33 results on '"Law, Steven"'
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2. Early relapse is an adverse prognostic marker in systemic immunoglobulin light chain (AL) Amyloidosis
- Author
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Ravichandran, Sriram, Law, Steven, Mahmood, Shameem, Wisniowski, Brenden, Foard, Darren, Fontana, Marianna, Martinez-Naharro, Ana, Whelan, Carol, Gillmore, Julian D., Lachmann, Helen J., Hawkins, Philip N., and Wechalekar, Ashutosh D.
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- 2022
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3. Long-term safety and efficacy of patisiran for hereditary transthyretin-mediated amyloidosis with polyneuropathy: 12-month results of an open-label extension study
- Author
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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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4. Impact of early response on outcomes in AL amyloidosis following treatment with frontline Bortezomib
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Ravichandran, Sriram, Cohen, Oliver C., Law, Steven, Foard, Darren, Fontana, Marianna, Martinez-Naharro, Ana, Whelan, Carol, Gillmore, Julian D., Lachmann, Helen J., Sachchithanantham, Sajitha, Mahmood, Shameem, Hawkins, Philip N., and Wechalekar, Ashutosh D.
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- 2021
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5. Tocilizumab (anti-IL-6) treatment for AA renal amyloidosis in a patient with advanced chronic kidney disease, a case report.
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Seneschall, Charlotte, Law, Steven, Roufosse, Candice, Woodham, Sarah, and Kousios, Andreas
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- 2024
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6. The successful use of pembrolizumab in a renal transplant recipient with metastatic melanoma
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Hanna, Daire L., Law, Steven J., Merrick, Sophie A., Heptinstall, Lauren, Bass, Paul, Dupont, Peter, and Sheri, Amna
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- 2020
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7. Karyomegalic interstitial nephritis with a novel FAN1 gene mutation and concurrent ALECT2 amyloidosis
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Law, Steven, Gillmore, Julian, Gilbertson, Janet A., Bass, Paul, and Salama, Alan D.
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- 2020
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8. An Integrative Suicide Prevention Program for Visitor Charcoal Burning Suicide and Suicide Pact
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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.
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- 2009
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9. Conventional heart failure therapy in cardiac ATTR amyloidosis.
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Ioannou, Adam, Massa, Paolo, Patel, Rishi K, Razvi, Yousuf, Porcari, Aldostefano, Rauf, Muhammad U, Jiang, Anita, Cabras, Giacomo, Filisetti, Stefano, Bolhuis, Roos E, Bandera, Francesco, Venneri, Lucia, Martinez-Naharro, Ana, Law, Steven, Kotecha, Tushar, Virsinskaite, Ruta, Knight, Daniel S, Emdin, Michele, Petrie, Aviva, and Lachmann, Helen
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CARDIAC amyloidosis ,HEART failure ,HEART failure patients ,MINERALOCORTICOID receptors ,ACE inhibitors ,VENTRICULAR ejection fraction - Abstract
Aims The aims of this study were to assess prescription patterns, dosages, discontinuation rates, and association with prognosis of conventional heart failure medications in patients with transthyretin cardiac amyloidosis (ATTR-CA). Methods and results A retrospective analysis of all consecutive patients diagnosed with ATTR-CA at the National Amyloidosis Centre between 2000 and 2022 identified 2371 patients with ATTR-CA. Prescription of heart failure medications was greater among patients with a more severe cardiac phenotype, comprising beta-blockers in 55.4%, angiotensin-converting enzyme inhibitors (ACEis)/angiotensin II receptor blockers (ARBs) in 57.4%, and mineralocorticoid receptor antagonists (MRAs) in 39.0% of cases. During a median follow-up of 27.8 months (interquartile range 10.6–51.3), 21.7% had beta-blockers discontinued, and 32.9% had ACEi/ARBs discontinued. In contrast, only 7.5% had MRAs discontinued. A propensity score-matched analysis demonstrated that treatment with MRAs was independently associated with a reduced risk of mortality in the overall population [hazard ratio (HR) 0.77 (95% confidence interval (CI) 0.66–0.89), P <.001] and in a pre-specified subgroup of patients with a left ventricular ejection fraction (LVEF) >40% [HR 0.75 (95% CI 0.63–0.90), P =.002]; and treatment with low-dose beta-blockers was independently associated with a reduced risk of mortality in a pre-specified subgroup of patients with a LVEF ≤40% [HR 0.61 (95% CI 0.45–0.83), P =.002]. No convincing differences were found for treatment with ACEi/ARBs. Conclusion Conventional heart failure medications are currently not widely prescribed in ATTR-CA, and those that received medication had more severe cardiac disease. Beta-blockers and ACEi/ARBs were often discontinued, but low-dose beta-blockers were associated with reduced risk of mortality in patients with a LVEF ≤40%. In contrast, MRAs were rarely discontinued and were associated with reduced risk of mortality in the overall population; but these findings require confirmation in prospective randomized controlled trials. [ABSTRACT FROM AUTHOR]
- Published
- 2023
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10. Tc-99m labelled bone scintigraphy in suspected cardiac amyloidosis.
