10 results on '"Plana, Josep Maria Campistol"'
Search Results
2. #2908 Renal progenitor cells an early non-invasive biomarker of silent kidney injury in Fabry disease
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Ugalde-Altamirano, Jessica, primary, Juarez, Jordi Rovira, additional, Tubita, Valeria, additional, Plana, Josep Maria Campistol, additional, Poch, Esteban, additional, Diekmann, Fritz, additional, Piñeiro, Gaston, additional, Cucchiari, David, additional, Azorin, Sebastian, additional, Banon, Elisenda, additional, Corral-Velez, Vicente, additional, and Prats, José Vicente Torregrosa, additional
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- 2024
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3. #3006 Therapeutic apheresis in Fabry disease
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Ugalde-Altamirano, Jessica, primary, Juarez, Jordi Rovira, additional, Plana, Josep Maria Campistol, additional, Lozano, Miquel, additional, Cid, Joan, additional, Piñeiro, Gaston, additional, Ramirez, Maria Jose, additional, Corral-Velez, Vicente, additional, Revuelta, Ignacio, additional, Roca, Ramon, additional, Calls, Jordi, additional, and Prats, José Vicente Torregrosa, additional
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- 2024
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4. Clinical and genetic profile of patients enrolled in the Transthyretin Amyloidosis Outcomes Survey (THAOS) : 14-year update
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Dispenzieri, Angela, Coelho, Teresa, Conceição, Isabel, Waddington-Cruz, Márcia, Wixner, Jonas, Kristen, Arnt V., Rapezzi, Claudio, Planté-Bordeneuve, Violaine, Gonzalez-Moreno, Juan, Maurer, Mathew S., Grogan, Martha, Chapman, Doug, Amass, Leslie, Pavia, Pablo Garcia, Tarnev, Ivaylo, Costello, Jose Gonzalez, Briseno, Maria Alejandra Gonzalez Duarte, Schmidt, Hartmut, Drachman, Brian, Barroso, Fabio Adrian, Yamashita, Taro, Lairez, Olivier, Sekijima, Yoshiki, Vita, Giuseppe, Jeon, Eun-Seok, Hanna, Mazen, Slosky, David, Luigetti, Marco, LoRusso, Samantha, Beamud, Francisco Munoz, Adams, David, Moelgaard, Henning, Press, Rayomand, Cirami, Calogero Lino, Nienhuis, Hans, Plana, Josep Maria Campistol, Inamo, Jocelyn, Jacoby, Daniel, Emdin, Michele, Quan, Dianna, Hummel, Scott, Witteles, Ronald, Dori, Amir, Shah, Sanjiv, Lenihan, Daniel, Azevedo, Olga, Murali, Srinivas, Zivkovic, Sasa, Low, Soon Chai, Nativi-Nicolau, Jose, Fine, Nowell, Tallaj, Jose, Tschoepe, Carsten, Torrón, Roberto Fernandéz, Polydefkis, Michael, Merlini, Giampaolo, Badelita, Sorina, Gottlieb, Stephen, Tauras, James, Correia, Edileide Barros, Ventura, Hector, Gess, Burkhard, Darstein, Felix, Oh, Jeeyoung, Marburger, Tessa, Van Cleemput, Johan, Salutto, Valeria Lujan, Parman, Yesim, Chao, Chi-Chao, Sarswat, Nitasha, Mueller, Christopher, Steidley, David, Ralph, Jeffrey, Warner, Alberta, Cotts, William, Hoffman, James, Rugiero, Marcelo, Misawa, Sonoko, Blanco, Jose Luis Munoz, Davila, Lucia Galan, Sadeh, Menachem, Luo, Jin, Kyriakides, Theodoros, Wang, Annabel, Kaufmann, Horacio, Dispenzieri, Angela, Coelho, Teresa, Conceição, Isabel, Waddington-Cruz, Márcia, Wixner, Jonas, Kristen, Arnt V., Rapezzi, Claudio, Planté-Bordeneuve, Violaine, Gonzalez-Moreno, Juan, Maurer, Mathew