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5. Long-term safety and efficacy of patisiran for hereditary transthyretin-mediated amyloidosis with polyneuropathy: 12-month results of an open-label extension study

Author

Vita, Giuseppe, Rizzo, Vincenzo, Russo, Massimo, Mazzeo, Anna, Gentile, Luca, Berk, John L, Brueckner, Caitlin, Lazzari, Victoria, Wiesman, Janice, DeLong, Douglas, Victory, Jennifer, Dalton, James, May, John, Gilmore, Catherine, Attarian, Shahram, Diallo, Saran, Delmont, Emilien, Pouget, Jean, Verschueren, Annie, Grapperon, Aude-Marie, Campana-Salort, Emmanuelle, Conceição, Isabel M, Lopes, Ana, Lamas, Filipa, Neves, Carlos, Castro, Jose, Pereira, Pedro, Castro, Isabel, Franco, Ana, Santos, Miguel Oliveira, de Azevedo Coutinho, Conceição, Falcao de Campos, Catarina, Coelho, Teresa, Hipólito Reis, Antonio, Correia, Nuno, Perez, Javier M, Martins da Silva, Ana, Alves, Cristina, Cardoso, Marcio, Valdrez, Katia, Monte, Julia R, Pessoa, Bernardete, Guimaraes, Nadia, Freitas, Monica, Ramalho, Joana, Ferreira, Natalia, Kuzume, Daisuke, Tard, Celine, Waucquier, Nawal, Rougeaux, Isabelle, Brice, Sylvie, Kasprzyk, Emmanuelle, Elrezzi, Elise, Meguig, Sayah, Hachulla, Eric, Gauvain, Clement, Migaud-Chervy, Maria-Claire, Deplanque, Dominique, Jozefowicz, Elsa, Lebellec, Loic, Adams, David, Balaya-Gouraya, Line, Jehan Lacour, Nathalie, Bournane, Halima, Martin, Nathalie, Elabed, Mongia, Sacko, Niamey, Boubrit, Yasmine, Gaouar, Amina, Rakotondratafika, Fetra, Théaudin-Saliou, Marie, Cauquil-Michon, Cécile, Labeyrie, Celine, Not, Adeline, Al-Salameh, Abdallah, Lecoq, Anne-Lise, Stephant, Maeva, Echaniz-Laguna, Andoni, Becquemont, Laurent, Beaudonnet, Guillemette, Algalarrondo, Vincent, Eliahou, Ludivine, Slama, Michel S, Rousseau, Antoine, Signate, Aissatou, Berthelot, Emeline, Inamo, Jocelyn, Planté-Bordeneuve, Violaine, Vervoitte, Laetitia, Focseneanu, Cecile, Gendre, Thierry, Arrouasse, Raphaele, Ayache, Samar S., Ernande, Laura, Le Corvoisier, Philippe, Salhi, Hayet, Choumert, Ariane, Ehinger, Vincent, Ruiz, Julie, Charlin, Cyril, Megelin, Thomas, Brannagan III, Thomas H, Fayerman, Raisy, Kim, Arreum, Paras, Allan, Gonzalez, Leidy J, Tsang, Steven, Wajnsztajn, Fernanda, Shije, Jeffrey, Ulane, Christina, Kleyman, Inna, Weimer, Louis, Cioroiu, Comana, Lambrianides, Sakis, Abu-Manneh, Rana, Zamba-Papanicolaou, Eleni, Agathangelou, Petros, Leonidou, Eleni, Tada, Satoshi, Fujita, Akemi, Nagai, Masahiro, Ando, Rina, Hosokawa, Yuko, Yamanishi, Yuki, Overcash, J. Scott, Giardino, Elena, Boyer, Leslie, Dang, Lien, Le, An, Nguyen, Tyler, Giang, Lien, Sellers, Peter, Tran, Leyla, Truong, Nghi, Vinas, Maita, Hrkman, Nicole, Miller, Sarah, Nguyen, David, Smith, Ashley, Pu, Helen, Li, Steve, Vuong, Thao, Dioso, Holly, Green, Sinikka, Lee, Kia, Chu, Hanh, Waters, Michael, Coskun, Derya J, Zepeda, Karla A, O'Riordan, William, Obici, Laura, Cortese, Andrea, Lozza, Alessandro, Merlini, Giampaolo, Rosti, Vittorio, Sabatelli, Mario, Bisogni, Giulia, Bernardo, Daniela, Luigetti, Marco, Di Paolantonio, Andrea, Guglielmino, Valeria, Romano, Angela, Nienhuis, Hans, Bulthuis-Kuiper, Janita, Kristen, Arnt V, Gerk, Olga, Ulbricht, Hannah, Taylor, Lenka, Meyle, Eva, Kleinschmidt, Natalia, Meyrath, David, Noe-Schwenn, Simone, Meng, Ulrike, Bauer, Ralf, aus dem Siepen, Fabian, Hein, Selina, Takahashi, Tetsuya, Oshita, Tomohiko, Koujin, Yoko, Neshige, Shuichiro, Nezu, Tomohisa, Segawa, Akiko, Ueno, Hiroki, Morino, Hiroyuki, Campistol, Josep M, Rodas Marin, Lida Maria, Blasco Pelicano, Josep Miquel, Dávila, Lucía Galán, Palacios, Marta, Pytel Cordoba, Vanesa, Guerrero Sola, Antonio, Horga, Alejandro, García Feijoo, Julián, Perez de Isla, Leopoldo, Marques Júnior, Wilson, Moscardini, Mariana, Litcanov, Debora Cristina, Viera Lima, Ana Flavia, Rodrigues, Leonardo, Marques Coutinho, Barbara, Moreira, Carolina Lavigne, Daccach Marques, Vanessa, Munoz Beamud, Francisco, Gragera Martínez, Álvaro, Borrachero, Cristina, Losada López, Inés Asunción, Cisneros Barroso, Eugenia, Rodríguez Rodríguez, Adrián, Sanz, Monica, Rigo Oliver, Elena, González Moreno, Juan, Gamez Martinez, Jose M, Descals, Cristina, Uson, Mercedes, Jose Vega, Francisco, Figuerola, Antoni, Montala, Carles, Waddington-Cruz, Márcia, Dias da Silva, Moises, Gervais de Santa Rosa, Renata, Pinto, Luiz Felipe, Pinto, Marcus Vinicius, Cardoso Berensztejn, Amanda, Barroso, Fabio, Lautre, Andrea, Orellana, Lucas G, González-Duarte Briseño, Maria Alejandra, Cárdenas-Soto, Karla, Jiménez López, Brenda Poled, Pérez-Castañeda, Sandra Lorena, Cantú Brito, Carlos Gerardo, Rivera de la Parra, David, Hernandez Reyes, Jose Pablo, del Mar Saniger Alba, Maria, Criollo Mora, Elia, Parman, Yesim, Rezzan, Kus Jülide, Sahin, Erdi, Serbest, Nail G, Durmus, Hacer, Cakar, Arman, Tugal Tutkun, Nuriye Ilknur, Karamursel, Sacit, Elitok, Ali, Sirin Inan, Nermin G, Altinkurt, Emre, Polydefkis, Michael, Ye, Jing, Allen, Adriane C, Chaudhry, Vinay, Jarrett, Raquel, Bressler, Neil, Burks, Kathleen L, Liu, Qingfeng, Khoshnoodi, Mohammad, Judge, Daniel P, Vista, Geno, Shah, Syed Mahmood, Hamaguchi, Hirotoshi, Oda, Junko, Fukase, Emi, Taniguchi, Ikuko, Oda, Tetsuya, Endo, Hironobu, Shimomura, Masahiro, Katanazaka, Kimitaka, Koto, Shusuke, Nakano, Takahiro, Scheid, Christof, Zueiter, Andreas, Pester, Lars, Walter, Doreen, Özdemir, Betül, Frenzel, Lukas F, Holtick, Udo, Oh, Jeeyoung, Kim, Hee Jin, Shin, Hyun Jin, Choi, Kyomin, Yamashita, Taro, Ueda, Mitsuharu, Masuda, Teruaki, Misumi, Yohei, Ueda, Akihiko, Nakahara, Keiichi, Yorita, Akiko, Tsuruhisa, Seiko, Taniwaki, Takayuki, Harada, Masaya, Moritaka, Taiga, Sakurada, Naonori, Mauricio, Elizabeth A, Baskin, Amber, Dimberg, Elliot, Dispenzieri, Angela, Fonder, Amie, Hobbs, Miriam, Russell, Stephen J, Dyck, Peter, Gonsalves, Wilson, Leung, Nelson, Witzig, Thomas E, Zeldenrust, Steven R, Hwa, Lisa, Kapoor, Prashant, Kumar, Shaji K, Lin, Yi, Lust, John A, Rajkumar, Vincent S, Dingli, David, Gertz, Morie A, Go, Ronald, Hayman, Suzanne R, Dalia, Samir, Carrillo, Esmeralda, Gorevic, Peter, Mason, Garnette, Chao, Chi-Chao, Lee, Ming-Jen, Su, Jen-Jen, Hsieh, Sung-Tsang, Tsai, Li-Kai, Yeh, Shin-Joe, Yang, Chih-Chao, Ajroud-Driss, Senda Ajroud-Driss, Casey, Patricia, Joslin, Benjamin C, Freimer, Miriam, Sankey, Alison, Kenepp, Amanda, Heintzman, Sarah, LoRusso, Samantha, Hokezu, Youichi, Kim, Byoung-Joon, Kim, JuHyeon, Lee, Ga Yeon, Cho, Eun Bin, Jeon, Eun-Seok, Min, Ju-Hong, Seok, Jin Myoung, Lee, Hye Lim, Park, Jae Hong, Sekijima, Yoshiki, Miyazawa, Chinatsu, Kato, Nagaaki, Kishida, Dai, Hineno, Akiyo, Kodaira, Minori, Yoshinaga, Tsuneaki, Miyahara, Teruyoshi, Imai, Akira, Matsumoto, Kazuhiko, Lin, Kon-Ping, Lee, Yi-Chung, Wixner, Jonas, Falk, Malin, Pilebro, Bjorn, Suhr, Ole, Lindqvist, Per, Soderberg, Karin, Pedrosa-Domellöf, Fatima, Anan, Intissar, Nordh, Erik, Tournev, Ivaylo, Zhelyazkova-Glaveeva, Sashka, Cherneva, Zheyna, Sarafov, Staiko, Chamova, Teodora, Cherninkova-Gopina, Sylvia, Schmidt, Hartmut H, Friebel, Frauke, Zibert, Andree, Mihailovic, Natasa, Schubert, Friederike, Vorona, Elena, Lahme, Larissa, Huesing-Kabar, Anna, Schilling, Matthias, Kabar, Iyad, Gillmore, Julian D, Martinez-Naharro, Ana, Chacko, Liza, Cohen, Oliver, Law, Steven, Rezk, Tamer, Lachmann, Helen J, Quan, Dianna, Blume, Brianna, Dixon, Stacy, Low, Soon Chai, Chan, Soo Looi, Lim, He Eng Li, Goh, Khean Jin, Mezei, Michelle M, Kraus, Deborah, Jack, Kristin, Wade, N. Kevin, Lopate, Glenn, Zwijack, Brittany, Florence, Julaine, Sommerville, R. Brian, Stewart, Graeme, Ryder, Julie, Mekhael, Linda, Taylor, Mark, Suan, Daniel, Wells, Karen, Stone, Paula, Itoya, Amenze, Owusu-Sekyere, Mercy, Thai, Desmond, Chahine, Ilonah, Pedrosa, Salve, Do, Thi Hoa (Therese), González-Duarte, Alejandra, Kyriakides, Theodoros, Ajroud-Driss, Senda, Mauricio, Elizabeth, Brannagan, Thomas H, III, Aldinc, Emre, Wang, Jing Jing, White, Matthew T, Vest, John, Berber, Erhan, and Sweetser, Marianne T

