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22 results on '"Gagliardi, C."'

1. Correlations of event activity with hard and soft processes in $p$ + Au collisions at $\sqrt{s_\mathrm{NN}}$ = 200 GeV at STAR

Author

STAR Collaboration, Abdulhamid, M. I., Aboona, B. E., Adam, J., Adamczyk, L., Adams, J. R., Aggarwal, I., Aggarwal, M. M., Ahammed, Z., Aschenauer, E. C., Aslam, S., Atchison, J., Bairathi, V., Cap, J. G. Ball, Barish, K., Bellwied, R., Bhagat, P., Bhasin, A., Bhatta, S., Bhosale, S. R., Bielcik, J., Bielcikova, J., Brandenburg, J. D., Broodo, C., Cai, X. Z., Caines, H., Sánchez, M. Calderón de la Barca, Cebra, D., Ceska, J., Chakaberia, I., Chaloupka, P., Chan, B. K., Chang, Z., Chatterjee, A., Chen, D., Chen, J., Chen, J. H., Chen, Z., Cheng, J., Cheng, Y., Christie, W., Chu, X., Crawford, H. J., Csanád, M., Dale-Gau, G., Das, A., Deppner, I. M., Dhamija, A., Dixit, P., Dong, X., Drachenberg, J. L., Duckworth, E., Dunlop, J. C., Engelage, J., Eppley, G., Esumi, S., Evdokimov, O., Eyser, O., Fatemi, R., Fazio, S., Feng, C. J., Feng, Y., Finch, E., Fisyak, Y., Flor, F. A., Fu, C., Gagliardi, C. A., Galatyuk, T., Gao, T., Geurts, F., Ghimire, N., Gibson, A., Gopal, K., Gou, X., Grosnick, D., Gupta, A., Guryn, W., Hamed, A., Han, Y., Harabasz, S., Harasty, M. D., Harris, J. W., Harrison-Smith, H., He, W., He, X. H., He, Y., Herrmann, N., Holub, L., Hu, C., Hu, Q., Hu, Y., Huang, H., Huang, H. Z., Huang, S. L., Huang, T., Huang, Y., Humanic, T. J., Isshiki, M., Jacobs, W. W., Jalotra, A., Jena, C., Jentsch, A., Ji, Y., Jia, J., Jin, C., Ju, X., Judd, E. G., Kabana, S., Kalinkin, D., Kang, K., Kapukchyan, D., Kauder, K., Keane, D., Khanal, A., Khyzhniak, Y. V., Kikoła, D. P., Kincses, D., Kisel, I., Kiselev, A., Knospe, A. G., Ko, H. S., Kołaś, J., Kosarzewski, L. K., Kumar, L., Labonte, M. C., Lacey, R., Landgraf, J. M., Lauret, J., Lebedev, A., Lee, J. H., Leung, Y. H., Li, C., Li, D., Li, H-S., Li, H., Li, W., Li, X., Li, Y., Li, Z., Liang, X., Liang, Y., Licenik, R., Lin, T., Lin, Y., Lisa, M. A., Liu, C., Liu, G., Liu, H., Liu, L., Liu, T., Liu, X., Liu, Y., Liu, Z., Ljubicic, T., Lomicky, O., Longacre, R. S., Loyd, E. M., Lu, T., Luo, J., Luo, X. F., Ma, L., Ma, R., Ma, Y. G., Magdy, N., Mallick, D., Manikandhan, R., Margetis, S., Markert, C., Matonoha, O., McNamara, G., Mezhanska, O., Mi, K., Mioduszewski, S., Mohanty, B., Mondal, B., Mondal, M. M., Mooney, I., Mrazkova, J., Nagy, M. I., Nain, A. S., Nam, J. D., Nasim, M., Neff, D., Nelson, J. M., Nie, M., Nigmatkulov, G., Niida, T., Nonaka, T., Odyniec, G., Ogawa, A., Oh, S., Okubo, K., Page, B. S., Pal, S., Pandav, A., Panday, A., Pandey, A. K., Pani, T., Paul, A., Pawlik, B., Pawlowska, D., Perkins, C., Pluta, J., Pokhrel, B. R., Posik, M., Protzman, T. L., Prozorova, V., Pruthi, N. K., Przybycien, M., Putschke, J., Qin, Z., Qiu, H., Racz, C., Radhakrishnan, S. K., Rana, A., Ray, R. L., Reed, R., Robertson, C. W., Robotkova, M., Aguilar, M. A. Rosales, Roy, D., Chowdhury, P. Roy, Ruan, L., Sahoo, A. K., Sahoo, N. R., Sako, H., Salur, S., Sato, S., Schaefer, B. C., Schmidke, W. B., Schmitz, N., Seck, F-J., Seger, J., Seto, R., Seyboth, P., Shah, N., Shanmuganathan, P. V., Shao, T., Sharma, M., Sharma, N., Sharma, R., Sharma, S. R., Sheikh, A. I., Shen, D., Shen, D. Y., Shen, K., Shi, S. S., Shi, Y., Shou, Q. Y., Si, F., Singh, J., Singha, S., Sinha, P., Skoby, M. J., Smirnov, N., Söhngen, Y., Song, Y., Srivastava, B., Stanislaus, T. D. S., Stefaniak, M., Stewart, D. J., Su, Y., Sumbera, M., Sun, C., Sun, X., Sun, Y., Surrow, B., Svoboda, M., Sweger, Z. W., Tamis, A. C., Tang, A. H., Tang, Z., Tarnowsky, T., Thomas, J. H., Timmins, A. R., Tlusty, D., Todoroki, T., Trentalange, S., Tribedy, P., Tripathy, S. K., Truhlar, T., Trzeciak, B. A., Tsai, O. D., Tsang, C. Y., Tu, Z., Tyler, J., Ullrich, T., Underwood, D. G., Upsal, I., Van Buren, G., Vanek, J., Vassiliev, I., Verkest, V., Videbæk, F., Voloshin, S. A., Wang, G., Wang, J. S., Wang, J., Wang, K., Wang, X., Wang, Y., Wang, Z., Webb, J. C., Weidenkaff, P. C., Westfall, G. D., Wielanek, D., Wieman, H., Wilks, G., Wissink, S. W., Witt, R., Wu, J., Wu, X., Xi, B., Xiao, Z. G., Xie, G., Xie, W., Xu, H., Xu, N., Xu, Q. H., Xu, Y., Xu, Z., Yan, G., Yan, Z., Yang, C., Yang, Q., Yang, S., Yang, Y., Ye, Z., Yi, L., Yu, Y., Zbroszczyk, H., Zha, W., Zhang, C., Zhang, D., Zhang, J., Zhang, S., Zhang, W., Zhang, X., Zhang, Y., Zhang, Z. J., Zhang, Z., Zhao, F., Zhao, J., Zhao, M., Zhou, S., Zhou, Y., Zhu, X., Zurek, M., and Zyzak, M.

Subjects
Nuclear Experiment
Abstract

With the STAR experiment at the BNL Relativisic Heavy Ion Collider, we characterize $\sqrt{s_\mathrm{NN}}$ = 200 GeV p+Au collisions by event activity (EA) measured within the pseudorapidity range $eta$ $in$ [-5, -3.4] in the Au-going direction and report correlations between this EA and hard- and soft- scale particle production at midrapidity ($\eta$ $\in$ [-1, 1]). At the soft scale, charged particle production in low-EA p+Au collisions is comparable to that in p+p collisions and increases monotonically with increasing EA. At the hard scale, we report measurements of high transverse momentum (pT) jets in events of different EAs. In contrast with the soft particle production, high-pT particle production and EA are found to be inversely related. To investigate whether this is a signal of jet quenching in high-EA events, we also report ratios of pT imbalance and azimuthal separation of dijets in high- and low-EA events. Within our measurement precision, no significant differences are observed, disfavoring the presence of jet quenching in the highest 30% EA p+Au collisions at $\sqrt{s_\mathrm{NN}}$ = 200 GeV., Comment: 12 page, 9 figures

Published
2024
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2. Strangeness production in sNN = 3 GeV Au+Au collisions at RHIC