S., Grogan, Martha, Chapman, Doug, Amass, Leslie, Pavia, Pablo Garcia, Tarnev, Ivaylo, Costello, Jose Gonzalez, Briseno, Maria Alejandra Gonzalez Duarte, Schmidt, Hartmut, Drachman, Brian, Barroso, Fabio Adrian, Yamashita, Taro, Lairez, Olivier, Sekijima, Yoshiki, Vita, Giuseppe, Jeon, Eun-Seok, Hanna, Mazen, Slosky, David, Luigetti, Marco, LoRusso, Samantha, Beamud, Francisco Munoz, Adams, David, Moelgaard, Henning, Press, Rayomand, Cirami, Calogero Lino, Nienhuis, Hans, Plana, Josep Maria Campistol, Inamo, Jocelyn, Jacoby, Daniel, Emdin, Michele, Quan, Dianna, Hummel, Scott, Witteles, Ronald, Dori, Amir, Shah, Sanjiv, Lenihan, Daniel, Azevedo, Olga, Murali, Srinivas, Zivkovic, Sasa, Low, Soon Chai, Nativi-Nicolau, Jose, Fine, Nowell, Tallaj, Jose, Tschoepe, Carsten, Torrón, Roberto Fernandéz, Polydefkis, Michael, Merlini, Giampaolo, Badelita, Sorina, Gottlieb, Stephen, Tauras, James, Correia, Edileide Barros, Ventura, Hector, Gess, Burkhard, Darstein, Felix, Oh, Jeeyoung, Marburger, Tessa, Van Cleemput, Johan, Salutto, Valeria Lujan, Parman, Yesim, Chao, Chi-Chao, Sarswat, Nitasha, Mueller, Christopher, Steidley, David, Ralph, Jeffrey, Warner, Alberta, Cotts, William, Hoffman, James, Rugiero, Marcelo, Misawa, Sonoko, Blanco, Jose Luis Munoz, Davila, Lucia Galan, Sadeh, Menachem, Luo, Jin, Kyriakides, Theodoros, Wang, Annabel, and Kaufmann, Horacio
- Abstract
Background: Transthyretin amyloidosis (ATTR amyloidosis) is a rare, life-threatening disease caused by the accumulation of variant or wild-type (ATTRwt amyloidosis) transthyretin amyloid fibrils in the heart, peripheral nerves, and other tissues and organs. Methods: Established in 2007, the Transthyretin Amyloidosis Outcomes Survey (THAOS) is the largest ongoing, global, longitudinal observational study of patients with ATTR amyloidosis, including both inherited and wild-type disease, and asymptomatic carriers of pathogenic TTR mutations. This descriptive analysis examines baseline characteristics of symptomatic patients and asymptomatic gene carriers enrolled in THAOS since its inception in 2007 (data cutoff: August 1, 2021). Results: This analysis included 3779 symptomatic patients and 1830 asymptomatic gene carriers. Symptomatic patients were predominantly male (71.4%) and had a mean (standard deviation [SD]) age of symptom onset of 56.3 (17.8) years. Val30Met was the most common genotype in symptomatic patients in South America (80.9%), Europe (55.4%), and Asia (50.5%), and more patients had early- versus late-onset disease in these regions. The majority of symptomatic patients in North America (58.8%) had ATTRwt amyloidosis. The overall distribution of phenotypes in symptomatic patients was predominantly cardiac (40.7%), predominantly neurologic (40.1%), mixed (16.6%), and no phenotype (2.5%). In asymptomatic gene carriers, mean (SD) age at enrollment was 42.4 (15.7) years, 42.4% were male, and 73.2% carried the Val30Met mutation. Conclusions: This 14-year global overview of THAOS in over 5000 patients represents the largest analysis of ATTR amyloidosis to date and highlights the genotypic and phenotypic heterogeneity of the disease. ClinicalTrials.gov Identifier: NCT00628745.
