscular atrophy and differing pulmonary requirements and conducted a semistructured qualitative interview among a subsample of caregivers at baseline, 6 months, and 10 months after the first nusinersen treatment. Patients were stratified according to ventilation modalities and age at study entry. Results: Most patients in our cohort remained stable (84/109 = 77%). More than 80% of the children treated before age 2 years survived, in contrast to the lower survival reported in natural history studies, and did so without tracheostomy or noninvasive ventilation (NIV) ≥16 hours. In those less than 2 years old, only 3 patients shifted from NIV ≤10 hours to NIV >10 hours, and the other 3 reduced the hours of NIV required. Most of the older patients remained stable; this included not only those on tracheostomy or NIV >10 hours but also 75% of those on NIV ≤10 hours. Conclusions: Our results suggest that nusinersen may produce some improvement in the progression of respiratory impairment, both in terms of survival and need for respiratory support ≥16 hours, especially before the age of 2 years.

Published
2020

2. Consensus-based care recommendations for adults with myotonic dystrophy type 1

Author

Ashizawa, T., Gagnon, C., Groh, W.J., Gutmann, L., Johnson, N.E., Meola, G., Moxley, R., 3rd, Pandya, S., Rogers, M.T., Simpson, E., Angeard, N., Bassez, G., Berggren, K.N., Bhakta, D., Bozzali, M., Broderick, A., Byrne, J.L., Campbell, C., Cup, E.H., Day, J.W., Mattia, E. De, Duboc, D., Duong, T., Eichinger, K., Ekstrom, A.B., Engelen, B. van, Esparis, B., Eymard, B., Ferschl, M., Gadalla, S.M., Gallais, B., Goodglick, T., Heatwole, C., Hilbert, J., Holland, V., Kierkegaard, M., Koopman, Wilma, Lane, K., Maas, D, Mankodi, A., Mathews, K.D., Monckton, D.G., Moser, D., Nazarian, S., Nguyen, L, Nopoulos, P., Petty, R., Phetteplace, J., Puymirat, J., Raman, S., Richer, L., Roma, E., Sampson, J., Sansone, V., Schoser, B., Sterling, L., Statland, J., Subramony, S.H., Tian, C., Trujillo, C., Tomaselli, G., Turner, C., Venance, S., Verma, A., White, M., Winblad, S., Ashizawa, T., Gagnon, C., Groh, W.J., Gutmann, L., Johnson, N.E., Meola, G., Moxley, R., 3rd, Pandya, S., Rogers, M.T., Simpson, E., Angeard, N., Bassez, G., Berggren, K.N., Bhakta, D., Bozzali, M., Broderick, A., Byrne, J.L., Campbell, C., Cup, E.H., Day, J.W., Mattia, E. De, Duboc, D., Duong, T., Eichinger, K., Ekstrom, A.B., Engelen, B. van, Esparis, B., Eymard, B., Ferschl, M., Gadalla, S.M., Gallais, B., Goodglick, T., Heatwole, C., Hilbert, J., Holland, V., Kierkegaard, M., Koopman, Wilma, Lane, K., Maas, D, Mankodi, A., Mathews, K.D., Monckton, D.G., Moser, D., Nazarian, S., Nguyen, L, Nopoulos, P., Petty, R., Phetteplace, J., Puymirat, J., Raman, S., Richer, L., Roma, E., Sampson, J., Sansone, V., Schoser, B., Sterling, L., Statland, J., Subramony, S.H., Tian, C., Trujillo, C., Tomaselli, G., Turner, C., Venance, S., Verma, A., White, M., and Winblad, S.

Abstract

Item does not contain fulltext, Purpose of review: Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that affects between 1 in 3,000 and 8,000 individuals globally. No evidence-based guideline exists to inform the care of these patients, and most do not have access to multidisciplinary care centers staffed by experienced professionals, creating a clinical care deficit. Recent findings: The Myotonic Dystrophy Foundation (MDF) recruited 66 international clinicians experienced in DM1 patient care to develop consensus-based care recommendations. MDF created a 2-step methodology for the project using elements of the Single Text Procedure and the Nominal Group Technique. The process generated a 4-page Quick Reference Guide and a comprehensive, 55-page document that provides clinical care recommendations for 19 discrete body systems and/or care considerations. Summary: The resulting recommendations are intended to help standardize and elevate care for this patient population and reduce variability in clinical trial and study environments.

