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1. Respiratory Diseases in Women

Author

Han, MeiLan K., Shteinberg, Michal, Assayag, Deborah, Schleich, Florence, Pengo, Martino, Scicluna, Victoria M., Lombardi, Carolina, Barrecheguren, Miriam, and Jara-Palomares, Luis

Published
2024
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2. Sex-Differences in Alpha-1 Antitrypsin Deficiency: Data From the EARCO Registry

Author

Ersöz, Hilal, Torres-Durán, María, Turner, Alice M., Tanash, Hanan, Rodríguez García, Carlota, Corsico, Angelo Guido, López-Campos, José Luis, Miravitlles, Marc, Clarenbach, Christian F., Chapman, Kenneth R., Hernández Pérez, José M., Guimarães, Catarina, Bartošovská, Eva, Greulich, Timm, Barrecheguren, Miriam, Koczulla, Andreas Rembert, Höger, Philipp, Olivares Rivera, Arturo, Herth, Felix, and Trudzinski, Franziska C.

Published
2024
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3. Correction: Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency: EARCO international registry

Author

Miravitlles, Marc, Turner, Alice M., Torres-Duran, María, Tanash, Hanan, Rodríguez-García, Carlota, López-Campos, José Luis, Chlumsky, Jan, Guimaraes, Catarina, Rodríguez-Hermosa, Juan Luis, Corsico, Angelo, Martinez-González, Cristina, Hernández-Pérez, José María, Bustamante, Ana, Parr, David G., Casas-Maldonado, Francisco, Hecimovic, Ana, Janssens, Wim, Lara, Beatriz, Barrecheguren, Miriam, González, Cruz, Stolk, Jan, Esquinas, Cristina, and Clarenbach, Christian F.

Published
2023
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5. Updated guidance on the management of COVID-19: from an American Thoracic Society/European Respiratory Society coordinated International Task Force (29 July 2020)

Author

Bai, Chunxue, Chotirmall, Sanjay H, Rello, Jordi, Alba, George A, Ginns, Leo C, Krishnan, Jerry A, Rogers, Robert, Bendstrup, Elisabeth, Burgel, Pierre-Regis, Chalmers, James D, Chua, Abigail, Crothers, Kristina A, Duggal, Abhijit, Kim, Yeon Wook, Laffey, John G, Luna, Carlos M, Niederman, Michael S, Raghu, Ganesh, Ramirez, Julio A, Riera, Jordi, Roca, Oriol, Tamae-Kakazu, Maximiliano, Torres, Antoni, Watkins, Richard R, Barrecheguren, Miriam, Belliato, Mirko, Chami, Hassan A, Chen, Rongchang, Cortes-Puentes, Gustavo A, Delacruz, Charles, Hayes, Margaret M, Heunks, Leo MA, Holets, Steven R, Hough, Catherine L, Jagpal, Sugeet, Jeon, Kyeongman, Johkoh, Takeshi, Lee, May M, Liebler, Janice, McElvaney, Gerry N, Moskowitz, Ari, Oeckler, Richard A, Ojanguren, Iñigo, O'Regan, Anthony, Pletz, Mathias W, Rhee, Chin Kook, Schultz, Marcus J, Storti, Enrico, Strange, Charlie, Thomson, Carey C, Torriani, Francesca J, Wang, Xun, Wuyts, Wim, Xu, Tao, Yang, Dawei, Zhang, Ziqiang, and Wilson, Kevin C

Subjects
Lung, Clinical Research, Clinical Trials and Supportive Activities, Advisory Committees, Betacoronavirus, COVID-19, Consensus, Coronavirus Infections, Europe, Humans, International Cooperation, Pandemics, Pneumonia, Viral, Pulmonary Medicine, SARS-CoV-2, Societies, Medical, United States, Medical Physiology, Respiratory System
Abstract

BackgroundCoronavirus disease 2019 (COVID-19) is a disease caused by severe acute respiratory syndrome-coronavirus-2. Consensus suggestions can standardise care, thereby improving outcomes and facilitating future research.MethodsAn International Task Force was composed and agreement regarding courses of action was measured using the Convergence of Opinion on Recommendations and Evidence (CORE) process. 70% agreement was necessary to make a consensus suggestion.ResultsThe Task Force made consensus suggestions to treat patients with acute COVID-19 pneumonia with remdesivir and dexamethasone but suggested against hydroxychloroquine except in the context of a clinical trial; these are revisions of prior suggestions resulting from the interim publication of several randomised trials. It also suggested that COVID-19 patients with a venous thromboembolic event be treated with therapeutic anticoagulant therapy for 3 months. The Task Force was unable to reach sufficient agreement to yield consensus suggestions for the post-hospital care of COVID-19 survivors. The Task Force fell one vote shy of suggesting routine screening for depression, anxiety and post-traumatic stress disorder.ConclusionsThe Task Force addressed questions related to pharmacotherapy in patients with COVID-19 and the post-hospital care of survivors, yielding several consensus suggestions. Management options for which there is insufficient agreement to formulate a suggestion represent research priorities.