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- 2022
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5. Clinical and genetic profile of patients enrolled in the Transthyretin Amyloidosis Outcomes Survey (THAOS)
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Dispenzieri, Angela, Coelho, Teresa, Conceição, Isabel, Waddington-Cruz, Márcia, Wixner, Jonas, Kristen, Arnt V., Rapezzi, Claudio, Planté-Bordeneuve, Violaine, Gonzalez-Moreno, Juan, Maurer, Mathew S., Grogan, Martha, Chapman, Doug, Amass, Leslie, Pavia, Pablo Garcia, Tarnev, Ivaylo, Costello, Jose Gonzalez, Briseno, Maria Alejandra Gonzalez Duarte, Schmidt, Hartmut, Drachman, Brian, Barroso, Fabio Adrian, Yamashita, Taro, Lairez, Olivier, Sekijima, Yoshiki, Vita, Giuseppe, Jeon, Eun-Seok, Hanna, Mazen, Slosky, David, Luigetti, Marco, LoRusso, Samantha, Beamud, Francisco Munoz, Adams, David, Moelgaard, Henning, Press, Rayomand, Cirami, Calogero Lino, Nienhuis, Hans, Plana, Josep Maria Campistol, Inamo, Jocelyn, Jacoby, Daniel, Emdin, Michele, Quan, Dianna, Hummel, Scott, Witteles, Ronald, Dori, Amir, Shah, Sanjiv, Lenihan, Daniel, Azevedo, Olga, Murali, Srinivas, Zivkovic, Sasa, Low, Soon Chai, Nativi-Nicolau, Jose, Fine, Nowell, Tallaj, Jose, Tschoepe, Carsten, Torrón, Roberto Fernandéz, Polydefkis, Michael, Merlini, Giampaolo, Badelita, Sorina, Gottlieb, Stephen, Tauras, James, Correia, Edileide Barros, Ventura, Hector, Gess, Burkhard, Darstein, Felix, Oh, Jeeyoung, Marburger, Tessa, Van Cleemput, Johan, Salutto, Valeria Lujan, Parman, Yesim, Chao, Chi-Chao, Sarswat, Nitasha, Mueller, Christopher, Steidley, David, Ralph, Jeffrey, Warner, Alberta, Cotts, William, Hoffman, James, Rugiero, Marcelo, Misawa, Sonoko, Blanco, Jose Luis Munoz, Davila, Lucia Galan, Sadeh, Menachem, Luo, Jin, Kyriakides, Theodoros, Wang, Annabel, and Kaufmann, Horacio
- Subjects
Male ,Amyloid Neuropathies, Familial ,Registry ,Neurologi ,Cardiomyopathy ,General Medicine ,Amyloidosis ,Genetic Profile ,Amyloid Neuropathies ,Transthyretin ,Phenotype ,Familial ,Neurology ,Surveys and Questionnaires ,Polyneuropathy ,Humans ,Prealbumin ,Pharmacology (medical) ,Female ,Genetics (clinical) - Abstract
Background Transthyretin amyloidosis (ATTR amyloidosis) is a rare, life-threatening disease caused by the accumulation of variant or wild-type (ATTRwt amyloidosis) transthyretin amyloid fibrils in the heart, peripheral nerves, and other tissues and organs. Methods Established in 2007, the Transthyretin Amyloidosis Outcomes Survey (THAOS) is the largest ongoing, global, longitudinal observational study of patients with ATTR amyloidosis, including both inherited and wild-type disease, and asymptomatic carriers of pathogenic TTR mutations. This descriptive analysis examines baseline characteristics of symptomatic patients and asymptomatic gene carriers enrolled in THAOS since its inception in 2007 (data cutoff: August 1, 2021). Results This analysis included 3779 symptomatic patients and 1830 asymptomatic gene carriers. Symptomatic patients were predominantly male (71.4%) and had a mean (standard deviation [SD]) age of symptom onset of 56.3 (17.8) years. Val30Met was the most common genotype in symptomatic patients in South America (80.9%), Europe (55.4%), and Asia (50.5%), and more patients had early- versus late-onset disease in these regions. The majority of symptomatic patients in North America (58.8%) had ATTRwt amyloidosis. The overall distribution of phenotypes in symptomatic patients was predominantly cardiac (40.7%), predominantly neurologic (40.1%), mixed (16.6%), and no phenotype (2.5%). In asymptomatic gene carriers, mean (SD) age at enrollment was 42.4 (15.7) years, 42.4% were male, and 73.2% carried the Val30Met mutation. Conclusions This 14-year global overview of THAOS in over 5000 patients represents the largest analysis of ATTR amyloidosis to date and highlights the genotypic and phenotypic heterogeneity of the disease. ClinicalTrials.gov Identifier: NCT00628745.