Published
2018

3. Consensus-based care recommendations for adults with myotonic dystrophy type 1

Author

Ashizawa, T., Gagnon, C., Groh, W.J., Gutmann, L., Johnson, N.E., Meola, G., Moxley, R., 3rd, Pandya, S., Rogers, M.T., Simpson, E., Angeard, N., Bassez, G., Berggren, K.N., Bhakta, D., Bozzali, M., Broderick, A., Byrne, J.L., Campbell, C., Cup, E.H., Day, J.W., Mattia, E. De, Duboc, D., Duong, T., Eichinger, K., Ekstrom, A.B., Engelen, B. van, Esparis, B., Eymard, B., Ferschl, M., Gadalla, S.M., Gallais, B., Goodglick, T., Heatwole, C., Hilbert, J., Holland, V., Kierkegaard, M., Koopman, Wilma, Lane, K., Maas, D, Mankodi, A., Mathews, K.D., Monckton, D.G., Moser, D., Nazarian, S., Nguyen, L, Nopoulos, P., Petty, R., Phetteplace, J., Puymirat, J., Raman, S., Richer, L., Roma, E., Sampson, J., Sansone, V., Schoser, B., Sterling, L., Statland, J., Subramony, S.H., Tian, C., Trujillo, C., Tomaselli, G., Turner, C., Venance, S., Verma, A., White, M., Winblad, S., Ashizawa, T., Gagnon, C., Groh, W.J., Gutmann, L., Johnson, N.E., Meola, G., Moxley, R., 3rd, Pandya, S., Rogers, M.T., Simpson, E., Angeard, N., Bassez, G., Berggren, K.N., Bhakta, D., Bozzali, M., Broderick, A., Byrne, J.L., Campbell, C., Cup, E.H., Day, J.W., Mattia, E. De, Duboc, D., Duong, T., Eichinger, K., Ekstrom, A.B., Engelen, B. van, Esparis, B., Eymard, B., Ferschl, M., Gadalla, S.M., Gallais, B., Goodglick, T., Heatwole, C., Hilbert, J., Holland, V., Kierkegaard, M., Koopman, Wilma, Lane, K., Maas, D, Mankodi, A., Mathews, K.D., Monckton, D.G., Moser, D., Nazarian, S., Nguyen, L, Nopoulos, P., Petty, R., Phetteplace, J., Puymirat, J., Raman, S., Richer, L., Roma, E., Sampson, J., Sansone, V., Schoser, B., Sterling, L., Statland, J., Subramony, S.H., Tian, C., Trujillo, C., Tomaselli, G., Turner, C., Venance, S., Verma, A., White, M., and Winblad, S.

Abstract

Item does not contain fulltext, Purpose of review: Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that affects between 1 in 3,000 and 8,000 individuals globally. No evidence-based guideline exists to inform the care of these patients, and most do not have access to multidisciplinary care centers staffed by experienced professionals, creating a clinical care deficit. Recent findings: The Myotonic Dystrophy Foundation (MDF) recruited 66 international clinicians experienced in DM1 patient care to develop consensus-based care recommendations. MDF created a 2-step methodology for the project using elements of the Single Text Procedure and the Nominal Group Technique. The process generated a 4-page Quick Reference Guide and a comprehensive, 55-page document that provides clinical care recommendations for 19 discrete body systems and/or care considerations. Summary: The resulting recommendations are intended to help standardize and elevate care for this patient population and reduce variability in clinical trial and study environments.

Published
2018

4. Efficacy of a new technique - INtubate-RECruit-SURfactant-Extubate - 'IN-REC-SUR-E' - in preterm neonates with respiratory distress syndrome: study protocol for a randomized controlled trial.