Published
2020

7. Comparison of the Determinants of the "Chronic Obstructive Pulmonary Disease Assessment Test" (CAT) and the "Asthma Control Test" (ACT) in Patients with Asthma–COPD Overlap.

Author

Aljama, Cristina, Granados, Galo, Callejas-González, Francisco Javier, Martínez-Rivera, Carlos, Pallarés-Sanmartín, Abel, Rodríguez-Pons, Laura, Cabrera-César, Eva, Márquez-Martín, Eduardo, Boldova-Loscertales, Ana, Naval-Sendra, Elsa, Abascal-Bolado, Beatriz, Cabrera-López, Carlos, Miravitlles, Marc, Esquinas, Cristina, and Barrecheguren, Miriam

Subjects
CHRONIC obstructive pulmonary disease, COMORBIDITY, MULTIVARIATE analysis, EX-smokers, ASTHMA
Abstract

Objective: The objective of this study was to investigate which of two short questionnaires, the Asthma Control Test (ACT) or the COPD Assessment Test (CAT), correlates better with severity variables and whether they share similar determinants in patients with asthma–COPD overlap. Method: This observational, cross-sectional, multicentric study included smokers and former smokers of more than 10 pack-years, with non-fully reversible airflow obstruction and either a concomitant diagnosis of asthma or signs of type 2 inflammation, from 15 centres in Spain. Results: A total of 157 patients were included, 109 (69.4%) were men, the mean age was 63.3 (SD: 9) years and the mean FEV1 (%) was 59.7% (SD: 20.5%). The mean CAT score was 14.5 (SD: 8.7), and the mean ACT score was 17.9 (SD: 5.2). Both scores showed good correlations (r = 0.717; p < 0.001). In the multivariate analysis, the Hospital Anxiety and Depression Scale and mMRC dyspnoea scores were independently and significantly associated with both the CAT and ACT scores; however, age was only significantly associated with the CAT, and the EQ-5D scores and the number of exacerbations in the previous year were only significantly associated with the ACT scores. The ACT had a slightly better predictive value for exacerbations than the CAT (AUC = 0.70 (95% CI: 0.62 to 0.79 vs. 0.65 (95% CI: 0.56 to 0.74))). Conclusions: There is a good correlation between ACT and CAT scores in patients with ACO. However, severe patients scored worse on the CAT than the ACT. Anxiety, depression and dyspnoea were significantly associated with both the CAT and ACT scores. The ACT was a slightly better predictor of exacerbations than the CAT in this population. [ABSTRACT FROM AUTHOR]

Published
2024
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10. Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency: EARCO international registry

Author

Miravitlles, Marc, Turner, Alice M., Torres-Duran, María, Tanash, Hanan, Rodríguez-García, Carlota, López-Campos, José Luis, Chlumsky, Jan, Guimaraes, Catarina, Rodríguez-Hermosa, Juan Luis, Corsico, Angelo, Martinez-González, Cristina, Hernández-Pérez, José María, Bustamante, Ana, Parr, David G., Casas-Maldonado, Francisco, Hecimovic, Ana, Janssens, Wim, Lara, Beatriz, Barrecheguren, Miriam, González, Cruz, Stolk, Jan, Esquinas, Cristina, and Clarenbach, Christian F.

Published
2022
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15. COVID-19 in lung transplant recipients: A multicenter study

Author

Saez-Giménez, Berta, Berastegui, Cristina, Barrecheguren, Miriam, Revilla-López, Eva, Los Arcos, Ibai, Alonso, Rodrigo, Aguilar, Myriam, Mora, Víctor M., Otero, Isabel, Reig, Juan P., Quezada, Carlos A., Pérez, Virginia, Valle, Manuel, Laporta, Rosalía, Deu, María, Sacanell, Judith, Bravo, Carles, Gavalda, Joan, Lopez-Meseguer, Manuel, and Monforte, Víctor

Published
2021
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17. Changes in Control Status of COPD Over Time and Their Consequences: A Prospective International Study

Author

Miravitlles, Marc, Esquinas, Cristina, Barrecheguren, Miriam, Nuñez, Alexa, Alcazar, Bernardino, García-Rivero, Juan Luis, Hueso, Karina, Roman-Rodríguez, Miguel, Sliwinski, Pawel, Iwan, Katarzyna, Kolakowski, Jacek, Rhee, Chin Kook, Ahn, Esther, Tan, Jessica, Laperre, Therese, Leng, Karen Tan Li, Chia, Nicole, How, Ong Thun, Shamsuddin, SyifaBinte, Gim, Sherine Lim Shu, Bee, Yap Chwee, Ya, Soh Rui, Tee, Augustine, Yan, Jun Jie, Hong, Samuel, Tan, William, Carter, Victoria, Hardaker, Latife, McLaughlin, Andrew, Gouder, Caroline, Costello, Richard W., Tan, Jessica H.Y., Lapperre, Therese Sophie, Kemppinen, Anu, Soler-Cataluña, Juan José, and Price, David B.