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- 2022
6. Efficacy and safety with >3 years of inotersen treatment for the polyneuropathy of hereditary transthyretin amyloidosis
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Coelho, Teresa, Whelan, Carol, Conceição, Isabel, Brannagan, Thomas, Wang, Annabel, Polydefkis, Michael, Dyck, Peter, Planté-Bordeneuve, Violaine, Berk, John, Merlini, Giampaolo, Obici, Laura, Drachman, Brian, Gorevic, Peter, Schmidt, Hartmut, Plana, Josep Maria Campistol, Gamez, Josep, Kristen, Arnt, Mazzeo, Anna, Narayana, Arvind, Olugemo, Kemi, Aquino, Peter, Benson, Merrill, and Gertz, Morie
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- 2021
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7. Characteristics of Patients with Late- vs. Early-Onset Val30Met Transthyretin Amyloidosis from the Transthyretin Amyloidosis Outcomes Survey (THAOS).
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Waddington-Cruz, Márcia, Wixner, Jonas, Amass, Leslie, Kiszko, Jan, Chapman, Doug, Ando, Yukio, the THAOS investigators, Barroso, Fabio Adrian, Rugiero, Marcelo, Van Cleemput, Johan, Tarnev, Ivaylo, Kyriakides, Theodoros, Kristen, Arnt, Schmidt, Hartmut, Darstein, Felix, Gess, Burkhard, Plana, Josep Maria Campistol, Moreno, Juan Gonzalez, Costello, Jose Gonzalez, and Pavia, Pablo Garcia
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- 2021
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8. Impact of genotype and phenotype on cardiac biomarkers in patients with transthyretin amyloidosis - Report from the Transthyretin Amyloidosis Outcome Survey (THAOS)
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Kristen, Arnt V., Maurer, Mathew S., Rapezzi, Claudio, Mundayat, Rajiv, Suhr, Ole B., Damy, Thibaud, Barroso, Fabio Adrian, Rugiero, Marcelo F, Van Cleemput, Johan J., Tournev, Ivailo, Cruz, Marcia Waddington, Fine, Nowell M., Kristen, Arnt Volko, Schmidt, Hartmut H. J., Zimmermann, Tim, Gess, Burkhard, Moelgaard, Henning, Plana, Josep Maria Campistol, Reines, Juan Buades, Costello, Jose Gonzalez, Pavia, Pablo Garcia, Blanco, Jose Luis Munoz, Plante Bordeneuve, Violaine, Adams, David, Inamo, Jocelyn, Vita, Giuseppe, Merlini, Giampaolo, Bergesio, Franco, Sekijima, Yoshiki, Ando, Yukio, Misawa, Sonoko, Lee, Ga Yeon, Jeeyoung, Oh, Briseno, Maria Alejandra Gonzalez Duarte, Hazenberg, Bouke P. C., Coelho, Teresa, Conceicao, Isabel M., Maurer, Mathew Shane, Shah, Sanjiv Jayendra, Quan, Dianna, Judge, Daniel Philip, Gottlieb, Stephen Scott, Sarswat, Nitasha, Murali, Srinivas C., Iyadurai, Stanley, Cotts, William Gerritt, Drachman, Brian M., Dispenzieri, Angela, Steidley, David Eric, Hummel, Scott L., Lenihan, Daniel J., Ventura, Hector Osvaldo, Jacoby, Daniel L., Hoffman, James E., Kristen, Arnt V., Maurer, Mathew S., Rapezzi, Claudio, Mundayat, Rajiv, Suhr, Ole B., Damy, Thibaud, Barroso, Fabio Adrian, Rugiero, Marcelo F, Van Cleemput, Johan J., Tournev, Ivailo, Cruz, Marcia Waddington, Fine, Nowell M., Kristen, Arnt Volko, Schmidt, Hartmut H.J., Zimmermann, Tim, Gess, Burkhard, Moelgaard, Henning, Plana, Josep Maria Campistol, Reines, Juan Buade, Costello, Jose Gonzalez, Pavia, Pablo Garcia, Blanco, Jose Luis Munoz, Plante-Bordeneuve, Violaine, Adams, David, Inamo, Jocelyn, Vita, Giuseppe, Merlini, Giampaolo, Bergesio, Franco, Sekijima, Yoshiki, Ando, Yukio, Misawa, Sonoko, Lee, Ga Yeon, Oh, Jeeyoung, Briseno, Maria Alejandra Gonzalez Duarte, Hazenberg, Bouke P.C., Coelho, Teresa, Conceicao, Isabel M., Maurer, Mathew Shane, Shah, Sanjiv Jayendra, Quan, Dianna, Judge, Daniel Philip, Gottlieb, Stephen Scott, Sarswat, Nitasha, Murali, Srinivas C., Iyadurai, Stanley, Cotts, William Gerritt, Drachman, Brian M., Dispenzieri, Angela, Steidley, David Eric, Hummel, Scott L., Lenihan, Daniel J., Ventura, Hector Osvaldo, Jacoby, Daniel L., Hoffman, James E., and Translational Immunology Groningen (TRIGR)