Author

Abdulhamid, M. I., Aboona, B. E., Adam, J., Adamczyk, L., Adams, J. R., Aggarwal, I., Aggarwal, M. M., Ahammed, Z., Aschenauer, E. C., Aslam, S., Atchison, J., Bairathi, V., Ball Cap, J. G., Barish, K., Bellwied, R., Bhagat, P., Bhasin, A., Bhatta, S., Bhosale, S. R., Bielcik, J., Bielcikova, J., Brandenburg, J. D., Broodo, C., Cai, X. Z., Caines, H., Calderón de la Barca Sánchez, M., Cebra, D., Ceska, J., Chakaberia, I., Chaloupka, P., Chan, B. K., Chang, Z., Chatterjee, A., Chen, D., Chen, J., Chen, J. H., Chen, Z., Cheng, J., Cheng, Y., Christie, W., Chu, X., Crawford, H. J., Csanád, M., Dale-Gau, G., Das, A., Deppner, I. M., Dhamija, A., Dixit, P., Dong, X., Drachenberg, J. L., Duckworth, E., Dunlop, J. C., Engelage, J., Eppley, G., Esumi, S., Evdokimov, O., Eyser, O., Fatemi, R., Fazio, S., Feng, C. J., Feng, Y., Finch, E., Fisyak, Y., Flor, F. A., Fu, C., Gagliardi, C. A., Galatyuk, T., Gao, T., Geurts, F., Ghimire, N., Gibson, A., Gopal, K., Gou, X., Grosnick, D., Gupta, A., Guryn, W., Hamed, A., Han, Y., Harabasz, S., Harasty, M. D., Harris, J. W., Harrison-Smith, H., He, W., He, X. H., He, Y., Herrmann, N., Holub, L., Hu, C., Hu, Q., Hu, Y., Huang, H., Huang, H. Z., Huang, S. L., Huang, T., Huang, Y., Huang, Y., Humanic, T. J., Isshiki, M., Jacobs, W. W., Jalotra, A., Jena, C., Jentsch, A., Ji, Y., Jia, J., Jin, C., Ju, X., Judd, E. G., Kabana, S., Kalinkin, D., Kang, K., Kapukchyan, D., Kauder, K., Keane, D., Khanal, A., Khyzhniak, Y. V., Kikoła, D. P., Kincses, D., Kisel, I., Kiselev, A., Knospe, A. G., Ko, H. S., Kołaś, J., Kosarzewski, L. K., Kumar, L., Labonte, M. C., Lacey, R., Landgraf, J. M., Lauret, J., Lebedev, A., Lee, J. H., Leung, Y. H., Li, C., Li, D., Li, H-S., Li, H., Li, W., Li, X., Li, Y., Li, Y., Li, Z., Liang, X., Liang, Y., Licenik, R., Lin, T., Lin, Y., Lisa, M. A., Liu, C., Liu, G., Liu, H., Liu, L., Liu, T., Liu, X., Liu, Y., Liu, Z., Ljubicic, T., Lomicky, O., Longacre, R. S., Loyd, E. M., Lu, T., Luo, J., Luo, X. F., Ma, L., Ma, R., Ma, Y. G., Magdy, N., Mallick, D., Manikandhan, R., Margetis, S., Markert, C., Matonoha, O., McNamara, G., Mezhanska, O., Mi, K., Mioduszewski, S., Mohanty, B., Mondal, B., Mondal, M. M., Mooney, I., Mrazkova, J., Nagy, M. I., Nain, A. S., Nam, J. D., Nasim, M., Neff, D., Nelson, J. M., Nie, M., Nigmatkulov, G., Niida, T., Nonaka, T., Odyniec, G., Ogawa, A., Oh, S., Okubo, K., Page, B. S., Pal, S., Pandav, A., Panday, A., Pandey, A. K., Pani, T., Paul, A., Pawlik, B., Pawlowska, D., Perkins, C., Pluta, J., Pokhrel, B. R., Posik, M., Protzman, T. L., Prozorova, V., Pruthi, N. K., Przybycien, M., Putschke, J., Qin, Z., Qiu, H., Racz, C., Radhakrishnan, S. K., Rana, A., Ray, R. L., Reed, R., Robertson, C. W., Robotkova, M., Rosales Aguilar, M. A., Roy, D., Roy Chowdhury, P., Ruan, L., Sahoo, A. K., Sahoo, N. R., Sako, H., Salur, S., Sato, S., Schaefer, B. C., Schmidke, W. B., Schmitz, N., Seck, F-J., Seger, J., Seto, R., Seyboth, P., Shah, N., Shanmuganathan, P. V., Shao, T., Sharma, M., Sharma, N., Sharma, R., Sharma, S. R., Sheikh, A. I., Shen, D., Shen, D. Y., Shen, K., Shi, S. S., Shi, Y., Shou, Q. Y., Si, F., Singh, J., Singha, S., Sinha, P., Skoby, M. J., Smirnov, N., Söhngen, Y., Song, Y., Srivastava, B., Stanislaus, T. D. S., Stefaniak, M., Stewart, D. J., Su, Y., Sumbera, M., Sun, C., Sun, X., Sun, Y., Sun, Y., Surrow, B., Svoboda, M., Sweger, Z. W., Tamis, A. C., Tang, A. H., Tang, Z., Tarnowsky, T., Thomas, J. H., Timmins, A. R., Tlusty, D., Todoroki, T., Trentalange, S., Tribedy, P., Tripathy, S. K., Truhlar, T., Trzeciak, B. A., Tsai, O. D., Tsang, C. Y., Tu, Z., Tyler, J., Ullrich, T., Underwood, D. G., Upsal, I., Van Buren, G., Vanek, J., Vassiliev, I., Verkest, V., Videbæk, F., Voloshin, S. A., Wang, G., Wang, J. S., Wang, J., Wang, K., Wang, X., Wang, Y., Wang, Y., Wang, Y., Wang, Z., Webb, J. C., Weidenkaff, P. C., Westfall, G. D., Wielanek, D., Wieman, H., Wilks, G., Wissink, S. W., Witt, R., Wu, J., Wu, J., Wu, X., Wu, X., Xi, B., Xiao, Z. G., Xie, G., Xie, W., Xu, H., Xu, N., Xu, Q. H., Xu, Y., Xu, Y., Xu, Z., Xu, Z., Yan, G., Yan, Z., Yang, C., Yang, Q., Yang, S., Yang, Y., Ye, Z., Ye, Z., Yi, L., Yu, Y., Zbroszczyk, H., Zha, W., Zhang, C., Zhang, D., Zhang, J., Zhang, S., Zhang, W., Zhang, X., Zhang, Y., Zhang, Y., Zhang, Y., Zhang, Y., Zhang, Z. J., Zhang, Z., Zhang, Z., Zhao, F., Zhao, J., Zhao, M., Zhou, S., Zhou, Y., Zhu, X., Zurek, M., and Zyzak, M.

Published
2024
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3. AB1507 HOW TO IMPROVE SEXUAL AND REPRODUCTIVE HEALTH CARE TO FEMALE AND MALE PATIENTS WITH AUTOIMMUNE RHEUMATIC DISEASES: THE EXPERIENCE OF A REPRODUCTIVE HEALTH COUNSELLING CLINIC

Author

Giacobbe, E., primary, Gerardi, M. C., additional, Gagliardi, C., additional, Benedetti, S., additional, Raimondo, G. DI, additional, Ascione, A., additional, Barichello, M., additional, Ughi, N., additional, Adinolfi, A., additional, Belloli, L., additional, Casu, C., additional, Cicco, M. DI, additional, Filippini, D. A., additional, Longhi, M., additional, Palermo, B. L., additional, Schettino, M., additional, Segatto, G., additional, Verduci, E., additional, and Epis, O. M., additional

Published
2024
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4. AB1472 MALE PERSPECTIVE ON REPRODUCTIVE HEALTH IN RHEUMATOLOGY PATIENTS: A CROSS-SECTIONAL STUDY

Author

Giacobbe, E., primary, Gerardi, M. C., additional, Gagliardi, C., additional, Benedetti, S., additional, Raimondo, G. DI, additional, Ascione, A., additional, Barichello, M., additional, Ughi, N., additional, Adinolfi, A., additional, Belloli, L., additional, Casu, C., additional, Cicco, M. DI, additional, Filippini, D. A., additional, Longhi, M., additional, Palermo, B. L., additional, Schettino, M., additional, Segatto, G., additional, Verduci, E., additional, and Epis, O. M., additional

Published
2024
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5. Management of pregnancy in autoimmune rheumatic diseases: maternal disease course, gestational and neonatal outcomes and use of medications in the prospective Italian P-RHEUM.it study