Published
2021
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10. Conventional heart failure therapy in cardiac ATTR amyloidosis

Author

Ioannou, Adam, primary, Massa, Paolo, additional, Patel, Rishi K, additional, Razvi, Yousuf, additional, Porcari, Aldostefano, additional, Rauf, Muhammad U, additional, Jiang, Anita, additional, Cabras, Giacomo, additional, Filisetti, Stefano, additional, Bolhuis, Roos E, additional, Bandera, Francesco, additional, Venneri, Lucia, additional, Martinez-Naharro, Ana, additional, Law, Steven, additional, Kotecha, Tushar, additional, Virsinskaite, Ruta, additional, Knight, Daniel S, additional, Emdin, Michele, additional, Petrie, Aviva, additional, Lachmann, Helen, additional, Wechelakar, Ashutosh, additional, Petrie, Mark, additional, Hughes, Alun, additional, Freemantle, Nick, additional, Hawkins, Philip N, additional, Whelan, Carol, additional, McMurray, John J V, additional, Gillmore, Julian D, additional, and Fontana, Marianna, additional

Published
2023
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11. Tc-99m labelled bone scintigraphy in suspected cardiac amyloidosis

Author

Rauf, Muhammad Umaid, primary, Hawkins, Philip N, additional, Cappelli, Francesco, additional, Perfetto, Federico, additional, Zampieri, Mattia, additional, Argiro, Alessia, additional, Petrie, Aviva, additional, Law, Steven, additional, Porcari, Aldostefano, additional, Razvi, Yousuf, additional, Bomsztyk, Joshua, additional, Ravichandran, Sriram, additional, Ioannou, Adam, additional, Patel, Rishi, additional, Starr, Neasa, additional, Hutt, David F, additional, Mahmood, Shameem, additional, Wisniowski, Brendan, additional, Martinez–Naharro, Ana, additional, Venneri, Lucia, additional, Whelan, Carol, additional, Roczenio, Dorota, additional, Gilbertson, Janet, additional, Lachmann, Helen J, additional, Wechalekar, Ashutosh D, additional, Rapezzi, Claudio, additional, Serenelli, Matteo, additional, Massa, Paolo, additional, Caponetti, Angelo Giuseppe, additional, Ponziani, Alberto, additional, Accietto, Antonella, additional, Giovannetti, Alessandro, additional, Saturi, Giulia, additional, Sguazzotti, Maurizio, additional, Gagliardi, Christian, additional, Biagini, Elena, additional, Longhi, Simone, additional, Fontana, Marianna, additional, and Gillmore, Julian D, additional

Published
2023
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12. Cardiac transplantation in transthyretin amyloid cardiomyopathy: Outcomes from three decades of tertiary center experience