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Genetics and Molecular Biology (all) ,Pathology ,Physiology ,medicine.medical_treatment ,Peptide Hormones ,lcsh:Medicine ,Medicine (all) ,Biochemistry ,Agricultural and Biological Sciences (all) ,030204 cardiovascular system & hematology ,Liver transplantation ,Pathology and Laboratory Medicine ,Body Mass Index ,0302 clinical medicine ,Genotype-phenotype distinction ,Surveys and Questionnaires ,Genotype ,Natriuretic Peptide, Brain ,Natriuretic peptide ,Medicine and Health Sciences ,Atrial natriuretic peptides ,Cardiac and Cardiovascular Systems ,lcsh:Science ,Multidisciplinary ,Kardiologi ,biology ,Amyloidosis ,Biochemical markers ,Troponin ,Phenotype ,Physiological Parameters ,cardiovascular system ,Anatomy ,hormones, hormone substitutes, and hormone antagonists ,Research Article ,medicine.medical_specialty ,medicine.drug_class ,Cardiology ,Surgical and Invasive Medical Procedures ,macromolecular substances ,NO ,03 medical and health sciences ,Digestive System Procedures ,Signs and Symptoms ,Troponin T ,Diagnostic Medicine ,Natriuretic Peptide ,medicine ,Humans ,cardiovascular diseases ,Heart Failure ,Amyloid Neuropathies, Familial ,Transplantation ,Biochemistry, Genetics and Molecular Biology (all) ,business.industry ,lcsh:R ,Troponin I ,Body Weight ,Biology and Life Sciences ,Proteins ,Renal System ,Organ Transplantation ,medicine.disease ,Hormones ,Liver Transplantation ,Transthyretin ,Cytoskeletal Proteins ,Heart failure ,biology.protein ,lcsh:Q ,business ,030217 neurology & neurosurgery ,Biomarkers - Abstract
AIM:Cardiac troponins and natriuretic peptides are established for risk stratification in light-chain amyloidosis. Data on cardiac biomarkers in transthyretin amyloidosis (ATTR) are lacking. METHODS AND RESULTS:Patients (n = 1617) with any of the following cardiac biomarkers, BNP (n = 1079), NT-proBNP (n = 550), troponin T (n = 274), and troponin I (n = 108), available at baseline in the Transthyretin Amyloidosis Outcomes Survey (THAOS) were analyzed for differences between genotypes and phenotypes and their association with survival. Median level of BNP was 68.0 pg/mL (IQR 30.5-194.9), NT-proBNP 337.9 pg/mL (IQR 73.0-2584.0), troponin T 0.03 μg/L (IQR 0.01-0.05), and troponin I 0.08 μg/L (IQR 0.04-0.13). NT-proBNP and BNP were higher in wild-type than mutant-type ATTR, troponin T and I did not differ, respectively. Non-Val30Met patients had higher BNP, NT-proBNP and troponin T levels than Val30Met patients, but not troponin I. Late-onset Val30Met was associated with higher levels of troponin I and troponin T compared with early-onset. 115 patients died during a median follow-up of 1.2 years. Mortality increased with increasing quartiles (BNP/NT-proBNP Q1 = 1.7%, Q2 = 5.2%, Q3 = 21.7%, Q4 = 71.3%; troponin T/I Q1 = 6.5%, Q2 = 14.5%, Q3 = 33.9%, Q4 = 45.2%). Three-year overall-survival estimates for BNP/NT-proBNP and troponin T/I quartiles differed significantly (p
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- 2017
9. Adoptive Cellular Immunotherapy for the Control of Primary Membranous Nephropathy
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Busquets, Ainhoa Garcia, Matilla, Marina, Arana, Carolt, Méndez, Sergi Betriu, Ramirez-Bajo, Maria Jose, Bañón-Maneus, Elisenda, Font, Marc Xipell, Plana, Josep Maria Campistol, Juan, Manel, Quintana, Luis F., Palou, Eduard, Diekmann, Fritz, and Rovira, Jordi
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- 2023
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10. Immunogenicity and Safety of the Adjuvanted Recombinant Zoster Vaccine in Chronically Immunosuppressed Adults Following Renal Transplant: A Phase 3, Randomized Clinical Trial.