Author

Andreoli, L, Gerardi, M, Gerosa, M, Rozza, D, Crisafulli, F, Erra, R, Lini, D, Trespidi, L, Padovan, M, Ruffilli, F, Serale, F, Cuomo, G, Raffeiner, B, Semeraro, P, Tani, C, Chimenti, M, Conigliaro, P, Hoxha, A, Nalli, C, Fredi, M, Lazzaroni, M, Filippini, M, Taglietti, M, Franceschini, F, Zatti, S, Loardi, C, Orabona, R, Ramazzotto, F, Zanardini, C, Fontana, G, Gozzoli, G, Barison, C, Bizioli, P, Caporali, R, Carrea, G, Ossola, M, Maranini, B, Silvagni, E, Govoni, M, Morano, D, Verteramo, R, Doria, A, Del Ross, T, Favaro, M, Calligaro, A, Tonello, M, Larosa, M, Zen, M, Zambon, A, Mosca, M, Zucchi, D, Elefante, E, Gori, S, Iannone, F, Anelli, M, Lavista, M, Abbruzzese, A, Fasano, C, D'Angelo, S, Cutro, M, Picerno, V, Carbone, T, Padula, A, Rovere-Querini, P, Canti, V, De Lorenzo, R, Cavallo, L, Ramoni, V, Montecucco, C, Codullo, V, Milanesi, A, Pazzola, G, Comitini, G, Marvisi, C, Salvarani, C, Epis, O, Benedetti, S, Di Raimondo, G, Gagliardi, C, Lomater, C, Crepaldi, G, Bellis, E, Bellisai, F, Garcia Gonzalez, E, Pata, A, Zerbinati, M, Urban, M, Mattioli, I, Iuliano, A, Sebastiani, G, Brucato, A, Bizzi, E, Cutolo, M, Santo, L, Tonetta, S, Landolfi, G, Carrara, G, Bortoluzzi, A, Scirè, C, Tincani, A, Andreoli, Laura, Gerardi, Maria Chiara, Gerosa, Maria, Rozza, Davide, Crisafulli, Francesca, Erra, Roberta, Lini, Daniele, Trespidi, Laura, Padovan, Melissa, Ruffilli, Francesca, Serale, Francesca, Cuomo, Giovanna, Raffeiner, Bernd, Semeraro, Paolo, Tani, Chiara, Chimenti, Maria Sole, Conigliaro, Paola, Hoxha, Ariela, Nalli, Cecilia, Fredi, Micaela, Lazzaroni, Maria Grazia, Filippini, Matteo, Taglietti, Marco, Franceschini, Franco, Zatti, Sonia, Loardi, Chiara, Orabona, Rossana, Ramazzotto, Francesca, Zanardini, Cristina, Fontana, Giulia, Gozzoli, Giorgia, Barison, Claudia, Bizioli, Paola, Caporali, Roberto Felice, Carrea, Giulia, Ossola, Manuela Wally, Maranini, Beatrice, Silvagni, Ettore, Govoni, Marcello, Morano, Danila, Verteramo, Rosita, Doria, Andrea, Del Ross, Teresa, Favaro, Maria, Calligaro, Antonia, Tonello, Marta, Larosa, Maddalena, Zen, Margherita, Zambon, Alessandra, Mosca, Marta, Zucchi, Dina, Elefante, Elena, Gori, Sabrina, Iannone, Florenzo, Anelli, Maria Grazia, Lavista, Marlea, Abbruzzese, Anna, Fasano, Carlo Giuseppe, D'Angelo, Salvatore, Cutro, Maria Stefania, Picerno, Valentina, Carbone, Teresa, Padula, Angela Anna, Rovere-Querini, Patrizia, Canti, Valentina, De Lorenzo, Rebecca, Cavallo, Ludovica, Ramoni, Véronique, Montecucco, Carlomaurizio, Codullo, Veronica, Milanesi, Alessandra, Pazzola, Giulia, Comitini, Giuseppina, Marvisi, Chiara, Salvarani, Carlo, Epis, Oscar Massimiliano, Benedetti, Sara, Di Raimondo, Giuseppina, Gagliardi, Clizia, Lomater, Claudia, Crepaldi, Gloria, Bellis, Elisa, Bellisai, Francesca, Garcia Gonzalez, Estrella, Pata, Anna Paola, Zerbinati, Martina, Urban, Maria Letizia, Mattioli, Irene, Iuliano, Annamaria, Sebastiani, Giandomenico, Brucato, Antonio Luca, Bizzi, Emanuele, Cutolo, Maurizio, Santo, Leonardo, Tonetta, Sara, Landolfi, Gianpiero, Carrara, Greta, Bortoluzzi, Alessandra, Scirè, Carlo Alberto, Tincani, Angela, Andreoli, L, Gerardi, M, Gerosa, M, Rozza, D, Crisafulli, F, Erra, R, Lini, D, Trespidi, L, Padovan, M, Ruffilli, F, Serale, F, Cuomo, G, Raffeiner, B, Semeraro, P, Tani, C, Chimenti, M, Conigliaro, P, Hoxha, A, Nalli, C, Fredi, M, Lazzaroni, M, Filippini, M, Taglietti, M, Franceschini, F, Zatti, S, Loardi, C, Orabona, R, Ramazzotto, F, Zanardini, C, Fontana, G, Gozzoli, G, Barison, C, Bizioli, P, Caporali, R, Carrea, G, Ossola, M, Maranini, B, Silvagni, E, Govoni, M, Morano, D, Verteramo, R, Doria, A, Del Ross, T, Favaro, M, Calligaro, A, Tonello, M, Larosa, M, Zen, M, Zambon, A, Mosca, M, Zucchi, D, Elefante, E, Gori, S, Iannone, F, Anelli, M, Lavista, M, Abbruzzese, A, Fasano, C, D'Angelo, S, Cutro, M, Picerno, V, Carbone, T, Padula, A, Rovere-Querini, P, Canti, V, De Lorenzo, R, Cavallo, L, Ramoni, V, Montecucco, C, Codullo, V, Milanesi, A, Pazzola, G, Comitini, G, Marvisi, C, Salvarani, C, Epis, O, Benedetti, S, Di Raimondo, G, Gagliardi, C, Lomater, C, Crepaldi, G, Bellis, E, Bellisai, F, Garcia Gonzalez, E, Pata, A, Zerbinati, M, Urban, M, Mattioli, I, Iuliano, A, Sebastiani, G, Brucato, A, Bizzi, E, Cutolo, M, Santo, L, Tonetta, S, Landolfi, G, Carrara, G, Bortoluzzi, A, Scirè, C, Tincani, A, Andreoli, Laura, Gerardi, Maria Chiara, Gerosa, Maria, Rozza, Davide, Crisafulli, Francesca, Erra, Roberta, Lini, Daniele, Trespidi, Laura, Padovan, Melissa, Ruffilli, Francesca, Serale, Francesca, Cuomo, Giovanna, Raffeiner, Bernd, Semeraro, Paolo, Tani, Chiara, Chimenti, Maria Sole, Conigliaro, Paola, Hoxha, Ariela, Nalli, Cecilia, Fredi, Micaela, Lazzaroni, Maria Grazia, Filippini, Matteo, Taglietti, Marco, Franceschini, Franco, Zatti, Sonia, Loardi, Chiara, Orabona, Rossana, Ramazzotto, Francesca, Zanardini, Cristina, Fontana, Giulia, Gozzoli, Giorgia, Barison, Claudia, Bizioli, Paola, Caporali, Roberto Felice, Carrea, Giulia, Ossola, Manuela Wally, Maranini, Beatrice, Silvagni, Ettore, Govoni, Marcello, Morano, Danila, Verteramo, Rosita, Doria, Andrea, Del Ross, Teresa, Favaro, Maria, Calligaro, Antonia, Tonello, Marta, Larosa, Maddalena, Zen, Margherita, Zambon, Alessandra, Mosca, Marta, Zucchi, Dina, Elefante, Elena, Gori, Sabrina, Iannone, Florenzo, Anelli, Maria Grazia, Lavista, Marlea, Abbruzzese, Anna, Fasano, Carlo Giuseppe, D'Angelo, Salvatore, Cutro, Maria Stefania, Picerno, Valentina, Carbone, Teresa, Padula, Angela Anna, Rovere-Querini, Patrizia, Canti, Valentina, De Lorenzo, Rebecca, Cavallo, Ludovica, Ramoni, Véronique, Montecucco, Carlomaurizio, Codullo, Veronica, Milanesi, Alessandra, Pazzola, Giulia, Comitini, Giuseppina, Marvisi, Chiara, Salvarani, Carlo, Epis, Oscar Massimiliano, Benedetti, Sara, Di Raimondo, Giuseppina, Gagliardi, Clizia, Lomater, Claudia, Crepaldi, Gloria, Bellis, Elisa, Bellisai, Francesca, Garcia Gonzalez, Estrella, Pata, Anna Paola, Zerbinati, Martina, Urban, Maria Letizia, Mattioli, Irene, Iuliano, Annamaria, Sebastiani, Giandomenico, Brucato, Antonio Luca, Bizzi, Emanuele, Cutolo, Maurizio, Santo, Leonardo, Tonetta, Sara, Landolfi, Gianpiero, Carrara, Greta, Bortoluzzi, Alessandra, Scirè, Carlo Alberto, and Tincani, Angela

Abstract

Objectives To investigate pregnancy outcomes in women with autoimmune rheumatic diseases (ARD) in the Italian prospective cohort study P-RHEUM.it. Methods Pregnant women with different ARD were enrolled for up to 20 gestational weeks in 29 Rheumatology Centres for 5 years (2018-2023). Maternal and infant information were collected in a web-based database. Results We analysed 866 pregnancies in 851 patients (systemic lupus erythematosus was the most represented disease, 19.6%). Maternal disease flares were observed in 135 (15.6%) pregnancies. 53 (6.1%) pregnancies were induced by assisted reproduction techniques, 61 (7%) ended in miscarriage and 11 (1.3%) underwent elective termination. Obstetrical complications occurred in 261 (30.1%) pregnancies, including 2.3% pre-eclampsia. Two cases of congenital heart block were observed out of 157 pregnancies (1.3%) with anti-Ro/SSA. Regarding treatments, 244 (28.2%) pregnancies were treated with glucocorticoids, 388 (44.8%) with hydroxychloroquine, 85 (9.8%) with conventional synthetic disease-modifying anti-rheumatic drugs and 122 (14.1%) with biological disease-modifying anti-rheumatic drugs. Live births were 794 (91.7%), mostly at term (84.9%); four perinatal deaths (0.5%) occurred. Among 790 newborns, 31 (3.9%) were small-for-gestational-age and 169 (21.4%) had perinatal complications. Exclusive maternal breast feeding was received by 404 (46.7%) neonates. The Edinburgh Postnatal Depression Scale was compiled by 414 women (52.4%); 89 (21.5%) scored positive for emotional distress. Conclusions Multiple factors including preconception counselling and treat-to-target with pregnancy-compatible medications may have contributed to mitigate disease-related risk factors, yielding limited disease flares, good pregnancy outcomes and frequency of complications which were similar to the Italian general obstetric population. Disease-specific issues need to be further addressed to plan preventative measures.