Author

Razvi, Yousuf, primary, Porcari, Aldostefano, additional, Di Nora, Concetta, additional, Patel, Rishi K., additional, Ioannou, Adam, additional, Rauf, Muhammad U., additional, Masi, Ambra, additional, Law, Steven, additional, Chacko, Liza, additional, Rezk, Tamer, additional, Ravichandran, Sriram, additional, Gilbertson, Janet, additional, Rowczenio, Dorota, additional, Blakeney, Iona J., additional, Kaza, Nandita, additional, Hutt, David F., additional, Lachmann, Helen, additional, Wechalekar, Ashutosh, additional, Moody, William, additional, Lim, Sern, additional, Chue, Colin, additional, Whelan, Carol, additional, Venneri, Lucia, additional, Martinez-Naharro, Ana, additional, Merlo, Marco, additional, Sinagra, Gianfranco, additional, Livi, Ugolino, additional, Hawkins, Philip, additional, Fontana, Marianna, additional, and Gillmore, Julian D., additional

Published
2023
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15. Long‐term outcomes in light chain deposition disease‐analysis of a UK cohort

Author

Ravichandran, Sriram, primary, Law, Steven, additional, Mahmood, Shameem, additional, Wisniowski, Brenden, additional, Foard, Darren, additional, Fontana, Marianna, additional, Martinez‐Naharro, Ana, additional, Whelan, Carol, additional, Hawkins, Philip N., additional, Gillmore, Julian D., additional, Lachmann, Helen J., additional, and Wechalekar, Ashutosh D., additional

Published
2022
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16. An Integrative Suicide Prevention Program for Visitor Charcoal Burning Suicide and Suicide Pact

Author

Wong, Paul W. C., Liu, Patricia M. Y., Chan, Wincy S. C., Law, Y. W., Law, Steven C. K., Fu, King-Wa, Li, Hana S. H., Tso, M. K., Beautrais, Annette L., and Yip, Paul S. F.

Abstract

An integrative suicide prevention program was implemented to tackle an outbreak of visitor charcoal burning suicides in Cheung Chau, an island in Hong Kong, in 2002. This study evaluated the effectiveness of the program. The numbers of visitor suicides reduced from 37 deaths in the 51 months prior to program implementation to 6 deaths in the 42 months post-implementation period. The number of visitor suicide pacts decreased from 7 pacts (15 individuals) to 1 pact (2 individuals). No statistically significant differences in the numbers of visitor suicide attempts and resident suicides were observed in the two time periods. No statistically significant changes in visitor suicides during the study period were observed on the comparison islands. The consistency and timing of reduction in visitor suicides correlated with the development and delivery of the integrative program on the intervention island, suggesting a causal association between program delivery and reduction of visitor suicides. The possibility of displacement seems small because there was no increase in visitor suicides on the comparison islands during the study period. This integrative approach in preventing target-specific suicides may serve as an example for other communities to develop suicide prevention programs that make use of the existing local resources.

Published
2009
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17. Cardiovascular magnetic resonance in light-chain amyloidosis to guide treatment

Author

Martinez-Naharro, Ana, primary, Patel, Rishi, additional, Kotecha, Tushar, additional, Karia, Nina, additional, Ioannou, Adam, additional, Petrie, Aviva, additional, Chacko, Liza A, additional, Razvi, Yousuf, additional, Ravichandran, Sriram, additional, Brown, James, additional, Law, Steven, additional, Quarta, Cristina, additional, Mahmood, Shameem, additional, Wisniowski, Brendan, additional, Pica, Silvia, additional, Sachchithanantham, Sajitha, additional, Lachmann, Helen J, additional, Moon, James C, additional, Knight, Daniel S, additional, Whelan, Carol, additional, Venneri, Lucia, additional, Xue, Hui, additional, Kellman, Peter, additional, Gillmore, Julian D, additional, Hawkins, Philip N, additional, Wechalekar, Ashutosh D, additional, and Fontana, Marianna, additional

Published
2022
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18. Prognostic importance of the 6 min walk test in light chain (AL) amyloidosis

Author

Cohen, Oliver C, primary, Sathyanath, Ananth, additional, Petrie, Aviva, additional, Ravichandran, Sriram, additional, Law, Steven, additional, Manwani, Richa, additional, Foard, Darren, additional, Sachchithanantham, Sajitha, additional, Mahmood, Shameem, additional, Martinez-Naharro, Ana, additional, Fontana, Marianna, additional, Whelan, Carol J, additional, Hawkins, Philip N, additional, Lachmann, Helen J, additional, Gillmore, Julian D, additional, and Wechalekar, Ashutosh D, additional

Published
2022
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19. Characteristics and natural history of early-stage cardiac transthyretin amyloidosis

Author

Law, Steven, primary, Bezard, Melanie, additional, Petrie, Aviva, additional, Chacko, Liza, additional, Cohen, Oliver C, additional, Ravichandran, Sriram, additional, Ogunbiyi, Olabisi, additional, Kharoubi, Mounira, additional, Ganeshananthan, Sashiananthan, additional, Ganeshananthan, Sharmananthan, additional, Gilbertson, Janet A, additional, Rowczenio, Dorota, additional, Wechalekar, Ashutosh, additional, Martinez-Naharro, Ana, additional, Lachmann, Helen J, additional, Whelan, Carol J, additional, Hutt, David F, additional, Hawkins, Philip N, additional, Damy, Thibaud, additional, Fontana, Marianna, additional, and Gillmore, Julian D, additional

Published
2022
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20. The experience of hereditary apolipoprotein A-I amyloidosis at the UK National Amyloidosis Centre

Author

Cohen, Oliver C., primary, Blakeney, Iona J., additional, Law, Steven, additional, Ravichandran, Sriram, additional, Gilbertson, Janet, additional, Rowczenio, Dorota, additional, Mahmood, Shameem, additional, Sachchithanantham, Sajitha, additional, Wisniowski, Brendan, additional, Lachmann, Helen J., additional, Whelan, Carol J., additional, Martinez-Naharro, Ana, additional, Fontana, Marianna, additional, Hawkins, Philip N., additional, Gillmore, Julian D., additional, and Wechalekar, Ashutosh D., additional

Published
2022
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21. Patisiran treatment in patients with hereditary transthyretin-mediated amyloidosis with polyneuropathy after liver transplantation

Author

Schmidt, Hartmut, Wixner, Jonas, Planté-Bordeneuve, Violaine, Muñoz-Beamud, Francisco, Lladó, Laura, Gillmore, Julian D., Mazzeo, Anna, Li, Xingyu, Arum, Seth, Jay, Patrick Y., Adams, David, Langestroer, Christel, Huesing-Kabar, Anna, Schilling, Matthias, Kabar, Iyad, Backlund, Rolf, Anan, Intissar, Nordh, Erik, Uneus, Erika, Pilebro, Björn, Englund, Ulrika, Coelho, Teresa, Novais, Marta, Perez, Javier, Martins da Silva, Ana, Pesseguerio Miranda, Helena, Ramalho, Joana, Monte, Raquel, Alves, Cristina, Cardaso, Ines, Guimaraes, Nádia, Gentile, Luca, Russo, Massimo, Di Bella, Gianluca, Gaouar, Amina, Cauquil-Michon, Cécile, Kounis, Ilias, Echaniz-Laguna, Andoni, Stéphant, Maëva, Rakotondratafika, Fetra, Boubrit, Yasmine, Labeyrie, Celine, Focsenaunu, Cecile, Le Corvoisier, Phillippe, Ayache, Samar S., Gendre, Thierry, Vervoitte, Laetitia, Arrouasse, Raphaele, Gragera Martinez, Alvaro, Borrachero, Cristina, Manovel, Ana, Diaz Rodriguez, Eusebio, Gutiérrez Gándara, Marta, Fabra Jiménez, Elena, Valentina Vélez Santamaría, Patricia, Martínez Vilar, Yurema, Cachero, Alba, Rannigan, Lisa, Fontana, Marianna, Orrell, Richard, Louth, Sarah, Chacko, Liza, Varughese, Sindhu, Throburn, Douglas, Cohen, Oliver, Law, Steven, Smit, Angelique, and Strehina, Svetla