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Rauf, Muhammad Umaid, Hawkins, Philip N, Cappelli, Francesco, Perfetto, Federico, Zampieri, Mattia, Argiro, Alessia, Petrie, Aviva, Law, Steven, Porcari, Aldostefano, Razvi, Yousuf, Bomsztyk, Joshua, Ravichandran, Sriram, Ioannou, Adam, Patel, Rishi, Starr, Neasa, Hutt, David F, Mahmood, Shameem, Wisniowski, Brendan, Martinez–Naharro, Ana, and Venneri, Lucia
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RADIONUCLIDE imaging ,CARDIAC amyloidosis ,IMMUNOGLOBULIN light chains ,CHRONIC kidney failure - Abstract
Aims To perform evaluation of widely embraced bone scintigraphy-based non-biopsy diagnostic criteria (NBDC) for ATTR amyloid cardiomyopathy (ATTR-CM) in clinical practice, and to refine serum free light chain (sFLC) ratio cut-offs that reliably exclude monoclonal gammopathy (MG) in chronic kidney disease. Methods and results A multi-national retrospective study of 3354 patients with suspected or histologically proven cardiac amyloidosis (CA) referred to specialist centres from 2015 to 2021; evaluations included radionuclide bone scintigraphy, serum and urine immunofixation, sFLC assay, eGFR measurement and echocardiography. Seventy-nine percent (1636/2080) of patients with Perugini grade 2 or 3 radionuclide scans fulfilled NBDC for ATTR-CM through absence of a serum or urine monoclonal protein on immunofixation together with a sFLC ratio falling within revised cut-offs incorporating eGFR; 403 of these patients had amyloid on biopsy, all of which were ATTR type, and their survival was comparable to non-biopsied ATTR-CM patients (p = 0.10). Grade 0 radionuclide scans were present in 1091 patients, of whom 284 (26%) had CA, confirmed as AL type (AL-CA) in 276 (97%) and as ATTR-CM in only one case with an extremely rare TTR variant. Among 183 patients with grade 1 radionuclide scans, 122 had MG of whom 106 (87%) had AL-CA; 60/61 (98%) without MG had ATTR-CM. Conclusion The NBDC for ATTR-CM are highly specific [97% (95% CI 0.91-0.99)] in clinical setting, and diagnostic performance was further refined here using new cut-offs for sFLC ratio in patients with CKD. A grade 0 radionuclide scan all but excludes ATTR-CM but occurs in most patients with AL-CA. Grade 1 scans in patients with CA and no MG are strongly suggestive of early ATTR-type, but require urgent histologic corroboration. [ABSTRACT FROM AUTHOR]
- Published
- 2023
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11. The effect of changing dialysate bicarbonate concentration on serum bicarbonate, body weight and normalized nitrogen appearance rate.
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Law, Steven and Davenport, Andrew
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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]
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- 2023
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12. The experience of hereditary apolipoprotein A-I amyloidosis at the UK National Amyloidosis Centre.
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Cohen, Oliver C., Blakeney, Iona J., Law, Steven, Ravichandran, Sriram, Gilbertson, Janet, Rowczenio, Dorota, Mahmood, Shameem, Sachchithanantham, Sajitha, Wisniowski, Brendan, Lachmann, Helen J., Whelan, Carol J., Martinez-Naharro, Ana, Fontana, Marianna, Hawkins, Philip N., Gillmore, Julian D., and Wechalekar, Ashutosh D.