Published
2024

6. New Insights into the Neuropsychological Profile and Intellectual Quotient Variability in Joubert Syndrome Compared to Other Congenital Cerebellar Malformations

Author

Butti, N., Oldrati, V., Ferrari, Emma, Romaniello, R., Gagliardi, Chiara, Borgatti, Renato, Urgesi, Cosimo, Ferrari E. (ORCID:0009-0000-3237-9551), Gagliardi C. (ORCID:0000-0001-9931-9879), Borgatti R. (ORCID:0000-0001-8165-4994), Urgesi C., Butti, N., Oldrati, V., Ferrari, Emma, Romaniello, R., Gagliardi, Chiara, Borgatti, Renato, Urgesi, Cosimo, Ferrari E. (ORCID:0009-0000-3237-9551), Gagliardi C. (ORCID:0000-0001-9931-9879), Borgatti R. (ORCID:0000-0001-8165-4994), and Urgesi C.

Abstract

The neuropsychological characteristics of the cerebellar cognitive affective syndrome (CCAS) in congenital, non-progressive malformations of the cerebellum have been scarcely investigated, and even less is known for Joubert syndrome (JS), an inherited, non-progressive cerebellar ataxia characterized by the so-called molar tooth sign. The few studies on this topic reported inconsistent results about intellectual functioning and specific neuropsychological impairments. The aim of this research is to examine the neuropsychological profile of JS compared to other congenital cerebellar malformations (CM), considering individual variability of intellectual quotient (IQ) in the two groups. Fourteen patients with JS and 15 patients with CM aged 6-25 years were tested through a comprehensive, standardized neuropsychological battery. Their scores in the neuropsychological domains were inspected through descriptive analysis and compared by mean of MANOVA and ANOVA models, then replicated inserting IQ as covariate. The two groups showed a largely overlapping neuropsychological profile, consistent with CCAS. However, the JS group showed worse performance in visual-spatial memory compared to CM patients, although this difference was mitigated when considering IQ. These findings highlight a divergence between JS and other CM in visual-spatial memory, which might suggest a critical role of the cerebellum in recalling task-relevant memories and might inform rehabilitative interventions.

Published
2024

7. News insights into social cognition in Williams syndrome from a comprehensive assessment and a virtual reality task.

Author

Butti N, Ferrari E, Oldrati V, Biffi E, Gagliardi C, Romaniello R, Strazzer S, Borgatti R, and Urgesi C

Subjects
Humans, Male, Female, Adolescent, Adult, Young Adult, Neuropsychological Tests, Social Perception, Middle Aged, Williams Syndrome psychology, Williams Syndrome physiopathology, Virtual Reality, Social Cognition
Abstract

Although the uneven neuropsychological profile of William Syndrome (WS) is well established, less is known about social perception and how profile characteristics may affect the ability to predict other's intentions, a main hallmark of social cognition. This study aimed at examining the neuropsychological profile, including social perception, of adolescents and adults with WS, and at verifying which neuropsychological outcome might account for their social prediction ability. Twenty-six individuals with WS were administered a comprehensive neuropsychological assessment, and a virtual reality scenario designed to assess social prediction in a dynamic, everyday life-like context. We found that social perception was a relative strength of the profile, although a dissociation emerged between impaired verbal ToM and relatively spared low-level components. Peaks and valleys were reported in other domains consistently with the expected profile. Both spatial and facial memory were significantly associated with the performance at the social prediction task. Results clarified that social perception per se should not be considered as typically impaired in WS. Weaknesses and strengths in specific abilities, particularly spatial and facial memory, might affect the ability to understand others' intentions in WS beyond domain-specific mechanisms. These findings might inform future syndrome-specific rehabilitative interventions., Competing Interests: Declarations Competing interests The authors declare no competing interests., (© 2024. The Author(s).)

Published
2024
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8. Incidence and predictors of sudden death in patients with cardiac amyloidosis.

Author

de Frutos F, Saturi G, Gonzalez-Lopez E, Sguazzotti M, Dominguez F, Ponziani A, Cabrera-Romero E, Caponetti AG, Lozano S, Massa P, Peiro-Aventin B, Accietto A, Mora-Ayestarán N, Giovannetti A, Castro-Urda V, Gagliardi C, Cobo-Marcos M, Rios-Tamayo R, Biagini E, Gomez-Bueno M, Galiè N, Segovia-Cubero J, Longhi S, and Garcia-Pavia P

Abstract

Introduction: Although sudden death (SD) is a recognized complication of cardiac amyloidosis, there is scarce data about its incidence, mechanisms, and predictors. The aim of this study was to describe incidence of SD and to analyze possible risk factors., Methods: Consecutive patients with ATTR or AL cardiac amyloidosis evaluated at two European centers were identified. SD was defined as unexpected death in clinically stable patients. Cox proportional hazard regression was performed to assess risk factors in univariate analysis. Those statistically significant were then assessed through age-adjusted multivariate analysis., Results: Analysis included 784 patients, 569 with ATTR amyloidosis (mean age 74.1 ± 12.1 years) and 215 with AL amyloidosis (mean age 64.5 ± 10.8 years). After a median follow-up of 1.9 years, SD rate at 2 years was 1.8% in ATTR. Previous pacemaker implantation (PPM) was associated with increased risk after age-adjusted analysis (HR 4.97; 95%CI: 1.39-17.7; p  = 0.01). SD rate in AL amyloidosis patients at 2 years was 8.0% after a median follow-up of 1.2 years. Betablockers and NYHA III-IV were independently associated with an increased risk after age-adjusted multivariate analysis (HR 7.06 95%CI (2.31-21.5) p  = 0.001) and (HR 4.56 95%CI (1.51-13.8) p  = 0.007) respectively., Conclusions: SD is more frequent in AL than in ATTR cardiac amyloidosis. SD is associated with different risk factors in both entities.

Published
2024
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9. Early diagnosis, disease stage and prognosis in wild-type transthyretin amyloid cardiomyopathy: The DIAMOND study.

Author

Tini G, Musumeci B, Milani P, Zampieri M, Caponetti AG, Fabris F, Foli A, Argirò A, Mazzoni C, Gagliardi C, Longhi S, Saturi G, Vergaro G, Aimo A, De Fazio L, Varrà GG, Serenelli M, Fabbri G, De Michieli L, Palmiero G, Ciliberti G, Carigi S, Zanoletti M, Mandoli GE, Lucchi GR, Rella V, Monti E, Gardini E, Bartolotti M, Crotti L, Merli E, Mussinelli R, Vianello PF, Cameli M, Marzo F, Guerra F, Limongelli G, Cipriani A, Perlini S, Obici L, Perfetto F, Barbato E, Porto I, Sinagra G, Merlo M, Emdin M, Biagini E, Cappelli F, Palladini G, and Canepa M

Abstract

Aims: Disease staging and prognostic scoring in wild-type transthyretin-related cardiac amyloidosis (ATTRwt-CA) can be captured by two systems (NAC and Columbia scores). However, uncertainty remains as epidemiology of the disease is evolving rapidly. We evaluated features associated with staging systems across ATTRwt-CA patients from different diagnostic pathways, and their association with prognosis., Methods: We performed an analysis on DIAMOND patients with available data to evaluate NAC and Columbia score. DIAMOND was a retrospective study from 17 Italian referral centres for CA, enrolling 1281 patients diagnosed between 2016 and 2021, and aimed at describing characteristics of pathways leading to ATTRwt-CA diagnosis. Of the original cohort, 811 patients were included in this analysis. Each patient had NAC and Columbia score calculated. Patients were grouped according to NAC and Columbia scoring classes. We described characteristics of patients according to staging classes and diagnostic pathways at diagnosis. Prevalence of early diagnoses, defined as NAC Ia, NYHA class I, no use of diuretics, no history of heart failure (HF) hospitalizations nor of atrial fibrillation prior to diagnosis, was investigated. Finally, prognostic variables were tested alone and grouped as NAC or Columbia scores in Cox univariate and multivariate regression analyses. Prognosis was investigated as all-cause mortality, in the whole population and dividing patients in HF versus other diagnostic pathways., Results: Only 1% of the study population had an early ATTRwt-CA diagnosis. Distribution of prognostic variables and of NAC and Columbia classes was heterogeneous across diagnostic pathways. The prevalence of NAC III and Columbia III was higher in the HF diagnostic pathway, but all NAC and Columbia classes were present in all pathways. Both NAC and Columbia scores were associated with all-cause mortality at univariate Cox regression analysis in the whole population, in patients from the HF diagnostic pathway and in those from other pathways. At multivariate analysis, Columbia score remained significantly associated with the outcome, together with age at diagnosis, left ventricular ejection fraction and maximal wall thickness., Conclusions: In this contemporary nationwide cohort, an ATTRwt-CA early diagnosis was very rare. Disease staging with NAC and Columbia scoring systems determined classes of patients with heterogeneous features. Both scores were significantly associated with mortality, but other variables also had prognostic significance., (© 2024 The Author(s). ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Published
2024
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10. Temporal implementation of a regional referral pathway in transthyretin cardiac amyloidosis: Emilia-Romagna experience.