Subjects
liver allograft function/dysfunction, Neurologi, Molecular biology, Medizin, Trasplantament hepàtic, clinical research/practice, Polyneuropathies, Clinical trials, patient survival, small interfering RNA [molecular biology], Immunology and Allergy, Humans, Prealbumin, Pharmacology (medical), RNA, Small Interfering, Biologia molecular, Transplantation, Amyloid Neuropathies, Familial, clinical trial, Amyloidosis, Liver Transplantation, Neurology, Quality of Life, Amiloïdosi, pharmacology, Hepatic transplantation, liver transplantation/hepatology, Assaigs clínics
Abstract

Hereditary transthyretin-mediated (hATTR) amyloidosis, or ATTRv amyloidosis, is a progressive disease, for which liver transplantation (LT) has been a long-standing treatment. However, disease progression continues post-LT. This Phase 3b, open-label trial evaluated efficacy and safety of patisiran in patients with ATTRv amyloidosis with polyneuropathy progression post-LT. Primary endpoint was median transthyretin (TTR) reduction from baseline. Twenty-three patients received patisiran for 12 months alongside immunosuppression regimens. Patisiran elicited a rapid, sustained TTR reduction (median reduction [Months 6 and 12 average], 91.0%; 95% CI: 86.1%–92.3%); improved neuropathy, quality of life, and autonomic symptoms from baseline to Month 12 (mean change [SEM], Neuropathy Impairment Score, −3.7 [2.7]; Norfolk Quality of Life-Diabetic Neuropathy questionnaire, −6.5 [4.9]; least-squares mean [SEM], Composite Autonomic Symptom Score-31, −5.0 [2.6]); and stabilized disability (Rasch-built Overall Disability Scale) and nutritional status (modified body mass index). Adverse events were mild or moderate; five patients experienced ≥1 serious adverse event. Most patients had normal liver function tests. One patient experienced transplant rejection consistent with inadequate immunosuppression, remained on patisiran, and completed the study. In conclusion, patisiran reduced serum TTR, was well tolerated, and improved or stabilized key disease impairment measures in patients with ATTRv amyloidosis with polyneuropathy progression post-LT (www.clinicaltrials.gov NCT03862807). in press

Published
2022

22. The effect of changing dialysate bicarbonate concentration on serum bicarbonate, body weight and normalized nitrogen appearance rate.

Author

Law, Steven and Davenport, Andrew

Subjects
*BICARBONATE ions, *BODY weight, *SERUM albumin, *NITROGEN, *ACID-base imbalances, *MULTIVARIATE analysis
Abstract

Introduction: Most hemodialysis machines deliver a fixed bicarbonate concentration. Higher concentrations may improve acidosis, but risk post‐hemodialysis alkalosis, whereas lower concentrations potentially increase acidosis but reduce alkalosis. We reviewed the effects of lowering dialysate bicarbonate. Methods: We reviewed peri‐dialysis chemistries in patients switching to a lower bicarbonate dialysate at 4 time points over 19 months. Results: We studied 126 patients, mean age 63.7 ± 16.3 years, 57.9% males. Post‐hemodialysis alkalosis fell from 1.6 to 0.3% sessions, but pre‐hemodialysis acidosis increased from 11.9 to 23.8% sessions (p = 0.005) reducing dialysate bicarbonate from 32 to 28 mmol/L. After 3 months, pre‐hemodialysis serum bicarbonate fell (21.1 ± 2.3 to 19.8 ± 2.2 mmol/L), and post‐hemodialysis (24.9 ± 2.1 to 22.5 ± 2.0 mmol/L, p < 0.001) with a fall in pre‐hemodialysis weight from 74.6 ± 20.7 to 71.7 ± 18.2 kg, normalized protein nitrogen accumulation rate 0.8 ± 0.28 to 0.77 ± 0.2 g/kg/day, p < 0.05, and serum albumin 39.7 ± 4.2 to 37.7 ± 4.9 g/L, p < 0.001. Thereafter, apart from pre‐ and post‐hemodialysis serum bicarbonate, weight and normalized protein nitrogen accumulation stabilized, although albumin remained lower (37.6 ± 4.0 g/L, p < 0.001). On multivariate logistic analysis, serum bicarbonate increased more with lower pre‐hemodialysis bicarbonate standardized coefficient β 0.5 (95% confidence interval −0.6 to −0.42), increased normalized protein nitrogen accumulation β 0.2 (0.96 to 2.38), p < 0.001, and session time β 0.09, (0.47 to 5.98), p < 0.022, and less with lower dialysate bicarbonate 0.0–0.23 (−1.54 to −0.74), p < 0.001. Conclusion: Increases in SE‐Bic with hemodialysis, depend on the bicarbonate gradient, session time and nPNA. Lower D‐Bic reduces post‐hemodialysis alkalosis but increases pre‐hemodialysis acidosis and may initially have adverse effects on weight and normalized protein nitrogen accumulation. [ABSTRACT FROM AUTHOR]

Published
2023
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23. P-024: Early relapse is an adverse prognostic marker in systemic immunoglobulin light chain (AL) Amyloidosis

Author

Ravichandran, Sriram, primary, Law, Steven, additional, Mahmood, Shameem, additional, Wisniowski, Brendan, additional, Foard, Darren, additional, Fontana, Marianna, additional, Martinez-Naharro, Ana, additional, Whelan, Carol, additional, Gillmore, Jullian, additional, Lachmann, Helen, additional, Hawkins, Philip, additional, and Wechalekar, Ashutosh, additional

Published
2021
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24. Longitudinal strain is an independent predictor of survival and response to therapy in patients with systemic AL amyloidosis

Author

Cohen, Oliver C, primary, Ismael, Andreia, additional, Pawarova, Babita, additional, Manwani, Richa, additional, Ravichandran, Sriram, additional, Law, Steven, additional, Foard, Darren, additional, Petrie, Aviva, additional, Ward, Sevda, additional, Douglas, Brooke, additional, Martinez-Naharro, Ana, additional, Chacko, Liza, additional, Quarta, Candida Cristina, additional, Mahmood, Shameem, additional, Sachchithanantham, Sajitha, additional, Lachmann, Helen J, additional, Hawkins, Philip N, additional, Gillmore, Julian D, additional, Fontana, Marianna, additional, Falk, Rodney H, additional, Whelan, Carol J, additional, and Wechalekar, Ashutosh D, additional

Published
2021
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25. Focal Segmental Glomerulosclerosis Complicating Therapy With Inotersen, an Antisense Oligonucleotide Inhibitor: A Case Report

Author

Law, Steven, Arnold, Julia, Rauf, Muhammad U., Heptinstall, Lauren, Gilbertson, Janet, Rowczenio, Dorota, Baharani, Jyoti, Langman, Gerald, Fontana, Marianna, and Gillmore, Julian D.