Published
2021
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18. Chronic Obstructive Lung Disease: Treatment Guidelines and Recommendations for Referral and Multidisciplinary Continuity of Care

Author

Miguel Díez, Javier De, Fernández Villar, Alberto, Doña Díaz, Esperanza, Padilla Bernáldez, Marta, Trillo Calvo, Eva, Molina París, Jesús, Barrecheguren, Miriam, Valero Pérez, José Miguel, Ramírez Prieto, María Teresa, Miguel Díez, Javier De, Fernández Villar, Alberto, Doña Díaz, Esperanza, Padilla Bernáldez, Marta, Trillo Calvo, Eva, Molina París, Jesús, Barrecheguren, Miriam, Valero Pérez, José Miguel, and Ramírez Prieto, María Teresa

Abstract

Chronic Obstructive Pulmonary Disease (COPD) constitutes a major public health problem, and it is anticipated that its prevalence will continue to increase in the future. Its progressive nature requires a continuous and well-coordinated care approach. The follow-up for COPD should primarily focus on disease screening and control, which includes monitoring of pulmonary function, prevention of exacerbations, identification of aggravating factors and comorbidities, as well as ensuring treatment adequacy and adherence. However, existing clinical practice guidelines and consensus documents offer limited recommendations for the follow-up. In this context, we undertake a review of COPD treatment and the continuity of care recommendations endorsed by several scientific societies. Moreover, we underscore the importance of the involvement of nursing and community pharmacy in this process, as well as the utilization of quality indicators in the provision of care for the disease., Depto. de Medicina, Fac. de Medicina, TRUE, pub, Descuento UCM

Published
2024

19. Personalised indication of augmentation therapy for emphysema associated with severe alpha-1 antitrypsin deficiency: a case series.

Author

Aljama, Cristina, Martin, Teresa, Granados, Galo, Miravitlles, Marc, and Barrecheguren, Miriam

Subjects
TRAVEL restrictions, INTRAVENOUS therapy, TRYPSIN inhibitors, LUNG diseases, ALPHA 1-antitrypsin deficiency, BLOOD donors
Abstract

Severe alpha-1 antitrypsin deficiency (AATD) is associated with an increased risk of emphysema. However, the clinical manifestations are very heterogeneous, and an individual prognosis is very difficult to establish. Intravenous augmentation therapy with alpha-1 antitrypsin (AAT) from pooled blood donors is the only specific treatment available, but it requires weekly or biweekly administration for life. Several guidelines provide the indication criteria for the initiation of AAT augmentation therapy. However, in clinical practice, there are situations in which the decision as to when to start treatment becomes uncertain and some studies have shown great variability in the indication of this treatment even among specialists. The usual dilemma is between initiating augmentation therapy in individuals who may not develop significant lung disease or in whom disease will not progress or delaying it in patients who may otherwise rapidly and irreversibly progress. We illustrate this dilemma with five clinical cases: from the case of a patient with normal lung function who requests initiation of therapy to a moderately stable patient without augmentation or a mild patient who, after several years of remaining stable without treatment, deterioration in lung function initiated and, consequently, augmentation therapy was begun. All the nuances associated with the indication of augmentation justify a personalised approach and the decision about initiating augmentation therapy must be made after careful consideration of the pros and cons with the patient in reference centres with experience in treatment. These reference centres can work in collaboration with local hospitals where patients can be closely followed and augmentation therapy can be administered to avoid unnecessary travelling, making periodical administrations more comfortable for the patient. [ABSTRACT FROM AUTHOR]

Published
2024
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20. Motivation and Confidence about Physical Activity in Chronic Obstructive Pulmonary Disease Patients: Health Benefits Matter to Patients.

Author

Aljama, Cristina, Granados, Galo, Ramon, Marian, Barrecheguren, Miriam, Loeb, Eduardo, Nuñez, Alexa, Pleguezuelos, Eulogio, García-Río, Francisco, and Miravitlles, Marc

Subjects
FEAR, HEALTH attitudes, PLEASURE, RESEARCH funding, SCIENTIFIC observation, ACCELEROMETERS, QUESTIONNAIRES, CONFIDENCE, MOTIVATION (Psychology), LONGITUDINAL method, WALKING, OBSTRUCTIVE lung diseases, HEALTH behavior, RESEARCH, FORCED expiratory volume, PHYSICAL activity, PATIENTS' attitudes
Abstract