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Vink, Peter, Torrell, Josep Maria Ramon, Fructuoso, Ana Sanchez, Kim, Sung-Joo, Kim, Sang-il, Zaltzman, Jeff, Ortiz, Fernanda, Plana, Josep Maria Campistol, Rodriguez, Ana Maria Fernandez, Rodrigo, Henar Rebollo, Marti, Magda Campins, Perez, Rafael, Roncero, Francisco Manuel González, Kumar, Deepali, Chiang, Yang-Jen, Doucette, Karen, Pipeleers, Lissa, Morales, Maria Luisa Agüera, Rodriguez-Ferrero, Maria Luisa, and Secchi, Antonio
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HERPES zoster prevention ,GLYCOPROTEINS ,GRAFT rejection ,HOMOGRAFTS ,IMMUNOGLOBULINS ,IMMUNOLOGIC diseases ,IMMUNOSUPPRESSION ,KIDNEY transplantation ,PATIENT safety ,RECOMBINANT proteins ,STATISTICAL sampling ,T cells ,TRANSPLANTATION of organs, tissues, etc. ,RANDOMIZED controlled trials ,HERPES zoster vaccines ,IMMUNOCOMPROMISED patients ,ADULTS - Abstract
Background The incidence of herpes zoster is up to 9 times higher in immunosuppressed solid organ transplant recipients than in the general population. We investigated the immunogenicity and safety of an adjuvanted recombinant zoster vaccine (RZV) in renal transplant (RT) recipients ≥18 years of age receiving daily immunosuppressive therapy. Methods In this phase 3, randomized (1:1), observer-blind, multicenter trial, RT recipients were enrolled and received 2 doses of RZV or placebo 1–2 months (M) apart 4–18M posttransplant. Anti–glycoprotein E (gE) antibody concentrations, gE-specific CD4 T-cell frequencies, and vaccine response rates were assessed at 1M post–dose 1, and 1M and 12M post–dose 2. Solicited and unsolicited adverse events (AEs) were recorded for 7 and 30 days after each dose, respectively. Solicited general symptoms and unsolicited AEs were also collected 7 days before first vaccination. Serious AEs (including biopsy-proven allograft rejections) and potential immune-mediated diseases (pIMDs) were recorded up to 12M post–dose 2. Results Two hundred sixty-four participants (RZV: 132; placebo: 132) were enrolled between March 2014 and April 2017. gE-specific humoral and cell-mediated immune responses were higher in RZV than placebo recipients across postvaccination time points and persisted above prevaccination baseline 12M post–dose 2. Local AEs were reported more frequently by RZV than placebo recipients. Overall occurrences of renal function changes, rejections, unsolicited AEs, serious AEs, and pIMDs were similar between groups. Conclusions RZV was immunogenic in chronically immunosuppressed RT recipients. Immunogenicity persisted through 12M postvaccination. No safety concerns arose. Clinical Trials Registration NCT02058589. [ABSTRACT FROM AUTHOR]
- Published
- 2020
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