Abstract

Inotersen is an antisense oligonucleotide inhibitor licensed for the treatment of polyneuropathy complicating hereditary transthyretin amyloidosis (ATTRv). Nephrotoxicity has been reported with inotersen, including progression to kidney failure. We describe what is to our knowledge the first reported case of inotersen-associated nephrotic syndrome secondary to focal segmental glomerulosclerosis (FSGS) and review the literature concerning inotersen-induced nephrotoxicity. We report a woman in her early 30s with ATTRv associated with the V50M transthyretin (TTR) variant, who presented with nephrotic syndrome 7 months after commencement of inotersen. Renal histology demonstrated FSGS and scanty glomerular amyloid deposition. Discontinuation of inotersen alone resulted in complete clinical and biochemical resolution of nephrotic syndrome. Inotersen is associated with significant nephrotoxicity. In the phase 3 NEURO-TTR clinical trial, 3% of patients in the treatment arm developed a crescentic glomerulonephritis. All affected patients carried the V50M TTR variant, which is known to be associated with renal amyloid deposition. This case adds to the spectrum of kidney disease associated with inotersen and indicates that discontinuation of the drug alone may result in resolution of renal complications without additional immunosuppression. Monitoring of kidney function is essential in patients with ATTRv receiving inotersen, particularly if there is evidence of existing renal amyloid.

Published
2023
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27. Haematologic responses and survival do not significantly decrease with subsequent lines of therapy in systemic immunoglobulin light chain amyloidosis: results from an analysis of real‐world longitudinal data

Author

Ravichandran, Sriram, primary, Cohen, Oliver C., additional, Law, Steven, additional, Sachchithanantham, Sajitha, additional, Mahmood, Shameem, additional, Foard, Darren, additional, Fontana, Marianna, additional, Martinez‐Naharro, Ana, additional, Whelan, Carol, additional, Gillmore, Julian D., additional, Lachmann, Helen J., additional, Hawkins, Philip N., additional, and Wechalekar, Ashutosh D., additional

Published
2021
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28. Change in N-terminal pro-B-type natriuretic peptide at 1 year predicts mortality in wild-type transthyretin amyloid cardiomyopathy

Author

Law, Steven, primary, Petrie, Aviva, additional, Chacko, Liza, additional, Cohen, Oliver C, additional, Ravichandran, Sriram, additional, Gilbertson, Janet A, additional, Rowczenio, Dorota, additional, Wechalekar, Ashutosh D, additional, Martinez-Naharro, Ana, additional, Lachmann, Helen J, additional, Whelan, Carol J, additional, Hutt, David F, additional, Hawkins, Philip N, additional, Fontana, Marianna, additional, and Gillmore, Julian D, additional

Published
2021
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29. Cardiac Magnetic Resonance–Derived Extracellular Volume Mapping for the Quantification of Hepatic and Splenic Amyloid

Author

Chacko, Liza, primary, Boldrini, Michele, additional, Martone, Raffaele, additional, Law, Steven, additional, Martinez-Naharrro, Ana, additional, Hutt, David F., additional, Kotecha, Tushar, additional, Patel, Rishi K., additional, Razvi, Yousuf, additional, Rezk, Tamer, additional, Cohen, Oliver C., additional, Brown, James T., additional, Srikantharajah, Mukunthan, additional, Ganesananthan, Sharmananthan, additional, Lane, Thirusha, additional, Lachmann, Helen J., additional, Wechalekar, Ashutosh D., additional, Sachchithanantham, Sajitha, additional, Mahmood, Shameem, additional, Whelan, Carol J., additional, Knight, Daniel S., additional, Moon, James C., additional, Kellman, Peter, additional, Gillmore, Julian D., additional, Hawkins, Philip N., additional, and Fontana, Marianna, additional

Published
2021
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30. 30.2 A 1Tb 4b/Cell 144-Tier Floating-Gate 3D-NAND Flash Memory with 40MB/s Program Throughput and 13.8Gb/mm2 Bit Density

Author

Khakifirooz, Ali, primary, Balasubrahmanyam, Sriram, additional, Fastow, Richard, additional, Gaewsky, Kristopher H., additional, Ha, Chang Wan, additional, Haque, Rezaul, additional, Jungroth, Owen W., additional, Law, Steven, additional, Madraswala, Aliasgar S., additional, Ngo, Binh, additional, Naveen Prabhu, V, additional, Rajwade, Shantanu, additional, Ramamurthi, Karthikeyan, additional, Shenoy, Rohit S., additional, Snyder, Jacqueline, additional, Sun, Cindy, additional, Thimmegowda, Deepak, additional, Pathak, Bharat M., additional, and Kalavade, Pranav, additional

Published
2021
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31. Renal transplant outcomes in amyloidosis

Author

Law, Steven, primary, Cohen, Oliver, additional, Lachmann, Helen J, additional, Rezk, Tamer, additional, Gilbertson, Janet A, additional, Rowczenio, Dorota, additional, Wechalekar, Ashutosh D, additional, Hawkins, Philip N, additional, Motallebzadeh, Reza, additional, and Gillmore, Julian D, additional

Published
2021
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33. Long-term safety and efficacy of patisiran for hereditary transthyretin-mediated amyloidosis with polyneuropathy: 12-month results of an open-label extension study