Author

Vento, Giovanni, Pastorino, Roberta, Boni, L, Cota, Francesco, Carnielli, V, Cools, F, Dani, C, Mosca, F, Pillow, J, Polglase, G, Tagliabue, Roberta Piera, van Kaam, Ah, Ventura, Ml, Tana, Milena, Tirone, Chiara, Aurilia, Claudia, Lio, Alessandra, Ricci, C, Gambacorta, Maria Antonietta, Consigli, C, D'Onofrio, D, Gizzi, C, Massenzi, L, Cardilli, V, Casati, A, Bottino, R, Pontiggia, Federico, Ciarmoli, E, Martinelli, S, Ilardi, L, Colnaghi, M, Matassa, Piero Giuseppe, Vendettuoli, Valentina, Villani, P, Fusco, Francesca Paola, Gazzolo, D, Ricotti, A, Ferrero, F, Stasi, I, Magaldi, R, Maffei, G, Presta, G, Perniola, R, Messina, F, Montesano, G, Poggi, C, Giordano, L, Roma, E, Grassia, C, Ausanio, G, Sandri, F, Mescoli, G, Giura, F, Garani, G, Solinas, A, Lucente, Michele, Nigro, G, Del Vecchio, Angela Maria, Petrillo, F, Orfeo, L, Grappone, L, Quartulli, L, Scorrano, Antonio, Messner, H, Staffler, Alex, Gargano, G, Balestri, E, Nobile, Stefano, Cacace, C, Meli, V, Dallaglio, S, Pasqua, B, Mattia, L, Gitto, E, Vitaliti, M, Re, Mp, Vedovato, S, Grison, A, Berardi, A, Torcetta, F, Guidotti, I, di Fabio, S, Maranella, E, Mondello, I, Visentin, S, Tormena, F., Vento, Giovanni (ORCID:0000-0002-8132-5127), Pastorino, Roberta (ORCID:0000-0001-5013-0733), Cota, Francesco (ORCID:0000-0002-9009-3997), Gambacorta, Maria Antonietta (ORCID:0000-0001-5455-8737), Nobile, S (ORCID:0000-0002-5304-1485), Vento, Giovanni, Pastorino, Roberta, Boni, L, Cota, Francesco, Carnielli, V, Cools, F, Dani, C, Mosca, F, Pillow, J, Polglase, G, Tagliabue, Roberta Piera, van Kaam, Ah, Ventura, Ml, Tana, Milena, Tirone, Chiara, Aurilia, Claudia, Lio, Alessandra, Ricci, C, Gambacorta, Maria Antonietta, Consigli, C, D'Onofrio, D, Gizzi, C, Massenzi, L, Cardilli, V, Casati, A, Bottino, R, Pontiggia, Federico, Ciarmoli, E, Martinelli, S, Ilardi, L, Colnaghi, M, Matassa, Piero Giuseppe, Vendettuoli, Valentina, Villani, P, Fusco, Francesca Paola, Gazzolo, D, Ricotti, A, Ferrero, F, Stasi, I, Magaldi, R, Maffei, G, Presta, G, Perniola, R, Messina, F, Montesano, G, Poggi, C, Giordano, L, Roma, E, Grassia, C, Ausanio, G, Sandri, F, Mescoli, G, Giura, F, Garani, G, Solinas, A, Lucente, Michele, Nigro, G, Del Vecchio, Angela Maria, Petrillo, F, Orfeo, L, Grappone, L, Quartulli, L, Scorrano, Antonio, Messner, H, Staffler, Alex, Gargano, G, Balestri, E, Nobile, Stefano, Cacace, C, Meli, V, Dallaglio, S, Pasqua, B, Mattia, L, Gitto, E, Vitaliti, M, Re, Mp, Vedovato, S, Grison, A, Berardi, A, Torcetta, F, Guidotti, I, di Fabio, S, Maranella, E, Mondello, I, Visentin, S, Tormena, F., Vento, Giovanni (ORCID:0000-0002-8132-5127), Pastorino, Roberta (ORCID:0000-0001-5013-0733), Cota, Francesco (ORCID:0000-0002-9009-3997), Gambacorta, Maria Antonietta (ORCID:0000-0001-5455-8737), and Nobile, S (ORCID:0000-0002-5304-1485)

Abstract

BACKGROUND: Although beneficial in clinical practice, the INtubate-SURfactant-Extubate (IN-SUR-E) method is not successful in all preterm neonates with respiratory distress syndrome, with a reported failure rate ranging from 19 to 69 %. One of the possible mechanisms responsible for the unsuccessful IN-SUR-E method, requiring subsequent re-intubation and mechanical ventilation, is the inability of the preterm lung to achieve and maintain an "optimal" functional residual capacity. The importance of lung recruitment before surfactant administration has been demonstrated in animal studies showing that recruitment leads to a more homogeneous surfactant distribution within the lungs. Therefore, the aim of this study is to compare the application of a recruitment maneuver using the high-frequency oscillatory ventilation (HFOV) modality just before the surfactant administration followed by rapid extubation (INtubate-RECruit-SURfactant-Extubate: IN-REC-SUR-E) with IN-SUR-E alone in spontaneously breathing preterm infants requiring nasal continuous positive airway pressure (nCPAP) as initial respiratory support and reaching pre-defined CPAP failure criteria. METHODS/DESIGN: In this study, 206 spontaneously breathing infants born at 24(+0)-27(+6) weeks' gestation and failing nCPAP during the first 24 h of life, will be randomized to receive an HFOV recruitment maneuver (IN-REC-SUR-E) or no recruitment maneuver (IN-SUR-E) just prior to surfactant administration followed by prompt extubation. The primary outcome is the need for mechanical ventilation within the first 3 days of life. Infants in both groups will be considered to have reached the primary outcome when they are not extubated within 30 min after surfactant administration or when they meet the nCPAP failure criteria after extubation. DISCUSSION: From all available data no definitive evidence exists about a positive effect of recruitment before surfactant instillation, but a rationale exists for testing the following h