Introduction: Physical activity (PA) has shown great benefits in patients with chronic obstructive pulmonary disease (COPD); however, their PA is below average. Motivational factors associated with PA in COPD have not been widely studied and could be a target for improving adherence to PA. The objective of our study was to identify and understand the different motivational and confidence factors related to low levels of PA in a COPD cohort. Method: Observational, prospective, multicenter study of COPD patients. Sociodemographic data, respiratory symptoms, comorbidities, spirometry, and exercise capacity were collected. PA was measured using the Dynaport accelerometer and patient motivation and confidence in PA were assessed by a questionnaire previously used in a COPD population in the USA. Results: Eighty six COPD patients were included, 68.6% being male, with a mean (SD) age of 66.6 (8.5) years and a mean forced expiratory volume in the first second (%) of 50.9% (17.3%). The mean walking time was 82.8 (37.8) minutes/day. Questions related to health benefits and enjoying exercise were ranked highest in the motivation questionnaire and statistically significant differences were found in PA measures between patients with low and high motivation. A lack of confidence regarding hot weather and health-related issues significantly influenced PA levels. Advice from third parties, including healthcare providers, was not associated with higher PA levels. Conclusions: Improving the health of COPD patients is their main motivation to perform PA. Lack of confidence when it is hot or when they fear for their health is related to low levels of PA. Advice from third parties, including healthcare professionals, is not associated with higher PA. These results are relevant for developing strategies to increase the adherence of COPD patients to PA programs. [ABSTRACT FROM AUTHOR]

Published
2024
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22. Serum Levels of Alpha1-antitrypsin and Their Relationship With COPD in the General Spanish Population

Author

Ancochea, Julio, Badiola, Carlos, Sánchez, Guadalupe, Duran, Enric, García Río, Francisco, Miravitlles, Marc, Muñoz, Luis, Sobradillo, Víctor, Soriano, Joan B., Borderias, Luis, Martínez, Jaime, Montemayor, Teodoro, Piñeiro, Luis, Serra, Joan, Soler-Cataluña, Juan José, Torres, Antoni, Viejo, José Luis, Janciauskiene, Sabina, DeLuca, David S., Barrecheguren, Miriam, and Welte, Tobias

Published
2020
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23. Chronic Obstructive Lung Disease: Treatment Guidelines and Recommendations for Referral and Multidisciplinary Continuity of Care

Author

De Miguel-Díez, Javier, primary, Fernández-Villar, Alberto, additional, Doña Díaz, Esperanza, additional, Padilla Bernáldez, Marta, additional, Trillo-Calvo, Eva, additional, Molina París, Jesús, additional, Barrecheguren, Miriam, additional, Valero Pérez, José Miguel, additional, and Ramírez Prieto, María Teresa, additional

Published
2024
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27. Liver Fibrosis and Metabolic Alterations in Adults With alpha-1-antitrypsin Deficiency Caused by the Pi*ZZ Mutation

Author

Hamesch, Karim, Mandorfer, Mattias, Pereira, Vítor M., Moeller, Linda S., Pons, Monica, Dolman, Grace E., Reichert, Matthias C., Schneider, Carolin V., Woditsch, Vivien, Voss, Jessica, Lindhauer, Cecilia, Fromme, Malin, Spivak, Igor, Guldiken, Nurdan, Zhou, Biaohuan, Arslanow, Anita, Schaefer, Benedikt, Zoller, Heinz, Aigner, Elmar, Reiberger, Thomas, Wetzel, Martin, Siegmund, Britta, Simões, Carolina, Gaspar, Rui, Maia, Luís, Costa, Dalila, Bento-Miranda, Mário, van Helden, Josef, Yagmur, Eray, Bzdok, Danilo, Stolk, Jan, Gleiber, Wolfgang, Knipel, Verena, Windisch, Wolfram, Mahadeva, Ravi, Bals, Robert, Koczulla, Rembert, Barrecheguren, Miriam, Miravitlles, Marc, Janciauskiene, Sabina, Stickel, Felix, Lammert, Frank, Liberal, Rodrigo, Genesca, Joan, Griffiths, William J., Trauner, Michael, Krag, Aleksander, Trautwein, Christian, and Strnad, Pavel

Published
2019
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29. Enhanced liver fibrosis (ELF) test as a biomarker of pulmonary fibrosis (PF) secondary to COVID-19 pneumonia.