Author

Adams, David, primary, Polydefkis, Michael, additional, González-Duarte, Alejandra, additional, Wixner, Jonas, additional, Kristen, Arnt V, additional, Schmidt, Hartmut H, additional, Berk, John L, additional, Losada López, Inés Asunción, additional, Dispenzieri, Angela, additional, Quan, Dianna, additional, Conceição, Isabel M, additional, Slama, Michel S, additional, Gillmore, Julian D, additional, Kyriakides, Theodoros, additional, Ajroud-Driss, Senda, additional, Waddington-Cruz, Márcia, additional, Mezei, Michelle M, additional, Planté-Bordeneuve, Violaine, additional, Attarian, Shahram, additional, Mauricio, Elizabeth, additional, Brannagan, Thomas H, additional, Ueda, Mitsuharu, additional, Aldinc, Emre, additional, Wang, Jing Jing, additional, White, Matthew T, additional, Vest, John, additional, Berber, Erhan, additional, Sweetser, Marianne T, additional, Coelho, Teresa, additional, Vita, Giuseppe, additional, Rizzo, Vincenzo, additional, Russo, Massimo, additional, Mazzeo, Anna, additional, Gentile, Luca, additional, Brueckner, Caitlin, additional, Lazzari, Victoria, additional, Wiesman, Janice, additional, DeLong, Douglas, additional, Victory, Jennifer, additional, Dalton, James, additional, May, John, additional, Gilmore, Catherine, additional, Diallo, Saran, additional, Delmont, Emilien, additional, Pouget, Jean, additional, Verschueren, Annie, additional, Grapperon, Aude-Marie, additional, Campana-Salort, Emmanuelle, additional, Lopes, Ana, additional, Lamas, Filipa, additional, Neves, Carlos, additional, Castro, Jose, additional, Pereira, Pedro, additional, Castro, Isabel, additional, Franco, Ana, additional, Santos, Miguel Oliveira, additional, de Azevedo Coutinho, Conceição, additional, Falcao de Campos, Catarina, additional, Hipólito Reis, Antonio, additional, Correia, Nuno, additional, Perez, Javier M, additional, Martins da Silva, Ana, additional, Alves, Cristina, additional, Cardoso, Marcio, additional, Valdrez, Katia, additional, Monte, Julia R, additional, Pessoa, Bernardete, additional, Guimaraes, Nadia, additional, Freitas, Monica, additional, Ramalho, Joana, additional, Ferreira, Natalia, additional, Kuzume, Daisuke, additional, Tard, Celine, additional, Waucquier, Nawal, additional, Rougeaux, Isabelle, additional, Brice, Sylvie, additional, Kasprzyk, Emmanuelle, additional, Elrezzi, Elise, additional, Meguig, Sayah, additional, Hachulla, Eric, additional, Gauvain, Clement, additional, Migaud-Chervy, Maria-Claire, additional, Deplanque, Dominique, additional, Jozefowicz, Elsa, additional, Lebellec, Loic, additional, Adams, David, additional, Balaya-Gouraya, Line, additional, Jehan Lacour, Nathalie, additional, Bournane, Halima, additional, Martin, Nathalie, additional, Elabed, Mongia, additional, Sacko, Niamey, additional, Boubrit, Yasmine, additional, Gaouar, Amina, additional, Rakotondratafika, Fetra, additional, Théaudin-Saliou, Marie, additional, Cauquil-Michon, Cécile, additional, Labeyrie, Celine, additional, Not, Adeline, additional, Al-Salameh, Abdallah, additional, Lecoq, Anne-Lise, additional, Stephant, Maeva, additional, Echaniz-Laguna, Andoni, additional, Becquemont, Laurent, additional, Beaudonnet, Guillemette, additional, Algalarrondo, Vincent, additional, Eliahou, Ludivine, additional, Rousseau, Antoine, additional, Signate, Aissatou, additional, Berthelot, Emeline, additional, Inamo, Jocelyn, additional, Vervoitte, Laetitia, additional, Focseneanu, Cecile, additional, Gendre, Thierry, additional, Arrouasse, Raphaele, additional, Ayache, Samar S., additional, Ernande, Laura, additional, Le Corvoisier, Philippe, additional, Salhi, Hayet, additional, Choumert, Ariane, additional, Ehinger, Vincent, additional, Ruiz, Julie, additional, Charlin, Cyril, additional, Megelin, Thomas, additional, Brannagan III, Thomas H, additional, Fayerman, Raisy, additional, Kim, Arreum, additional, Paras, Allan, additional, Gonzalez, Leidy J, additional, Tsang, Steven, additional, Wajnsztajn, Fernanda, additional, Shije, Jeffrey, additional, Ulane, Christina, additional, Kleyman, Inna, additional, Weimer, Louis, additional, Cioroiu, Comana, additional, Lambrianides, Sakis, additional, Abu-Manneh, Rana, additional, Zamba-Papanicolaou, Eleni, additional, Agathangelou, Petros, additional, Leonidou, Eleni, additional, Tada, Satoshi, additional, Fujita, Akemi, additional, Nagai, Masahiro, additional, Ando, Rina, additional, Hosokawa, Yuko, additional, Yamanishi, Yuki, additional, Overcash, J. Scott, additional, Giardino, Elena, additional, Boyer, Leslie, additional, Dang, Lien, additional, Le, An, additional, Nguyen, Tyler, additional, Giang, Lien, additional, Sellers, Peter, additional, Tran, Leyla, additional, Truong, Nghi, additional, Vinas, Maita, additional, Hrkman, Nicole, additional, Miller, Sarah, additional, Nguyen, David, additional, Smith, Ashley, additional, Pu, Helen, additional, Li, Steve, additional, Vuong, Thao, additional, Dioso, Holly, additional, Green, Sinikka, additional, Lee, Kia, additional, Chu, Hanh, additional, Waters, Michael, additional, Coskun, Derya J, additional, Zepeda, Karla A, additional, O'Riordan, William, additional, Obici, Laura, additional, Cortese, Andrea, additional, Lozza, Alessandro, additional, Merlini, Giampaolo, additional, Rosti, Vittorio, additional, Sabatelli, Mario, additional, Bisogni, Giulia, additional, Bernardo, Daniela, additional, Luigetti, Marco, additional, Di Paolantonio, Andrea, additional, Guglielmino, Valeria, additional, Romano, Angela, additional, Nienhuis, Hans, additional, Bulthuis-Kuiper, Janita, additional, Gerk, Olga, additional, Ulbricht, Hannah, additional, Taylor, Lenka, additional, Meyle, Eva, additional, Kleinschmidt, Natalia, additional, Meyrath, David, additional, Noe-Schwenn, Simone, additional, Meng, Ulrike, additional, Bauer, Ralf, additional, aus dem Siepen, Fabian, additional, Hein, Selina, additional, Takahashi, Tetsuya, additional, Oshita, Tomohiko, additional, Koujin, Yoko, additional, Neshige, Shuichiro, additional, Nezu, Tomohisa, additional, Segawa, Akiko, additional, Ueno, Hiroki, additional, Morino, Hiroyuki, additional, Campistol, Josep M, additional, Rodas Marin, Lida Maria, additional, Blasco Pelicano, Josep Miquel, additional, Dávila, Lucía Galán, additional, Palacios, Marta, additional, Pytel Cordoba, Vanesa, additional, Guerrero Sola, Antonio, additional, Horga, Alejandro, additional, García Feijoo, Julián, additional, Perez de Isla, Leopoldo, additional, Marques Júnior, Wilson, additional, Moscardini, Mariana, additional, Litcanov, Debora Cristina, additional, Viera Lima, Ana Flavia, additional, Rodrigues, Leonardo, additional, Marques Coutinho, Barbara, additional, Moreira, Carolina Lavigne, additional, Daccach Marques, Vanessa, additional, Munoz Beamud, Francisco, additional, Gragera Martínez, Álvaro, additional, Borrachero, Cristina, additional, Cisneros Barroso, Eugenia, additional, Rodríguez Rodríguez, Adrián, additional, Sanz, Monica, additional, Rigo Oliver, Elena, additional, González Moreno, Juan, additional, Gamez Martinez, Jose M, additional, Descals, Cristina, additional, Uson, Mercedes, additional, Jose Vega, Francisco, additional, Figuerola, Antoni, additional, Montala, Carles, additional, Dias da Silva, Moises, additional, Gervais de Santa Rosa, Renata, additional, Pinto, Luiz Felipe, additional, Pinto, Marcus Vinicius, additional, Cardoso Berensztejn, Amanda, additional, Barroso, Fabio, additional, Lautre, Andrea, additional, Orellana, Lucas G, additional, González-Duarte Briseño, Maria Alejandra, additional, Cárdenas-Soto, Karla, additional, Jiménez López, Brenda Poled, additional, Pérez-Castañeda, Sandra Lorena, additional, Cantú Brito, Carlos Gerardo, additional, Rivera de la Parra, David, additional, Hernandez Reyes, Jose Pablo, additional, del Mar Saniger Alba, Maria, additional, Criollo Mora, Elia, additional, Parman, Yesim, additional, Rezzan, Kus Jülide, additional, Sahin, Erdi, additional, Serbest, Nail G, additional, Durmus, Hacer, additional, Cakar, Arman, additional, Tugal Tutkun, Nuriye Ilknur, additional, Karamursel, Sacit, additional, Elitok, Ali, additional, Sirin Inan, Nermin G, additional, Altinkurt, Emre, additional, Ye, Jing, additional, Allen, Adriane C, additional, Chaudhry, Vinay, additional, Jarrett, Raquel, additional, Bressler, Neil, additional, Burks, Kathleen L, additional, Liu, Qingfeng, additional, Khoshnoodi, Mohammad, additional, Judge, Daniel P, additional, Vista, Geno, additional, Shah, Syed Mahmood, additional, Hamaguchi, Hirotoshi, additional, Oda, Junko, additional, Fukase, Emi, additional, Taniguchi, Ikuko, additional, Oda, Tetsuya, additional, Endo, Hironobu, additional, Shimomura, Masahiro, additional, Katanazaka, Kimitaka, additional, Koto, Shusuke, additional, Nakano, Takahiro, additional, Scheid, Christof, additional, Zueiter, Andreas, additional, Pester, Lars, additional, Walter, Doreen, additional, Özdemir, Betül, additional, Frenzel, Lukas F, additional, Holtick, Udo, additional, Oh, Jeeyoung, additional, Kim, Hee Jin, additional, Shin, Hyun Jin, additional, Choi, Kyomin, additional, Yamashita, Taro, additional, Masuda, Teruaki, additional, Misumi, Yohei, additional, Ueda, Akihiko, additional, Nakahara, Keiichi, additional, Yorita, Akiko, additional, Tsuruhisa, Seiko, additional, Taniwaki, Takayuki, additional, Harada, Masaya, additional, Moritaka, Taiga, additional, Sakurada, Naonori, additional, Mauricio, Elizabeth A, additional, Baskin, Amber, additional, Dimberg, Elliot, additional, Fonder, Amie, additional, Hobbs, Miriam, additional, Russell, Stephen J, additional, Dyck, Peter, additional, Gonsalves, Wilson, additional, Leung, Nelson, additional, Witzig, Thomas E, additional, Zeldenrust, Steven R, additional, Hwa, Lisa, additional, Kapoor, Prashant, additional, Kumar, Shaji K, additional, Lin, Yi, additional, Lust, John A, additional, Rajkumar, Vincent S, additional, Dingli, David, additional, Gertz, Morie A, additional, Go, Ronald, additional, Hayman, Suzanne R, additional, Dalia, Samir, additional, Carrillo, Esmeralda, additional, Gorevic, Peter, additional, Mason, Garnette, additional, Chao, Chi-Chao, additional, Lee, Ming-Jen, additional, Su, Jen-Jen, additional, Hsieh, Sung-Tsang, additional, Tsai, Li-Kai, additional, Yeh, Shin-Joe, additional, Yang, Chih-Chao, additional, Ajroud-Driss, Senda Ajroud-Driss, additional, Casey, Patricia, additional, Joslin, Benjamin C, additional, Freimer, Miriam, additional, Sankey, Alison, additional, Kenepp, Amanda, additional, Heintzman, Sarah, additional, LoRusso, Samantha, additional, Hokezu, Youichi, additional, Kim, Byoung-Joon, additional, Kim, JuHyeon, additional, Lee, Ga Yeon, additional, Cho, Eun Bin, additional, Jeon, Eun-Seok, additional, Min, Ju-Hong, additional, Seok, Jin Myoung, additional, Lee, Hye Lim, additional, Park, Jae Hong, additional, Sekijima, Yoshiki, additional, Miyazawa, Chinatsu, additional, Kato, Nagaaki, additional, Kishida, Dai, additional, Hineno, Akiyo, additional, Kodaira, Minori, additional, Yoshinaga, Tsuneaki, additional, Miyahara, Teruyoshi, additional, Imai, Akira, additional, Matsumoto, Kazuhiko, additional, Lin, Kon-Ping, additional, Lee, Yi-Chung, additional, Falk, Malin, additional, Pilebro, Bjorn, additional, Suhr, Ole, additional, Lindqvist, Per, additional, Soderberg, Karin, additional, Pedrosa-Domellöf, Fatima, additional, Anan, Intissar, additional, Nordh, Erik, additional, Tournev, Ivaylo, additional, Zhelyazkova-Glaveeva, Sashka, additional, Cherneva, Zheyna, additional, Sarafov, Staiko, additional, Chamova, Teodora, additional, Cherninkova-Gopina, Sylvia, additional, Friebel, Frauke, additional, Zibert, Andree, additional, Mihailovic, Natasa, additional, Schubert, Friederike, additional, Vorona, Elena, additional, Lahme, Larissa, additional, Huesing-Kabar, Anna, additional, Schilling, Matthias, additional, Kabar, Iyad, additional, Martinez-Naharro, Ana, additional, Chacko, Liza, additional, Cohen, Oliver, additional, Law, Steven, additional, Rezk, Tamer, additional, Lachmann, Helen J, additional, Blume, Brianna, additional, Dixon, Stacy, additional, Low, Soon Chai, additional, Chan, Soo Looi, additional, Lim, He Eng Li, additional, Goh, Khean Jin, additional, Kraus, Deborah, additional, Jack, Kristin, additional, Wade, N. Kevin, additional, Lopate, Glenn, additional, Zwijack, Brittany, additional, Florence, Julaine, additional, Sommerville, R. Brian, additional, Stewart, Graeme, additional, Ryder, Julie, additional, Mekhael, Linda, additional, Taylor, Mark, additional, Suan, Daniel, additional, Wells, Karen, additional, Stone, Paula, additional, Itoya, Amenze, additional, Owusu-Sekyere, Mercy, additional, Thai, Desmond, additional, Chahine, Ilonah, additional, Pedrosa, Salve, additional, and Do, Thi Hoa (Therese), additional