Author

Longhi S, Biagini E, Guaraldi P, Carigi S, Dossi MC, Bartolotti M, Gardini E, Merli E, Marzo F, Luisi GA, Postiglione E, Serenelli M, Tugnoli V, De Gennaro R, Caponetti AG, Gagliardi C, Saturi G, Ponziani A, Perugini E, Rinaldi R, Barbieri A, Bonatti S, Ariatti A, Leuzzi C, Codeluppi L, Serra W, Allegri I, Lanati G, Terracciano C, Cortelli P, Galiè N, and Boriani G

Subjects
Humans, Retrospective Studies, Male, Female, Aged, Time Factors, Italy, Middle Aged, Aged, 80 and over, Health Care Surveys, Time-to-Treatment, Predictive Value of Tests, Critical Pathways, Amyloid Neuropathies, Familial diagnosis, Amyloid Neuropathies, Familial therapy, Amyloid Neuropathies, Familial mortality, Cardiomyopathies diagnosis, Cardiomyopathies therapy, Delayed Diagnosis, Referral and Consultation statistics & numerical data
Abstract

Aims: Transthyretin cardiac amyloidosis (ATTR-CA) is a rare and progressive cardiomyopathy caused by amyloid fibril deposition in myocardial tissue. Diagnostic challenges have historically hampered timely detection. Recent advances in noninvasive diagnostic techniques have facilitated ATTR-CA diagnosis. We aimed to examine the development of a regional network for the diagnosis and management of ATTR-CA and describe a cohort of patients with ATTR-CA, investigate diagnostic pathways and assess clinical outcomes according to diagnosis periods., Methods: We performed a survey study analyzing answers from 11 cardiology centers and we conducted a retrospective study including patients with ATTR-CA attending a referral center between 1 January 2012 and 31 December 2022, and categorized by the period of diagnosis (2012-2016 and 2017-2022)., Results: Over the years, a growing number of patients reached a diagnosis and were treated in the surveyed nonreferral centers of the region. The retrospective study showed a more significant diagnostic delay in the earlier period rather than the later one [13.4 (5-30.2) vs. 10.6 (5.0-17.9) months, P = 0.04]. Patients diagnosed after 2017 showed a greater survival rate than those diagnosed earlier ( P = 0.02). In the multivariate analysis, the year of diagnosis from 2017 remained independently associated with mortality [hazard ratio (HR) 0.46, 95% confidence interval (CI) 0.28-0.79; P = 0.005]., Conclusion: This study emphasized the shift toward noninvasive diagnostic criteria. It revealed a positive impact on patient survival and disease management with the use of disease-modifying therapies and diagnostic developments in more recent years. The findings underscore the importance of disease awareness and networking to reduce diagnostic delays and enhance patient journeys for ATTR-CA., (Copyright © 2024 Italian Federation of Cardiology - I.F.C. All rights reserved.)

Published
2024
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11. Long-term treatment of hereditary transthyretin amyloidosis with patisiran: multicentre, real-world experience in Italy.

Author

Gentile L, Mazzeo A, Briani C, Casagrande S, De Luca M, Fabrizi GM, Gagliardi C, Gemelli C, Forcina F, Grandis M, Guglielmino V, Iabichella G, Leonardi L, Lozza A, Manganelli F, Mussinelli R, My F, Occhipinti G, Fenu S, Russo M, Romano A, Salvalaggio A, Tagliapietra M, Tozza S, Palladini G, Obici L, and Luigetti M

Subjects
Humans, Italy, Male, Female, Middle Aged, Retrospective Studies, Aged, Treatment Outcome, RNA, Small Interfering therapeutic use, Quality of Life, Amyloid Neuropathies, Familial drug therapy, Amyloid Neuropathies, Familial complications
Abstract

Background: Hereditary transthyretin (ATTRv, v for variant) amyloidosis with polyneuropathy is a rare disease caused by mutations in the transthyretin gene. In ATTRv amyloidosis, multisystem extracellular deposits of amyloid cause tissue and organ dysfunction. Patisiran is a small interfering RNA molecule drug that reduces circulating levels of mutant and wild-type TTR proteins. Prior to its regulatory approval, patisiran was available in Italy through a compassionate use programme (CUP). The aim of this study was to analyse the long-term outcomes of patients who entered into the CUP., Methods: This was a multicentre, observational, retrospective study of patients with ATTRv amyloidosis treated with patisiran. The analysis included change from baseline to 12, 24, 36 and 48 months in familial amyloid polyneuropathy (FAP) stage, polyneuropathy disability (PND) class, neuropathy impairment score (NIS), modified body mass index (mBMI), Compound Autonomic Dysfunction Test (CADT), Karnofsky Performance Status (KPS) scale and Norfolk Quality of Life-Diabetic Neuropathy (QoL-DN) questionnaire. Safety data were also analysed., Results: Forty patients from 11 Italian centres were enrolled: 23 in FAP 1 (6 in PND 1 and 17 in PND 2) and 17 in FAP 2 (8 in PND 3a and 9 in PND 3b) stage. In this population, the mean NIS at baseline was 71.4 (± 27.8); mBMI, 917.1 (± 207) kg/m 2 ; KPS, 67.1 (± 14.0); Norfolk QoL-DN, 62.2 (± 25.2); and CADT, 13.2 (± 3.3). Statistical analysis showed few significant differences from baseline denoting disease stability. No new safety signals emerged., Conclusions: Patisiran largely stabilised disease in patients with ATTRv amyloidosis., (© 2024. The Author(s).)

Published
2024
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12. Correction to: Long-term treatment of hereditary transthyretin amyloidosis with patisiran: multicentre, real-world experience in Italy.

Author

Gentile L, Mazzeo A, Briani C, Casagrande S, De Luca M, Fabrizi GM, Gagliardi C, Gemelli C, Forcina F, Grandis M, Guglielmino V, Iabichella G, Leonardi L, Lozza A, Manganelli F, Mussinelli R, My F, Occhipinti G, Fenu S, Russo M, Romano A, Salvalaggio A, Tagliapietra M, Tozza S, Palladini G, Obici L, and Luigetti M

Published
2024
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13. Observation of Strong Nuclear Suppression in Exclusive J/ψ Photoproduction in Au+Au Ultraperipheral Collisions at RHIC.