Author

Nuñez Dubon, Alexa Gabriela, primary, Aljama, Cristina, additional, Esquinas, Cristina, additional, Orriols, Gerard, additional, Farago, Georgina, additional, Granados, Galo, additional, Gabriel-Medina, Pablo, additional, Rodriguez-Frias, Francisco, additional, Pons, Mònica, additional, Miravitlles, Marc, additional, and Barrecheguren, Miriam, additional

Published
2023
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32. Recommendations for the Implementation of the Self-Administration of Alpha-1 Antitrypsin

Author

Torres-Durán, María, primary, López-Campos, José Luis, additional, Calle Rubio, Myriam, additional, Montero-Martínez, Carmen, additional, Priegue Carrera, Ana, additional, Amaro Rodríguez, Rosanel, additional, Barrecheguren, Miriam, additional, Barrio Guirado, María Ángeles, additional, Callejas-González, Francisco Javier, additional, Casas-Maldonado, Francisco, additional, Diab-Cáceres, Layla, additional, García-Meseguer, Pilar, additional, Hernández-Pérez, José María, additional, Lázaro-Asegurado, Lourdes, additional, Martínez-González, Cristina, additional, Martínez Rivera, Carlos, additional, Michel, Francisco Javier, additional, Montoro-Ronsano, José-Bruno, additional, Sánchez, Raquel, additional, Ortiz-Pica, Marta, additional, Parra, Isabel, additional, Quintero García, José Pablo, additional, Ruiz-Serrano-de la Espada, María del Rosario, additional, Tortajada-Goitia, Begoña, additional, and Miravitlles, Marc, additional

Published
2023
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33. Acquired tracheomalacia due to SARS‐CoV‐2 pneumonia.

Author

Espejo, David, Zapata, Marta, Omari, Saliha, Muñoz, Xavier, Cruz, Maria‐Jesús, Andreu, Jordi, Arjona, Marta, Berastegui, Cristina, Barrecheguren, Miriam, Cardoso, José, Clofent, David, Felipe, Almudena, Granados, Galo‐David, Jiménez, Ma Ángeles, López, Manuel, Ojanguren, Íñigo, Osorio, Jeisson, Pallero, Mercedes, Pilia, María‐Florencia, and Ramon, Ma Antonia

Subjects
SARS-CoV-2, PULMONARY function tests, COMPUTED tomography, PNEUMONIA, HOSPITAL admission & discharge
Abstract

Introduction: Several studies mentioned parenchymal findings after SARS‐CoV‐2 pneumonia, but few studies have mentioned alterations in the airways. The aim of this study was to estimate the prevalence of tracheomalacia and to analyse the clinical characteristics in a cohort of patients with SARS‐CoV‐2. Methods: The study population consisted of all patients with SARS‐CoV‐2 admitted a hospital serving a population of 500 000 inhabitants. Patients were visited between 2 and 6 months after hospital discharge. In this visit, all patients were subjected to an exhaustive clinical questionnaire and underwent clinical examination, pulmonary function tests and chest CT. Results: From February 2020 to August 2021, 1920 patients were included in the cohort and tracheomalacia was observed in 15 (0.8%) on expiratory HRCT imaging. All patients with tracheomalacia also presented ground glass opacities in the CT scan and 12 patients had airway sequelae. Conclusions: Tracheomalacia is an exceptional sequela of SARS‐CoV‐2 survivors. [ABSTRACT FROM AUTHOR]

Published
2024
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35. Recommendations for the Implementation of the Self-Administration of Alpha-1 Antitrypsin

Author

Torres-Durán,María, López-Campos,José Luis, Calle Rubio,Myriam, Montero-Martínez,Carmen, Priegue Carrera,Ana, Amaro Rodríguez,Rosanel, Barrecheguren,Miriam, Barrio Guirado,María Ángeles, Callejas-González,Francisco Javier, Casas-Maldonado,Francisco, Diab-Cáceres,Layla, García-Meseguer,Pilar, Hernández-Pérez,José María, Lázaro-Asegurado,Lourdes, Martínez-González,Cristina, Martínez Rivera,Carlos, Michel,Francisco Javier, Montoro-Ronsano,José-Bruno, Sánchez,Raquel, Ortiz-Pica,Marta, Parra,Isabel, Quintero García,José Pablo, Ruiz-Serrano-de la Espada,María del Rosario, Tortajada-Goitia,Begoña, Miravitlles,Marc, Torres-Durán,María, López-Campos,José Luis, Calle Rubio,Myriam, Montero-Martínez,Carmen, Priegue Carrera,Ana, Amaro Rodríguez,Rosanel, Barrecheguren,Miriam, Barrio Guirado,María Ángeles, Callejas-González,Francisco Javier, Casas-Maldonado,Francisco, Diab-Cáceres,Layla, García-Meseguer,Pilar, Hernández-Pérez,José María, Lázaro-Asegurado,Lourdes, Martínez-González,Cristina, Martínez Rivera,Carlos, Michel,Francisco Javier, Montoro-Ronsano,José-Bruno, Sánchez,Raquel, Ortiz-Pica,Marta, Parra,Isabel, Quintero García,José Pablo, Ruiz-Serrano-de la Espada,María del Rosario, Tortajada-Goitia,Begoña, and Miravitlles,Marc