Published
2021
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34. The Impact of Longitudinal Strain on Haematological and Cardiac Response and Survival in Patients with Systemic AL Amyloidosis

Author

Cohen, Oliver C, primary, Ismael, Andreia, additional, Manwani, Richa, additional, Ravichandran, Sriram, additional, Law, Steven, additional, Foard, Darren, additional, Martinez-Naharro, Ana, additional, Mahmood, Shameem, additional, Sachchithananthan, Sajitha, additional, Hawkins, Philip N, additional, Lachmann, Helen J, additional, Gillmore, Julian D, additional, Fontana, Marianna, additional, Falk, Rodney H, additional, Whelan, Carol, additional, and Wechalekar, Ashutosh D., additional

Published
2020
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35. The Prognostic Importance of the 6-Minute Walk Test in AL Amyloidosis

Author

Cohen, Oliver C, primary, Sathyanath, Ananth, additional, Ravichandran, Sriram, additional, Law, Steven, additional, Manwani, Richa, additional, Foard, Darren, additional, Sachchithanantham, Sajitha, additional, Mahmood, Shameem, additional, Martinez-Naharro, Ana, additional, Fontana, Marianna, additional, Whelan, Carol, additional, Hawkins, Philip N, additional, Lachmann, Helen J, additional, Gillmore, Julian D, additional, and Wechalekar, Ashutosh D., additional

Published
2020
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36. Disease progression in cardiac transthyretin amyloidosis is indicated by serial calculation of National Amyloidosis Centre transthyretin amyloidosis stage

Author

Law, Steven, primary, Petrie, Aviva, additional, Chacko, Liza, additional, Cohen, Oliver C., additional, Ravichandran, Sriram, additional, Gilbertson, Janet A., additional, Rowczenio, Dorota, additional, Wechalekar, Ashutosh, additional, Martinez‐Naharro, Ana, additional, Lachmann, Helen J., additional, Whelan, Carol J., additional, Hutt, David F., additional, Hawkins, Philip N., additional, Fontana, Marianna, additional, and Gillmore, Julian D., additional

Published
2020
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37. Rapid response to single agent daratumumab is associated with improved progression-free survival in relapsed/refractory AL amyloidosis

Author

Cohen, Oliver C., primary, Brodermann, Maximillian H., additional, Blakeney, Iona J., additional, Mahmood, Shameem, additional, Sachchithanantham, Sajitha, additional, Ravichandran, Sriram, additional, Law, Steven, additional, Lachmann, Helen J., additional, Whelan, Carol J., additional, Popat, Rakesh, additional, Rabin, Neil, additional, Yong, Kwee, additional, Kyriakou, Charalampia, additional, Shah, Raakhee, additional, Cheesman, Simon, additional, Worthington, Sarah, additional, Hawkins, Philip, additional, Gillmore, Julian D., additional, and Wechalekar, Ashutosh D., additional

Published
2020
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38. Change in N-terminal pro-B-type natriuretic peptide at 1 year predicts mortality in wild-type transthyretin amyloid cardiomyopathy.