Author

Abdulhamid MI, Aboona BE, Adam J, Adamczyk L, Adams JR, Aggarwal I, Aggarwal MM, Ahammed Z, Aschenauer EC, Aslam S, Atchison J, Bairathi V, Cap JGB, Barish K, Bellwied R, Bhagat P, Bhasin A, Bhatta S, Bhosale SR, Bielcik J, Bielcikova J, Brandenburg JD, Broodo C, Cai XZ, Caines H, Sánchez MCB, Cebra D, Ceska J, Chakaberia I, Chaloupka P, Chan BK, Chang Z, Chatterjee A, Chen D, Chen J, Chen JH, Chen Z, Cheng J, Cheng Y, Choudhury S, Christie W, Chu X, Crawford HJ, Csanád M, Dale-Gau G, Das A, Deppner IM, Dhamija A, Dixit P, Dong X, Drachenberg JL, Duckworth E, Dunlop JC, Engelage J, Eppley G, Esumi S, Evdokimov O, Eyser O, Fatemi R, Fazio S, Feng CJ, Feng Y, Finch E, Fisyak Y, Flor FA, Fu C, Gagliardi CA, Galatyuk T, Gao T, Geurts F, Ghimire N, Gibson A, Gopal K, Gou X, Grosnick D, Gupta A, Guryn W, Hamed A, Han Y, Harabasz S, Harasty MD, Harris JW, Harrison-Smith H, He W, He XH, He Y, Herrmann N, Holub L, Hu C, Hu Q, Hu Y, Huang H, Huang HZ, Huang SL, Huang T, Huang X, Huang Y, Huang Y, Humanic TJ, Isshiki M, Jacobs WW, Jalotra A, Jena C, Jentsch A, Ji Y, Jia J, Jin C, Ju X, Judd EG, Kabana S, Kalinkin D, Kang K, Kapukchyan D, Kauder K, Keane D, Khanal A, Khyzhniak YV, Kikoła DP, Kincses D, Kisel I, Kiselev A, Knospe AG, Ko HS, Kosarzewski LK, Kumar L, Labonte MC, Lacey R, Landgraf JM, Lauret J, Lebedev A, Lee JH, Leung YH, Lewis N, Li C, Li D, Li HS, Li H, Li W, Li X, Li Y, Li Y, Li Z, Liang X, Liang Y, Licenik R, Lin T, Lin Y, Lisa MA, Liu C, Liu G, Liu H, Liu L, Liu T, Liu X, Liu Y, Liu Z, Ljubicic T, Lomicky O, Longacre RS, Loyd EM, Lu T, Luo J, Luo XF, Ma L, Ma R, Ma YG, Magdy N, Mallick D, Manikandhan R, Margetis S, Markert C, McNamara G, Mezhanska O, Mi K, Mioduszewski S, Mohanty B, Mondal MM, Mooney I, Mrazkova J, Nagy MI, Nain AS, Nam JD, Nasim M, Neff D, Nelson JM, Nemes DB, Nie M, Nigmatkulov G, Niida T, Nonaka T, Odyniec G, Ogawa A, Oh S, Okubo K, Page BS, Pak R, Pal S, Pandav A, Pandey AK, Pani T, Paul A, Pawlik B, Pawlowska D, Perkins C, Pluta J, Pokhrel BR, Posik M, Protzman T, Prozorova V, Pruthi NK, Przybycien M, Putschke J, Qin Z, Qiu H, Racz C, Radhakrishnan SK, Rana A, Ray RL, Reed R, Robertson CW, Robotkova M, Aguilar MAR, Roy D, Chowdhury PR, Ruan L, Sahoo AK, Sahoo NR, Sako H, Salur S, Sato S, Schaefer BC, Schmidke WB, Schmitz N, Seck FJ, Seger J, Seto R, Seyboth P, Shah N, Shanmuganathan PV, Shao T, Sharma M, Sharma N, Sharma R, Sharma SR, Sheikh AI, Shen D, Shen DY, Shen K, Shi SS, Shi Y, Shou QY, Si F, Singh J, Singha S, Sinha P, Skoby MJ, Smirnov N, Söhngen Y, Song Y, Srivastava B, Stanislaus TDS, Stefaniak M, Stewart DJ, Su Y, Sumbera M, Sun C, Sun X, Sun Y, Sun Y, Surrow B, Svoboda M, Sweger ZW, Tamis AC, Tang AH, Tang Z, Tarnowsky T, Thomas JH, Timmins AR, Tlusty D, Todoroki T, Trentalange S, Tribedy P, Tripathy SK, Truhlar T, Trzeciak BA, Tsai OD, Tsang CY, Tu Z, Tyler J, Ullrich T, Underwood DG, Upsal I, Van Buren G, Vanek J, Vassiliev I, Verkest V, Videbæk F, Voloshin SA, Wang F, Wang G, Wang JS, Wang J, Wang K, Wang X, Wang Y, Wang Y, Wang Y, Wang Z, Webb JC, Weidenkaff PC, Westfall GD, Wielanek D, Wieman H, Wilks G, Wissink SW, Witt R, Wu J, Wu J, Wu X, Wu X, Xi B, Xiao ZG, Xie G, Xie W, Xu H, Xu N, Xu QH, Xu Y, Xu Y, Xu Z, Xu Z, Yan G, Yan Z, Yang C, Yang Q, Yang S, Yang Y, Ye Z, Ye Z, Yi L, Yip K, Yu Y, Zbroszczyk H, Zha W, Zhang C, Zhang D, Zhang J, Zhang S, Zhang W, Zhang X, Zhang Y, Zhang Y, Zhang Y, Zhang Y, Zhang ZJ, Zhang Z, Zhang Z, Zhao F, Zhao J, Zhao M, Zhou J, Zhou S, Zhou Y, Zhu X, Zurek M, and Zyzak M

Abstract

We report a measurement of exclusive J/ψ and ψ(2s) photoproduction in Au+Au ultraperipheral collisions at sqrt[s_{NN}]=200  GeV using the STAR detector. For the first time, (i) the ψ(2s) photoproduction in midrapidity at the Relativistic Heavy-Ion Collider has been experimentally measured; (ii) nuclear suppression factors are measured for both the coherent and incoherent J/ψ production. At average photon-nucleon center-of-mass energy of 25.0 GeV, the coherent and incoherent J/ψ cross sections of Au nuclei are found to be 71±10% and 36±7%, respectively, of that of free protons. The stronger suppression observed in the incoherent production provides a new experimental handle to study the initial-state parton density in heavy nuclei. Data are compared with theoretical models quantitatively.

Published
2024
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14. [Cardiac amyloidosis and aortic stenosis: what do we know?]

Author

Sena G, Ruotolo I, Zaccaro A, Ponziani A, Saturi G, Gagliardi C, Biagini E, and Longhi S

Subjects
Humans, Prognosis, Amyloidosis diagnosis, Amyloidosis therapy, Amyloidosis complications, Cardiomyopathies etiology, Cardiomyopathies therapy, Cardiomyopathies diagnosis, Amyloid Neuropathies, Familial complications, Amyloid Neuropathies, Familial diagnosis, Amyloid Neuropathies, Familial therapy, Echocardiography, Aortic Valve Stenosis etiology, Aortic Valve Stenosis diagnosis, Aortic Valve Stenosis surgery
Abstract

Aortic valve stenosis and cardiac amyloidosis, particularly transthyretin-related, often coexist and share a common clinical and demographic profile. Several pathophysiological hypotheses have been proposed regarding the causes of this association, neither of which fully substantiated in practice. The key to detect the coexistence of cardiac amyloidosis and aortic valve stenosis lies in clinical suspicion. It is possible to hypothesize concurrent cardiac amyloidosis in patients with aortic valve stenosis with the aid of clinical, electrocardiographic, echocardiographic, and extracardiac "red flags". Subsequent non-invasive diagnostic steps are often sufficient to establish a definitive diagnosis. The early diagnosis of this condition is pivotal since the presence of dual pathology worsens patient's prognosis, especially without intervention. Available data on treatment show a better outcome in terms of survival and cardiovascular events in patients undergoing percutaneous correction of valvular heart disease rather than medical therapy alone, regardless of the presence of cardiac amyloidosis. Furthermore, it seems that cardiac amyloidosis does not impact survival after transcatheter aortic valve replacement, even if higher rates of rehospitalizations have been described. Indeed, percutaneous treatment of valvular heart disease is currently considered the primary therapeutic option. Subsequently a disease-modifying treatment for transthyretin amyloidosis may be considered in order to delay disease progression and improve outcomes, even if specific data are still lacking.

Published
2024
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15. Patisiran for the treatment of patients with p.Ile88Leu hereditary transthyretin amyloidosis: an Italian real-life experience.

Author

Urbinati G, Cani I, Currò Dossi M, Longhi S, Carigi S, Gagliardi C, Biagini E, Galiè N, Cortelli P, and Guaraldi P

Abstract

Objectives: Evidence on the activity of patisiran therapy in specific subgroups of patients with hereditary transthyretin amyloidosis variant (ATTRv) is still scarce. This prospective real-world study was designed to provide the first in-depth clinical data on the effectiveness of patisiran in patients with ATTRv reporting the p.Ile88Leu variant, the most widespread variant in the Emilia-Romagna regional area, which has been less represented in previous clinical trials., Patients and Methods: This prospective study evaluated all the patients with genetically proven ATTRv (p.Ile88Leu) and polyneuropathy treated with patisiran in the Emilia-Romagna referral centers for ATTRv (Institute of Neurological Sciences in Bologna and Division of Neurology in Rimini) from March 2021 to April 2023. All subjects underwent clinical and neurological evaluations at baseline and after 9-12 months of treatment., Results: A total of 22 patients were included in the study; the median age was 73 years (IQR: 9), the age at diagnosis was 72 years (IQR: 10), and the disease duration was 1.6 years (IQR: 2.3). We observed stability of all considered neurological and cardiological parameters at 9-12 months after the beginning of patisiran treatment., Conclusion: Our findings support the clinical data regarding the effectiveness of patisiran in stabilizing the disease course and extend this activity to the subset of patients with the p.Ile88Leu variant., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Urbinati, Cani, Currò Dossi, Longhi, Carigi, Gagliardi, Biagini, Galiè, Cortelli and Guaraldi.)

Published
2024
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16. Characterization and natural history of different phenotypes in hereditary transthyretin amyloidosis: 40-year experience at a single Italian referral centre.