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APOLIPOPROTEIN A ,AMYLOIDOSIS ,CARDIAC amyloidosis ,KIDNEY failure ,AGE of onset ,CHRONIC kidney failure ,KIDNEY transplantation - Abstract
Hereditary apolipoprotein A-I (AApoAI) amyloidosis is a rare heterogeneous disease with variable age of onset and organ involvement. There are few series detailing the natural history and outcomes of solid organ transplantation across a range of causative APOA1 gene mutations. We identified all patients with AApoAI amyloidosis who presented to the National Amyloidosis Centre (NAC) between 1986 and 2019. In total, 57 patients with 14 different APOA1 mutations were identified including 18 patients who underwent renal transplantation (5 combined liver-kidney (LKT) and 2 combined heart-kidney (HKT) transplants). Median age of presentation was 43 years and median time from presentation to referral was 3 (0–31 years). Involvement of the kidneys, liver and heart by amyloid was detected in 81%, 67% and 28% of patients, respectively. Renal amyloidosis was universal in association with the most commonly identified variant (Gly26Arg, n = 28). Across all variants, patients with renal amyloidosis had a median creatinine of 159 µmol/L and median urinary protein of 0.3 g/24 h at the time of diagnosis of AApoAI amyloidosis and median time from diagnosis to end-stage renal disease was 15.0 (95% CI: 10.0–20.0) years. Post-renal transplantation, median allograft survival was 22.0 (13.0–31.0) years. There was one early death following transplantation (infection-related at 2 months post-renal transplant) and no episodes of early rejection leading to graft failure. Liver transplantation led to regression of amyloid in all four cases in whom serial
123 I-SAP scintigraphy was performed. AApoAI amyloidosis is a slowly progressive disease that is challenging to diagnose. The outcomes of transplantation are encouraging and graft survival is excellent. [ABSTRACT FROM AUTHOR]- Published
- 2022
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13. Cardiovascular magnetic resonance in light-chain amyloidosis to guide treatment.
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Martinez-Naharro, Ana, Patel, Rishi, Kotecha, Tushar, Karia, Nina, Ioannou, Adam, Petrie, Aviva, Chacko, Liza A, Razvi, Yousuf, Ravichandran, Sriram, Brown, James, Law, Steven, Quarta, Cristina, Mahmood, Shameem, Wisniowski, Brendan, Pica, Silvia, Sachchithanantham, Sajitha, Lachmann, Helen J, Moon, James C, Knight, Daniel S, and Whelan, Carol
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BRAIN natriuretic factor ,MAGNETIC resonance ,IMMUNOGLOBULIN light chains ,AMYLOIDOSIS ,CARDIAC amyloidosis - Abstract
Aims To assess the ability of cardiovascular magnetic resonance (CMR) to (i) measure changes in response to chemotherapy; (ii) assess the correlation between haematological response and changes in extracellular volume (ECV); and (iii) assess the association between changes in ECV and prognosis over and above existing predictors. Methods and results In total, 176 patients with cardiac AL amyloidosis were assessed using serial N-terminal pro-B-type natriuretic peptide (NT-proBNP), echocardiography, free light chains and CMR with T1 and ECV mapping at diagnosis and subsequently 6, 12, and 24 months after starting chemotherapy. Haematological response was graded as complete response (CR), very good partial response (VGPR), partial response (PR), or no response (NR). CMR response was graded by changes in ECV as progression (≥0.05 increase), stable (<0.05 change), or regression (≥0.05 decrease). At 6 months, CMR regression was observed in 3% (all CR/VGPR) and CMR progression in 32% (61% in PR/NR; 39% CR/VGPR). After 1 year, 22% had regression (all CR/VGPR), and 22% had progression (63% in PR/NR; 37% CR/VGPR). At 2 years, 38% had regression (all CR/VGPR), and 14% had progression (80% in PR/NR; 20% CR/VGPR). Thirty-six (25%) patients died during follow-up (40 ± 15 months); CMR response at 6 months predicted death (progression hazard ratio 3.82; 95% confidence interval 1.95–7.49; P < 0.001) and remained prognostic after adjusting for haematological response, NT-proBNP and longitudinal strain (P < 0.01). Conclusions Cardiac amyloid deposits frequently regress following chemotherapy, but only in patients who achieve CR or VGPR. Changes in ECV predict outcome after adjusting for known predictors. [ABSTRACT FROM AUTHOR]
- Published
- 2022
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14. Prognostic importance of the 6 min walk test in light chain (AL) amyloidosis.
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Cohen, Oliver C., Sathyanath, Ananth, Petrie, Aviva, Ravichandran, Sriram, Law, Steven, Manwani, Richa, Foard, Darren, Sachchithanantham, Sajitha, Mahmood, Shameem, Martinez-Naharro, Ana, Fontana, Marianna, Whelan, Carol J., Hawkins, Philip N., Lachmann, Helen J., Gillmore, Julian D., and Wechalekar, Ashutosh D.