Author

Law, Steven, Petrie, Aviva, Chacko, Liza, Cohen, Oliver C., Ravichandran, Sriram, Gilbertson, Janet A., Rowczenio, Dorota, Wechalekar, Ashutosh D., Martinez-Naharro, Ana, Lachmann, Helen J., Whelan, Carol J., Hutt, David F., Hawkins, Philip N., Fonta, Marianna, Gillmore, Julian D., and Fontana, Marianna

Subjects
CARDIAC amyloidosis, BRAIN natriuretic factor, TRANSTHYRETIN, AMYLOID, CARDIOMYOPATHIES
Abstract

Objectives: Wild-type transthyretin amyloid cardiomyopathy (wtATTR-CM) is a progressive and fatal condition. Although prognosis can be determined at the time of diagnosis according to National Amyloidosis Centre (NAC) transthyretin amyloidosis (ATTR) stage, the clinical course varies substantially between individuals. There are currently no established measures of rate of disease progression. Through systematic analysis of functional, biochemical and echocardiographic disease-related variables we aimed to identify prognostic markers of disease progression in wtATTR-CM.Methods: This is a retrospective observational study of 432 patients with wtATTR-CM diagnosed at the UK NAC, none of whom received disease-modifying therapy. The association between mortality from the 12-month timepoint and change from diagnosis to 12 months in a variety of disease-related variables was explored using Cox regression.Results: Change in N-terminal pro-B-type natriuretic peptide concentration (∆ NT-proBNP) at 12 months from diagnosis was the strongest predictor of ongoing mortality and was independent of both change in other disease-related variables (HR 1.04 per 500 ng/L increase (95% CI 1.01 to 1.07); p=0.003) and a range of known prognostic variables at the time of diagnosis (HR 1.07 per 500 ng/L increase (95% CI 1.02 to 1.13); p=0.007). An increase in NT-proBNP of >500 ng/L, >1000 ng/L and >2000 ng/L during the first year of follow-up occurred in 45%, 35% and 16% of patients, respectively.Conclusion: Change in NT-proBNP concentration during the first year of follow-up is a powerful independent predictor of mortality in wtATTR-CM. [ABSTRACT FROM AUTHOR]

Published
2022
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39. Longitudinal strain is an independent predictor of survival and response to therapy in patients with systemic AL amyloidosis.

Author

Cohen, Oliver C, Ismael, Andreia, Pawarova, Babita, Manwani, Richa, Ravichandran, Sriram, Law, Steven, Foard, Darren, Petrie, Aviva, Ward, Sevda, Douglas, Brooke, Martinez-Naharro, Ana, Chacko, Liza, Quarta, Candida Cristina, Mahmood, Shameem, Sachchithanantham, Sajitha, Lachmann, Helen J, Hawkins, Philip N, Gillmore, Julian D, Fontana, Marianna, and Falk, Rodney H

Subjects
CARDIAC amyloidosis, ECHOCARDIOGRAPHY, LONGITUDINAL method, PROGNOSIS, NATRIURETIC peptides
Abstract

Aims Cardiac involvement, a major determinant of prognosis in AL (light-chain immunoglobulin) amyloidosis, is characterized by an impairment of longitudinal strain (LS%). We sought to evaluate the utility of LS% in a prospectively observed series of patients. Methods and results A total of 915 serial newly diagnosed AL patients with comprehensive baseline assessments, inclusive of echocardiography, were included. A total of 628/915 (68.6%) patients had cardiac involvement. The LS% worsened with advancing cardiac stage with mean −21.1%, −17.1%, −12.9%, and −12.1% for stages I, II, IIIa, and IIIb, respectively (P < 0.0001). There was a highly significant worsening of overall survival (OS) with worsening LS% quartile: LS% ≤−16.2%: 80 months, −16.1% to −12.2%: 36 [95% confidence interval (CI) 20.9–51.1] months, −12.1% to −9.1%: 22 (95% CI 9.1–34.9) months, and ≥−9.0%: 5 (95% CI 3.2–6.8) months (P < 0.0001). Improvement in LS% was seen at 12 months in patients achieving a haematological complete response (CR) (median improvement from −13.8% to −14.9% in those with CR and difference between involved and uninvolved light chain <10 mg/L). Strain improvement was associated with improved OS (median not reached at 53 months vs. 72 months in patients without strain improvement, P = 0.007). Patients achieving an LS% improvement and a standard N-terminal pro-brain natriuretic peptide-based cardiac response survived longer than those achieving a biomarker-based cardiac response alone (P < 0.0001). Conclusion Baseline LS% is a functional marker that correlates with worsening cardiac involvement and is predictive of survival. Baseline LS% and an absolute improvement in LS% are useful additional measures of prognosis and response to therapy in cardiac AL amyloidosis, respectively. [ABSTRACT FROM AUTHOR]

Published
2022
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40. Industrial wood-fired: boiler optimization and emissions compliance: with the contemplation of potential compliance changes for small industrial boiler operations and emissions by Federal regulators, compliance should be based upon what makes sense for the environment and the economy

Author

Kaminski, Paul, Taylor, Fred, Reusing, Gordon, Law, Steven, and MacRae, John

Subjects
Ethan Allen Inc. -- Waste management, Furniture industry -- Waste management -- Case studies, Boilers -- Case studies -- Waste management, Business, Environmental services industry, Waste management, Case studies
Abstract

This study of stack testing data and process optimization evaluations was completed for 13 Ethan Allen industrial boilers and several other boilers evaluated by Conestoga-Rovers & Associates. The boilers range [...]

Published
2002

43. Marketing Yourself on the Web

Author

Law, Steven

Subjects
Internet marketing -- Usage, Spam (Junk email) -- Usage, Web sites -- Design and construction, Authors -- Marketing, Consumer preferences, Unsolicited bulk e-mail, Company Web site/Web page, Company marketing practices, Literature/writing, Publishing industry
Published
2004

48. Law as Narrative: Narrative Interpretation and Appropriation as an Element of Theft.

Author

Law, Steven Cammiss Senior Lecturer in

Subjects
THEFT -- Law & legislation, STATUTORY interpretation, LEGAL justification, PROPERTY rights, APPROPRIATION (Torts), LEGAL judgments, COMMON law
Abstract

The act requirement for the offence of theft requires that the defendant appropriates property belonging to another. The concept of appropriation, thought by the legislation's drafters to be straightforward, has proved to be difficult to interpret; controversies in the cases and commentary focus upon whether the consent of the owner is relevant to appropriation, and whether the concept includes an adverse interference with property rights. This paper explores these controversies through a different lens, that of law as narrative. Drawing upon law as literature, particularly law and narrative, I argue that narrative interpretations in law result in the creation of 'hard cases', where the application of appropriation, as envisaged by its framers, leads to difficult results. In so doing, I show how the normative syllogism is merely a justification for legal interpretation, with the law best understood in narrative terms. Applying the law is, therefore, not an exercise of correspondence between abstract law and particular facts, but instead an exercise in comparison between different narratives. [ABSTRACT FROM AUTHOR]

Published
2019
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49. Justice for our Guatemalan sisters and brothers!

Author

Law, Steven

Subjects
Mineral industry -- Cases -- Social aspects, Murder -- Cases, Mining industry -- Cases -- Social aspects, Violence -- Social aspects -- Political aspects, Native people's land claims -- Political aspects, Company legal issue, Political science
Abstract

CANADIANS VIE FOR IT, want it, and when they play hockey, they demand it. Gold. For most Canadians it's a medal they would like to see hanging around Sidney Crosby's [...]

Published
2013

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