Author

Caponetti AG, Sguazzotti M, Accietto A, Saturi G, Ponziani A, Giovannetti A, Massa P, Ruotolo I, Sena G, Zaccaro A, Parisi V, Bonfiglioli R, Guaraldi P, Gagliardi C, Cortelli P, Galie N, Biagini E, and Longhi S

Subjects
Humans, Male, Female, Italy epidemiology, Retrospective Studies, Middle Aged, Aged, Time Factors, Adult, Prognosis, Genetic Predisposition to Disease, Disease Progression, Risk Factors, Amyloid Neuropathies, Familial genetics, Amyloid Neuropathies, Familial mortality, Amyloid Neuropathies, Familial diagnosis, Phenotype, Mutation, Prealbumin genetics
Abstract

Aims: Hereditary transthyretin amyloidosis (ATTRv) is one of the leading aetiologies of systemic amyloidosis with more than 135 mutations described and a broad spectrum of clinical manifestations. We aimed to provide a systematic description of a population of individuals carrying pathogenic mutations of transthyretin (TTR) gene and to investigate the major clinical events during follow-up., Methods and Results: This was an observational, retrospective, cohort study including consecutive patients with mutations of TTR gene, admitted to a tertiary referral centre in Bologna, Italy, between 1984 and 2022. Three hundred twenty-five patients were included: 106 asymptomatic carriers, 49 cardiac phenotype, 49 neurological phenotype, and 121 mixed phenotype. Twenty-two different mutations were found, with Ile68Leu (41.8%), Val30Met (19%), and Glu89Gln (10%) being the most common. After a median follow-up of 51 months, 111 patients (38.3%) died and 9 (11.5%) of the 78 asymptomatic carriers developed ATTRv. Carriers had a prognosis comparable with healthy population, while no significant differences were seen among the three phenotypes adjusted by age. Age at diagnosis, New York Heart Association class III, left ventricular ejection fraction, modified polyneuropathy disability score IV, and disease-modifying therapy were independently associated with survival., Conclusion: This study offers a wide and comprehensive overview of ATTRv from the point of view of a tertiary referral centre in Italy. Three main phenotypes can be identified (cardiac, neurological, and mixed) with specific clinical and instrumental features. Family screening programmes are essential to identify paucisymptomatic affected patients or unaffected carriers of the mutation, to be followed through the years. Lastly, disease-modifying therapy represents an evolving cornerstone of the management of ATTRv, with a great impact on mortality., Competing Interests: Conflict of interest: none declared., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Published
2024
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17. Management of pregnancy in autoimmune rheumatic diseases: maternal disease course, gestational and neonatal outcomes and use of medications in the prospectiveItalian P-RHEUM.it study.

Author

Andreoli L, Gerardi MC, Gerosa M, Rozza D, Crisafulli F, Erra R, Lini D, Trespidi L, Padovan M, Ruffilli F, Serale F, Cuomo G, Raffeiner B, Semeraro P, Tani C, Chimenti MS, Conigliaro P, Hoxha A, Nalli C, Fredi M, Lazzaroni MG, Filippini M, Taglietti M, Franceschini F, Zatti S, Loardi C, Orabona R, Ramazzotto F, Zanardini C, Fontana G, Gozzoli G, Barison C, Bizioli P, Caporali RF, Carrea G, Ossola MW, Maranini B, Silvagni E, Govoni M, Morano D, Verteramo R, Doria A, Del Ross T, Favaro M, Calligaro A, Tonello M, Larosa M, Zen M, Zambon A, Mosca M, Zucchi D, Elefante E, Gori S, Iannone F, Anelli MG, Lavista M, Abbruzzese A, Fasano CG, D'Angelo S, Cutro MS, Picerno V, Carbone T, Padula AA, Rovere-Querini P, Canti V, De Lorenzo R, Cavallo L, Ramoni V, Montecucco C, Codullo V, Milanesi A, Pazzola G, Comitini G, Marvisi C, Salvarani C, Epis OM, Benedetti S, Di Raimondo G, Gagliardi C, Lomater C, Crepaldi G, Bellis E, Bellisai F, Garcia Gonzalez E, Pata AP, Zerbinati M, Urban ML, Mattioli I, Iuliano A, Sebastiani G, Brucato AL, Bizzi E, Cutolo M, Santo L, Tonetta S, Landolfi G, Carrara G, Bortoluzzi A, Scirè CA, and Tincani A

Subjects
Adult, Female, Humans, Infant, Newborn, Pregnancy, Antirheumatic Agents therapeutic use, Antirheumatic Agents adverse effects, Glucocorticoids therapeutic use, Hydroxychloroquine therapeutic use, Hydroxychloroquine adverse effects, Italy epidemiology, Prospective Studies, Autoimmune Diseases epidemiology, Autoimmune Diseases drug therapy, Pregnancy Complications epidemiology, Pregnancy Complications drug therapy, Pregnancy Outcome epidemiology, Rheumatic Diseases drug therapy, Rheumatic Diseases epidemiology, Rheumatic Diseases complications
Abstract

Objectives: To investigate pregnancy outcomes in women with autoimmune rheumatic diseases (ARD) in the Italian prospective cohort study P-RHEUM.it., Methods: Pregnant women with different ARD were enrolled for up to 20 gestational weeks in 29 Rheumatology Centres for 5 years (2018-2023). Maternal and infant information were collected in a web-based database., Results: We analysed 866 pregnancies in 851 patients (systemic lupus erythematosus was the most represented disease, 19.6%). Maternal disease flares were observed in 135 (15.6%) pregnancies. 53 (6.1%) pregnancies were induced by assisted reproduction techniques, 61 (7%) ended in miscarriage and 11 (1.3%) underwent elective termination. Obstetrical complications occurred in 261 (30.1%) pregnancies, including 2.3% pre-eclampsia. Two cases of congenital heart block were observed out of 157 pregnancies (1.3%) with anti-Ro/SSA. Regarding treatments, 244 (28.2%) pregnancies were treated with glucocorticoids, 388 (44.8%) with hydroxychloroquine, 85 (9.8%) with conventional synthetic disease-modifying anti-rheumatic drugs and 122 (14.1%) with biological disease-modifying anti-rheumatic drugs. Live births were 794 (91.7%), mostly at term (84.9%); four perinatal deaths (0.5%) occurred. Among 790 newborns, 31 (3.9%) were small-for-gestational-age and 169 (21.4%) had perinatal complications. Exclusive maternal breast feeding was received by 404 (46.7%) neonates. The Edinburgh Postnatal Depression Scale was compiled by 414 women (52.4%); 89 (21.5%) scored positive for emotional distress., Conclusions: Multiple factors including preconception counselling and treat-to-target with pregnancy-compatible medications may have contributed to mitigate disease-related risk factors, yielding limited disease flares, good pregnancy outcomes and frequency of complications which were similar to the Italian general obstetric population. Disease-specific issues need to be further addressed to plan preventative measures., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Published
2024
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18. Impact of a Discontinuous Training Program on Sedentary Behavior in Italian Type 2 Diabetes Older Patients: The Results of the TRIPL-A Randomized Controlled Trial.

Author

Bonfigli AR, Gagliardi C, Protic O, Lamedica AM, Luconi MP, Turchi F, Tortato E, Di Rosa M, Lucertini F, and Spazzafumo L

Abstract

Background : Physical activity is an important predictor of quality of life in older adults with type 2 diabetes (T2D). Unfortunately, most T2D adults adopt a sedentary lifestyle. The randomized, controlled TRIPL-A trial aims to verify the effect of a personalized, discontinuous exercise program on a sedentary lifestyle of T2D older adults. Methods : A total of 305 T2D patients (mean age ± SD: 68.8 ± 3.3 years) were divided into a control arm receiving only behavioral counseling and an intervention arm of an 18-month supervised discontinuous exercise program (ERS). The primary outcomes were the changes in sitting time (ST) and metabolic equivalent (MET) values, both evaluated by the International Physical Activity Questionnaire short form. A repeated measures ANOVA with Bonferroni correction for multiple comparisons was used to compare study outcomes. Results : The ST and MET differed significantly during the study compared to the control group ( p = 0.028 and p = 0.004, respectively). In the intervention group, a decrease from baseline in ST at 6 months ( p = 0.01) and an increase in MET values at 6 months ( p = 0.01) up to 12 months ( p < 0.01) were found. No significant differences were found for the other variables. Conclusions : Beneficial lifestyle changes were found within the first year of intervention. These results align with the theory of change.

Published
2024
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19. Clinical and Prognostic Implications of Right Ventricular Uptake on Bone Scintigraphy in Transthyretin Amyloid Cardiomyopathy.