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AMYLOIDOSIS ,PROGNOSIS ,IMPACT of Event Scale - Abstract
Objectives: In AL amyloidosis, organ response assessment is based on surrogates (eg, cardiac biomarkers). An objective functional test, such as the 6 min walk test (6MWT), capturing overall clinical improvement, is required. We aimed to evaluate the prognostic impact of the 6MWT at baseline and change following chemotherapy.Methods: This study evaluated the outcomes of patients who enrolled in a prospective observational study at the UK National Amyloidosis Centre (2012-2017). Patients underwent comprehensive assessments inclusive of blood testing, echocardiogram and 6MWT at baseline and annually thereafter.Results: In total, 799 patients were included within the study. Median baseline 6 min walk distance (6MWD) was 362 m (IQR: 231 m). 6MWD progressively decreased with worsening cardiac disease stage (458 m, 404 m, 331 m and 168 m for cardiac Mayo stages I, II, IIIa and IIIb, respectively (p<0.0001)). In patients with a baseline 6MWT of ≥350 m, the median overall survival was not reached (vs 30.0 (95% CI 23.2 to 36.8) months if <350 m and 5.0 (95% CI 2.8 to 7.2) months if unable to attempt 6MWT (p<0.0001). Following chemotherapy, only patients in a complete haematological response improved their 6MWD by 12 months (p=0.001). Improvement in 6MWD prolonged survival in patients with cardiac amyloidosis (p=0.005).Conclusion: The 6MWT is prognostic in AL amyloidosis. A baseline distance of ≥350 m independently predicts better survival. These data suggest that 6MWT has utility in AL amyloidosis for baseline prognosis and assessing response. [ABSTRACT FROM AUTHOR]- Published
- 2022
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15. Characteristics and natural history of early-stage cardiac transthyretin amyloidosis.
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Law, Steven, Bezard, Melanie, Petrie, Aviva, Chacko, Liza, Cohen, Oliver C, Ravichandran, Sriram, Ogunbiyi, Olabisi, Kharoubi, Mounira, Ganeshananthan, Sashiananthan, Ganeshananthan, Sharmananthan, Gilbertson, Janet A, Rowczenio, Dorota, Wechalekar, Ashutosh, Martinez-Naharro, Ana, Lachmann, Helen J, Whelan, Carol J, Hutt, David F, Hawkins, Philip N, Damy, Thibaud, and Fontana, Marianna
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CARDIAC amyloidosis ,BRAIN natriuretic factor ,NATURAL history - Abstract
Aims Transthyretin amyloid cardiomyopathy (ATTR-CM) is increasingly diagnosed at an early stage of the disease natural history, defined as National Amyloidosis Centre (NAC) ATTR Stage I. The natural history of early-stage ATTR-CM remains poorly characterized. Methods and results A retrospective multi-centre observational study of 879 patients with ATTR-CM, either wild-type TTR genotype or carrying the p.V142I TTR variant, and NAC ATTR Stage I biomarkers at the time of diagnosis who did not receive disease-modifying therapy for amyloidosis. Disease characteristics at diagnosis that were independently associated with mortality by Cox regression analysis were N-terminal pro-B-type natriuretic peptide (NT-proBNP), TTR genotype, and troponin T. Patients were categorized into NAC ATTR Stage Ia, defined as a furosemide equivalent diuretic requirement of <0.75 mg/kg and an NT-proBNP ≤500 ng/L or ≤1000 ng/L in the presence of atrial fibrillation, and NAC ATTR Stage Ib comprising all remaining Stage I patients. Median estimated survival among the 88% NAC ATTR Stage Ib patients was 75 (95% CI 57–93) months compared with >100 months in the 12% with Stage Ia disease [hazard ratio for death 5.06 (95% confidence interval 1.23–20.87); P = 0.025] despite significant cardiovascular morbidity at the time of diagnosis which increased during follow-up, including among patients diagnosed in NAC ATTR Stage Ia. Estimated survival among UK NAC ATTR Stage Ia patients was comparable to UK general population controls (P = 0.297). Conclusion Patients with NAC ATTR Stage I ATTR-CM can be further stratified according to NT-proBNP concentration and diuretic requirement at diagnosis. Patients with Stage Ia ATTR-CM have significant cardiovascular morbidity despite good short- and mid-term survival. [ABSTRACT FROM AUTHOR]
- Published
- 2022
- Full Text
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16. Change in N-terminal pro-B-type natriuretic peptide at 1 year predicts mortality in wild-type transthyretin amyloid cardiomyopathy.