Abstract

María Torres-Durán,1 José Luis López-Campos,2,3 Myriam Calle Rubio,4 Carmen Montero-Martínez,5 Ana Priegue Carrera,6 Rosanel Amaro Rodríguez,7 Miriam Barrecheguren,8 María Ángeles Barrio Guirado,9 Francisco Javier Callejas-González,10 Francisco Casas-Maldonado,11 Layla Diab-Cáceres,12 Pilar García-Meseguer,9 José María Hernández-Pérez,13 Lourdes Lázaro-Asegurado,14 Cristina Martínez-González,15 Carlos Martínez Rivera,16 Francisco Javier Michel,17 José-Bruno Montoro-Ronsano,18 Raquel Sánchez,19 Marta Ortiz-Pica,20 Isabel Parra,21 José Pablo Quintero García,22 María del Rosario Ruiz-Serrano-de la Espada,23 Begoña Tortajada-Goitia,24 Marc Miravitlles8 1Pneumology Department, Hospital Álvaro Cunqueiro, NeumoVigo I+i Research Group, IIS Galicia Sur, Vigo, Spain; 2Instituto de Salud Carlos III, Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Madrid, Spain; 3Medical and Surgery Unit for Respiratory Diseases, Instituto de Biomedicina de Sevilla (IBiS), Hospital Universitario Virgen del Rocío/Universidad de Sevilla, Seville, Spain; 4Pneumology Department, Research Institute of Hospital Clínico San Carlos (IdISSC), Department of Medicine, Faculty of Medicine, University Complutense of Madrid, Madrid, Spain; 5Pneumology Department, Hospital Universitario de A Coruña, A Coruña, Spain; 6Nursing Unit, Hospital Álvaro Cunqueiro, Pontevedra, Spain; 7Pneumology Department, Hospital Clínic, Barcelona, Spain; 8Pneumology Department, Hospital Universitari Vall d’Hebron, Vall d’Hebron Institut de Recerca (VHIR), Vall d’Hebron Barcelona Hospital Campus, Barcelona, Spain; 9Nursung Unit, Hospital Universitari Vall d’Hebron, Barcelona, Spain; 10Pneumology Department, Complejo Hospitalario Universitario de Albacete, Albacete, Spain; 11Pneumology Department, Hospital Universitario Clínico San Cecilio, Granada, Spain; 12Pneumology Department, Hospital Universitario 12 de Octubre, Madrid, Spain; 13Pneumology Department

Published
2023

36. Recommendations for the Implementation of the Self-Administration of Alpha-1 Antitrypsin

Author

CSL Behring, Torres-Durán, María, López-Campos, J. L., Calle Rubio, Myriam, Montero-Martínez, Carmen, Priegue Carrera, Ana, Amaro Rodríguez, Rosanel, Barrecheguren, Miriam, Barrio Guirado, María Ángeles, Callejas González, Francisco Javier, Casas-Maldonado, Francisco, Diab-Cáceres, Layla, García-Meseguer, Pilar, Hernández-Pérez, José María, Lázaro-Asegurado, Lourdes, Martínez-González, Cristina, Martínez Rivera, Carlos, Michel de la Rosa, Francisco Javier, Montoro, J. Bruno, Sánchez, Raquel, Ortiz-Pica, Marta, Parra, Isabel, Quintero-García, José Pablo, Ruiz-Serrano-de la Espada, María del Rosario, Tortajada-Goitia, Begoña, Miravitlles, Marc, CSL Behring, Torres-Durán, María, López-Campos, J. L., Calle Rubio, Myriam, Montero-Martínez, Carmen, Priegue Carrera, Ana, Amaro Rodríguez, Rosanel, Barrecheguren, Miriam, Barrio Guirado, María Ángeles, Callejas González, Francisco Javier, Casas-Maldonado, Francisco, Diab-Cáceres, Layla, García-Meseguer, Pilar, Hernández-Pérez, José María, Lázaro-Asegurado, Lourdes, Martínez-González, Cristina, Martínez Rivera, Carlos, Michel de la Rosa, Francisco Javier, Montoro, J. Bruno, Sánchez, Raquel, Ortiz-Pica, Marta, Parra, Isabel, Quintero-García, José Pablo, Ruiz-Serrano-de la Espada, María del Rosario, Tortajada-Goitia, Begoña, and Miravitlles, Marc