Author

Porcari A, Fontana M, Canepa M, Biagini E, Cappelli F, Gagliardi C, Longhi S, Pagura L, Tini G, Dore F, Bonfiglioli R, Bauckneht M, Miceli A, Girardi F, Martini AL, Barbati G, Costanzo EN, Caponetti AG, Paccagnella A, Sguazzotti M, La Malfa G, Zampieri M, Sciagrà R, Perfetto F, Rowczenio D, Gilbertson J, Hutt DF, Hawkins PN, Rapezzi C, Merlo M, Sinagra G, and Gillmore JD

Subjects
Humans, Prealbumin genetics, Prognosis, Tomography, Emission-Computed, Single-Photon, Cardiomyopathies diagnosis
Abstract

Background: The extent of myocardial bone tracer uptake with technetium pyrophosphate, hydroxymethylene diphosphonate, and 3,3-diphosphono-1,2-propanodicarboxylate in transthyretin amyloid cardiomyopathy (ATTR-CM) might reflect cardiac amyloid burden and be associated with outcome., Methods: Consecutive patients with ATTR-CM who underwent diagnostic bone tracer scintigraphy with acquisition of whole-body planar and cardiac single-photon emission computed tomography (SPECT) images from the National Amyloidosis Centre and 4 Italian centers were included. Cardiac uptake was defined according to the Perugini classification: 0=absent cardiac uptake; 1=mild uptake less than bone; 2=moderate uptake equal to bone; and 3=high uptake greater than bone. Extent of right ventricular (RV) uptake was defined as focal (basal segment of the RV free wall only) or diffuse (extending beyond basal segment) on the basis of SPECT imaging. The primary outcome was all-cause mortality., Results: Among 1422 patients with ATTR-CM, RV uptake accompanying left ventricular uptake was identified by SPECT imaging in 100% of cases at diagnosis. Median follow-up in the whole cohort was 34 months (interquartile range, 21 to 50 months), and 494 patients died. By Kaplan-Meier analysis, diffuse RV uptake on SPECT imaging (n=936) was associated with higher all-cause mortality compared with focal (n=486) RV uptake (77.9% versus 22.1%; P <0.001), whereas Perugini grade was not associated with survival ( P =0.27 in grade 2 versus grade 3). On multivariable analysis, after adjustment for age at diagnosis (hazard ratio [HR], 1.03 [95% CI, 1.02-1.04]; P <0.001), presence of the p.(V142I) TTR variant (HR, 1.42 [95% CI, 1.20-1.81]; P =0.004), National Amyloidosis Centre stage (each category, P <0.001), stroke volume index (HR, 0.99 [95% CI, 0.97-0.99]; P =0.043), E/e' (HR, 1.02 [95% CI, 1.007-1.03]; P =0.004), right atrial area index (HR, 1.05 [95% CI, 1.02-1.08]; P =0.001), and left ventricular global longitudinal strain (HR, 1.06 [95% CI, 1.03-1.09]; P <0.001), diffuse RV uptake on SPECT imaging (HR, 1.60 [95% CI, 1.26-2.04]; P <0.001) remained an independent predictor of all-cause mortality. The prognostic value of diffuse RV uptake was maintained across each National Amyloidosis Centre stage and in both wild-type and hereditary ATTR-CM ( P <0.001 and P =0.02, respectively)., Conclusions: Diffuse RV uptake of bone tracer on SPECT imaging is associated with poor outcomes in patients with ATTR-CM and is an independent prognostic marker at diagnosis., Competing Interests: Disclosures The authors report no conflicts in relation to the submitted work. They report the following conflicts outside the submitted work: Dr Rapezzi served on the Italian scientific advisory board of Pfizer and received unrestricted research grants and personal fees from Pfizer and personal fees from Alnylam Pharmaceuticals. Dr Biagini received advisory board fees from Sanofi, Genzyme, and Takeda. Dr Cappelli received advisory board fees from Pfizer and Akcea and research grants from Pfizer. Dr Sinagra received personal fees for occasional educational activities from Biotronik, Boston Scientific, AstraZeneca, and Novartis. Dr Canepa received speaker and advisor fees from Akcea Therapeutics, Menarini, Novartis, Pfizer, Sanofi e Sanofi Genzyme, and Vifor Pharma, as well as 2 investigator-initiated grants from Pfizer. Dr Fontana is supported by a British Heart Foundation intermediate clinical research fellowship (FS/18/21/33447). Dr Merlo received congress fees from Novartis and Vifor Pharma and research grant and congress fees from Pfizer. Dr Gillmore receives advisory board fees from Pfizer, Alnylam, ATTRalus, Intellia, AstraZeneca, and BridgeBio.

Published
2024
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20. New Insights into the Neuropsychological Profile and Intellectual Quotient Variability in Joubert Syndrome Compared to Other Congenital Cerebellar Malformations.

Author

Butti N, Oldrati V, Ferrari E, Romaniello R, Gagliardi C, Borgatti R, and Urgesi C

Subjects
Humans, Cerebellum abnormalities, Abnormalities, Multiple psychology, Kidney Diseases, Cystic genetics, Kidney Diseases, Cystic psychology, Eye Abnormalities psychology, Retina abnormalities, Cerebellar Diseases
Abstract

The neuropsychological characteristics of the cerebellar cognitive affective syndrome (CCAS) in congenital, non-progressive malformations of the cerebellum have been scarcely investigated, and even less is known for Joubert syndrome (JS), an inherited, non-progressive cerebellar ataxia characterized by the so-called molar tooth sign. The few studies on this topic reported inconsistent results about intellectual functioning and specific neuropsychological impairments. The aim of this research is to examine the neuropsychological profile of JS compared to other congenital cerebellar malformations (CM), considering individual variability of intellectual quotient (IQ) in the two groups. Fourteen patients with JS and 15 patients with CM aged 6-25 years were tested through a comprehensive, standardized neuropsychological battery. Their scores in the neuropsychological domains were inspected through descriptive analysis and compared by mean of MANOVA and ANOVA models, then replicated inserting IQ as covariate. The two groups showed a largely overlapping neuropsychological profile, consistent with CCAS. However, the JS group showed worse performance in visual-spatial memory compared to CM patients, although this difference was mitigated when considering IQ. These findings highlight a divergence between JS and other CM in visual-spatial memory, which might suggest a critical role of the cerebellum in recalling task-relevant memories and might inform rehabilitative interventions., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Published
2024
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21. Mystery of the memory engram: History, current knowledge, and unanswered questions.

Author

Lopez MR, Wasberg SMH, Gagliardi CM, Normandin ME, and Muzzio IA

Subjects
Humans, Brain physiology, Neurons physiology
Abstract

The quest to understand the memory engram has intrigued humans for centuries. Recent technological advances, including genetic labelling, imaging, optogenetic and chemogenetic techniques, have propelled the field of memory research forward. These tools have enabled researchers to create and erase memory components. While these innovative techniques have yielded invaluable insights, they often focus on specific elements of the memory trace. Genetic labelling may rely on a particular immediate early gene as a marker of activity, optogenetics may activate or inhibit one specific type of neuron, and imaging may capture activity snapshots in a given brain region at specific times. Yet, memories are multifaceted, involving diverse arrays of neuronal subpopulations, circuits, and regions that work in concert to create, store, and retrieve information. Consideration of contributions of both excitatory and inhibitory neurons, micro and macro circuits across brain regions, the dynamic nature of active ensembles, and representational drift is crucial for a comprehensive understanding of the complex nature of memory., Competing Interests: Declaration of Competing Interest The authors do not have conflicts of interest., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Published
2024
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22. Preact to lower the risk of falling by customized rehabilitation across Europe: the feasibility study protocol of the PRECISE project in Italy.

Author

D'Ambrosio F, Harbo M, Contiero D, Bonfigli AR, Cicconi D, Heuer N, Roos A, Fischer Pedersen C, Fabbietti P, and Gagliardi C

Subjects
Aged, Humans, Artificial Intelligence, Europe, Feasibility Studies, Italy, User-Computer Interface, Clinical Trials as Topic, Accidental Falls prevention & control, Activities of Daily Living
Abstract

Introduction: Falls are a major worldwide health problem in older people. Several physical rehabilitation programs with home-based technologies, such as the online DigiRehab platform, have been successfully delivered. The PRECISE project combines personalized training delivered through the application with an artificial intelligence-based predictive model (AI-DSS platform) for fall risk assessment. This new system, called DigiRehab, will enable early identification of significant risk factors for falling and propose an individualized physical training plan to attend to these critical areas., Methods: The study will test the usability of the DigiRehab platform in generating personalized physical rehabilitation programs at home. Fifty older adults participants will be involved, 20 of them testing the beta version prototype, and 30 participants testing the updated version afterwards. The inclusion criteria will be age ≥65, independent ambulation, fall risk (Tinetti test), Mini Mental State Examination ≥24, home residents, familiarity with web applications, ability and willingness to sign informed consent. Exclusion criteria will be unstable clinical condition, severe visual and/or hearing impairment, severe impairment in Activities of Daily Living and absence of primary caregiver., Discussion: The first part of the screening consists in a structured questionnaire of 10 questions regarding the user's limitations, including the risk of falling, while the second consists in 10 physical tests to assess the functional status. Based on the results, the program will help define the user's individual profile upon which the DSS platform will rate the risk of falling and design the personalized exercise program to be carried out at home. All measures from the initial screening will be repeated and the results will be used to optimize the predictive algorithms in order to prepare the tool in its final version. For the usability assessment, the System Usability Scale will be administered. The follow-up will take place after the 12-week intervention at home. A semi-structured satisfaction questionnaire will also be administered to verify whether the project will meet the needs of older adults and their family caregiver., Conclusion: We expect that personalized training prescribed by DigiRehab platform could help to reduce the need for care in older adults subjects and the care burden. Clinical trial registration : [https://clinicaltrials.gov/], identifier [NCT05846776]., Competing Interests: MH and NH were employed by DigiRehab A/S. AR is employed by DigiRehab Benelux, B.V. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 D’Ambrosio, Harbo, Contiero, Bonfigli, Cicconi, Heuer, Roos, Fischer Pedersen, Fabbietti and Gagliardi.)

Published
2024
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