- Author
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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
- Full Text
- View/download PDF
17. Long‐term outcomes in light chain deposition disease‐analysis of a UK cohort.
- Author
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Ravichandran, Sriram, Law, Steven, Mahmood, Shameem, Wisniowski, Brenden, Foard, Darren, Fontana, Marianna, Martinez‐Naharro, Ana, Whelan, Carol, Hawkins, Philip N., Gillmore, Julian D., Lachmann, Helen J., and Wechalekar, Ashutosh D.
- Published
- 2022
- Full Text
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18. Longitudinal strain is an independent predictor of survival and response to therapy in patients with systemic AL amyloidosis.
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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
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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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19. 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.
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Ravichandran, Sriram, Cohen, Oliver C., Law, Steven, Sachchithanantham, Sajitha, Mahmood, Shameem, Foard, Darren, Fontana, Marianna, Martinez‐Naharro, Ana, Whelan, Carol, Gillmore, Julian D., Lachmann, Helen J., Hawkins, Philip N., and Wechalekar, Ashutosh D.
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AMYLOIDOSIS ,SEROTHERAPY ,OVERALL survival ,SURVIVAL rate ,NATURAL history ,IMMUNOGLOBULIN light chains - Abstract
Summary: Systemic immunoglobulin light chain amyloidosis (AL) is an incurable disorder, and the natural history is incompletely understood. In this study, we describe its natural history based on an analysis of real‐world longitudinal data. All patients seen at the National Amyloidosis Centre, UK, between February 2010 and August 2019 and treated with up‐front bortezomib are included. In all, 1 276 patients received the first‐line treatment; 259, 85, and 32 patients received second, third, and fourth treatment lines, respectively. Among patients requiring further treatment after the first line, 77·2% started the second line within two years of the first line; 50·5%, 50·6%, 40·1% and 40·6% of patients had achieved at least very good partial response after the first, second, third and fourth treatment lines. Median overall survival (OS) from first, second, third and fourth lines was 45 months, 56 months, 37 months and not reached, respectively (P = 0·109). In summary, although relapses occur in AL amyloidosis, the outcomes and responses do not worsen with each subsequent relapse, making it attractive to design therapeutics with curative intent. [ABSTRACT FROM AUTHOR]
- Published
- 2021
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20. Renal transplant outcomes in amyloidosis.
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Law, Steven, Cohen, Oliver, Lachmann, Helen J, Rezk, Tamer, Gilbertson, Janet A, Rowczenio, Dorota, Wechalekar, Ashutosh D, Hawkins, Philip N, Motallebzadeh, Reza, and Gillmore, Julian D
- Subjects
- *
POLYCYSTIC kidney disease , *AMYLOIDOSIS , *KIDNEY transplantation , *PROPENSITY score matching - Abstract
Background Outcomes after renal transplantation have traditionally been poor in systemic amyloid A (AA) amyloidosis and systemic light chain (AL) amyloidosis, with high mortality and frequent recurrent disease. We sought to compare outcomes with matched transplant recipients with autosomal dominant polycystic kidney disease (ADPKD) and diabetic nephropathy (DN), and identify factors predictive of outcomes. Methods We performed a retrospective cohort study of 51 systemic AL and 48 systemic AA amyloidosis patients undergoing renal transplantation. Matched groups were generated by propensity score matching. Patient and death-censored allograft survival were compared via Kaplan–Meier survival analyses, and assessment of clinicopathological features predicting outcomes via Cox proportional hazard analyses. Results One-, 5- and 10-year death-censored unadjusted graft survival was, respectively, 94, 91 and 78% for AA amyloidosis, and 98, 93 and 93% for AL amyloidosis; median patient survival was 13.1 and 7.9 years, respectively. Patient survival in AL and AA amyloidosis was comparable to DN, but poorer than ADPKD [hazard ratio (HR) = 3.12 and 3.09, respectively; P < 0.001]. Death-censored allograft survival was comparable between all groups. In AL amyloidosis, mortality was predicted by interventricular septum at end diastole (IVSd) thickness >12 mm (HR = 26.58; P = 0.03), while survival was predicted by haematologic response (very good partial or complete response; HR = 0.07; P = 0.018). In AA amyloidosis, recurrent amyloid was associated with elevated serum amyloid A concentration but not with outcomes. Conclusions Renal transplantation outcomes for selected patients with AA and AL amyloidosis are comparable to those with DN. In AL amyloidosis, IVSd thickness and achievement of deep haematologic response pre-transplant profoundly impact patient survival. [ABSTRACT FROM AUTHOR]
- Published
- 2021
- Full Text
- View/download PDF
21. Disease progression in cardiac transthyretin amyloidosis is indicated by serial calculation of National Amyloidosis Centre transthyretin amyloidosis stage.