Published
2016

6. Examination of the conformational restraints to a chiral diimine bridged 2,2′-bipyridine

Author

Prabaharan, Ravi, Oakes, Roma E., Fletcher, Nicholas C., Nieuwenhuyzen, Mark, Prabaharan, Ravi, Oakes, Roma E., Fletcher, Nicholas C., and Nieuwenhuyzen, Mark

Abstract

The synthesis of a new bis(2,2′-bipyridine), bridged by a Schiff base cyclohexane moiety is described. Surprisingly, this compound does not appear to form discrete oligonuclear metal complexes on the addition of zinc(II) and iron(II) cations. In order to rationalise this behaviour, the compound's conformation has been explored using a combination of circular dichroism, X-ray crystallography and DFT calculations, indicating that at least two energy barriers need to be overcome to orientate the ligand in a suitable conformation to permit the formation of coordination helicates with control over the metal centred stereochemistry.

Published
2004

8. Papeles varios en italiano [Manuscrito]

Author

Firenze, Duca de fl. 1569 Lettera del Duca di Firenze all'altro di Savoia circa il titolo datoli di gran duca della Santita di Papa Pio V in consistoro publico. Turino, 28 decembre 1569, Contarini, Tommaso 1542-1617 Relatione fatta dall'Ilmo. Sig. Tommaso Contarini tornato ambasciatore dalli Stati d'Olanda, a di 10 ottobri 1610, Valle, Giovanni Andrea della fl. 1612 Lettera nella qual s'intendere la morte di sette principali baroni fati morire dal Duca de Parma et Piasenza per la congiura contra la sua persona et la sua casa., Marcelli, Marco Antonio Historia nella quale si contiene come ha havuto principio il dominio di Roma, e quanto habia acquistato la Santa Sede Apostolica, Firenze, Duca de fl. 1569 Lettera del Duca di Firenze all'altro di Savoia circa il titolo datoli di gran duca della Santita di Papa Pio V in consistoro publico. Turino, 28 decembre 1569, Contarini, Tommaso 1542-1617 Relatione fatta dall'Ilmo. Sig. Tommaso Contarini tornato ambasciatore dalli Stati d'Olanda, a di 10 ottobri 1610, Valle, Giovanni Andrea della fl. 1612 Lettera nella qual s'intendere la morte di sette principali baroni fati morire dal Duca de Parma et Piasenza per la congiura contra la sua persona et la sua casa., and Marcelli, Marco Antonio Historia nella quale si contiene come ha havuto principio il dominio di Roma, e quanto habia acquistato la Santa Sede Apostolica

Abstract

Índice en h. 1, Relacione de Venecia (h.2-12). Brevissima descritione de Arsenale di Venecia (h.13-20). Copia d'una lettera nella qual s'intende la morte di sette principali Baroni fati morire dal Duca di Parma et Piasenza per la congiura facta contra la sua persona et la sua casa, por Giovanni Andrea della Valle. Parma, 20 maggio 1612 (h.21-31). Capitoli di confederatione tra la Serma. Republica di Venetia et le tre leghe de signori Grisoni (h.32-40v). Relatione del stato, governo, sito... della Signoria di Genova (h.41-55). Discorso di precedenza tra Francia et Spagna (h.56-122). Discorso intorno l'attioni et disegni del cattolico Re di Spagna in materia delle cose d'Italia (h.123-134). Risposta al discorso sopra detto (h.135-188). Lettera del Duca de Firenza all'altro di Savoia circa il titolo datoli di gran Duca della Santita di Papa Pio quinto in consistoro publico. Turino 27 decembre 1569 (h.189-191v). Historia de Marco Antonio Marcelli, nobile venetiano, nella quale si contiene come ha havuto principio il dominio di Roma, e quanto habbia acquistato la Santa Sede Apostolica (h. 192-310). Relatione fatta dall'Ilmo. Sign. Tommaso Contarini tornato ambasciatore dalli Stati d'Olanda a di 10 ottobri 1610 (h.311-321), Pascual de Gayangos

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