Abstract

Purpose: Administration of exogenous alpha-1 antitrypsin (AAT) is the only specific therapy for the management of pulmonary morbidity in patients with AAT deficiency. It requires weekly or biweekly intravenous infusions, which may impact patient independence and quality of life. Self-administration of AAT therapy is an alternative to reduce the burden for patients who require AAT therapy. We presented herein experts’ recommendations for the implementation of a program for the self-administration of AAT. Methods: This project was conducted using a modified nominal group technique and was undertaken in two online meetings involving the participation of 25 experts: specialists in pulmonology (n=17), nurses (n=5) and hospital pharmacists (n=3). Results: The following issues were discussed, and several recommendations were agreed upon on the following topics: a) patient profile and clinical evaluation, establishing selection criteria that should include clinical as well as social criteria; b) role of health care professionals, suggested roles for specialists in pulmonology, nurses, and hospital pharmacists; c) training by the nurse, including recommendations before initiating the training and the content of the training sessions; and d) logistic issues and follow-up, adherence, and patient support. Conclusion: We expect this proposal to increase awareness of this therapeutic alternative and facilitate the implementation of self-administration programs, thus contributing to optimizing the patient experience with AAT therapy. Further research on the outcomes of these programs, especially from the patient perspective, will also help to improve their design and implementation.

Published
2023

37. Characteristics of individuals with alpha-1 antitrypsin deficiency from Northern and Southern European countries: EARCO international registry

Author

Grifols, CSL Behring, Kamada, pH Pharma, Takeda Pharmaceutical Company, Miravitlles, Marc, Turner, Alice M., Torres-Durán, María, Tanash, Hanan, Rodríguez-García, Carlota, López-Campos, J. L., Chlumsky, Jan, Guimaraes, Catarina, Rodríguez-Hermosa, Juan Luis, Corsico, Angelo, Martínez-González, Cristina, Hernández-Pérez, José María, Bustamante, Ana, Parr, David G., Casas-Maldonado, Francisco, Hecimovic, Ana, Janssens, Wim, Lara, Beatriz, Barrecheguren, Miriam, González, Cruz, Stolk, Jan, Esquinas, Cristina, Clarenbach, Christian F., for the EARCO study investigators, Grifols, CSL Behring, Kamada, pH Pharma, Takeda Pharmaceutical Company, Miravitlles, Marc, Turner, Alice M., Torres-Durán, María, Tanash, Hanan, Rodríguez-García, Carlota, López-Campos, J. L., Chlumsky, Jan, Guimaraes, Catarina, Rodríguez-Hermosa, Juan Luis, Corsico, Angelo, Martínez-González, Cristina, Hernández-Pérez, José María, Bustamante, Ana, Parr, David G., Casas-Maldonado, Francisco, Hecimovic, Ana, Janssens, Wim, Lara, Beatriz, Barrecheguren, Miriam, González, Cruz, Stolk, Jan, Esquinas, Cristina, Clarenbach, Christian F., and for the EARCO study investigators

Abstract

Alpha-1 antitrypsin deficiency (AATD) is characterised by low serum levels of alpha-1 antitrypsin (AAT) and predisposes to the development of pulmonary emphysema and liver disease [1]. This deficiency is considered a rare condition and its prevalence ranges from one in 2400 to one in 6000 individuals in Western European countries [2]. Due to its low prevalence, large observational prospective registries are needed to collect information about the clinical characteristics and prognosis of the individuals affected.

Published
2023

38. The prevalence of bronchiectasis in patients with alpha-1 antitrypsin deficiency: initial report of EARCO

Author

Stockley, Robert A; https://orcid.org/0000-0003-3726-1207, Pye, Anita, De Soyza, Joshua, Turner, Alice M, Miravitlles, Marc, Torres-Duran, María, Tanash, Hanan, Rodríguez-García, Carlota, López-Campos, José Luis, Chlumsky, Jan, Guimaraes, Catarina, Rodríguez-Hermosa, Juan Luis, Corsico, Angelo, Martinez-González, Cristina, Hernández-Pérez, José María, Bustamante, Ana, Parr, David G, Casas-Maldonado, Francisco, Hecimovic, Ana, Janssens, Wim, Lara, Beatriz, Barrecheguren, Miriam, González, Cruz, Stolk, Jan, Clarenbach, Christian F, Stockley, Robert A; https://orcid.org/0000-0003-3726-1207, Pye, Anita, De Soyza, Joshua, Turner, Alice M, Miravitlles, Marc, Torres-Duran, María, Tanash, Hanan, Rodríguez-García, Carlota, López-Campos, José Luis, Chlumsky, Jan, Guimaraes, Catarina, Rodríguez-Hermosa, Juan Luis, Corsico, Angelo, Martinez-González, Cristina, Hernández-Pérez, José María, Bustamante, Ana, Parr, David G, Casas-Maldonado, Francisco, Hecimovic, Ana, Janssens, Wim, Lara, Beatriz, Barrecheguren, Miriam, González, Cruz, Stolk, Jan, and Clarenbach, Christian F