- Author
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Law, Steven, Petrie, Aviva, Chacko, Liza, Cohen, Oliver C., Ravichandran, Sriram, Gilbertson, Janet A., Rowczenio, Dorota, Wechalekar, Ashutosh, Martinez‐Naharro, Ana, Lachmann, Helen J., Whelan, Carol J., Hutt, David F., Hawkins, Philip N., Fontana, Marianna, and Gillmore, Julian D.
- Subjects
TRANSTHYRETIN ,DISEASE progression ,AMYLOIDOSIS - Abstract
Aims: Cardiac transthyretin amyloidosis (ATTR‐CM) is a progressive and fatal condition. Prognosis can be determined at diagnosis according to the National Amyloidosis Centre (NAC) transthyretin amyloidosis (ATTR) stage. We sought to examine how NAC ATTR stage changes during follow‐up and whether it maintains its prognostic value throughout the disease course. Methods and results: We performed a retrospective study of 945 patients with wild‐type ATTR‐CM (wtATTR‐CM) or hereditary ATTR‐CM associated with the V122I variant (V122I‐hATTR‐CM) who were diagnosed and serially evaluated at the UK NAC. Patients who commenced any disease‐modifying therapy for amyloidosis were censored at the time of doing so. Landmark Kaplan–Meier survival analyses were performed at diagnosis (n = 945) and at 6 ± 1 (n = 432), 12 ± 3 (n = 562), and 24 ± 3 (n = 316) months and stratified by recalculated NAC ATTR stage at the relevant time point. Cox regression analyses were performed to assess the prognostic significance during follow‐up of an increase in NAC ATTR stage from Stage I at diagnosis. Mortality in ATTR‐CM was predicted by NAC ATTR stage at each time point [Stage II vs. I, hazard ratios (HRs) 1.95–2.67; P < 0.001; Stage III vs. II, HRs 1.64–2.25; P < 0.001–0.013]. An increase from NAC ATTR Stage I, which occurred in 21%, 32%, and 44% of evaluable patients at 6, 12, and 24 months of follow‐up respectively, was highly predictive of ongoing mortality at each time point (HRs 2.58–3.22; P < 0.001) and in each genotypic subgroup (HRs 1.86–4.38; P < 0.05). Increase in NAC ATTR stage occurred earlier in V122I‐hATTR‐CM than in wtATTR‐CM (43% vs. 27% at 12 months of follow‐up; P = 0.003). Conclusions: National Amyloidosis Centre ATTR stage predicts ongoing survival throughout the disease natural history in ATTR‐CM, and an increase from NAC ATTR Stage I at diagnosis to a higher NAC ATTR stage predicts mortality throughout follow‐up. Serial calculation of NAC ATTR stage suggests a more aggressive phenotype in V122I‐hATTR‐CM than in wtATTR‐CM. [ABSTRACT FROM AUTHOR]
- Published
- 2020
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- View/download PDF
22. Rapid response to single agent daratumumab is associated with improved progression-free survival in relapsed/refractory AL amyloidosis.
- Author
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Cohen, Oliver C., Brodermann, Maximillian H., Blakeney, Iona J., Mahmood, Shameem, Sachchithanantham, Sajitha, Ravichandran, Sriram, Law, Steven, Lachmann, Helen J., Whelan, Carol J., Popat, Rakesh, Rabin, Neil, Yong, Kwee, Kyriakou, Charalampia, Shah, Raakhee, Cheesman, Simon, Worthington, Sarah, Hawkins, Philip, Gillmore, Julian D., and Wechalekar, Ashutosh D.