Abstract

Background: Although bronchiectasis has been recognised as a feature of some patients with Alpha1-Antitrypsin deficiency the prevalence and characteristics are not widely known. We wished to determine the prevalence of bronchiectasis and patient characteristics. The first cohort of patients recruited to the EARCO (European Alpha1 Research Collaboration) International Registry data base by the end of 2021 was analysed for radiological evidence of both emphysema and bronchiectasis as well as baseline demographic features. Results: Of the first 505 patients with the PiZZ genotype entered into the data base 418 (82.8%) had a reported CT scan. There were 77 (18.4%) with a normal scan and 38 (9.1%) with bronchiectasis alone. These 2 groups were predominantly female never smokers and had lung function in the normal range. The remaining 303 (72.5%) ZZ patients all had emphysema on the scan and 113 (27%) had additional evidence of bronchiectasis. Conclusions: The data indicates the bronchiectasis alone is a feature of 9.1% of patients with the PiZZ genotype of Alpha1-antitrypsin deficiency but although emphysema is the dominant lung pathology bronchiectasis is also present in 27% of emphysema cases and may require a different treatment strategy.

Published
2023

49. Pulmonologists’ Opinion on the Use of Inhaled Corticosteroids in Chronic Obstructive Pulmonary Disease Patients in Spain: A Cross-Sectional Survey

Author

Miravitlles, Marc, primary, González-Torralba, Fernando, additional, Represas-Represas, Cristina, additional, Pomares, Xavier, additional, Márquez-Martín, Eduardo, additional, González, Cruz, additional, Amado, Carlos, additional, Forné, Carles, additional, Alonso, Soledad, additional, Alcázar, Bernardino, additional, Barrecheguren, Miriam, additional, Jurado Mirete, Juan María, additional, and Naval, Elsa, additional

Published
2022
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50. Pulmonologists’ Opinion on the Use of Inhaled Corticosteroids in Chronic Obstructive Pulmonary Disease Patients in Spain: A Cross-Sectional Survey

Author

Miravitlles,Marc, González-Torralba,Fernando, Represas-Represas,Cristina, Pomares,Xavier, Márquez-Martín,Eduardo, González,Cruz, Amado,Carlos, Forné,Carles, Alonso,Soledad, Alcázar,Bernardino, Barrecheguren,Miriam, Jurado Mirete,Juan María, Naval,Elsa, Miravitlles,Marc, González-Torralba,Fernando, Represas-Represas,Cristina, Pomares,Xavier, Márquez-Martín,Eduardo, González,Cruz, Amado,Carlos, Forné,Carles, Alonso,Soledad, Alcázar,Bernardino, Barrecheguren,Miriam, Jurado Mirete,Juan María, and Naval,Elsa

Abstract

Marc Miravitlles,1,2 Fernando González-Torralba,3 Cristina Represas-Represas,4 Xavier Pomares,2,5 Eduardo Márquez-Martín,2,6 Cruz González,7 Carlos Amado,8 Carles Forné,9,10 Soledad Alonso,11 Bernardino Alcázar,12 Miriam Barrecheguren,1 Juan María Jurado Mirete,13 Elsa Naval14 1Pneumology Department, Hospital Universitari Vall d’Hebron, Vall d’Hebron Institut de Recerca (VHIR), Vall d’Hebron Barcelona Hospital Campus, Barcelona, Spain; 2CIBER de Enfermedades Respiratorias (CIBERES), ISCIII, Madrid, Spain; 3Pneumology Department, Hospital Universitario del Tajo, Aranjuez, Spain; 4Pneumology Department, Hospital Álvaro Cunqueiro, Vigo, Spain; 5Pneumology Department, Corporació Sanità ria Parc Taulí, Sabadell, Spain; 6Medical-Surgical Unit for Respiratory Diseases, Hospital Universitario Virgen del Rocío, Sevilla, Spain; 7Pneumology Department, Hospital Clínico de Valencia, Valencia, Spain; 8Pneumology Department, Hospital Universitario Marqués de Valdecilla, Santander, Spain; 9Heorfy Consulting, Lleida, Spain; 10Basic Medical Sciences Department, University of Lleida, Lleida, Spain; 11Pneumology Department, Hospital Universitario de Torrejón, Torrejón de Ardoz, Spain; 12Pneumology Department, Hospital Universitario Virgen de las Nieves, Granada, Spain; 13Scientific Department, GOC Health Consulting, Barcelona, Spain; 14Pneumology Department, Hospital Universitario de La Ribera, Alzira, SpainCorrespondence: Marc Miravitlles, Pneumology Department, Hospital Universitari Vall d’Hebron, P. Vall d’Hebron 119-129, Barcelona, 08035, Spain, Tel/Fax +34 932746083, Email marcm@separ.esIntroduction: Identifying the variables that guide decision-making in relation to the use of inhaled corticosteroids (ICS) can contribute to the appropriate use of these drugs. The objective of this study was to identify the clinical variables that physicians consider most relevant for prescribing or withdrawing ICS in COPD.Methods: A cross-sectional survey was conducted i

Published
2022

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