- Subjects
PROGRESSION-free survival ,CD38 antigen ,PLASMA cells ,AMYLOIDOSIS ,DARATUMUMAB - Abstract
Background: Daratumumab is a monoclonal antibody, which targets CD38; an antigen expressed on malignant plasma cells in AL amyloidosis thus providing a rationale for its use. Method: Patients treated with daratumumab monotherapy (2016–2019) for relapsed/refractory systemic AL amyloidosis were identified from the database at the UK National Amyloidosis Centre. Results: Of 50 evaluable patients, haematological responses at 3 months were: CR – 19 (38%), VGPR – 14 (28%), PR – 9 (18%) and no response – 8 (16%). Median time to response was 1 (1–6) month. Of assessable patients, cardiac, renal and hepatic responses were seen in 43.8%, 25.0% and 0% of patients whilst progression occurred in 25.0%, 12.5% and 37.5% respectively. Patients achieving a CR had longer median OS (not reached vs. 22.7 months [95% CI 17.0–28.4 months]) (p =.036). Furthermore, patients achieving a rapid response (at 1 month) had a longer median PFS (not reached vs. 9 months [95% CI 5.8–12.2 months]) (p =.013). Conclusion: Daratumumab monotherapy is effective in multiply-relapsed systemic AL amyloidosis and should be considered, if available, in patients who have not received prior daratumumab therapy. Responses are achieved rapidly and overall response rate was 84%. CR predicts overall survival whilst speed of response is predictive of a longer PFS. [ABSTRACT FROM AUTHOR]
- Published
- 2020
- Full Text
- View/download PDF
23. Glucose absorption from peritoneal dialysate is associated with a gain in fat mass and a reduction in lean body mass in prevalent peritoneal dialysis patients.
- Author
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Law, Steven and Davenport, Andrew
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ADIPOSE tissues ,CONFIDENCE intervals ,FAT substitutes ,GLUCOSE ,PERITONEAL dialysis ,DESCRIPTIVE statistics ,ODDS ratio - Abstract
The majority of peritoneal dialysates use glucose to generate an osmotic gradient for the convective removal of water and Na. Although glucose can potentially be absorbed, previous studies have failed to establish whether this leads to increased fat weight gain. We measured body composition using bioimpedance in peritoneal dialysis (PD) patients, electively starting PD, attending for their first assessment of peritoneal membrane function after 2–3 months, and then after 12 months. We studied 143 patients: eighty-nine (62·2 %) males, fifty-three (37·1 %) diabetics, mean age 61·3 (SD 14·9) years, with ninety (62·1 %) patients treated by automated PD cyclers with a daytime icodextrin exchange and thirty-seven (25·9 %) by continuous ambulatory PD. Median fat mass increased by 1·8 (–0·5 to 4·1) kg, whereas fat-free mass fell –1·3 (–2·9 to 1·0) kg, and the increase in fat mass was negatively associated with the fall in soft lean mass (r –0·41, P < 0·001). Increased fat mass was associated with measured peritoneal glucose absorption (r 0·69, P < 0·001), and glucose absorption was associated with the amount of 22·7 g/l glucose dialysate (OR 2·0, 95 % CI 1·5, 2·5, P < 0·001), peritoneal urea clearance (OR 9·5, 95 % CI 2·4, 37·1, P = 0·001) and male sex (OR 4·8, 95 % CI 1·5, 14·9, P = 0·008). We report an observational study in prevalent PD patients following body composition from their first assessment of PD membrane function for approximately 12 months, and despite the majority of patients prescribed icodextrin, we have demonstrated not only an association between intra-peritoneal glucose absorption and fat weight gain but also loss of fat-free mass. [ABSTRACT FROM AUTHOR]
- Published
- 2020
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24. The effect of glucose absorption from peritoneal dialysates on changes in lipid profiles in prevalent peritoneal dialysis patients.
- Author
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Law, Steven and Davenport, Andrew
- Published
- 2021
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25. Law as Narrative: Narrative Interpretation and Appropriation as an Element of Theft.
- Author
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Law, Steven Cammiss Senior Lecturer in
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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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26. Long-Term Settlement of a Large Water Standpipe.
- Author
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Law, Steven and Kasprzak, John
- Subjects
- *
STANDPIPES , *TANK industry - Abstract
Provides information on Accokeek standpipe in Washington D.C. Water capacity; Features; Subsurface site conditions.
- Published
- 2001
27. P-024: Early relapse is an adverse prognostic marker in systemic immunoglobulin light chain (AL) Amyloidosis.
- Author
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Ravichandran, Sriram, Law, Steven, Mahmood, Shameem, Wisniowski, Brendan, Foard, Darren, Fontana, Marianna, Martinez-Naharro, Ana, Whelan, Carol, Gillmore, Jullian, Lachmann, Helen, Hawkins, Philip, and Wechalekar, Ashutosh
- Published
- 2021
- Full Text
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28. Cardiac Magnetic Resonance–Derived Extracellular Volume Mapping for the Quantification of Hepatic and Splenic Amyloid.
- Author
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Chacko, Liza, Boldrini, Michele, Martone, Raffaele, Law, Steven, Martinez-Naharrro, Ana, Hutt, David F., Kotecha, Tushar, Patel, Rishi K., Razvi, Yousuf, Rezk, Tamer, Cohen, Oliver C., Brown, James T., Srikantharajah, Mukunthan, Ganesananthan, Sharmananthan, Lane, Thirusha, Lachmann, Helen J., Wechalekar, Ashutosh D., Sachchithanantham, Sajitha, Mahmood, Shameem, and Whelan, Carol J.
- Abstract
Background: Systemic amyloidosis is characterized by amyloid deposition that can involve virtually any organ. Splenic and hepatic amyloidosis occurs in certain types, in some patients but not others, and may influence prognosis and treatment. SAP (serum amyloid P component) scintigraphy is uniquely able to identify and quantify amyloid in the liver and spleen, thus informing clinical management, but it is only available in 2 centers globally. The aims of this study were to examine the potential for extracellular volume (ECV) mapping performed during routine cardiac magnetic resonance to: (1) detect amyloid in the liver and spleen and (2) estimate amyloid load in these sites using SAP scintigraphy as the reference standard. Methods: Five hundred thirty-three patients referred to the National Amyloidosis Centre, London, between 2015 and 2017 with suspected systemic amyloidosis who underwent SAP scintigraphy and cardiac magnetic resonance with T1 mapping were studied. Results: The diagnostic performance of ECV to detect splenic and hepatic amyloidosis was high for both organs (liver: area under the curve, −0.917 [95% CI, 0.880–0.954]; liver ECV cutoff, 0.395; sensitivity, 90.7%; specificity, 77.7%; P <0.001; spleen: area under the curve, −0.944 [95% CI, 0.925–0.964]; spleen ECV cutoff, 0.385; sensitivity, 93.6%; specificity, 87.5%; P <0.001). There was good correlation between liver and spleen ECV and amyloid load assessed by SAP scintigraphy (r=0.504, P <0.001; r=0.693, P <0.001, respectively). There was high interobserver agreement for both the liver and spleen (ECV liver intraclass correlation coefficient, 0.991 [95% CI, 0.984–0.995]; P <0.001; ECV spleen intraclass correlation coefficient, 0.995 [95% CI, 0.991–0.997]; P <0.001) with little bias across a wide range of ECV values. Conclusions: Our study demonstrates that ECV measurements obtained during routine cardiac magnetic resonance scans in patients with suspected amyloidosis can identify and measure the magnitude of amyloid infiltration in the liver and spleen, providing important clues to amyloid type and offering a noninvasive measure of visceral amyloid burden that can help guide and track treatment. [ABSTRACT FROM AUTHOR]
- Published
- 2021
- Full Text
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29. Sewer Outfall Replacement Relies on Piers.
- Author
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Law, Steven
- Subjects
- *
PIPELINES , *SEWAGE purification , *SEWERAGE - Abstract
Discusses the reconstruction of the sewer pipelines of the Washington Suburban Sanitary Commission for the Riverview subdivision in Prince George's County, Maryland. Measurements of the pipelines; Topography of the pipeline area; Installation process of the pipelines.
- Published
- 2002
30. Extensive subcortical white matter, deep grey matter and brainstem oedema in a confused patient: Answer.
- Author
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Blakeway, Bryony, Law, Steven, and Rajakulendran, Sanjeev
- Published
- 2017
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31. Extensive subcortical white matter, deep grey matter and brainstem oedema in a confused patient: Question.
- Author
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Blakeway, Bryony, Law, Steven, and Rajakulendran, Sanjeev
- Published
- 2017
- Full Text
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32. Analysis of Consolidation Behavior of Mica Dam
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Eisenstein, Zdenek and Law, Steven T. C.
- Published
- 1977
- Full Text
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33. Sources of metals in municipal incinerator emissions
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Gordon, Glen E. and Law, Steven L.
- Published
- 1979
- Full Text
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